Nephrology

A.1.
A 63-year-old man presents to the Respiratory Clinic, having been referred by his General
Practitioner following investigations that revealed high serum calcium and a chronic cough.
He is a non-smoker, with no significant past medical history of note, other than hypertension, for which
he takes amlodipine 5 mg once a day. He has no significant history of nosebleeding, weight loss, fatigue
or malaise. Other than occasional paracetamol, he takes no regular medication.
On examination, there are fine crepitations throughout both lung fields, as well as a non-tender,
distended abdomen. A rectal examination is consistent with faecal loading.
The rash shown below is seen on his right leg:
Investigations:
Investigation Result Normal Values
Haemoglobin (Hb) 120 g/l 115–155 g/l
White cell count (WCC) 8.0 × 109/l 4.0–11.0 × 109/l
C-reactive protein (CRP) 4.0 mg/l 0–10 mg/l
Corrected calcium (Ca2+) 3.0 mmol/l 2.0–2.6 mmol/l
Creatinine (Cr) 145 µmol/l 50–120 µmol/l
Urea 8.0 mmol/l 2.5–6.5 mmol/l
Urine protein ++
Urine blood ++
He is referred for a kidney biopsy.
What would you expect to be the most likely result seen on this kidney biopsy?
Your answer was correct
‘Basket weave’ pattern of basement membrane
Crescentic glomerulonephritis
Granulomatous tubulointerstitial nephritis
Immune complex deposits
Normal kidney biopsy
Explanation
Granulomatous tubulointerstitial nephritis
This patient has presented with a history of hypercalcaemia and constipation, in the presence of clinical
evidence of fibrotic lung disease and a skin rash consistent with erythema nodosum. This man likely has
an underlying diagnosis of sarcoidosis, a granuloma-forming illness. Kidney biopsies in patients with
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sarcoidosis and evidence of renal involvement appear to have granulomas, with surrounding
inflammation of the renal interstitium. Importantly, sarcoidosis is associated with hypercalcaemia in 10–
20% of patients. Very few patients are symptomatic.
Image source: Erythema Nodosum, provided by Wiki Commons

https://commons.wikimedia.org/wiki/File:Erythema_Nodosum.jpg
‘Basket weave’ pattern of basement membrane
The ‘basket weave’ pattern appearance of the glomerular basement membrane is a pathognomonic
feature of Alport syndrome, a genetic disorder characterised by sensorineural hearing loss and
microscopic haematuria, with progression to renal failure. The majority of patients have a history of X-
linked dominant inheritance (85%). The remainder have either autosomal recessive (more common) or
autosomal dominant inheritance.
Crescentic glomerulonephritis
Crescentic glomerulonephritis refers to a group of illnesses, including antiglomerular basement

membrane disease and antineutrophil cytoplasmic antibody-associated vasculitis. A crescent is an
inflammatory destructive process of the glomeruli that presents with acute, rapidly progressive chronic
kidney disease and symptoms of vasculitis (which this man lacks). It would be unusual for this to present
with hypercalcaemia.
Immune complex deposits
There are a variety of disorders associated with the formation of immune complexes and renal failure.
These include lupus nephritis, endocarditis and post-streptococcal glomerulonephritis. These would
likely present with a prodromal history, which this man lacks. In lupus nephritis, one would expect a
prodromal history consistent with a diagnosis of lupus in the majority, but not all, cases. Similarly, it
would be unusual for a man of this age to present with lupus, particularly with these clinical findings.
Normal kidney biopsy
This patient has clinical features consistent with a nephritic process. He has haematoproteinuria and a
suspicious rash. Given his history, it is highly unlikely that a kidney biopsy will be entirely normal.
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A.2.An 80-year-old White woman is admitted to the Emergency Department following a fall and a
fractured distal radius. She has a past medical history of aching hips and knees, for which she uses
topical ibuprofen and oral paracetamol. She takes no other regular medication. Her most recent
creatinine level, taken two years ago, was recorded at 45 µmol/l.
On examination, she is an overweight woman who is currently euvolaemic. Her clinical examination is
normal. Her blood pressure is 110/65 mmHg.
Investigations:
Investigation Result Normal value
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Sodium (Na+) 139 mmol/l 135–145 mmol/l
Potassium (K+) 3.7 mmol/l 3.5–5.0 mmol/l
Urine dipstick protein Positive
Urine dipstick blood Positive
Antinuclear antibody (ANA) 1/40
Complement C32.2 g/l 0.8–2.1 g/l
Complement C40.49 g/l 0.15–0.5 g/l
Antineutrophil cytoplasm antibodies (ANCA) Negative
Antiglomerular basement membrane (anti-GBM) antibodies Negative
Creatine kinase (CK) 250 U/l 33–211 U/l
An abdominal ultrasound (US) is conducted which is demonstrated below.
What is the most likely diagnosis?
Your answer was incorrect
Drug-induced tubulointerstitial nephritis (TIN)
Lupus nephritis
Multiple myeloma (MM)
Obstructive uropathy
Prerenal acute kidney injury (AKI)
Explanation
Drug-induced tubulointerstitial nephritis (TIN)
Important causes of drug-induced TIN include contrast agents, antibiotics and non-steroidal anti-
inflammatory drugs (NSAIDs). In this case, the patient uses topical NSAIDs, which causes an
inflammatory response in the tubulointerstitium, characterised by mild haematoproteinuria. Topical
NSAIDs are an important, often overlooked cause of acute kidney injury (AKI).
Normal kidney ultrasound, courtesy of Wiki Commons

[https://commons.wikimedia.org/wiki/File:Kidney_ultrasound_110315132820_1329070.jpg]
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The US shows a normal kidney, with a normal corticomedullary differential, a normal and healthy-
looking collecting system and no evidence of calculus or haematoma. This means that based on the
current evidence, there is no suggestion of obstructive uropathy.
Lupus nephritis
There are a variety of disorders associated with the formation of immune complexes and renal failure.
These include lupus nephritis, endocarditis and post-streptococcal glomerulonephritis. These would
likely present with a prodromal history, which this patient lacks. In lupus nephritis, one would expect, in
the majority of cases, a prodromal history consistent with a diagnosis of lupus. Similarly, it would be
unusual for a patient of this age to present with lupus, particularly with these clinical findings.
Additionally, the serological finding of ANA positivity of 1/40 is very weakly positive. It would be unlikely
that this could be attributed to an underlying lupus diagnosis. Additional evidence against lupus is the
normal complement levels. Active lupus nephritis results in immune complex formation, which
therefore depletes complement.
Multiple myeloma (MM)
Multiple myeloma (MM) is a very plausible differential diagnosis in an 80-year-old patient with kidney
disease. There are, however, important negatives in this case. The history, in this case, is acute; there
are no significant bony aches and Ca2+ levels are normal. A longer history characterised by chronic
aches and hypercalcaemia would be more likely to point in the direction of MM.
Prerenal acute kidney injury (AKI)
Prerenal AKI occurs as a result of a loss of pressure gradient across the glomerulus. There is inadequate
pressure across the afferent arteriole, and this results in ultrafiltration at the glomerulus. This can result
from either a drop in arterial pressure or a rise in venous pressure (and consequently efferent arteriolar
pressure). This patient was not hypotensive and is reportedly euvolaemic.
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A.3.A 65-year-old obese man in hospital is investigated for unilateral hydronephrosis which was
incidentally identified on an ultrasound (US) scan performed for fatty liver disease. He has a significant
smoking history of 40 pack years and poorly controlled hypertension. He has no history of abdominal
pain, haematuria, weight loss, fevers or night sweats. He has no symptoms of lower urinary tract
obstruction but does complain of a dull ache in his abdomen and painfully cold feet.
A computerised tomography (CT) scan is performed:
Benign prostatic hypertrophy (BPH)
Prostate cancer
Retroperitoneal fibrosis
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Transitional cell cancer (TCC) of the bladder
Ureteric stricture
Explanation
The CT abdomen in the lateral view shows a large abdominal aortic aneurysm. The abdominal aorta
spans the retroperitoneal space; consequently, its inflammatory dilatation can result in direct
compression of other structures, inflammatory aortitis and retroperitoneal fibrosis. The fibrotic process
can subsequently result in organ dysfunction. In this case, the patient’s painfully cold lower limbs should
raise red flags for ischaemic aortic disease. Retroperitoneal fibrosis is indicated by the presence of
unilateral obstruction.
Abdominal Aortic Aneurysm, courtesy Wiki Commons,

[https://commons.wikimedia.org/wiki/File:Sagital_aaa.JPG]
Ureteric stricture
Ureteric strictures are an important cause of unilateral hydronephrosis. Ureteric strictures can develop
following renal calculi, blood clots, trauma and malignancy, among other causes. Ureteric strictures
cause unilateral hydronephrosis, driven by kidney outflow obstruction. Importantly, there is usually
some compensation by the contralateral kidney, so this may not present with significant acute kidney
injury. It can be diagnosed on non-contrast CT abdominal X-ray imaging and contrast CT urography.
Ureteric strictures are treated by management of the obstruction, including nephrostomy insertion,
followed by stenting the ureteric obstruction, if it is possible to do so. In this case, there is a clear
abdominal aortic aneurysm and it is likely that this is the causative agent of obstruction rather than a
second separate pathology.
Benign prostatic hypertrophy (BPH)
In middle-aged and older men, prostatic hypertrophy can result in circumferential narrowing of the
urethra and consequently in bladder outflow obstruction. As the case progresses, symptoms of lower
urinary tract obstruction become clinically evident, including lower abdominal discomfort, difficulty
initiating urination, starting and stopping, dribbling and the sensation of an incompletely emptied
bladder. Patients are predisposed to urinary sepsis and eventual complete obstruction. Given that the
obstruction is slow low down in the urinary tract, it presents classically with bilateral hydronephrosis
and hydroureter and may require a suprapubic catheter or a long-term urinary catheter to relieve the
obstruction. A transurethral resection of the prostate can provide sustained relief.
Prostate cancer
As with BPH, prostate cancer can cause circumferential urethral narrowing and subsequent obstruction
to bladder outflow. In this case, it is important to note that the patient has a unilateral obstruction. It
would be unlikely for a low renal tract obstruction to affect just one kidney rather than both. Prostate
cancer tends to have a reasonably slow onset of disease without the classical picture of cachexia and
systemic unwellness, but may well present with lower urinary tract symptoms and bladder outflow
obstruction.
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Transitional cell cancer (TCC) of the bladder
Like prostate cancer and BPH, TCC of the bladder can cause low obstruction and, therefore, result in
bilateral hydronephrosis. That being said, TTC occurring in the upper poles of the bladder can result in
obstruction at the vesicoureteric junction. This would be best diagnosed on cystoscopy. Importantly, in
this patient, there is a clear abdominal aortic aneurysm and it is likely that this is the causative agent of
obstruction rather than a second separate pathology.
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A.4.A 23-year-old man presents to the Nephrology Clinic for his annual follow-up appointment. He
initially presented to clinic in his teenage years with a history of recurrent muscle spasms and
intermittent weakness. He has a strong family and personal history of hypertension, which he details to
you in the following family tree. His mother, sister and several of his maternal cousins, an aunt and a
grandfather presented to medical services with severe hypertension and muscle spasm.
--He is indicated by the black arrow.
Investigations:
Investigation Result Normal values
Renin Low
Aldosterone Normal to low
pH 7.50 7.35–7.45
What is the most appropriate way to treat this patient’s hypertension?
Amiloride
Amlodipine
Bendroflumethiazide
Doxazosin
Ramipril
Explanation
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Amiloride
This patient has presented to the clinic with hypertension, hypokalaemia, alkalosis, hyporeninaemic
hypoaldosteronism and a strong family history of hypertension distributed in an autosomal dominant-
type inheritance pattern. This is a typical presentation of Liddle syndrome, which is caused by an
activating mutation of the epithelial sodium channel located in the principal cell of the cortical collecting
tubules. It is an inheritable K+-wasting disease that results in hypertension. It is best treated using a K+-
sparing diuretic with antihypertensive properties, such as amiloride.
Doxazosin
In the majority of cases, hypertensive treatment requires a stepwise approach, initially guided by age
and ethnicity. There are, however, some specific drug treatments for secondary hypertension, such as in
this case. Doxazosin is useful in essential hypertension as an adjunctive treatment after angiotensin-
converting enzyme (ACE) inhibitors, calcium channel blockers and thiazides have been unsuccessful.
Doxazosin is an alpha-blocker – its primary mode of action is as a muscle relaxant; it can, therefore, be a
useful adjunctive treatment in patients with both hypertension and benign prostatic hypertrophy (BPH).
Additionally, doxazosin alters the activity of alpha receptors and is also useful in patients with
phaeochromocytoma.
Amlodipine
Calcium channel blockers are important antihypertensives but, in this case, will not treat the underlying
problem or improve this patient’s hypokalaemia. In those over the age of 55 and in Black African
patients, amlodipine is the first-line antihypertensive treatment. While amlodipine may help to manage
this patient’s hypertension, it does not treat the underlying Liddle syndrome.
Bendroflumethiazide
Bendroflumethiazide is a thiazide diuretic with important antihypertensive properties. It functions by

modifying the activity of the sodium–chloride transporters found in the distal convoluted tubules. Its use
would result in significant K+ wasting and worsen the electrolyte picture in this patient.
Ramipril
ACE inhibitors cause the dilation of the efferent arteriole and reduce glomerular pressure. Their use
results in a meaningful decrease in blood pressure, as well as causing a decline in renal function and
exacerbation of hyperkalaemia. Ramipril is not a targeted treatment for Liddle syndrome, the condition
seen in this patient. Importantly, when a young patient with hypertension presents to the clinic, it is of
utmost importance to thoroughly rule out the primary causes of hypertension first.
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A.5.A 68-year-old Ghanaian man, with a 10-year history of type 2 diabetes, presented with a 3-week
history of generalised oedema. In spite of insulin treatment, his diabetes has been poorly controlled. He
has background retinopathy.
Investigations revealed normal renal function, albumin 12 g/l, negative ANA, negative ANCA, negative
GBM, negative HBsAg, negative hepatitis C antibody, negative cryoglobulins, negative serum
electrophoresis. 24 h urine protein excretion was 15.6.
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He underwent a renal biopsy shown below:
Focal segmental glomerulosclerosis Membranous nephropathy Light-chain disease Diabetic

glomerulosclerosis Minimal change disease
Explanation
Diabetic glomerulosclerosis
The duration of poorly controlled diabetes and evidence of microvascular disease make diabetic
nephropathy the most likely diagnosis. The biopsy shows mesangial expansion with a Kimmelstiel-
Wilson nodule at the top of the glomerulus, typical of diabetic glomerulosclerosis.
Focal segmental glomerulosclerosis
The renal biopsy findings are not consistent with this, along with the long duration of diabetes.
Membranous nephropathy
The incidence of membranous nephropathy is increased in diabetics compared with non-diabetic

patients but on light microscopy the glomeruli will show capillary loop thickening and normal
architecture respectively.
Light-chain disease
Inconsistent with the history of poorly controlled diabetes and normal electrophoresis.
Minimal change disease
The incidence of minimal-change disease is increased in diabetics compared with non-diabetic patients
but on light microscopy the glomeruli will show no change at all or at most mild mesangial proliferation.
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A.6.A 32-year-old man is referred to the Neurology Clinic with a 4-month history of hypertension,
headaches and impaired vision. Two weeks ago, he noticed blood in his urine. He had a nephrectomy in
his early twenties.
Below is a T1-weighted MRI scan with a contrast of his abdomen and brain:
Autosomal dominant polycystic kidney disease Autosomal recessive polycystic kidney disease
Tuberous sclerosis
Von Hippel–Lindau disease
Histiocytosis X
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Explanation
Von Hippel–Lindau disease
Von Hippel–Lindau disease is an autosomal dominant condition that manifests in the CNS
haemangioblastoma, renal and pancreatic cysts, renal carcinoma and phaeochromocytomas. Renal cysts
are usually multiple and bilateral and are often associated with solid tumours. Other causes of renal
cystic disease include autosomal dominant polycystic kidney disease, autosomal recessive polycystic
kidney disease, tuberous sclerosis complex, medullary cystic disease and renal cystic dysplasia. The MRI
scan here shows a cerebellar haemangioblastoma, as well as renal cysts.
Autosomal dominant polycystic kidney disease
This would not explain the MRI brain findings. Autosomal dominant polycystic kidney disease commonly
presents in adulthood and may be associated with intracranial berry aneurysms but not with
haemangioblastomas, as in this patient.
Autosomal recessive polycystic kidney disease
Autosomal recessive polycystic kidney disease commonly presents either at antenatal ultrasound
screening or in childhood. It is associated with renal impairment and hepatic fibrosis and not with
haemangioblastomas, as in this patient.
Tuberous sclerosis
TSC is an inherited neurocutaneous disorder. Eighty per cent of affected individuals have seizures, while
the majority of patients have cognitive impairment and present with angiomyolipomas in the skin and
visceral organs at a young age.
Histiocytosis X
Langerhans cell histiocytosis, also called histiocytosis X, is incorrect. It is a multisystem disease with
predominant bone manifestations. CNS manifestations, when present, are typically ataxia and cognitive
dysfunction rather than haemangioblastomas, as in this patient.
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A.7.A 45-year-old man presents to the Emergency Department with a history of collapse and
haemoptysis. He smokes ten cigarettes a day and is treated for hypertension with amlodipine 5 mg once
a day.
On clinical examination, he is alert and distressed. Crackles are heard bilaterally on auscultation of his
chest. He shows you a handkerchief stained with bright red blood.
He is transferred to critical care for respiratory support and a chest radiograph is shown below:
Investigations:
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Eosinophil count 0.1 × 109/l 0.04–0.4 × 109/l
Urea 35 mmol/l 1.7–8.3 mmol/l
Myeloperoxidase (MPO) < 0.3 mmol/l < 0.64 mmol/l
Proteinase 3 (PR3) < 0.3 U/ml < 3.5 U/ml
Antinuclear antibody (ANA) 1/80 speckled
Which of the following is the most appropriate treatment?
Azathioprine
Cyclophosphamide
Hydroxychloroquine
Plasma exchange
Rituximab
Explanation
Plasma exchange
Goodpasture’s (or antiglomerular basement membrane) disease is an autoimmune disorder

characterised by antibodies against the alpha-3 subunit of type IV collagen found in the basement
membrane of the lungs and kidneys. Its presentation is heterogeneous – it may present with either
alveolar haemorrhage or rapidly progressive glomerulonephritis. It is treated with steroids and plasma
exchange. The goal of treatment is first to dampen down the inflammatory response, then to follow
with antibody removal using plasma exchange. The process is monitored by measuring antibody titres
with treatment. The chest radiograph shows diffuse infiltrative opacification in consistent with
pulmonary haemorrhage. A left internal jugular venous catheter is also present.
https://radiopaedia.org/cases/diffuse-alveolar-haemorrhage-goodpasture-syndrome-3
Azathioprine
Azathioprine is an immunosuppressing drug which is used for a variety of disorders. Primarily, it is used
to treat autoimmune conditions as a steroid-sparing regimen. Additionally, it can be used as an
immunosuppressant for patients with a transplanted organ. It is noteworthy that azathioprine is used in
transplanted patients who are pregnant. It has no role in the induction of Goodpasture’s disease.
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Cyclophosphamide
Cyclophosphamide is a powerful immunosuppressing drug which is used in the induction of vasculitis.

There are a variety of risks associated with cyclophosphamide, for which the patient must be counselled,
including sterility. It is used in the induction of ANCA-associat ed vasculitis, which this patient does not
have.
Hydroxychloroquine
Hydroxychloroquine is a Toll-like receptor blocker used in the treatment of systemic lupus

erythematosus (SLE). It would be unusual for SLE to present with pulmonary/renal syndrome. Similarly,
the ANA is only weakly positive. Additionally, for active SLE, the primary treatment would be steroids
with the intention of dampening the inflammatory process.
Rituximab
Rituximab is a monoclonal antibody targeted against the B-lymphocyte membrane receptor CD20. It has
a variety of uses as an immunosuppressing drug, including management of autoimmune disorders and
transplant rejection. This case is a pulmonary/renal vasculitic process which is antineutrophil cytoplasm
antibody (ANCA) negative. Likely, this is Goodpasture’s disease, for which the use of rituximab is not
validated. Trial evidence suggests that rituximab does not improve renal outcomes in antiglomerular
basement membrane disease.
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A.8.A 45-year-old man presents to the General Nephrology Clinic for a follow-up appointment. He has a
past medical history of type I diabetes and several complications. These include peripheral vascular
disease, neuropathy and chronic kidney disease (CKD) – with an estimated glomerular filtration rate
(eGFR) of 19 ml/min per 1.73 m2.
He was diagnosed to have brain abscess a magnetic resonance imaging (MRI) scan, one month ago. On
examination, he appears emaciated. There is fine telangiectasia across his face, accompanied by small,
white patches.
A photograph of his hands is shown below:
Sclerodactyly, courtesy of Wiki Commons [https://commons.wikimedia.org/wiki/File:CREST1.JPG]
Autoantibody tests report the following:
Investigation Result
Antinuclear antibody (ANA) 1/80 speckled appearance
Anti-Scl 70 Negative
Anti-ribonucleic acid (RNA) Negative
Anti-Ro Negative
Anti-double-stranded deoxyribonucleic acid (dsDNA) Negative
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Anti-centromere antibody (ACA) Negative
Anti-URRNP Negative
Anti-tyrosine hydroxylase (TH) Negative
Diffuse cutaneous systemic sclerosis (DCSS)
Limited cutaneous systemic sclerosis (LCSS)
Mixed connective tissue disorder (MCTD)
Nephrogenic systemic fibrosis (NSF)
Systemic lupus erythematosus (SLE)
Explanation
Nephrogenic systemic fibrosis (NSF)
Nephrogenic systemic fibrosis (NSF) is a very rare phenomenon that occurs in patients with kidney
disease after exposure to gadolinium-based magnetic resonance imaging (MRI) contrast agents. Its
incidence is comparatively more common with older MRI gadolinium agents and as such, it is now rare
but still occurs, and remains an area of contention among Nephrologists. It presents with symptoms and
signs similar to that of systemic sclerosis but, notably, in the absence of autoimmune antibodies. Of
note, post-MRI dialysis does not reduce the incidence of NSF.
Diffuse cutaneous systemic sclerosis (DCSS)
Diffuse cutaneous systemic sclerosis (DCSS) is a less common, more severe form of scleroderma, which
is associated with trunk, arms and thigh disease. Likewise, in the absence of antibody positivity, this
diagnosis is less likely. Scleroderma is usually treated with immunosuppressing drugs such as
methotrexate and cyclophosphamide.
Limited cutaneous systemic sclerosis (LCSS)
This man has scleroderma-type features, including sclerodactyly, telangiectasia and calcinosis. He does
not have any antibody-positive results, making this diagnosis significantly less likely. Limited cutaneous
systemic sclerosis (LCSS) is associated with the formation of CREST symptoms (calcinosis, Raynaud’s,
oesophageal dysmotility, sclerodactyly and telangiectasia).
Mixed connective tissue disorder (MCTD)
Mixed connective tissue disorder (MCTD) refers to an illness that does not fit into any single category
based on its clinical phenotype. Often, these patients demonstrate a variety of clinical features,
including skin manifestations similar to sclerodactyly and myositis, with significant underlying organ
disease, including serositis, pulmonary fibrosis and pericarditis. This illness is almost always associated
with anti-ribonucleoprotein positivity.
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A small, but significant population of healthy individuals are weakly positive for antinuclear antibodies
(ANAs). In the absence of any other significantly positive antibodies, the presence of weakly positive
ANA in this patient is unlikely to be of any clinical significance to his current presentation.
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A.9.A 53-year-old diabetic, hypertensive man is admitted to the Emergency Department with a right-
sided, middle cerebral artery stroke. He is a heavy smoker with known coronary artery disease. At the
time of admission, he was within four hours of symptom onset and was successfully thrombolysed.
Several investigations are performed, including an electrocardiogram (ECG), which shows atrial
fibrillation. After this, he is appropriately warfarinised. A right-sided carotid artery stenosis is noted on
ultrasound, and while he is an inpatient, an endarterectomy is performed.
Forty-eight hours after the endarterectomy, he is referred to the Renal Registrar with acute kidney
injury (AKI).
His clinical examination reveals the presentation on his leg shown below.
The remainder of his clinical examination is otherwise unremarkable. His blood pressure (BP) is 130/80
mmHg.
His current medications include ramipril 2.5 mg once daily, atorvastatin 80 mg at night, warfarin 2 mg
once daily, aspirin 75 mg once daily and bisoprolol 2.5 mg once daily.
Investigations:
Eosinophils 3.2 × 109/l 0.04–0.4 × 109/l
Haemoglobin (Hb) 9.5 g/l 135–175 g/l
What is the most likely cause of this patient’s AKI?
Antineutrophil cytoplasm autoantibody (ANCA)-associated vasculitis
Cholesterol embolism
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Ramipril toxicity
Renal artery stenosis (RAS)
Explanation
Cholesterol embolism is an important common cause of acute kidney injury (AKI), with eosinophilia
often accompanied by a distal limb rash of livedo reticularis, as seen here. Cholesterol embolism is
secondary to procedures and drugs which result in cholesterol becoming dislodged. These include
angiography with stenting, procedures to treat abdominal aortic aneurysms, carotid endarterectomies
and initiation of warfarin. This patient has both undergone an endarterectomy (which is rarely
associated with cholesterol embolism) and recently been started on warfarin.
Livedo reticularis, courtesy of Wiki Commons

[https://commons.wikimedia.org/wiki/File:Livedo_reticularis_of_left_leg.jpg]
Antineutrophil cytoplasm autoantibody (ANCA)-associated vasculitis
Antineutrophil cytoplasm autoantibody (ANCA)-associated vasculitis is a crescent-forming inflammatory

disease. A crescent is an inflammatory destructive process of the glomeruli that results in acute, rapidly
progressive renal failure and symptoms of vasculitis (which this man lacks). ANCA-associated vasculitis is
treated by immunosuppression, initially with steroids. The rash presented is suspicious, but it would be
extremely unlikely for vasculitis to occur de novo in a patient hospitalised for something else,
particularly when there is a clear, alternative explanation.
While obstruction is an important cause of kidney injury in any patient with AKI, the absence of lower
urinary symptoms, the acute deterioration, the lack of pain and the presence of eosinophilia and a rash
point strongly away from this as the likely diagnosis.
Ramipril toxicity
Angiotensin-converting enzyme (ACE) inhibitors inhibit efferent arteriolar vasoconstriction and reduce
glomerular pressure and proteinuria. They also improve BP parameters and are therefore ideal
interventions for proteinuric chronic kidney disease in diabetic patients. Although stopping ramipril will
be part of the ongoing treatment of this patient, it is unlikely that, given his long-term stability, ramipril
is the causative agent of this AKI.
Renal artery stenosis (RAS)
Renal artery stenosis (RAS) can occur in the older population as a consequence of atherosclerotic
disease and, among young people, secondary to disorders such as fibromuscular dysplasia, which
appears as ‘beaded’ narrowing of the renal arteries. Classically, this results in secondary
hyperaldosteronism, hypertension and occasionally hypokalaemic metabolic alkalosis. Evidence suggests
that stenting these stenotic renal arteries does not improve outcome secondary to standard medical
care; however, this practice is still performed in patients with flash pulmonary oedema, as this group
was not included in the initial trials. This patient has some important risk factors for RAS, but the
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timeline of the history does not support this as the cause of this acute renal impairment. More common
modes of presentation include intractable hypertension or a rapid, dramatic deterioration in renal
function after initiating ACE inhibitors.
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A.10.A 55-year-old man who has worked as a painter and decorator for most of his adult life presents to
the Emergency Department with bilateral lower limb oedema. He is a heavy smoker and has diabetes.
He has well-controlled hypertension.
Clinical examination reveals a man with a body mass index (BMI) of 34 kg/m2. He has bilateral oedema
extending up to his thighs, with some testicular oedema and ascites. His blood pressure is 170/85
mmHg.
His current medications include gliclazide 40 mg once a day, amlodipine 5 mg once a day and
atorvastatin 10 mg at night.
Investigations:
White cell count (WCC) 10 × 109/l 4–11 × 109/l
Glycated haemoglobin (HbA1c) 7.2 mmol/l 4–7 mmol/l
Urine protein : creatinine ratio (uPCR) 800 mg/mmol < 50 mg/mmol
A chest X-ray (CXR) is conducted:
Amyloidosis
Congestive cardiac failure
Diabetic nephropathy
Minimal change disease (MCD)
Explanation
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There are two different kinds of membranous nephropathy: primary and secondary. Primary
membranous nephropathy is a protein loss disorder, which is driven by an immune process – typically,
phospholipase A2 receptor antibodies. Membranous nephropathy is treated using an aggressive
immunosuppression regimen. Secondary membranous nephropathy commonly occurs as a result of an
underlying malignancy. It is also characterised by heavy protein loss and nephrotic syndrome. The
classical pattern of histopathology in membranous nephropathy is ‘spike’ formation of the basement
membrane. In this case, the patient has presented with heavy proteinuria in the context of a significant
smoking history and has evidence of malignancy on his CXR.
Lung cancer, courtesy wiki commons

[https://commons.wikimedia.org/wiki/File:Lung_Cancer_on_Chest_X-Ray.jpg]
Amyloidosis
Amyloidosis refers to the deposition of abnormal amyloid proteins in organs, leading to their
dysfunction. It is an important downstream effect of chronic inflammatory states and is classically seen
in the late stages of inflammatory illnesses such as rheumatoid arthritis. In nephrology, it classically
presents with massive proteinuria and oedema, which increases slowly. Given this is usually seen in
patients with a long-standing chronic inflammatory illness, it does not fit with the picture seen here. It is
diagnosed by positive Congo red-stained kidney biopsies.
Congestive cardiac failure
Cardiac failure is an important cause of significant oedema. There are a variety of causes of heart failure,
including primary diseases of the heart, such as hypertrophic obstructive cardiomyopathy, and
secondary disorders, including ischaemic cardiomyopathy and left ventricular hypertrophy secondary to
hypertension. The characteristics of congestive cardiac failure include oedema, shortness of breath,
elevated brain natriuretic peptide and cardiac anomalies on echocardiography. Importantly, an elevated
protein : creatinine ratio makes the diagnosis of congestive cardiac failure unlikely to be the causative
agent in this patient’s presentation.
Diabetic nephropathy is a chronic disorder of glomerular injury characterised by proteinuria and

progressive renal failure, which often coexists with other diseases such as hypertension. Massive
proteinuria and oedema are entirely possible, but in the absence of any other features of poorly
controlled diabetes, such as neuropathy, visual impairment or significantly deranged HbA1c, it is
important to rule out other causes of this patient’s nephropathy.
Minimal change disease (MCD)
Minimal change disease (MCD) is predominantly a childhood disorder characterised by heavy

proteinuria, which resolves when treated with immunosuppression. Effacement of podocyte foot
processes is seen in MCD but is an electron microscopy finding. The classical light microscopy finding is
of a normal-appearing glomerulus. The important negatives, in this case, include the patient’s age group
and a variety of important causes, which are significantly more likely, including an underlying
malignancy.
16
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A.11.An 87-year-old man with a recent diagnosis of a retroperitoneal sarcoma presents to the
Emergency Department (ED) after collapsing at home. Over the course of the last ten years, his general
health has slowly deteriorated. He is able to mobilise with a Zimmer frame for short periods of time. In
view of his frailty, a multidisciplinary approach to conservative care has been decided. He is cognitively
intact and explains that he slipped on the way to the bathroom. He carries a pendant alarm and was
able to get help urgently.
Clinical examination reveals a thin, elderly man with a soft ejection systolic murmur. He has a mild
postural drop in blood pressure (BP) which corrects after fluid resuscitation and a normal abdominal
examination. A digital rectal examination is performed, which identifies soft faeces and a normal-sized
prostate.
The patient has an abnormal renal function. Three years before presentation, his estimated glomerular
filtration rate (eGFR) was 65 ml/min/1.73 m2 (normal range: > 60 ml/min/1.73 m2). Now, on admission
to the ED, it is recorded as 20 ml/min/1.73 m2.
A computerised tomography (CT) scan is shown below:
Bilateral hydronephrosis, courtesy of Wiki Commons

[https://commons.wikimedia.org/wiki/File:BilateralHydro.png]
What is the most likely cause of this man’s acute kidney injury?
Drug-induced nephritis
Prostate cancer
Renal calculi
Rhabdomyolysis
Explanation
The CT scan reveals bilateral hydronephrosis and similarly small kidneys. This underpins two
observations. Firstly, the disease process is affecting both kidneys structurally and is, therefore, unlikely
to be a single ureteric illness such as a malignancy or stone. This means that it either affects both
ureters, the bladder or the urethra. Secondly, given the small size of the kidneys, there is likely a chronic
element to this presentation. This patient has a retroperitoneal sarcoma, which is known to cause
retroperitoneal fibrosis and can itself cause hydronephrosis; hence, obstructive uropathy is the most
likely diagnosis. Further evidence of retroperitoneal fibrosis includes the evidence clinically of peripheral
vascular insufficiency, which occurs as a result of impairment of the blood supply to the lower limbs by a
fibrosed retroperitoneal space.
17
Drug-induced nephritis
Several drugs are implicated in the cause of renal dysfunction. Of note, interstitial nephritis does not
cause hydronephrotic kidneys. Additionally, in this case, none of the medications this man is on
classically cause interstitial nephritis, which is more commonly seen with antibiotics, contrast,
gentamicin and ibuprofen.
Prostate cancer
Prostate cancer and benign prostatic hypertrophy are important causes of bilateral hydronephrosis and
renal failure. Classically, it presents with sensate urinary outflow obstruction, bilateral hydronephrosis
and obstructive uropathy and resolves with catheter insertion and transurethral resection of the
prostate (TURP). In this case, a rectal examination was normal, suggesting that the prostate is not to
blame.
Renal calculi
Renal calculi can cause hydronephrosis, but more often than not, it will be unilateral, as it is uncommon
to have bilateral obstructing calculi. Renal calculi are formed due to a variety of reasons, including
dehydration, medication use, prior surgery, diet and underlying medical disorders. They are classically
associated with pain radiating from the loin to the groin, with acute kidney injury and microscopic
haematuria. The vast majority of stones are visible on CT imaging. No stone is visible on this scan.
Rhabdomyolysis
Rhabdomyolysis causes renal failure by a variety of mechanisms. Firstly, in the classical case of an older
patient who falls and has a long lie, there is likely a degree of dehydration, which results in prerenal
injury. Secondly, rhabdomyolysis causes an acute inflammatory response within the interstitium.
Myoglobin casts result in tubular obstruction. It is associated with a rise in creatine kinase and is treated
predominantly with fluid resuscitation and replacement. In this case, the patient had a short lie on the
ground and did not have significant rhabdomyolysis that would explain his renal function. Similarly,
rhabdomyolysis would not explain the radiological finding.
*************************************************************************************
A.12.A 65-year-old woman presents to the Renal Clinic for review for an inherited condition. She brings
with her a pedigree of her family affected by a genetic disease linked to progressive kidney disease,
hearing loss and haematuria. She has progressively worsening renal impairment detected by her general
practitioner (GP). Currently, she is not taking any medications.
On examination, her blood pressure (BP) is 140/80 mmHg, and her chest and abdominal examination is
unremarkable.
Given the suspected diagnosis, which of the following correctly reflects the inheritance pattern for this
condition?
Autosomal dominant Autosomal recessive X-linked dominant X-linked recessive X-linked recessive
plus spontaneous mutation
18
Explanation
X-linked dominant
The symptoms described here are suggestive of a diagnosis of Alport’s syndrome, 85% of which is
inherited as an X-linked dominant condition. This accounts for the fact that both men and women are
affected. With respect to Alport’s, women usually present with milder, later onset disease.
Autosomal dominant
The fact that this is a renal condition suggestive of Alport’s drives us towards X-linked dominant as the
likely diagnosis. If an abnormal gene is inherited from the affected parent, the offspring will get the
disease in both men and women. One example of autosomal dominant inherited renal disease is
polycystic kidney disease.
Autosomal recessive
The high rates of heritability from generation to generation, (up to 50% of offspring), are much more
suggestive of either X-linked dominant or autosomal dominant inheritance. Adult polycystic kidney
disease may be inherited as an autosomal recessive condition.
X-linked recessive
The fact that women are affected by the condition effectively rules out X-linked recessive inheritance.
An example of X-linked recessive renal disease is nephrolithiasis (Dent disease).
X-linked recessive plus spontaneous mutation
This would not be consistent with the number of women affected by the condition.
***********************************************************************************
A.13.A 19-year-old man presented with a 2-day history of a painful rash, constipation, lethargy and
generalised body pain. He has no significant past medical history apart from hospitalisation as a child for
peritonitis secondary to a ruptured appendix.
Examining his legs, you note the following:
Investigations:
Creatinine (Cr) 162 µmol/l 50 - 120 µmol/l
LFTs normal
FBC normal
Urine microscopy red cell casts
ANCA negative
ASOT normal
19
Hepatitis B/C negative
What is the diagnosis?
IgA nephropathy Infective endocarditis Henoch-Schönlein purpura Erythema nodosum Polyarteritis

nodosa
Explanation
Henoch-Schönlein purpura
In a young patient with microscopic haematuria and renal impairment, the differential diagnosis
includes HSP, IgA nephropathy and thin-membrane disease. HSP is uncommon after the second decade
of life and the renal involvement is often transient. The rash is a purpuric vasculitis, usually spreading on
the extensor surfaces. Histology shows a leucocytoclastic vasculitis with IgA deposits in blood vessel
walls.
IgA nephropathy
Both IgA nephropathy (IgAN) and Henoch-Schönlein nephritis are characterised by mesangial IgA
deposition. The latter is differentiated from IgAN by extrarenal manifestations, such as purpura (as in
this gentleman), polyarthralgia, and abdominal pain caused by gut vasculitis with IgA deposition.
Infective endocarditis
It does not explain all the extra-renal manifestations and normal FBC.
Erythema nodosum
Erythema nodosum is characterised by red or violet subcutaneous nodules that usually develop in a
pretibial location.
Polyarteritis nodosa
PAN typically presents with systemic symptoms (fatigue, weight loss, weakness, fever, arthralgias) and
signs (skin lesions, hypertension, renal insufficiency, neurologic dysfunction, abdominal pain) of
multisystem involvement.
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A.14.A 30-year-old woman presents to her General Practitioner with a 3-day history of cramping
abdominal pain and vomiting.
A week previously, she had been absent from her job as a paint factory worker, with flu-like symptoms.
She has now developed a rash (see image). Her platelet count is normal at 282 x 109/l.
What complication might she be at the most risk of developing?
Epistaxis
20
Haemarthrosis
Pulmonary haemorrhage
Visual loss
Glomerulonephritis
Explanation
Glomerulonephritis
The rash is that of Henoch–Schönlein purpura (HSP), an immunoglobulin A (IgA)-mediated small-vessel

vasculitis characterised by purpura, arthritis, abdominal pain and haematuria. The cause is unknown;
IgA-dominant immune complexes are present in smaller venules, capillaries and arterioles, representing
a diffuse vasculitis that is secondary to hypersensitivity. HSP affects predominantly children (75% of
cases occur between the ages of 2 and 11 years) and males are affected twice as commonly as females.
However, adults can rarely be affected. Renal disease is the most common serious sequela (haematuria,
proteinuria, nephritis, nephritic syndrome). Possible rarer complications include hepatosplenomegaly,
myocardial infarction, pulmonary haemorrhage, pleural effusion, unnecessary abdominal surgery,
intussusception, gastrointestinal bleeding, bowel infarction and nervous system vasculitic involvement.
Epistaxis
HSP doesn’t increase the risk of bleeding or cause a significant increase in blood pressure for the
majority of cases, meaning that the risk of epistaxis is low.
Haemarthrosis
Joint haemorrhage is rarely seen in association with HSP, although polyarthritis is a common feature.
Pulmonary haemorrhage
Pulmonary haemorrhage is a more usual feature of Goodpasture syndrome and granulomatosis with
polyangitis.
Visual loss
Episcleritis and episodes of central retinal artery occlusion are seen in HSP, although they are very much
rarer than other manifestations such as glomerulonephritis and small-joint polyarthritis.
***********************************************************************************
A.15.A 24-year-old South Asian woman presented with acute onset of left-sided flank pain with
microscopic haematuria. She had a KUB X-ray performed.
She has a history of recurrent urinary tract infections since childhood and in the past was investigated
and treated by a Urology Specialist. Her maternal first cousin is on peritoneal dialysis for chronic kidney
disease.
Investigations:
21
Corrected calcium (Ca2+) 2.48 mmol/l 2.2 - 2.7 mmol/l
Phosphate (PO43-) 1.02 mmol/l 1.1 - 1.45 mmol/l
Albumin 40 g/l 35 - 55 g/l
24-h urinary protein collection 0.5 g < 0.2 g
Creatinine clearance 68 ml/min 88 - 128 ml/min
24-h urine calcium 10 mmol < 7.5 mmol
24-h urine oxalate 1.4 mmol < 0.36 mmol
24-h urine urate 3.2 mmol < 0.45 mmol
Her KUB X-ray is shown below:
is the most likely diagnosis?
Primary hyperoxaluria Primary hyperparathyroidism Secondary hyperparathyroidism Nephrogenic

diabetes insipidus Surreptitious antacid ingestion
Explanation
Primary hyperoxaluria
Elevated urinary oxalate levels may be due to increased dietary intake, malabsorption or an inherited
enzyme deficiency leading to decreased metabolism of oxalate (primary hyperoxaluria). There are three
types: types I and III are due to an enzyme defect in the liver glyoxalatepathway and in type II there is
failure of reduction of glyoxalate to glycolate. Type I is the commonest and results in widespread
calcium oxalate deposition throughout the body.
Treatment of primary hyperoxaluria is aimed at increasing urinary pH to make calcium oxalate more
soluble. This is by administering supplemental citrate and magnesium. Renal insufficiency is common
and patients require a combined liver and kidney transplant in type I disease. Primary hyperoxaluria also
leads to urine which is super-saturated with urate and urinary calcium which is just above the normal
range.
Primary hyperparathyroidism
Her normal serum calcium and phosphate levels are not consistent with hyperparathyroidism.
Secondary hyperparathyroidism
Her normal serum calcium and phosphate levels are not consistent with hyperparathyroidism.
Nephrogenic diabetes insipidus
22
This is characterised by polyuria and polydipsia. The acquired form may be due to electrolyte
abnormalities (hypokalaemia, hypercalcaemia), chronic kidney disease or lithium therapy.
Nephrocalcinosis is uncommon.
Surreptitious antacid ingestion
The recurrent childhood urinary tract infections and family history of kidney disease make this unlikely.
************************************************************************************
A.16.A 44-year-old woman presented with haematuria and loin pain.
Her computed tomography (CT) scan is shown below:
Medullary sponge kidney
Hydatid disease
Nephronophthisis
von Hippel–Lindau syndrome
Explanation
The CT scan shows the classic appearance of polycystic kidneys (red arrows) with multiple hepatic cysts
(one cyst identified with yellow arrow) which occur in 30–70% of patients. Autosomal dominant
polycystic kidney disease is an autosomal dominant condition and is a common cause of chronic kidney
disease. It can present at any age from the second decade onwards. Presenting features include loin
pain, haematuria, hypertension and abdominal mass. Associated features include intracranial aneurysms
(8%) and subarachnoid haemorrhage, mitral valve prolapse and renal calculi. Cysts may become infected
or haemorrhaged. Children and siblings of patients with this condition should be screened with
ultrasound after 20 years of age.
Medullary sponge kidney is a benign, common condition where there is diffuse medullary cyst
formation. It is a developmental disorder that can be detected on imaging.
Hydatid disease
Hydatid disease is caused by an infection with the tapeworm Echinococcus. The liver is the most
common site of infection. Treatment is frequently with surgery.
Nephronophthisis
23
Nephronophthisis is a renal disorder that affects children. Unlike medullary sponge kidney, it is a genetic
disorder that is transmitted in an autosomal recessive manner. Although it is rare, it is the most
common genetic cause of childhood kidney disease.
von Hippel–Lindau syndrome
We would expect other signs and symptoms to be present in this hereditary condition. This includes
headaches, problems with balance and walking, limb weakness and hypertension. Signs would include
angiomatosis. Conditions which are associated include phaeochromocytoma, renal cell carcinoma and
pancreatic cysts.
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A.17.A 39-year-old man comes to the clinic for follow-up after a short hospital admission for an episode
of renal colic.
At the time of admission, the patient had an intravenous pyelogram performed that showed mid-
ureteric calculi on the left side. There was delayed uptake and excretion of the contrast in the left
kidney, consistent with obstruction. There was also a filling defect at the level of L5 consistent with the
calcification seen on pre-contrast films.
While in the hospital, he underwent ureteroscopic stone extraction of the left mid-ureteral calculi. The
calculi were sent to the laboratory for chemical analysis, and the image is shown below. He denies any
history of calculi in the past or history of calculi in the family.
Image of calculi:
What will be the likely composition of the stones?
Calcium oxalate
Cysteine
Oxalic acid
Magnesium-ammonium-phosphate
Uric acid
Explanation
Calcium oxalate
This patient has a radio-opaque calculus. Calcium oxalate-containing stones account for nearly 60% of all
kidney stones. They are radio-opaque. Cysteine stones result from cysteinuria, which is due to an
inherited autosomal recessive disorder of renal tubular reabsorption of four amino acids. Uric acids
stones are not radio-opaque. Magnesium-ammonium-phosphate stones are infectious in aetiology. The
most common organisms are Proteus, Kleibsiella and Pseudomonas.
Cysteine
24
Cysteine stones are suspected in childhood stone formers. Mutation in the gene encoding an amino acid
transporter leads to wasting of the cationic amino acids cysteine, ornithine, lysine and arginine.
Oxalic acid
Hyperoxaluria can be associated with increased dietary oxalate or enhanced oxalate uptake due to ileal
inflammation or resection, such as in inflammatory disease, and may lead to the formation of oxalate
stones. Normally stones are in combination with calcium rather than oxalate alone.
Magnesium-ammonium-phosphate
These stones are formed as a result of infection by Proteus, Klebsiella or Serratia, which are capable of
splitting urea to ammonium and hydroxyl ions (thus increasing urinary pH), predisposing towards
struvite stones. Such stones act as a reservoir for infection, and often expand to fill as much of the
collecting system. Typically seen on KUB.
Uric acid
Pure uric acid stones are uncommon. Increase in urinary urate with reduction in urinary pH to less than
5.5 leads to the formation of insoluble uric acid stones. Increase in urinary urate occurs when there is
hyperuricaemia (10-20% will have frank gout) of any cause. Uric acid stones are radiolucent on KUB.
*********************************************************************************
A.18.A 45-year-old man presents with a history of poor urine output over the past two days. He has
recently returned from a holiday in Thailand, after which he had suffered diarrhoea about a week ago;
otherwise he has no other significant history.
On examination, he had a blood pressure of 98/50 mmHg and a pulse rate of 110 bpm. A diagnosis is
reached of haemolytic-uraemic syndrome associated with diarrhoea. Gram stain of the organism found
in the stool is shown below.
What would be the most useful treatment for this patient?
Antibiotics
Diuretics
Heparin
Intravenous immunoglobulins
Supportive (dialysis, fluids, blood transfusions)
Explanation
Supportive (dialysis, fluids, blood transfusions)
Supportive treatment is the mainstay of treatment for haemolytic–uraemic syndrome (HUS). Although in
some cases, plasma exchange will remove the inhibitor of the von Willebrand factor protease enzyme.
25
About 70% of affected patients will recover with no long-term problems with supportive treatment
alone.
Intravenous immunoglobulins
There is no role of immunoglobulin replacement in HUS. There are some immune disorders whereby
replacement of immunoglobulins may be useful such as multiple sclerosis, but not in this case.
Antibiotics
There is no evidence that antibiotics will shorten the duration of illness in HUS. For infective diarrhoea,
in general, giving antibiotics may prolong infection.
Diuretics
Giving diuretics may potentially worsen the acute kidney disease by causing further hypotension. This
man needs fluid resuscitation as evidenced by the low blood pressure and tachycardia.
Heparin
Anticoagulation is contraindicated in HUS, particularly in children, because the condition is frequently

complicated by both bleeding and hypertension. In adults, heparin increases mortality because of
increased bleeding.
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A.19.A 44-year-old woman presents to her GP for review. She has attended the GP on a number of
occasions during the past year for sinusitis and is now concerned that the bridge of her nose has
collapsed and that she may require cosmetic surgery.
Upon examination, blood pressure is 160/70 mmHg, chest is clear and abdominal examination is
unremarkable.
Chest X-ray is abnormal and reveals multiple nodules. Routine blood testing reveals a creatinine of 205
μmol/l.
Renal biopsy reveals:
Which of the following is the best initial treatment for this condition?
Azathioprine
Corticosteroids
Cyclophosphamide
Corticosteroids and cyclophosphamide
Methotrexate
Explanation
26
Corticosteroids and cyclophosphamide
The clinical history is highly suggestive of Granulomatosis with polyangiitis. Often patients present with
severe rhinorrhoea, complicated by nasal ulceration and later cough with haemoptysis. Chest X-ray
reveals nodular changes ± pneumonic features. Renal involvement is characterised by microvascular
glomerulonephritis, which is what is illustrated in the picture. Treatment is with cyclophosphamide in
combination with corticosteroids in patients with severe disease.
Azathioprine
Azathioprine may be used in the maintenance therapy for Granulomatosis with polyangiitis when the
patient is in remission.
Corticosteroids
Corticosteroids would treat the inflammation, but would not be used alone in therapy. Before the
introduction of steroids, over 75% of patients who had vital organ involvement and vasculitis would die.
However, after the introduction of steroids, 5-year mortality fell to 50% and further decreased to 12%
with the addition of cytotoxic drugs for polyarteritis nodosa. Few data exist for the use of steroids alone
for Granulomatosis with polyangiitis, but we would expect a similar reduction in death; therefore the
addition of cytotoxic medications in addition to steroids is important.
Cyclophosphamide
Cyclophosphamide is a well-established immunosuppressant therapy for vasculitis, but it would not be

used alone in the case of severe disease.
Methotrexate
Methotrexate would be used in the treatment of rheumatoid vasculitis but not in Granulomatosis with
polyangiitis.
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A.20.A 70-year-old white man with a history of intermittent microscopic haematuria presented with
generalised fatigue, cough, and vague abdominal pain and diarrhoea. He has a history of hypertension
and was on irbesartan. Baseline renal function was normal when checked by his general practitioner
(GP) three weeks previously.
Initial investigations:
Haemoglobin (Hb) 78 g/l 135 - 175 g/l
Haematocrit (HCT) 0.4 0.45 - 0.54 (male)
Platelets (PLT) 98 × 109/l 150 - 400 × 109/l
C-reactive Protein (CRP) 4 mg/l 0-10 mg/l
27
Amylase 150 u/l 23 - 85 u/l
LFTs normal
Urine microscopy was not done on admission.
Ultrasound scans of abdomen and pelvis were normal.
The chest X-ray is shown below:
He was treated with ciprofloxacin for a presumed chest infection. He then developed acute ischaemic
bowel requiring resection, with non-specific pathological findings. The hospital course was complicated
by hypotension and multi-organ failure. The creatinine rose to 636 μmol/l with anuria; haemodialysis
was initiated.
Urine microscopy Numerous red blood cells
Protein excretion 3 g/24 h < 0.2 g/24h
ANA, ANCA negative
HBsAg negative
HCV negative
Cryoglobulins negative
C3 107 mg/dl 65 - 190 mg/dl
C4 38 mg/dl 15 - 50 mg/dl
Protein electrophoresis normal pattern
Renal biopsy was performed which showed crescentic glomerulonephritis with linear IgG staining on
immunofluorescence.
Acute pyelonephritis
Anti-glomerular basement membrane disease
Haemolytic uraemic syndrome
Microscopic polyangiitis
Post-streptococcal glomerulonephritis (PSGN)
Explanation
Anti-glomerular basement membrane disease
28
The initial presentation was of a patient with haematuria, subclinical lung haemorrhage and acute
kidney injury. Often, lung haemorrhage may present without haemoptysis and in this case was
incorrectly diagnosed as chest infection. The clinical presentation and subsequent rapid deterioration
was consistent with the syndrome of alveolar haemorrhage and kidney disease caused by anti-
glomerular basement membrane (GBM) antibodies (Goodpasture syndrome).
Diagnosis is made by the presence of circulating anti-GBM antibodies and on renal biopsy. The normal
CRP is also more supportive of anti-GBM disease rather than vasculitis. Typical appearances are of
diffuse proliferative glomerulonephritis with variable degrees of necrosis, crescent formation as in this
case, glomerulosclerosis and tubular loss. Treatment is with cytotoxic immunosuppression and plasma
exchange. Poor prognostic factors are advanced kidney disease on presentation and the degree of
crescent formation.
Type 1 RPGN is associated with anti-GBM disease, where linear staining related to IgG and C3 is seen.
Type 2 is immune complex mediated and is associated with granular glomerular staining with diffuse IgG
and complement deposition. Type 3 is pauci immune with much reduced staining for both IgG and
complement versus the other types.
Microscopic polyangiitis
Microscopic polyangiitis may also cause Goodpasture syndrome, but the negative ANCA makes it
unlikely. Microscopic polyangiitis is a vasculitis which can affect kidneys, lungs, nerves, skin and joints.
The pathological evidence would show necrotising granulomatous inflammation. P-ANCA is positive.
Acute pyelonephritis is incorrect and does not explain the pulmonary findings. Acute pyelonephritis
would present with acute fevers, chills and rigors. There may be loin pain. Treatment is with antibiotics
and/or drainage of any abscess that are present.
Haemolytic uraemic syndrome is incorrect, as the renal biopsy findings point against this. Haemolytic
uraemic syndrome is a disease characterised by haemolytic anaemia, acute kidney disease and
thrombocytopenia. The renal biopsy would show thrombotic microangiopathy.
C3 was normal and PSGN rarely results in nephrotic range proteinuria. Renal biopsy is not performed in
most patients to confirm the diagnosis of PSGN, since the resolution of PSGN typically begins within one
week of presentation.
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A.21.A 28-year-old Botswanan woman presented to the Emergency Department with a 3-week history
of generalised weakness, anorexia, decreased appetite and weight loss. She was noted to be increasingly
short of breath and had intermittent headaches. She worked as a cleaner and has been in the UK for
three years. She was unable to give a sexual history. There was no history of blood transfusions.
29
On examination, she appeared cachectic, afebrile, pulse 80/min and regular, BP 130/80 mmHg, jugular
venous pressure (JVP) not seen. There was no peripheral oedema. Chest examination was normal.
Fundoscopy revealed grade 2 hypertensive retinopathy. The remainder of the examination was normal.
Investigations:
Investigation
Result
Normal Values
Haemoglobin (Hb)
92 g/l
135 - 175 g/l
White cell count (WCC)
4.1 × 109/l
4.0 – 11.0 × 109/l
Platelets (PLT)
100 × 109/l
150 - 400 × 109/l
Blood film
Microcytosis
Sodium (Na+)
138 mmol/l
135 - 145 mmol/l
Potassium (K+)
3.2 mmol/l
3.5 - 5.0 mmol/l
Urea
18 mmol/l
2.5 - 6.5 mmol/l
Creatinine (Cr)
205 μmol/l
30
50 - 120 µmol/l
Total protein
58 g/l
60 - 83 g/l
Albumin
18 g/l
35 - 55 g/l
LFTs
Normal
Bone profile
Normal
Urine microscopy
Intact red blood cells/granular casts, protein ++
Blood/urine culture
Negative
Chest X-ray
Normal
ECG
Normal
Renal ultrasound scan
Highly echogenic kidneys; right kidney 13.5 cm, left kidney 14 cm
She underwent a native renal biopsy, the results of which are shown below:
Heroin-associated nephropathy
HIV-associated nephropathy
Malignant hypertension
Primary focal segmental glomerulosclerosis
Schistosomiasis
31
Explanation
HIV-associated nephropathy
HIV-associated nephropathy (HIVAN) is associated with massive proteinuria, microscopic haematuria

and kidney disease, and predominantly occurs in black patients with HIV infection. In the era prior to
combination antiretroviral therapy, this led to rapid progression to end-stage kidney disease. The classic
lesion on renal histology is the collapsing variant of secondary focal segmental glomerulosclerosis, which
you can see here with collapse of the glomerular tuft and oblieration of capillary loops.
Heroin-associated nephropathy
While the histological features may also represent heroin-associated nephropathy, there are no clinical
pointers towards this, and would not explain the hypergammaglobulinaemia.
Malignant hypertension
The blood pressure is the upper limit of normal with only grade 2 hypertensive retinopathy. Malignant
hypertension is characterised by vascular fibrinoid necrosis and loss of precapillary arteriolar
autoregulation. It is a medical emergency because the blood pressure rises acutely and there is risk of
stroke.
Primary focal segmental glomerulosclerosis
This would not explain the high total protein count caused by hypergammaglobulinaemia. Primary focal
segmental glomerulosclerosis is a common cause of nephrotic syndrome in adults and one of the leading
causes of kidney disease.
Schistosomiasis
Schistosoma haematobium infection of the lower urinary tract is associated with ureteral strictures and
bladder cancer. It may cause an immune-complex-mediated mesangial glomerulonephritis.
************************************************************************************
A.22.A 32-year-old man has returned from India and presents to the Emergency Department with severe
diarrhoea. He is opening his bowels up to ten times per day with watery stool and feels he is unable to
keep up with his gastrointestinal losses. He also complains of general muscle aches and light-
headedness. He was previously fit and well and has been travelling for three months on a career break.
On examination, his blood pressure is 105/80 mmHg; pulse is 95 bpm and regular. There is a postural
drop of 25 mmHg on standing.
Investigations:
Investigation
Result
Normal value
Haemoglobin (Hb)
32
129 g/l
135–175 g/l
11.2 × 109/l
4–11 × 109/l
Platelets (PLT)
201 × 109/l
150–400 × 109/l
Sodium (Na+)
138 mmol/l
135–145 mmol/l
Potassium (K+)
5.4 mmol/l
3.5–5.0 mmol/l
Creatinine (Cr)
185 µmol/l
50–120 µmol/l
Urine osmolality
280 mOsm/kg
Urine sodium
45 mmol/l
Urine dipstick
Blood and protein positive
Which of the following is the most likely cause of the elevated creatinine seen here?
Acute tubular necrosis Haemolytic uraemic syndrome Muscle necrosis Pre-renal failure Post-renal
failure
Explanation
Acute tubular necrosis
33
The clinical vignette and urine test results are in keeping with acute tubular necrosis.
Acute tubular necrosis occurs because of ischaemic damage to the renal tubules; this leads to ineffective
sodium reabsorption and the inability to concentrate the urine. The normal renal tubules avidly
reabsorb sodium within the proximal tubule. When the urinary sodium is high, this reflects the tubule's
failure to reabsorb sodium. Patients often have a large urine output with a urine osmolality of less than
450 mOsm/kg because of the inability to concentrate urine. The patient's cause of renal failure is likely
due to the persistent hypotension during the period of illness. The treatment of choice is fluid
replacement in this case.
Pre-renal failure
Pre-renal failure is associated with retained ability to concentrate the urine and retain sodium which is
not evident from the investigations.
Haemolytic uraemic syndrome, although it can cause renal impairment, is often associated with
haemolysis and low platelets which is not currently evident from the blood results.
Muscle necrosis
There is nothing in the history to suggest significant muscle necrosis as a possible cause for this patient’s
renal impairment.
Post-renal failure
There is nothing in the history to suggest the inability to pass urine and post obstructive uropathy is not
associated with high urinary sodium. Patients often have a low urinary sodium as result of obstructive
uropathy.
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A.23.A 19-year-old woman from West Africa is referred to the Renal Clinic. She gives a history of several
months of pitting oedema, which is now up to the mid-leg level, abdominal swelling, general malaise
and intermittent fevers. There was an episode of headache, upper abdominal pain and vomiting with
associated diarrhoea two years ago, but nothing else of note. She was working on a fish farm during her
time in Africa.
Examination reveals evidence of ascites and marked lower limb oedema. Cardiovascular and respiratory
examinations are unremarkable.
Investigations:
34
Alkaline phosphatase (ALP) 90 U/l 30–150 U/l
Alanine aminotransferase (ALT) 125 U/l 7 - 55 U/l
Hepatic ultrasound Evidence of hepatosplenomegaly
Urine dipstick Blood +, protein +++
Which of the following investigations is the next most appropriate?
Cardiac ultrasound scan
Hepatitis serology
Liver biopsy
Renal biopsy
Urinary protein estimation
Explanation
Urinary protein estimation
This patient has marked ascites and lower limb oedema. There are no signs of cardiac dysfunction on
examination. Renal protein loss is one likely explanation for the oedema and hypoalbuminaemia, and it
can be easily excluded. Significant exposure to mosquitoes is likely, as this patient worked on a fish farm.
She has a history suggestive of chronic malarial infection. It is well known that Plasmodium malariae
may be associated with membranous glomerulonephritis, the most likely cause of her significant
proteinuria. Chloroquine would be an appropriate treatment in this case.
Hepatitis serology
The presentation is not in keeping with hepatitis. Hepatosplenomegaly is not a feature of hepatitis,
although acute hepatitis may present with fever, general malaise and jaundice.
Cardiac ultrasound scan
Given the lack of cardiorespiratory signs on examination, it would seem unlikely that cardiac failure or
cardiomyopathy is the cause of the peripheral oedema. Therefore, cardiac ultrasound scanning is
unlikely to be helpful.
Liver biopsy
A liver biopsy would not help in the diagnosis of malaria. It would be useful in the grading of severity of
liver injury, although not in malaria. It may be useful also in cases where the cause of liver dysfunction is
unknown and infiltrative disorders are thought to be responsible.
35
Renal biopsy
A renal biopsy in malaria may show glomerulonephritis, acute tubular necrosis and acute interstitial
nephritis. However, it would not be the next most appropriate investigation.
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A.24.A 28-year-old woman with type I diabetes for the past 20 years is referred to the Renal Clinic. Her
General Practitioner has become concerned that her renal function has been slowly deteriorating at her
annual review over the past three years. Recent glycated haemoglobin (Hb A1c) is elevated at 58
mmol/mol. She smokes five cigarettes per day and is otherwise well. She is taking a basal-bolus insulin
regime and the combined oral contraceptive pill. Her blood pressure in the clinic is 135/86 mmHg.
Investigation Result Normal Value
Creatinine 130 μmol/l 50–120 μmol/l
Estimated glomerular filtration rate (eGFR) 75 ml/min/1.73 m2
Diabetic nephropathy is suspected and therefore, further investigations are completed.
Which of the following represents the key diagnostic criteria for chronic kidney disease (CKD) in this
patient?
Bilateral small kidneys on renal ultrasound
eGFR < 89 ml/min/1.73 m2 for at least six months
Mesangial expansion, fibrosis and Kimmelstiel–Wilson nodules on kidney biopsy
Urine albumin : creatinine ratio (ACR) > 3 mg/mmol for at least three months
Urine dip positive for protein on two samples, one month apart
Explanation
Urine albumin : creatinine ratio (ACR) > 3 mg/mmol for at least three months
This patient has type I diabetes with poor blood sugar control, as evidenced by her Hb A1c. Diabetic
nephropathy develops in around 20% of patients with type I diabetes and is more common in patients
with childhood-onset of disease. It is commonly associated with other microvascular complications such
as retinopathy. Although this patient’s renal function is said to have deteriorated over the past three
years, her blood pressure is currently at target and no other features of microvascular complications are
described. Diabetic nephropathy is the most common secondary cause of the nephrotic syndrome and
therefore results in microalbuminuria. Key diagnostic tests look for the presence of albumin via urine
dipstick and urine ACR measurements. Chronic kidney disease (CKD) is diagnosed if there is persistent
eGFR < 60 ml/min/1.73 m2 and/or urine ACR > 3 mg/mmol over at least three months. The key
36
treatment of choice to prevent deterioration in renal function is the use of angiotensin-converting
enzyme inhibitors or angiotensin receptor blockers.
Mesangial expansion, fibrosis and Kimmelstiel–Wilson nodules on kidney biopsy
The findings described in this answer option are common findings in diabetic nephropathy. In a patient
with CKD where the cause is unclear, a kidney biopsy may be used to confirm the cause, and therefore
to initiate appropriate treatment. However, kidney biopsy is not used to confirm the diagnosis of CKD;
this diagnosis can be made using non-invasive techniques such as blood tests, urine collection and
ultrasound.
Bilateral small kidneys on renal ultrasound
Kidney ultrasound in diabetic nephropathy is usually used to exclude other causes of deteriorating renal
function. Kidneys are usually of normal size in diabetic nephropathy, and this is used to help identify
causes such as obstruction, infection, cysts or mass. The presence of bilateral small kidneys is not used
for the diagnosis of CKD.
eGFR < 89 ml/min/1.73 m2 for at least six months
This patient has an eGFR of 75 ml/min/1.73 m2 and therefore can only be diagnosed with CKD if there
are markers of kidney damage such as the presence of microalbuminuria.
Urine dip positive for protein on two samples, one month apart
Using urine dipstick alone, CDK cannot be diagnosed. A urine dipstick should be completed for this
patient and will likely be positive for protein due to the presence of microalbuminuria in diabetic
nephropathy. If urine dipstick is positive, further investigation with early morning urine for ACR should
be completed, alongside kidney ultrasound and/or Doppler to exclude other causes.
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A.25.A 31-year-old woman presents to the Renal Clinic for a follow-up review. She is 12 weeks pregnant
and has pre-existing diabetic nephropathy.
Her glycated haemoglobin has been relatively satisfactory in the past few years, averaging 55.19
mmol/mol (7.2%), and the last recorded creatinine level was 145 µmol/l. She has already stopped her
angiotensin-converting enzyme (ACE) inhibitor but wants to know about the prognosis with respect to
her kidney disease.
Which of the following is the best prognosis with respect to this patient’s diabetic nephropathy during
pregnancy?
10% chance of worsening renal function
Likely to develop acute kidney disease
37
Less than 5% chance of worsening of her renal function
Explanation
A recent case series demonstrated that there is a worsening of renal function in up to 45% of patients
who get pregnant with pre-existing end-stage renal failure secondary to diabetic nephropathy, although
in patients with mild renal impairment (creatinine level under 150 µmol/l), the proportion of patients is
lower at 16%. In patients with creatinine levels of 150–240 µmol/l, the frequency is roughly 20%.
Worsening hypertension is strongly associated with the degree of renal progression. A generally poorer
prognosis during pregnancy is associated with lupus as the underlying cause of renal impairment.
It is thought that there is a worsening of renal function in up to 45% of patients who get pregnant with
pre-existing end-stage renal failure secondary to diabetic nephropathy.
A recent case series demonstrated that there is a worsening of renal function in up to 45% of patients
who get pregnant with pre-existing end-stage renal failure secondary to diabetic nephropathy.
Likely to develop acute kidney disease
While this patient needs close monitoring of renal function, outcomes are usually favourable and this
would be inappropriate.
Less than 5% chance of worsening of her renal function
A recent case series demonstrated that there is worsening of renal function in between 16% and 45% of
patients who get pregnant with pre-existing diabetic nephropathy, depending on the severity of renal
failure.
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A.26.A 72-year-old man is referred to the Renal Clinic with swollen ankles and proteinuria. He has a past
medical history of hypertension, which is managed with ramipril, indapamide and amlodipine.
On examination in the clinic, his BP is 149/91 mmHg and his heart rate is 88 bpm and regular. He has
bilateral pitting oedema. His BMI is 38 kg/m2.
Investigations:
White Cell Count (WCC) 5.9 × 109/l 4.0–11.0 × 109/l
Platelets (PLT) 201 × 109/l 150–400 × 109/l
38
Calcium (Ca++) 2.1 mmol/l 2.2–2.7 mmol/l
Immunoglobulin A (IgA) 0.7 g/l 0.8-3.0 g/l
Immunoglobulin G (IgG) 8.9 g/l 6.0-13.0 g/l
Immunoglobulin M (IgM) 1.0 g/l 0.4-2.5 g/l
24-hour urinary protein 350 mg <100 mg
Urinalysis 3+ protein, 1+ blood
Which of the following is the most likely primary diagnosis?
Amyloid light-chain amyloidosis
Myeloma kidney
Explanation
Focal segmental glomerulosclerosis (FSGS) is the main differential for proteinuria here, especially against
a background of hypertension and obesity. The cause is unknown, and the presentation is proteinuria
with microscopic haematuria and hypertension. Impaired renal function is common. It is more common
in young black men. There is often profound hypoalbuminaemia (albumin below 20 g/l), as well as renal
impairment running a severe course, leading to chronic kidney disease. Pointers towards FSGS as the
answer, rather than membranous nephropathy, are the relatively older age of the patient, coupled with
FSGS being the most common cause of nephrotic syndrome in the older adult.
Membranous nephropathy is the most common cause of nephrotic syndrome in younger adults.
Additionally, the majority of patients present with normotension rather than with hypertension, as seen
here. Secondary causes of membranous nephropathy include malignancy, accounting for 5–10% of
cases, autoimmune conditions, such as systemic lupus erythematosus, infectious diseases, such as
hepatitis B, and drugs, such as non-steroidal analgesia, being responsible.
Amyloid light-chain amyloidosis
Given the lack of associated chronic disease, amyloidosis is unlikely. Amyloid light-chain amyloidosis is
caused by monoclonal light chains (usually lambda) or light chain fragments produced by a plasma cell
39
dyscrasia that forms amyloid sheets. It is more common in men than in women, with a ratio of 2 : 1, and
occurs most frequently in patients over 50 years.
Minimal change disease is a more common cause of nephrotic syndrome in children. Renal biopsy is
normal on light microscopy, and immunofluorescence is negative. Electron microscopy reveals diffuse
effacement of podocyte foot processes.
Myeloma kidney
In myeloma, an increase in monoclonal proteins would be expected. In the presence of near-normal

immunoglobulins (IgA is only very slightly reduced), myeloma kidney is not likely to be the underlying
diagnosis.
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A.27.A 45-year-old woman who has diabetes presents to the Emergency Department with sepsis
secondary to cellulitis in her right calf. She is promptly initiated on intravenous (IV) co-amoxiclav and
undergoes fluid resuscitation. She makes a rapid recovery.
She is admitted to the hospital for monitoring. 48 hours after admission, you are asked to review her as
her morning blood tests show acute kidney injury (AKI). She has no other symptoms of note.
Clinical examination is unremarkable and her blood pressure is 130/80 mmHg.
Investigations:
White cell count (WCC) 11 × 109/l 4.0–11.0 × 109/l
Eosinophils 0.9 × 109/l 0.04–0.4 × 109/l
Urine dipstick protein Positive
Urine dipstick blood Positive
Urine microscopy Eosinophils
Acute screen Pending
Ultrasound (US) abdominal scan NAD
40
Acute tubulointerstitial nephritis (ATIN)
Acute tubular necrosis (ATN)
Antineutrophil cytoplasm antibodies (ANCA)-associated vasculitis
Goodpasture’s disease
Prerenal AKI
Explanation
Acute tubulointerstitial nephritis (ATIN)
Drug-induced tubulointerstitial nephritis (TIN) is common. An important, but often overlooked cause of
TIN is penicillin-derived antibiotics. In this case of cellulitis, the initiation of co-amoxiclav has resulted in
drug-induced TIN, evidenced by mild haematoproteinuria, with eosinophilia. It can be treated by a short
course of high-dose steroid, with gastrointestinal and bone protection agents. It usually requires a
kidney biopsy to confirm the diagnosis.
Acute tubular necrosis (ATN)
Acute tubular necrosis (ATN) is a process of significant injury to the tubular cells of the nephron which
occurs secondary to insults such as hypotension and drug toxicity. In this case, there was no significant
hypotension and the patient is not on any drugs which classically cause tubular necrosis such as
aminoglycosides and contrast.
Antineutrophil cytoplasm antibodies (ANCA)-associated vasculitis
Antineutrophil cytoplasm antibodies (ANCA)-associated vasculitis is a crescent-forming inflammatory

disease. A crescent is an inflammatory destructive process of the glomeruli that presents with acute,
rapidly progressive renal failure and symptoms of vasculitis (which this man lacks). It is treated by
immunosuppression, initially with steroids.
Goodpasture’s disease is an autoimmune disorder characterised by antibodies against the alpha-3

subunit of type IV collagen found in the basement membranes of the lungs and kidneys. Its presentation
is heterogeneous; alveolar haemorrhage or rapidly progressive glomerulonephritis may be found.
Goodpasture’s disease is treated with steroids and plasma exchange. In this case, the history suggests a
more likely alternative diagnosis.
Prerenal AKI
Prerenal AKI occurs as a result of a loss of pressure gradient across the glomerulus. Inadequate pressure
in the afferent arteriole results in ultrafiltration at the glomerulus. This can be as a result of either a drop
in arterial pressure or a rise in venous pressure (and consequently efferent arteriolar pressure). In this
case, this patient presented with cellulitis without significant hypotension and was promptly treated.
41
There is also a delay in onset between presentation and AKI. It is unlikely, therefore, that prerenal AKI is
responsible.
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A.28.A 45-year-old woman with a history of type I diabetes and a recent renal transplant some three
months earlier is referred to the Emergency Department by her General Practitioner because of a rise in
her creatinine level of > 30% of post-transplant baseline. She has taken cyclosporin since her transplant.
She has recently started new medication for paroxysmal atrial fibrillation (AF) and you are concerned
about a drug interaction. Clinical examination is unremarkable; her blood pressure is 142/70 mmHg, and
pulse 70 bpm (regular).
Which of the following medications is most likely to be responsible?
Amiodarone
Metoprolol
Digoxin Diltiazem Flecainide
Explanation
Diltiazem
All of these agents are possible treatments for AF, although only diltiazem is metabolised solely by
CYP3A4, and co-prescription of 90 mg or more of diltiazem can lead to a 50% or greater increase in
ciclosporin levels.
Amiodarone
Amiodarone is only a weak inhibitor of 3A4 and 2D6 CYP450 enzymes, meaning that it is much less likely
to lead to an increase in ciclosporin levels.
Metoprolol
Metoprolol is metabolised by CYP2D6. As ciclosporin is metabolised by CYP3A4, there is no potential for

drug interaction with metoprolol.
Digoxin
Digoxin is excreted largely unchanged by the kidney. It is not metabolised by CYP enzymes, meaning that
it is unlikely to be the cause of increased ciclosporin levels here.
Flecainide
Flecainide, like metoprolol, is metabolised by CYP2D6. It is unlikely to be the cause of the increased
ciclosporin levels here. Flecainide is not usually deployed for chronic AF. It is more likely to be used for
cardioversion of acute AF in a patient with significant circulatory compromise.
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42
A.29.A 28-year-old woman presents to the Renal Clinic for a follow-up appointment post-renal
transplant 13 months ago. She reports feeling occasionally fatigued but has otherwise been able to
resume her university studies. Her other past history of note includes bilateral hearing loss and
worsening vision over the past few years, for which she has gone through three pairs of glasses in the
past five years.
On examination, she is a slim-looking woman with a body mass index of 21 kg/m2. Her heart rate is 77
bpm and regular, and her blood pressure is 155/90 mmHg. She has a soft abdomen with a palpable
transplanted kidney in the left iliac fossa.
Investigations:
Investigation Previous result Result today Normal value
Haemoglobin (Hb) 122 g/l 120 g/l 135–175 g/l
White cell count (WCC) 4.2 × 109/l 4.1 × 109/l 4–11 × 109/l
Potassium (K+) 3.7 mmol/l 5.4 mmol/l 3.5–5.0 mmol/l
Sodium (Na+) 136 mmol/l 138 mmol/l 135–145 mmol/l
Creatinine (Cr) 100 µmol/l 195 µmol/l 50–120 µmol/l
Urea 4.6 mmol/l 7.1 mmol/l 2.5–6.5 mmol/l
Urinalysis: ++ protein, ++ blood.
What is the most likely cause of this patient’s presentation?
Antiglomerular basement membrane (anti-GBM) antibodies
Cytomegalovirus infection
Graft-versus-host disease
Human leukocyte antigen (HLA) mismatch
Pyelonephritis
Explanation
Antiglomerular basement membrane (anti-GBM) antibodies
This patient likely has Alport syndrome. Patients present with haematuria and proteinuria. Ocular
manifestations include anterior lenticonus, dot and fleck retinopathy and corneal dystrophy.
Leiomyomatosis has also been observed in some families with Alport syndrome. In Europe, Alport
syndrome accounts for around 0.6% of patients presenting with end-stage renal disease. Around 90%
are said to develop kidney disease by the age of 40 years. It occurs due to a gene mutation coding for
type IV collagen and a number of different mutations have been characterised. Specifically, mutations in
the alpha 3, 4 and 5 chains of type IV collagen are the ones responsible for Alport syndrome, as these
43
code for the glomerular basement membrane (GBM). Treatment of end-stage renal disease is with a
renal transplant. This patient’s deteriorating renal function and proteinuria and haematuria on urinalysis
suggest failure of the transplant. Failure of renal transplant in patients with Alport syndrome is often
secondary to the presence of anti-GBM antibodies, causing a clinical picture similar to GBM disease
(Goodpasture syndrome).
Cytomegalovirus (CMV) is a common cause of renal transplant failure, particularly given the use of
immunosuppressant agents. It typically causes acute graft rejection in less than six months. However,
this patient is presenting with signs of chronic graft failure (> 12 months’ duration), making a recurrence
of the disease the more likely cause.
Graft-versus-host disease
Graft-versus-host disease refers to transplanted stem cells and bone marrow where the donor’s own
white blood cells from the graft attack the host’s organs, causing widespread organ inflammation. It is
different from transplant rejection where the host attacks the donor’s graft.
Human leukocyte antigen (HLA) mismatch
Human leukocyte antigen mismatching is responsible for hyperacute and acute organ transplant
rejection, and this process takes place within six months. It would not explain this prolonged
presentation.
Pyelonephritis
Without symptoms of a urinary tract infection or the presence of infection on blood tests or urinalysis, it
is unlikely for pyelonephritis to be the underlying disease process.
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A.30.A 40-year-old man who is now approaching end-stage renal disease requires haemodialysis three
times a week. His brother, who is an identical twin, has perfectly normal kidney function and agrees to
donate a kidney.
During the initial work-up for the kidney transplant, his twin brother is cardiovascularly fit for surgery.
The kidney is placed anteriorly in the abdomen.
His twin brother's investigations:
Sodium (Na+) 140 mmol/l 135 - 145 mmol/l
Potassium (K+) 4.2 mmol/l 3.5 - 5.0 mmol/l
Urea 7.2 mmol/l 2.5 - 6.5 mmol/l
44
White cell count (WCC) 5.1 × 109/l 4.0 - 11.0 × 109/l
Urine dipstick Trace protein
This type of transplantation is described as:
Heterotopic isograft
Heterotopic xenograft
Orthotopic allograft
Orthotopic autograft
Orthotopic isograft
Explanation
Heterotopic isograft
Transplants are classified as:
autografts: in which the same individual acts as both donor and recipient;
isografts: in which the donor and recipient are genetically identical;
allografts: where the donor and recipient are genetically dissimilar but belong to the same species;
xenografts: in which the donor and recipient belong to different species.
In orthotopic transplants the transplanted part is placed in its normal anatomical location, while in
heterotopic transplants it is placed in a different anatomical location.
Orthotopic allograft
Donor and recipient are genetically dissimilar but belong to the same species.
Heterotopic xenograft
Xenografts are from different species such as pig and human.
Orthotopic autograft
The same individual acts as both donor and recipient.
Orthotopic isograft
The transplanted part is placed in the normal anatomical location.
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A.31.A 24-year-old man is referred to the Renal Clinic following abnormal urinalysis results at a new
patient appointment with his General Practitioner. He was noted to have a BP of 159/89 mmHg, while
urinalysis revealed 2+ blood. The patient feels well and denies any significant past medical history.
45
As he was adopted, he cannot say whether there is no family history of renal disease.
On examination, he has a BP of 154/96 mmHg. The rest of the clinical examination is unremarkable.
Investigations:
Urine microscopy Red blood cells visualised
Creatinine (Cr) 95 μmol/l 50 - 120 µmol/l
Complement C3108 mg/dl 65 - 190 mg/dl
Renal ultrasound scan:
Right kidney 10 cm, with one simple cysts
Left kidney 10 cm, normal morphology
What is the next most appropriate course of action?
Check anti-glomerular basement membrane antibodies
Carry out genetic testing for adult polycystic kidney disease
Check immunoglobulin levels
Check antinuclear antibodies
Prescribe an antihypertensive drug
Explanation
Prescribe an antihypertensive drug
The differential diagnosis includes immunoglobulin A (IgA) nephropathy and thin basement membrane
disease. Although the gold standard diagnostic test is a renal biopsy, it would not help management at
present. A renal biopsy confers risk, and therefore, there is no point in carrying out a potentially kidney-
threatening procedure for little gain. The patient’s hypertension, however, does need treatment, so he
should be commenced on an antihypertensive drug. He should be monitored periodically in the Renal
Clinic for any progression of proteinuria or worsening renal function.
Check anti-glomerular basement membrane antibodies
46
Anti-glomerular basement membrane antibodies are positive in the majority of patients with
Goodpasture syndrome. This presents with rapidly progressive glomerulonephritis and alveolar
haemorrhage, and patients are typically systemically unwell. The lack of these features points away from
this diagnosis.
Carry out genetic testing for adult polycystic kidney disease
Adult polycystic kidney disease is usually indicated if large kidneys with ten or more cysts are found in
each kidney. The two simple cysts in the normal-sized right kidney are incidental and have no clinical
significance. Therefore, this patient does not need to be screened for adult polycystic kidney disease.
Check immunoglobulin levels
The differential diagnosis includes immunoglobulin A (IgA) nephropathy and thin basement membrane
disease. If the patient has IgA disease, his serum IgA levels may well be abnormal. However, checking
the levels would not constitute a diagnostic test and is therefore unlikely to contribute to this patient’s
management here.
Check antinuclear antibodies
Antinuclear antibodies have a high sensitivity and specificity for systemic lupus erythematosus. While
lupus nephritis is a differential for a patient presenting with renal impairment in this manner, it would
be unusual for it to present without systemic features of lupus such as dermatological manifestations,
constitutional symptoms, arthralgia and fevers. The levels of C3 and C4 would also be expected to be
low.
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A.32.An 82-year-old woman is admitted following a fall while getting up to go to the toilet at night. She
was found at 1100 this morning when her carer attended for her morning visit. Her past medical history
includes osteoarthritis, hypertension and mild cognitive impairment. She is on regular co-codamol,
amlodipine and ibuprofen gel. On admission, she is disorientated, with an abbreviated mental test score
(AMTS) of 10. On examination, she is grimacing on palpation of the lower abdomen and over the hips.
Appropriate imaging is arranged.
Investigations:
Urea 15 mmol/l 2.5 - 6.5 mmol/l
Urine dipstick Blood +++
47
Nephrolithiasis
Pubic ramus fracture
Profound dehydration
Rhabdomyolysis
Urinary tract infection (UTI)
Explanation
Rhabdomyolysis
Rhabdomyolysis is a syndrome that occurs in the setting of muscle injury, whereby the intracellular
contents of myocytes are released into the plasma. Falls, with or without immobilisation, are a common
cause of rhabdomyolysis in older patients. Complications may be life-threatening and include severe
electrolyte imbalances (hyperkalaemia, hyperuricaemia, hyperphosphataemia, hypocalcaemia),
myoglobinuric kidney disease, metabolic acidosis and disseminated intravascular coagulation. Evidence
of acute kidney injury in this population is associated with shorter overall survival. Urine dipstick testing
confuses myoglobin with haemoglobin from red blood cells, hence the reason for the results seen here.
Nephrolithiasis
Nephrolithiasis would cause flank pain, which is not present in this case. The finding of lower abdominal
pain is more in keeping with constipation due to her use of opioid medication for osteoarthritis.
Pubic ramus fracture
Although a pubic ramus fracture is a possible cause of the patient’s hip and lower abdominal pain, this
would not be in keeping with the blood and urine results. All older patients who present with a fall from
standing should be treated as major trauma, with appropriate scanning to rule out injuries.
Profound dehydration
Dehydration causes a pre-kidney disease with no blood in the urine. Urinalysis may show ketonuria.
Urinary tract infection (UTI)
A urinary tract infection (UTI) may be the reason that this patient was getting up in the night to go to the
toilet. In older patients, UTI may cause urinary retention, causing deranged kidney function with an
obstructive picture. However, this patient has been able to pass urine to produce a urine dipstick. A
urine dipstick is not sufficient to diagnose a UTI in older patients. Therefore, this is not the correct
diagnosis of this patient.
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48
A.33.A 48-year-old man was admitted for an endoscopic retrograde cholangiopancreatography for a
common bile duct stone. Unfortunately, he became systemically unwell with fever, hypotension and
tachycardia. His BP is 88/50 mmHg and he was treated for septicaemia.
After four days, he remained jaundiced but was afebrile and haemodynamically stable. There was no
palpable bladder on examining his abdomen, and his urine output was 40 ml/hour.
Investigations Results Normal Values
Creatinine kinase level 600 IU/l23–175 IU/l
Venous blood gas 138 mmol/l 135–145 mmol/l
pH 7.39 7.35–7.45
Bicarbonate (HCO3−) 24.4 mmol/l 24–30 mmol/l
Which of the following treatments should be considered in addition to administering intravenous fluids?
High-dose furosemide
Low-dose dopamine infusion
Low-dose dopamine + high-dose furosemide infusion
Mannitol infusion
Sodium bicarbonate
Explanation
Sodium bicarbonate
The development of an acute kidney injury (AKI) following an episode of hypotension is a common
complication of surgery and sepsis. Management of such patients has been controversial. Low-dose
dopamine and diuretics have been an established practice for many years. Evidence-based practice has,
however, led to a re-evaluation of such therapies based on retrospective analyses and controlled trials.
Early and aggressive intravenous crystalloid administration is the major preventive therapy for patients
at risk of AKI.
Appropriate patients with AKI secondary to rhabdomyolysis, usually those with a normal calcium level
who are not alkalaemic and have a serum bicarbonate level less than 30, may benefit from urinary
alkalinisation with appropriately monitored bicarbonate therapy.
High-dose furosemide
49
In the absence of fluid overload, loop diuretics are not advised because they provoke or worsen
hypocalcaemia as they induce calciuria and increase the risk of cast formation.
Low-dose dopamine infusion
Despite previous widespread use in Intensive Care, dopamine can actually reduce renal perfusion and is
now a ‘Do Not Do Recommendation’ by the National Institute of Healthcare Excellence (NICE) in acute
kidney injury (AKI) care.
Low-dose dopamine + high-dose furosemide infusion
Historically, both loop diuretics and low-dose dopamine infusions were used in Intensive Care settings
for acute renal failure. Dopamine can actually reduce renal perfusion and is now a ‘Do Not Do
Recommendation’ according to the NICE, while in the absence of fluid overload, loop diuretics are not
advised as they provoke or worsen hypocalcaemia and increase the risk of cast formation.
Mannitol infusion
Mannitol is not recommended in AKI in the setting of rhabdomyolysis. Without close monitoring, it can
quickly lead to volume depletion, hypernatraemia and hyperkalaemia. Even when monitored
appropriately, there is no high-quality evidence that the diuresis it produces leads to improved
outcomes.
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A.34.A 61-year-old man with a history of well-controlled type I diabetes for the past 20 years comes to
the clinic for review. His General Practitioner is concerned as he has tested positive for microscopic
haematuria on three occasions over the last six months, although there is no culture evidence of urinary
tract infection. He is otherwise well, with no significant complications related to his diabetes or other
systemic illnesses.
On examination, his blood pressure is 142/82 mmHg and pulse 70 bpm and regular. There are no
abnormal findings.
Investigations:
White cell count (WCC) 12.2 × 109/l 4–11 × 109/l
Platelets (PLT) 203 × 109/l 150–400 × 109/l
Creatinine 108 µmol/l 50–120 µmol/l
Glucose 8.1 mmol/l 3.5–5.5 mmol/l
HbA1c 53 mmol/mol (7.0%) 20–42 mmol/mol (4–6%)
50
Urine dipstick: Positive haematuria, no protein
Which of the following is the most appropriate next step?
24-hour urine for albumin : creatinine ratio
Renal biopsy
Repeat sample in six months
Ultrasound scan (USS) abdomen
Urology referral
Explanation
Urology referral
This patient has a number of positive samples for microscopic haematuria against absent urinary protein
and well-controlled type 1 diabetes, making an alternative diagnosis such as diabetic nephropathy much
less likely. He also fits the criteria from the NICE guidelines, see external link, (age > 60 and microscopic
haematuria with no other obvious cause). Further investigations run the risk of waiting too long to refer
a patient with an underlying urological cancer.
Renal biopsy
Renal biopsy is only indicated here for potential glomerulonephritis. In this case, with no proteinuria and
no evidence of systemic vasculitis, there is no role for renal biopsy.
24-hour urine for albumin : creatinine ratio
Proteinuria is absent here, suggesting that an underlying malignancy, rather than glomerulonephritis or
diabetic nephropathy, is the underlying cause.
Repeat sample in six months
This risks increasing significantly the potential lag time to diagnosing an underlying urological
malignancy. The multiple positive samples mean urological investigation is essential.
Ultrasound scan (USS) abdomen
Ultrasound is relatively insensitive for detecting an underlying urological malignancy. Cystoscopy,

followed by computed tomography (CT) of the abdomen and pelvis with contrast, would have a much
higher yield for ruling out a urological malignancy.
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A.35.A 39-year-old woman presents to the Renal Clinic, having recently been diagnosed with autosomal
dominant polycystic kidney disease (ADPKD). She has a past medical history of migraines with aura but is
otherwise well. She is taking regular ramipril and amlodipine, with sumatriptan for migraine attacks. Her
father died by suicide when she was six; her mother also suffers from migraines, and her maternal
51
grandfather died of a ruptured aortic aneurysm. At diagnosis, it was found that she also has cysts in her
liver and she would like to know more about the other possible complications associated with her
diagnosis and what the management may be.
Which of the following represents a need to screen the patient for an intracranial aneurysm?
Diagnosis in the fourth decade of life
Family history of abdominal aneurysm
History of migraines with aura
Patient anxiety
Presence of liver cysts
Explanation
Patient anxiety
Cerebral aneurysms affect 9–12% of patients with polycystic kidney disease, compared to 2–3% of the
general population. However, the risk of rupture does not appear to be greater than in the general
population, and the main risk factor is a family history of rupture. The Kidney Disease: Improving Global
Outcomes (KDIGO) organisation does not recommend routine screening of autosomal dominant
polycystic kidney disease (ADPKD) patients, except in the following situations:
family history of intracerebral aneurysm (ICA) or subarachnoid haemorrhage
personal history of previous ICA rupture
patients with a high-risk profession (eg aeroplane pilot)
patient anxiety despite being given adequate information regarding risk.
Presence of liver cysts
The presence of liver cysts does not confer an increased risk of ICA and does not indicate a need for
screening.
Diagnosis in the fourth decade of life
Diagnosis in the fourth decade does not indicate a need for screening.
Family history of abdominal aneurysm
A family history of intracerebral aneurysms should prompt screening for autosomal dominant polycystic
kidney disease (ADPKD), not a history of abdominal aortic aneurysms. It is likely that this patient
inherited the ADPKD from the patient's father, as her mother is well. Therefore, she should be
encouraged to investigate whether her father suffered from an intracerebral aneurysm (ICA).
History of migraines with aura
52
A history of migraine is not a reason to screen patients with polycystic disease. Migraines are a common
condition with a preponderance in women. The onset of acute, severe non-migrainous headache may
prompt the need for investigation for ruptured ICA.
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A.36.A 31-year-old housewife attends the Diabetes Clinic for a follow-up appointment. She is a patient
who has had type I diabetes for eight years and her blood sugar level is usually well controlled on long-
acting insulin therapy. She leads a sedentary lifestyle, is obese and smokes about ten cigarettes per day.
On examination, her blood pressure is 140/95 mmHg and her heart rate is 68 bpm and regular. Her
cardiorespiratory and abdominal examinations are unremarkable.
Investigations:
Mean corpuscular volume (MCV) 78 fl 80 - 100 fl
Platelets (PLT) 214 × 109/l 150 - 400 × 109/l
Glycated Hb (HbA1c) 55 mmol/mol 9–53 mmol/mol (3–7 %)
Random glucose 7.8 mmol/l 4–11.1 mmol/l
Urinary 24-hour protein 200 mg < 150 mg
Given this patient’s presentation, what is the next most appropriate drug to initiate?
Aspirin
Gliclazide
Metformin
Ramipril
Simvastatin
Explanation
53
Ramipril
This patient has proteinuria, and there is clear evidence that angiotensin-converting enzyme (ACE)
inhibitors, such as ramipril, slow the rate of decline in renal function in patients with type II diabetes and
established diabetic nephropathy. The MICRO-HOPE study also showed that there is a mortality benefit
in this group of patients.
Aspirin
There is no evidence that aspirin reduces renal disease progression. In fact, there may be an increased
risk of bleeding.
Gliclazide
Although this patient is hypertensive, ramipril is more renal-protective in patients with hypertension and
diabetes and should be given over other agents.
Metformin
Metformin, although useful in type II diabetes, as it promotes modest weight loss and glucose control,
has no significant role in the treatment of type I diabetes. Furthermore, if the estimated glomerular
filtration rate (eGFR) is < 30%, there is a risk of lactic acidosis.
Simvastatin
Cholesterol control is important in primary cardiovascular prevention in patients with diabetes.

However, although there is trial evidence from the SHARPS trial that in patients with chronic kidney
disease, there is a mortality benefit, there is no evidence that it reduces renal progression in diabetic
nephropathy.
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A.37.A 75-year-old woman presents to the Emergency Department with dysuria and abdominal pain in
the suprapubic and left flank regions. She has a past medical history of type II diabetes and
hypertension.
On examination, she has a temperature of 37.8 °C, her BP is 125/80 mmHg and her heart rate is 75 bpm.
Investigations:
Platelets (PLT) 202 × 109/l 150–400 × 109/l
54
Urine White cells ++, Protein ++
Which of the following is the most appropriate therapy?
IV amoxicillin
Oral amoxicillin
Oral cefalexin
Oral nitrofurantoin
Oral trimethoprim
Explanation
Oral cefalexin
Current National Institute for Health and Care Excellence guidelines recommend cefalexin as a first-line
oral therapy for pyelonephritis, as seen here. If culture results indicate susceptibility, then trimethoprim
or co-amoxiclav may be reasonable alternatives.
Oral trimethoprim
Trimethoprim resistance is an increasing problem. While it can be used to treat both lower urinary tract
infections and pyelonephritis, it should only be used when sensitivities are known.
IV amoxicillin
IV therapy should be reserved for sepsis, which is not indicated here, given the normal BP and heart
rate. There is significant resistance to amoxicillin for urinary tract pathogens, so amoxicillin should only
be used if sensitivities are known.
Oral amoxicillin
Significant resistance to amoxicillin exists with respect to urinary tract pathogens, being present in up to
50% of isolates in some areas. Therefore, amoxicillin should only be used if sensitivities are known. Co-
amoxiclav effectively overcomes this resistance problem.
Oral nitrofurantoin
Nitrofurantoin may be associated with peripheral neuropathy and is not a first-line choice. However, it is
one of the few drugs commonly used in pregnancy to treat urinary tract infections. Other drugs that
may be safe for urinary tract infections in pregnancy include cefalexin, amoxicillin and pivmecillinam.
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A.38.A 52-year-old man with diabetes presents with weight loss, fevers and dull, persistent left loin pain.
The general practitioner has been treating him for relapsing urinary tract infections with oral antibiotics.
On examination, his temperature is 37.9 °C, blood pressure is 130/80 mmHg and chest and abdominal
examination is unremarkable.
55
Investigations:
White cell count (WCC) 18 × 109/l 4.0 - 11.0 × 109/l
Platelets (PLT) 510 × 109/l 150 - 400 × 109/l
Erythrocyte sedimentation rate (ESR) 67 mm/h 1 - 20 mm/h
Computerised tomography scan shows a heterogeneous non-enhancing mass on the left kidney, which
is hydronephrotic. The right kidney is normal.
Renal biopsy shows lipid-laden macrophages with lymphocytes and polymorphonuclear leukocytes.
What is the definitive treatment in this patient?
Antituberculous treatment
Intravenous antibiotics
Left nephrectomy
Lithotripsy
Radiotherapy
Explanation
Left nephrectomy
Xanthogranulomatous pyelonephritis can be difficult to distinguish from a renal cell carcinoma. It

typically presents with symptoms of fever, weight loss and loin pain. It is more common in diabetics, the
immunocompromised and patients with obstructive uropathy. The most common organism is Proteus
mirabilis. Histology combined with clinical and radiological evidence confirms the diagnosis. The
definitive treatment is nephrectomy, as medical therapy is insufficient.
Antituberculous treatment
This man does not have renal tuberculosis. Tuberculosis of the kidneys would present with sterile
pyuria. Diagnosis may be obtained with three early morning urine cultures.
56
It is difficult for intravenous antibiotics alone to treat xanthogranulomatous pyelonephritis completely.
Lithotripsy
Lithotripsy is an ultrasound treatment for renal stones. It is not utilised in this case.
Radiotherapy
Radiotherapy will not help in the treatment for this mass. As it is an infection, it is important to include
antibiotics and nephrectomy for treatment.
*******************************************************************************
A.39.A 40-year-old man with life-long episodes of fever and acute severe abdominal and chest pain
presents to outpatients. He has lived most of his life in Turkey and several members of his family have
suffered with similar symptoms, often being admitted as emergencies to local hospitals. He has been
taking high doses of painkillers and anti-inflammatory medications for many years to help with his
symptoms.
On physical examination, his temperature is 38 °C, blood pressure is 130/80 mmHg and chest and
abdominal examination is unremarkable.
He was last seen over a year ago and a comparison of his blood results from that attendance and this
shows:
Results now:
Results 14 months ago:
The single most useful test to confirm the pathological basis of his renal dysfunction would be?
Intravenous urogram (IVU)
57
MEFV gene analysis for familial Mediterranean fever (FMF)
Rectal biopsy
Renal biopsy
Serum amyloid P component (SAP scan)
Explanation
Renal biopsy
This man is likely to have familial Mediterranean FEVER (FMF), an inherited condition resulting in
repeated episodes of polyserositis mimicking acute abdominal surgical presentations such as acute
appendicitis, and often leading to unnecessary surgical exploration until the diagnosis is made. It is
relatively common in the Middle East, especially in Turkey, Syria, Lebanon, Israel and Egypt. It relates to
abnormal triggering of the inflammatory response and can lead to AA-type amyloid deposition and
therefore kidney disease. However, patients with this condition frequently take high doses of analgesics
and anti-inflammatory drugs, which puts them at risk of analgesic nephropathy.
MEFV gene analysis for familial Mediterranean fever (FMF)
Although gene testing might confirm the suspected diagnosis, it would not conclusively indicate the
cause of the renal impairment.
IVU might show characteristic ‘cup and spill’ calices suggesting possible analgesic nephropathy, but this
would not be confirmatory.
Rectal biopsy
Rectal biopsy followed by examination with polarised light leading to ‘apple-green’ birefringence might
confirm amyloidosis, but this may not indicate the cause for the renal impairment if not identified.
Serum amyloid P component (SAP scan)
Serum amyloid P component (SAP) scan may likewise confirm the presence of amyloid, but a renal
biopsy would be diagnostic for either analgesic- or amyloid-associated nephropathy.
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A.40.A 63-year-old man with known chronic kidney disease (CKD) was admitted to another hospital six
months ago with hypertension and congestive cardiac failure (CCF) where he was treated with
furosemide (frusemide) 120 mg and enalapril 5 mg. His haemoglobin (Hb) concentration at that time
was 11.0 g/dl and his creatinine level was 300 µmol/l. He has now presented to his regular hospital with
lethargy.
During the intervening period, his GP has uptitrated his enalapril to 20 mg per day. His blood pressure is
115/70 mmHg, his jugular venous pressure (JVP) is visible and not raised. Chest is clear and abdomen is
soft and non-tender. There is no peripheral oedema.
58
Investigations show:
Urine dipstick Protein 3+, blood none
What would be the most appropriate management of this patient?
Reduce enalapril
Reduce furosemide (frusemide)
Stop all drugs
Stop enalapril
Stop furosemide (frusemide)
Explanation
Reduce enalapril
In chronic kidney disease, angiotensin-converting enzyme (ACE) inhibitors can reduce the rate of loss of
renal function even if the blood pressure is normal. This is especially true if there is proteinuria.
However, ACE inhibitors should be used with caution in kidney disease, preferably under specialist
supervision, and their response monitored. Hyperkalaemia and other side-effects from their use are
more common in renal impairment. At this stage there is still some room for manoeuvre and his enalpril
can be downtitrated to establish the effect on his creatinine and his potassium. This is the most
appropriate option here as the creatinine deterioration has corresponded with the uptitration of
enalapril by his GP, whereas the worsening anaemia is due to decreased erythropoietin production in
kidney as the kidney function deteriorated.
Stop furosemide (frusemide)
Temporary withdrawal of the loop diuretic may cause rebound pulmonary oedema.
Reduce furosemide (frusemide)
Reduction of the loop diuretic dose would contribute to the hyperkalaemia.
Stop all drugs
59
Stopping all medications may cause hypertension with stopping the enalapril. There would be a risk of
rebound pulmonary oedema with stopping the diuretic.
Stop enalapril
Using ACE inhibitors is important for proteinuria control; therefore to stop enalapril altogether would
not be beneficial. There is room for manoeuvre; therefore reduction with careful monitoring would be a
more appropriate option.
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A.41.A 32-year-old man is being treated with atazanavir containing HAART regime for HIV infection.
Other medication of note includes co-trimoxazole which was recently started as prophylaxis against PCP.
He has noticed increasing nausea and tiredness over the past few weeks.
Investigations reveal the following:
Platelets (PLT) 180 × 109/l 150–400 × 109/l
Creatinine (Cr) 276 μmol/l 50–120 µmol/l
Urine Blood +, Protein +
Given the most likely renal diagnosis, which of the following is the most appropriate therapy for this
patient?
IV hydrocortisone
Co-amoxiclav
IV methylprednisolone and cyclophosphamide
IV normal saline
IV sodium bicarbonate
Explanation
IV normal saline
This patient has crystal nephropathy, a condition known to be associated with atazanavir use, which is a
common component of HAART. Certain drug-drug interactions lead to increased atazanavir
60
concentration, one of which is co-trimoxazole. Adequate rehydration is crucial to avoid crystal
nephropathy, and patients taking atazanavir are recommended to take at least 2-3 litres of fluid per day.
IV rehydration is the treatment of choice for crystal nephropathy and, given adequate fluid replacement,
atazanavir therapy can often continue.
IV sodium bicarbonate
Sodium bicarbonate therapy may be used in some renal stone disease but not for indinavir crystals. An
example is that in one short-term study it has been reported that sodium bicarbonate is an effective
treatment for hypocitraturic calcium oxalate stones.
IV hydrocortisone
IV hydrocortisone is not a treatment for crystal nephropathy. Hydrocortisone may be used for treatment
of adrenal failure as a replacement for corticosteroids.
Co-amoxiclav
Co-amoxiclav would treat any bacterial urinary tract infection. Although blood and protein in the urine
may be mistaken for a urinary tract infection (UTI), you would expect other symptoms such as dysuria
and urgency of urination. In addition, the white cell count may be raised in a UTI.
IV methylprednisolone and cyclophosphamide
IV methylprednisolone and cyclophosphamide is not used for treatment of renal stones, but it may be
used in this combination to treat renal vasculitis. In this condition there is a rapidly progressive renal
dysfunction with blood and protein in the urine.
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A.42.A 40-year-old man is undergoing regular haemodialysis is receiving erythropoietin, but his Hb is still
low. There is no evidence of active or past infection. Other than chronic kidney disease of unknown
cause, he has no other past medical history.
He is on oral iron, which was started after a confirmed low ferritin.
Upon examination, his BMI is 28 kg/m2, and chest and abdominal examination was unremarkable.
Investigations showed:
C-reactive protein (CRP) 20 mg/l < 10 mg/l
61
Platelets (PLT) 560 × 109/l 150 - 400 × 109/l
What is the next step in his management?
Check for antibodies against erythropoietin
Give higher doses of iron tablets
Give folic acid
Give IV iron
Transfuse
Explanation
Give IV iron
Iron given orally is poorly absorbed in patients with chronic kidney disease. The gold standard method of
replacement recommended by renal physicians is IV. According to the UK Renal Association guidelines,
in renal patients we would aim to achieve a ferritin of >100 mg/l and TSAT >20% (or <10% hypochromic
red cells).
Check for antibodies against erythropoietin
Antibodies to erythropoietin can occur but iron should be given IV to dialysis patients, as in this case.
Antibodies are not routinely checked.
Give higher doses of iron tablets
Iron is poorly absorbed orally in chronic kidney disease; therefore higher doses will only increase risk of
side effects such as constipation, with little gain.
Give folic acid
There is no evidence of folic acid deficiency in this case. This patient has an iron-deficiency anaemia.
Transfuse
The first step is to give intravenous iron. Transfusion should only be given with caution in patients with
renal disease because of risk of fluid overload and sensitisation with antibodies which may render the
patient difficult for future renal transplant.
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A.43.A 64-year-old man presents with a 2-month history of dysuria, urinary urgency and pelvic
discomfort. He was investigated last year for proteinuria and haematuria picked up on urinalysis at the
general practitioner’s surgery, although no specific cause was found. He smokes ten cigarettes per day
and has travelled extensively as a missionary in the Asian subcontinent working as a teacher.
62
Repeated urine cultures sent at this time showed no growth. He also reports weight loss over the last
year of two stone.
Investigations:
Platelets (PLT) 479 × 109/l 150 - 400 × 109/l
Mean corpuscular haemoglobin (MCH) 32 g/dl 27 - 33 g/dl
C-reactive protein (CRP) 56 mg/l < 10 mg.l
Urinalysis:
Protein 1+
Blood 1+
Leucoctyes 3+
Glucose Negative
Two recent urine cultures have shown mixed growth on one and no growth on a second.
Chronic/recurrent E. coli infection
Chronic prostatitis
Renal cell carcinoma
Renal tract tuberculosis
Squamous cell carcinoma of the bladder
Explanation
63
Renal tract tuberculosis
This man has very long-standing urinalysis abnormalities with weight loss and symptoms of bladder
irritation. In addition, he has evidence of an inflammatory response with a mild anaemia. These raise the
suspicion of either malignant disease or severe chronic infective disease such as tuberculosis. The renal
tract is a common site for tuberculous infection after the lungs, and is commonly involved in association
with primary TB infection (albeit often without being detected clinically). It can be difficult to diagnose
and requires repeated early morning urine cultures and occasionally more invasive steps such as urinary
tract biopsy on cystoscopy.
Chronic/recurrent E. coli infection
Occult bacterial infection is unlikely to be the cause in view of the repeated negative urinary cultures.
Chronic prostatitis
Although prostatic symptoms are present, such as dysuria and urgency. Penile discharge may be present
and we would expect the urinary growth to be positive for bacteria.
The symptoms of bladder irritation are unlikely to occur with renal cell carcinoma.
Although squamous cell carcinoma of the bladder is a possibility, this would be more likely to be a
transitional cell lesion.
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A.44.A 49-year-old man with a history of type 1 diabetes is admitted to the Emergency Room with an
epileptic seizure. He has chronic kidney disease and has been receiving dialysis for the past three
months.
Medication includes ramipril, amlodipine, aspirin, calcium and vitamin D, and periodic injections of
recombinant erythropoietin, which were begun recently.
On examination, his blood pressure (BP) is 145/85 mmHg, with a temperature of 37.8 °C; his Glasgow
Coma Scale (GCS) score is 13.
Investigations:
Platelets (PLT) 295 × 109/l 150–400 × 109/l
64
Serum Glucose 3.9 mmol/l 3.9–7.1 mmol/l
Computed tomography (CT) head No focal lesion identified
Which of the following is the most likely cause of his seizure?
Bacterial meningitis
Erythropoietin-induced epilepsy
Ischaemic stroke
Viral meningitis
Uraemic encephalopathy
Explanation
Erythropoietin-induced epilepsy
This is erythropoietin-induced epilepsy. This rare, adverse event is seen within 90 days of erythropoietin
initiation. His white blood cell count is normal, making sepsis less likely, and the elevated temperature
here is just likely to be the result of his fit.
The creatinine level of 256 μmol/l is most likely due to the interval between his dialysis sessions. The
exact mechanism related to the seizures is unclear but may be related to veno-occlusive disease or the
rise in haemoglobin associated with erythropoietin use.
He has been receiving dialysis for the last three months; it would be unlikely that his urea level would be
raised, providing he was getting adequate dialysis.
Bacterial meningitis
This is incorrect because we would have expected the white cell count to be raised. In bacterial
meningitis, there may be symptoms of fever, skin rash and meningism, such as photophobia and neck
stiffness.
Lumbar puncture for bacterial meningitis usually shows an elevated opening pressure (> 25 cm of
cerebrospinal fluid) and a white blood cell count of > 100 cells/µl, with > 90% of polymorphs; glucose
level is low (< 40% of serum glucose) and protein level is elevated (> 50 mg/dl).
Ischaemic stroke
CT head shows no focal lesion. The other symptoms of an ischaemic stroke depend on the location of
the stroke, but may include hemiplegia, visual disturbances and dysphasia.
Viral meningitis
65
We would have expected the white cell count to be abnormal. Viral meningitis may present with
symptoms of meningism, such as photophobia and neck stiffness.
Lumbar puncture for viral meningitis shows a normal or elevated opening pressure, a white blood cell
count of 10–1000 cells/µl, classically lymphocytes, but there may be polymorphs early on, and glucose
levels normally > 60% of serum levels, but they may be low in herpes simplex virus (HSV) infection.
Protein levels are elevated (> 50 mg/dl).
The common culprits are HSV, varicella-zoster virus and adenovirus/enteroviruses. Therefore, a lumbar
puncture sample should be sent for viral polymerase chain reaction (PCR).
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A.45.A 23-year-old man was admitted to hospital with severe left loin pain and vomiting. Prior to this he
described a 5-week history of worsening generalised weakness and anorexia. He was previously well and
on no regular medication.
On examination, he was in obvious discomfort and hyperventilating. There was tenderness and guarding
in the left renal angle, but no other significant findings.
Investigation results are below:
Platelets (PLT) 200 × 109/l 150 - 400 × 109/l
Bicarbonate (HCO3-) 16 mmol/l 22 - 29 mmol/l
Chloride (Cl-) 120 mmol/l 98 - 106 mmol/l
Urate 3.2 mmol/dl 3.4 - 7.0 mmol/dl
Arterial blood gases (on air):
pH 7.210 7.35 - 7.45
pO2 12.6 kPa > 10.5 kPa
66
pCO2 4.8 kPa 4.7 - 6.0 kPa
Urinalysis pH 6.3, blood ++
Abdominal X-ray Speckled calcification in the left and right upper quadrants
Fanconi syndrome
Type 1 (distal) renal tubular acidosis
Type 2 (proximal) renal tubular acidosis
Type 4 renal tubular acidosis
Explanation
The kidneys are unable to create an acid urine due to failure of hydrogen ion excretion in the distal
tubule. Urine pH will be greater than 5.5, often in the presence of a severe systemic acidosis. There is
almost invariably hypokalaemia, which can cause severe weakness. Hypercalciuria and
hyperphosphaturia occur due to the release of calcium phosphate from bone in order to buffer excess
hydrogen ions during acidosis, and the direct effects of acidosis on tubular reabsorption of these ions.
Other clinical features include bone pain due to osteomalacia, renal stones, constipation, fatigue and
anorexia. Nephrocalcinosis may be seen. Renal function is usually preserved. In children it commonly
presents with polyuria, thirst and failure to thrive. Biochemically, there is a hyperchloraemic metabolic
acidosis with a normal anion gap. Causes of type 1 renal tubular acidosis include autoimmune diseases
(eg systemic lupus erythematosus), drugs (eg amphotecerin B), or it may be inherited or idiopathic.
Treatment is with bicarbonate replacement (1–3 mmol/kg/day); however hypokalaemia must be
corrected before the acidosis in order to prevent a further fall in the potassium and the risk of cardiac
arrest. Nephrocalcinosis is not a feature of proximal renal tubular acidosis.
There is impaired retention of bicarbonate in the proximal tubule leading to bicarbonate wasting and
systemic acidosis. There is hypercholoraemic metabolic acidosis. Common causes include myeloma,
amyloidosis, cystinosis, Wilson’s disease and heavy metal toxicity.
Fanconi syndrome
Fanconi syndrome is a generalised descriptive term for proximal tubular dysfunction. There is a failure of
the proximal tubules to reabsorb many filtered substances and classically there is phosphate wasting.
There is bone pain and osteomalacia. The causes are as for proximal renal tubular acidosis (see below).
Investigations would show a metabolic acidosis, reduced serum phosphate, reduced serum urate,
glycosuria, proteinuria and increased urinary phosphate and citrate.
67
Medullary sponge kidney is a benign common condition characterised by diffuse medullary cystic
formation. Impaired calcium handling leads to formation of calcium-containing stones. There are no
other biochemical abnormalities such as acidosis seen in this case.
This is known as hyporeninaemic hypoaldosteronism. Aldosterone promotes urinary potassium loss so

its absence leads to hyperkalaemia. Investigations would show hyperkalaemia, low bicarbonate (rarely
less than 16 mmol/l), normal anion gap with increased chloride, negative urinary anion gap, reduced
urinary citrate. Urine pH is variable but often less than 5.3.
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A.46.A 50-year-old man with end-stage renal disease had a renal transplant four months ago. His
baseline creatinine is 150 µmol/L. On physical examination his blood pressure is 160/70 mmHg and he
has tenderness over his transplant kidney. His blood results reveal urea 22 mmol/l, creatinine 500
µmol/l.
Which of the following renal pathologies is most likely to recur in a renal transplant patient?
Diabetic renal disease
Minimal-change glomerulonephritis
Membranous glomerulonephritis
Membranoproliferative glomerulonephritis
Explanation
Membranoproliferative glomerulonephritis
Membranoproliferative glomerulonephritis (MPGN) recurs at a high rate after kidney transplantation. It

is associated with a 30–90% recurrence rate post renal transplant (type 2 much greater than type 1). The
risk of MPGN recurrence increases with preemptive transplantation, living related donation, low
complement level, and the presence of monoclonal gammopathy. Recurrence of MPGN leads to
allograft failure in half of the cases.
Diabetic renal disease
It takes years to appear in a transplanted kidney.
Whilst the recurrence rate is also high with FSGS (40%), it does not quite approach the levels seen with
membranoproliferative glomerulonephritis.
68
Minimal-change glomerulonephritis
This is not usually a cause of recurrence within renal transplant.
The recurrence rates from case series approach 30%, and therefore not as high as option E.
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A.47.A 75-year-old man who is hypertensive and has type II diabetes mellitus managed with insulin on a
basal-bolus regimen presents to the hospital with an acute, right-sided middle cerebral artery stroke.
He is quickly admitted to the Hyperacute Stroke Unit, but due to hypertension, he is not thrombolysed.
He is subsequently transferred to the Stroke Ward for long-term management and rehabilitation. His
past medical history includes angina, for which he is awaiting angiography.
His preadmission medications include insulin Levemir® 28 units at night, insulin NovoRapid® 14 units
twice a day, amlodipine 10 mg, bisoprolol 5 mg once a day, glyceryl nitrate (GTN) spray as required and
atorvastatin 80 mg at night.
On examination, he is alert and aphasic, with gross left-sided weakness affecting his arms and legs. His
blood pressure is 190/110 mmHg. His body mass index is 32 kg/m2.
A battery of investigations are performed, and he is identified on carotid ultrasound to have right-sided,
tight, carotid stenosis. An echocardiogram (ECHO) is also performed during his admission and he is
found to have left ventricular hypertrophy and an ejection fraction of 35%. He is initiated on ramipril 2.5
mg once a day.
Investigations:
Investigation On admission After two days Normal value
White cell count
(WCC) 4.0 × 109/l 5.3 × 109/l 4.0–11.0 × 109/l
Haemoglobin
(Hb) 110 g/l 105 g/l 135–175 g/l
Potassium
(K+) 5.1 mmol/l 5.9 mmol/l 3.5–5.0 mmol/l
Ultrasound scanning of the renal tract is performed, which reveals a left-sided kidney measuring 10 cm
without hydronephrosis and a right-sided kidney measuring 9.8 cm.
69
What is the most likely underlying diagnosis?
Fibromuscular dysplasia (FMD)
Ramipril toxicity
Bilateral renal artery stenosis (RAS)
Explanation
Bilateral renal artery stenosis (RAS)
Renal artery stenosis (RAS) can occur in the older population as a consequence of atherosclerotic
disease. Typically, this results in secondary hyperaldosteronism, hypertension and, occasionally,
hypokalaemic metabolic alkalosis. Evidence suggests that stenting these stenotic renal arteries does not
improve outcomes secondary to standard medical care; however, this practice is still performed in
individuals with flash pulmonary oedema, as this group was not included in the initial trials.
This patient has some important risk factors for RAS – he is diabetic and has vasculopathy, as shown by
the presence of carotid artery stenosis and coronary artery disease. Additionally, he has severe
hypertension and has had a sudden drop in his renal function after initiating angiotensin-converting
enzyme (ACE) inhibitors. This should prompt consideration of bilateral RAS.

progressive renal failure, which often coexists with other diseases, such as hypertension. It is unlikely to
cause AKI. Kidney biopsies of those with diabetic nephropathy typically reveal Kimmelstiel–Wilson
nodules. Good diabetes management and antihypertensive, proteinuria-reducing medications are the
cornerstones of prevention.
Cholesterol embolism is a common cause of acute kidney injury (AKI) with eosinophilia, often
accompanied by a distal limb rash of livedo reticularis. Cholesterol embolism often occurs secondary to
procedures and drugs, which result in cholesterol becoming dislodged. This includes angiograms with
stenting, procedures for abdominal aortic aneurysms, carotid endarterectomies and the initiation of
warfarin. Of note, in this case, this patient was not thrombolysed and has not been initiated on a vitamin
K antagonist. Additionally, his white cell count (WCC) is normal and there is no mention of eosinophilia
or rash of livedo reticularis.
Fibromuscular dysplasia (FMD)
Fibromuscular dysplasia (FMD) is rare. It is a disorder characterised by intractable hypertension,

commonly in young people and is a secondary hypertensive disease. It is a form of RAS, which on
70
imaging is reported as ‘beading’ of the renal artery. Importantly, in young, hypertensive patients with
FMD, the characteristic history is of flash pulmonary oedema or a sudden, brisk deterioration in renal
function after the initiation of ACE inhibitors (secondary to a gross, overexaggerated response to the
drop in glomerular pressure) is seen. In this case, while this patient does have a sudden drop in his renal
function following ACE inhibition, another cause of his hypertension is more likely than a rare disorder
of young adults; particularly considering that he is not considered young.
Ramipril toxicity
Ramipril and other ACE inhibitors are important first-line anti-hypertensive agents. They function by
inhibiting efferent arteriolar vasoconstriction, and therefore reduce glomerular pressure. Consequently,
they reduce proteinuria (in patients with proteinuric chronic kidney disease), reduce blood pressure and
increase potassium. Importantly, because of the reduction in glomerular pressure and ultrafiltration, a
moderate rise of 20% in creatinine (Cr) is expected. A greater reduction in Cr should prompt
consideration of alternative diagnoses.
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A.48.You review a 19-year-old woman who has been referred by the Emergency Department after
attending with a faint. She is noted, on routine blood testing in the department, to have a potassium
level of 2.9 mmol/l with a serum bicarbonate of 30 mmol/l. In addition, her blood pressure on admission
was elevated at 155/90 mmHg. She has no past medical history of note and is on no medications. You
arrange some further blood tests, which reveal low renin and aldosterone levels. A urine diuretic screen
is negative.
Which of the following represents the best long-term treatment for her?
Potassium replacement
Ramipril
Spironolactone
Amiloride Bumetanide
Explanation
Amiloride
This woman presents with a hypokalaemic alkalosis and suppressed renin and aldosterone levels in the
presence of hypertension. This is strongly suggestive of Liddle syndrome, an autosomal dominant
condition thought to arise from a mutation in genes mapped to chromosome 16. This mutation leads to
abnormality in the beta- or gamma-subunit of the highly selective epithelial sodium channel in the distal
nephron. This results in activation of sodium/potassium exchange independent of circulating
mineralocorticoid. Hypertension and hypokalaemia respond well to amiloride but not spironolactone,
because amiloride acts directly on the sodium channel whereas spironolactone acts on the
mineralocorticoid receptor. Hypokalaemia and alkalosis in normotensive individuals who have elevated
plasma renin and aldosterone levels is suggestive of either Bartter or Gitelman syndrome.
71
Potassium replacement
Potassium replacement is of value in the treatment of Bartter syndrome and Gitelman syndrome, but it
isn’t an option for Liddle syndrome because it doesn’t improve blood pressure control.
Ramipril
Angiotensin-converting enzyme (ACE) inhibition is of most value in the treatment of hyporeninaemic

hyperaldosteronism, but it is less effective in the treatment of Liddle syndrome compared to amiloride.
Spironolactone
Spironolactone is an intervention of choice in the treatment of hyporeninaemic hyperaldosteronism.
Bumetanide
Bumetanide is a loop diuretic that is most useful in the treatment of fluid overload related to left
ventricular failure.
*********************************************************************************
A.49.A 43-year-old man is brought into hospital during the night. He was found on the street smelling
strongly of alcohol. Staff recall that he also has a history of intravenous drug use.
He is disorientated, localises pain and will open his eyes in response to a painful stimulus. Reflexes are
symmetrically brisk. Pupils are 2 mm in diameter. Blood pressure 95/60 mmHg, respiratory rate 12/min,
oxygen saturation 97% on air
Initial Investigations reveal the following:
Electrocardiogram (ECG) Sinus tachycardia, inverted p wave aVr
Chest X-ray (CXR) Hyperinflated lung fields, old healed rib fractures
Urine dipstick Protein +, blood +++
Platelets (PLT) 420 × 109/l 150–400 × 109/l
Phosphate (PO43-) 1.69 mmol/l 0.70–1.40 mmol/l
72
His ECG is shown below.
What appropriate step should be taken next?
Intravenous calcium gluconate
Vitamin D replacement therapy
Catheterise and intravenous saline
Intravenous naloxone
Explanation
Catheterise and intravenous saline
The diagnosis is rhabdomyolysis. The patient has high risk factors with alcohol and intravenous drug use.
He has most likely been immobile on the street, or could have suffered injury while intoxicated. The ECG
shows a sinus tachycardia of 115bpm, but otherwise no sinister features. Myoglobinuria gives a ‘false-
positive’ on urine dipstick for blood. Tests reveal typically hyperkalaemia, hypocalcaemia, raised
creatinine:urea ratio, hyperphosphataemia. Further confirmatory investigations include urine
myoglobin, serum creatine kinase and serum myoglobin. Fluid resuscitation is needed with urine output
measurement.
Image source:
https://upload.wikimedia.org/wikipedia/commons/8/81/Sinustachycaria_%28CardioNetworks_ECGpedi
a%29.png
Calcium gluconate helps stabilise cardiac membrane in hyperkalaemia. In this case rehydration will help
hyperkalaemia. There are no ECG changes consistent with severe hyperkalaemia. The latter stages of
rhabdomyolysis may result in hypercalcaemia as calcium is released from the muscles. This is more likely
to occur if supplementary calcium has been given.
Vitamin D replacement therapy
During later stages of rhabdomylosis, the accumulated calcium is released from the storage sites. This is
often associated with hyperparathyroidism and hypervitaminosis D and overt hypercalcaemia. However,
the hyperparathyroidism and hypervitaminosis D are not seen in all cases. Therefore, vitamin D is not a
treatment.
There is no evidence of any infection. The white cell count is normal. Therefore, antibiotics would not be
the treatment of choice.
Intravenous naloxone
73
He does not have symptoms of opioid overdose which are sufficiently severe enough to require
naloxone, and intravenous calcium can increase the destruction of muscle. The hyperkalaemia needs
monitoring but no active treatment is needed currently as fluid hydration will help.
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A.50.A 60-year-old man visits his General Practitioner with a 5-week history of intermittent claudication
in his lower limbs which improves after a period of rest. He has also noticed progressive swelling in his
legs. He has no past medical history of note other than completing a course of antibiotics for Treponema
pallidum infection two months ago. There is no history of smoking and he has a moderately healthy diet.
On examination, he appears well, with normal observations. On auscultation, his chest is clear and heart
sounds are normal, with a capillary refill time of < 2 seconds. There is no palpable abdominal tenderness
or organomegaly. He has pitting oedema to his knees. His dorsalis pedis pulses are present bilaterally.
Investigations:
Mean cell volume (MCV) 82 fl 80–100 fl
Neutrophils 6.1 × 109/l 2.5–7.5 × 109/l
Lymphocytes 3.7 × 109/l 0.8–5.0 × 109/l
Platelets (PLT) 213 × 109/l 150–400 × 109/l
Estimated glomerular filtration rate (eGFR) 40 ml/min/1.73 m2 60+ ml/min/1.73 m2
Albumin 40 g/l 35–55 g/l
Granulomatosis with polyangiitis
Nephrogenic systemic fibrosis
74
Non-Hodgkin’s lymphoma
Psoas abscess
Explanation
This man has retroperitoneal fibrosis, a chronic inflammatory process causing fibrosis of retroperitoneal
structures. Symptoms can initially be very vague and range from lower back and abdominal or flank
pain, along with general malaise and lethargy. Compression of the ureters causes ureteric obstruction,
combined with deranged kidney function and compression of the arterial supply and venous return from
the lower limbs can cause intermittent claudication and bilateral limb swelling, respectively. Chronic
normocytic anaemia is also common on routine laboratory testing, in addition to raised inflammatory
markers. Retroperitoneal fibrosis is exceedingly rare and most cases are idiopathic. However, the
disease is associated with a number of other illnesses such as malignancy, sarcoidosis, radiotherapy,
surgery and infection, including Mycobacterium tuberculosis and T. pallidum.
Psoas abscess
A psoas abscess is a collection of pus in the psoas muscle that can spread into the retroperitoneal space.
It is commonly diagnosed late and can cause severe illness. Risk factors include immunosuppression,
tuberculosis and intravenous drug use. Presenting symptoms include severe lower back or buttock pain,
along with fever and point tenderness on examination. The absence of risk factors, fever or a raised
WCC points away from a diagnosis of a psoas abscess.
Granulomatosis with polyangiitis is a type of small vessel vasculitis associated with respiratory and renal
involvement. Presenting features include nasal crusting and congestion, sinusitis and
glomerulonephritis. In more severe disease, haemoptysis and pulmonary haemorrhage can be seen.
However, granulomatosis with polyangiitis does not cause symptoms of retroperitoneal fibrosis.
Nephrogenic systemic fibrosis is a progressive disease characterised by rapid skin and soft tissue
thickening and fibrosis in patients with kidney impairment (commonly late-stage chronic kidney disease
or dialysis patients) following gadolinium contrast exposure. It does not explain this patient’s
intermittent claudication or progressive leg swelling.
Non-Hodgkin’s lymphoma
Non-Hodgkin’s lymphoma refers to a group of haematological malignancies associated with enlarged

lymph nodes, fever, night sweats and weight loss. Lymphomas are associated with a variety of illnesses,
including autoimmune diseases, immunosuppression and infections such as Helicobacter pylori and
hepatitis C. If located in the retroperitoneal space, lymphomas can have a similar radiological
appearance to retroperitoneal fibrosis and may cause similar symptoms secondary to compression of
75
retroperitoneal structures. However, the absence of constitutional symptoms (weight loss, fever and
night sweats), lymphadenopathy and raised WCC or lymphocyte count suggests an alternative diagnosis.
*************************************************************************************
A.51.A 40-year-old man is referred by his GP to Accident and Emergency with severe acute left-sided
flank pain. This is the first time he has had such severe sudden pain. He has no past medical history of
cardiovascular or kidney disease.
On examination, his BMI is 30 kg/m2 and pulse 78 bpm and regular. His jugular venous pressure is not
raised, heart sounds are normal and his chest is clear. His liver is not palpable but he has a mass in both
flanks, larger on the left than the right.
Mean corpuscular volume (MCV) 81 fl 80–100 fl
Platelets (PLT) 243 × 109 /l 150–400 × 109/l
Erythrocyte sedimentation rate (ESR) 8 mm/hour 1–20 mm/hour
Urine dipstick blood +++, protein +
What other disorder is this patient most likely to suffer from?
Aortic valve prolapse
Berry aneurysms in 50–75%
Hypertension
Gallstones
Diabetes mellitus
Explanation
Hypertension
76
The most likely diagnosis in this patient is adult polycystic kidney disease. It is a common autosomal-
dominant disease with a gene defect on the short arm of chromosome 16 and usually presents in the
fourth and fifth decades. In this patient, it was most likely complicated by haemorrhage into a cyst, and
hence the pain. It usually presents with haematuria, hypertension, loin pain, urinary tract infection and
kidney disease. It is associated with hepatic, pancreatic and ovarian cysts, and subarachnoid
haemorrhage, renal calculi, mitral valve prolapse, polycythaemia and anaemia of chronic kidney disease.
Berry aneurysms in 50–75%
The incidence of berry aneurysms in ADPKD is a lot lower, in the region of 20–25%.
Aortic valve prolapse
The common valvular condition in autosomal dominant polycystic kidney disease (ADPKD) is mitral valve
prolapse rather than aortic valve.
Gallstones
With respect to a diagnosis of gallstones, that seems unlikely here: there is no association between
APKD and gallstones, and no symptoms to suggest that as the underlying acute problem.
Diabetes mellitus
Diabetes mellitus may co-exist if we are suspecting a diagnosis of maturity onset diabetes of the young
(MODY), but he would have been expected to present with diabetes before the age of 42.
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A.52.A 37-year-old woman with a history of type 2 diabetes comes to the clinic for review. Medications
include metformin for control of blood glucose, and she is using over-the-counter orlistat in an attempt
to control her weight. She informs you that she has recently got married and wishes to start a family as
soon as possible. Her blood pressure is 132/72 mmHg, and pulse 70 bpm and regular. Her body mass
index (BMI) is 30 kg/m2.
Investigations:
Platelets (PLT) 181 × 109/l 150–400 × 109/l
HbA1c 53 mmol/mol (7.0%) <53 mmol/mol (<7.0%)
Urine albumin : creatinine ratio, 57 mg/l (< 30mg/l)
77
Which of the following is most appropriate with respect to anti-hypertensive medication?
Avoid anti-hypertensive medication currently Start Bendroflumethiazide Start Labetalol Start Ramipril
Start Valsartan
Explanation
Avoid anti-hypertensive medication currently
In this situation, an angiotensin-converting enzyme (ACE) inhibitor could be considered to delay

progression of diabetic nephropathy, although both ACE inhibitors and angiotensin receptor blockers
(ARBs) are teratogenic and are therefore best avoided in a patient who is contemplating pregnancy.
Bendroflumethiazide is also potentially teratogenic and is therefore avoided in this situation. Blood
pressure would reduce in first trimester and hence monitoring may require.During pregnancy,
hypertension is defined as systolic blood pressure ≥140 mmHg and/or diastolic blood pressure ≥90
mmHg. Severe hypertension is defined as systolic blood pressure ≥160 mmHg and/or diastolic blood
pressure ≥110 mmHg.
Start Bendroflumethiazide
Bendroflumethiazide is best avoided in this situation because of its teratogenetic effects on the fetus.
Thiazide diuretics can reduce placental blood flow to the fetus if used in the second or third trimester;
this causes jaundice, low platelet levels and fetal electrolyte disturbances.
Start Labetalol
Labetalol is a treatment for hypertension in pregnancy, though it will have no effects on the progression
of this patient’s diabetic nephropathy.
Start Ramipril
Ramipril is best avoided in this situation because of its links with increased fetal and neonatal death.
Start Valsartan
Valsartan is best avoided in pregnancy because of its links with spontaneous abortion, oligohydramnios
and newborn renal dysfunction.
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A.53.A 44-year-old man with a background of B-cell lymphoma is admitted to the Haematology Ward
with fevers, nausea and vomiting. He is diagnosed with sepsis secondary to his indwelling peripherally
inserted central catheter line and is started on broad-spectrum intravenous antibiotics. The
Haematology Team also commence filgrastim to support his immune function.
During his admission, it was noted there were some derangements in his blood tests:
Investigation Results on admission Results today Normal value
78
White cell count (WCC) 1.7 × 109/l 2.5 × 109/l 4.0–11.0 × 109/l
Platelets (PLT) 91 × 109/l 115 × 109/l 150–400 × 109/l
C-reactive protein (CRP) 44 mg/l 20 mg/l < 10 mg/l
Additional investigations were requested:
Investigation Results today Normal value
Urine sodium 50 mmol/l 40–220 mmol/day
Urine osmolality 300 mOsm/kg 50 to 1200 mOsm/kg
Cyclizine
Filgrastim
Meropenem
Ondansetron
Vancomycin
Explanation
Vancomycin
This patient has developed a significant acute kidney injury based on the worsening urea and creatinine
blood tests during hospital admission. In the assessment of an acute kidney injury, it is important to
establish whether the cause is prerenal (eg hypoperfusion secondary to sepsis), renal (eg acute tubular
necrosis, ATN) or post-renal (eg urinary obstruction). Taking a closer look at this patient’s blood tests,
there are several indicators that this patient’s acute kidney injury is caused by ATN.
In patients with ATN, due to the intrinsic damage to the renal tubular epithelium, the kidneys are less
able to hold on to electrolytes, causing an elevated urinary sodium level (ie over 40 mmol/l). There is
also reduced reabsorption of water, electrolytes and urea, causing a fairly dilute urine with a normal-to-
low urine osmolality (below 450 mOsmol/kg) and a urea-to-creatinine ratio of below 40 : 1 (calculated as
plasma urea / plasma creatinine / 1000). This patient has a urea-to-creatinine ratio of 39.4. In contrast,
patients with a prerenal acute kidney injury will have concentrated urine with low urinary sodium levels,
as the kidneys are trying to preserve volume.
79
Vancomycin is associated with tubulointerstitial nephritis and ATN and, therefore, should be stopped in
this patient.
Filgrastim
Filgrastim is used in the upregulation of neutrophil production in the bone marrow by binding to the
granulocyte-colony-stimulating-factor receptor. Common reactions include dyspnoea, haemoptysis and
hypersensitivity reactions. However, it is not known to cause ATN.
Cyclizine
Cyclizine is an antimuscarinic antiemetic and, therefore, common side effects include dry mouth and
drowsiness, followed by constipation and urinary retention. It is does not cause renal impairment and
would not explain this patient’s laboratory results.
Meropenem
Meropenem is a carbapenem-based antibiotic that can be used in the empirical treatment of

neutropenic sepsis. More common side effects include gastrointestinal upset, including abdominal pain,
nausea, vomiting and diarrhoea. However, more significant, albeit less common, side effects include
agranulocytosis, leukopenia and thrombocytopenia. However, it is not associated with renal
impairment.
Ondansetron
Ondansetron is a 5-HT3-receptor antagonist used in the treatment of nausea and vomiting. It can cause
constipation, headache and hot flushes, and in more severe cases, arrhythmias and QTc prolongation,
oculogyric crisis and seizures. However, it is not linked to renal impairment.
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A.54.A 35-year-old woman is referred to the Nephrology Outpatient Clinic following an incidental finding
of deranged kidney function on routine blood tests with her General Practitioner. She denies increased
frequency or urgency but does report needing to get up at least twice each evening to pass urine. There
is no history of fever, arthralgia or abdominal pain. There is no past medical history of note.
On examination, she appears well. Her observations are recorded within the normal range. On
auscultation, her chest is clear and heart sounds are normal. Her abdomen is soft and non-tender, with
no palpable organomegaly. There are tender palpable lesions shown below.
Neutrophils 5.2 × 109/l 2.5–7.5 × 109/l
Lymphocytes 3.4 × 109/l 0.8–5.0 × 109/l
80
Erythrocyte sedimentation rate (ESR) 40 mm/hour 0–29 mm/hour
Platelets (PLT) 399 × 109/l 150–400 × 109/l
Calcium (Ca2+) 2.9 mmol/l 2.2–2.6 mmol/l
Urinalysis: ++ protein.
Renal biopsy: granulomatous tubulointerstitial nephritis (TIN).
What is the most appropriate step in the management of this patient?
Corticosteroids
Cyclophosphamide
Intravenous 0.9% sodium chloride
Plasma exchange
Renal transplant
Explanation
Corticosteroids
This patient has sarcoidosis, a multisystem granulomatous disorder. Most commonly, women between
the age of 30 and 40 years are diagnosed. Typically, the respiratory system is involved, causing a dry
cough or shortness of breath. However, in up to 20% of patients, sarcoidosis presents as chronic TIN.
Chronic TIN may not necessarily present as drug-induced acute TIN with fever, arthralgia and
eosinophilia. Instead, polyuria or nocturia may be seen or symptoms of hypercalcaemia and
hypercalciuria such as ureteric calculi. Non-caseating granulomas are seen on renal biopsy. This patient
has evidence of impaired renal function, along with hypercalcaemia, raised ESR and normocytic anaemia
on laboratory tests. The raised erythematous lesions on this patient’s shins likely represent erythema
nodosum, of which sarcoidosis is the most common cause. Renal sarcoidosis responds very well to
corticosteroids and these should be tried initially.
Image source: https://commons.wikimedia.org/wiki/File:Erythema_nodosum_new_image.jpg
81
Cyclophosphamide
Cyclophosphamide is an immunosuppressant used in the treatment of a variety of renal disorders such

as nephrotic syndrome and vasculitis such as granulomatosis with polyangiitis (GPA). GPA is a small
vessel vasculitis causing lesions in the upper respiratory tract, lungs and kidneys. It can present as
rhinorrhoea and nasal ulceration in milder disease or with an acute kidney injury or haemoptysis, with
lung infiltrates seen on a chest X-ray, in more severe disease. In contrast to granulomatous TIN, a renal
biopsy in GPA demonstrates microvascular glomerulonephritis. Cyclophosphamide is not routinely used
in the first stages of sarcoidosis.
Intravenous 0.9% sodium chloride
Intravenous fluids are often given in the management of an acute kidney injury, especially if a prerenal
cause is suspected. However, it is likely that this patient’s kidney impairment is not from dehydration
alone and is secondary to an alternative disease process. This is further supported by the absence of
ketones in her urine and normal observations.
Plasma exchange
Plasma exchange is a management option for severe cases of vasculitis, including GPA or Goodpasture
syndrome (or anti-glomerular basement membrane disease). Goodpasture syndrome is a rare
autoimmune disease characterised by general malaise, weight loss and fever, along with haemoptysis
(and pulmonary haemorrhage in severe disease) and glomerulonephritis. Plasma exchange is not used in
the treatment of sarcoidosis.
Renal transplant
A renal transplant would be a much later consideration in the management of renal sarcoidosis when
other treatments have failed to improve renal function. As this patient has not yet started treatment,
corticosteroids should be tried as first line.
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A.55.A 64-year-old man with a history of type II diabetes comes to the clinic for review. He has been
unable to tolerate an angiotensin-converting enzyme (ACE) inhibitor because of hyperkalaemia and is
now taking a combination of anti-hypertensives, including diltiazem and indapamide, to control his
blood pressure. Other medication includes twice-daily mixed insulin and liraglutide.
On examination, his blood pressure is 138/82 mmHg and pulse is 75 bpm and regular. There are no
murmurs, and his chest is clear. His abdomen is soft and non-tender, and his body mass index (BMI) is 32
kg/m2.
Investigations:
Platelets (PLT) 179 × 109/l 150–400 × 109/l
82
Sodium (Na+) 132 mmol/l 135–145 mmol/
Bicarbonate 19 mmol/l 22–30 mmol/l
Chloride (Cl-) 118 mmol/l 95–106 mmol/l
Which of the following is the most likely cause of his hyperkalaemia related to ACE inhibition?
Addison’s disease Renal artery stenosis Renal tubular acidosis type 1 Renal tubular acidosis type 2
Renal tubular acidosis type 4
Explanation
Renal tubular acidosis type 4, which leads to insensitivity to aldosterone, is seen in diabetes mellitus,
systemic lupus erythematosus and tubulointerstitial disease. It presents with hyperkalaemic,
hyperchloraemic metabolic acidosis, and it is likely that, in this case, the use of an ACE inhibitor has
driven hyperkalaemia via this mechanism. Treatment is withdrawal of the precipitating medication.
Fludrocortisone can be considered in patients with persistent significant hyperkalaemia with metabolic
acidosis despite discontinuing the ACE inhibitor. Other medications known to precipitate renal tubular
acidosis type 4 include beta-blockers and non-steroidal anti-inflammatory agents.
Addison’s disease
The normal blood pressure here counts against adrenal insufficiency as the underlying diagnosis, and
the precipitant hyperkalaemia related to ACE inhibitor use without a rise in creatinine levels is more
suggestive of deficient aldosterone action and renal tubular acidosis type 4.
Renal artery stenosis
Renal dysfunction related to renal artery stenosis which is precipitated by ACE inhibitor use is more
likely to lead to a rise in creatinine levels, as well as a rise in serum potassium levels.
Renal tubular acidosis type 1 is associated with hypokalaemia and metabolic acidosis, rather than the
increased potassium level seen here. Its development is associated with autoimmune disease,
nephrocalcinosis and medullary sponge kidney.
Renal tubular acidosis type 2 is associated with proximal tubular dysfunction. Causes include Fanconi
syndrome and light chain nephropathy due to multiple myeloma.
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83
A.56.A patient with chronic kidney disease related to diabetes mellitus comes to the clinic. She is
managed with a number of anti-hypertensive medications and BD insulin to control her blood sugar. She
also takes atorvastatin and aspirin.
On examination, her BP is 142/82 mmHg, pulse is 85 bpm and regular. Her chest and abdominal
examination are unremarkable.
Platelets (PLT) 201 × 109/l 150–400 × 109/l
Creatinine (Cr) 230 μmol/l 50–120 μmol/l
Parathyroid hormone (PTH) 16.1 pmol/l 0.9–5.4 pmol/l
Ergocalciferol
Alphacalcidol
Calcitonin
Sevelamer
Cinacalcet
Explanation
Alphacalcidol
Hyperparathyroidism in renal disease can be managed with the use of alphacalcidol, because the drive
for increased PTH comes at least in part from low levels of 1, 25-OH vitamin D. If vitamin D replacement
fails to achieve a reduction in PTH levels, then surgery may be considered.
Sevelamer
Sevelamer is a phosphate-binding agent. Therefore, it will act to lower the phosphate levels but the
phosphate is normal in this scenario. The disadvantages of sevelamer compared with other phosphate
84
binders include its cost, its side effects of GI intolerance, the large doses that are usually needed and the
potential to cause mild metabolic acidosis.
Ergocalciferol
Ergocalciferol is vitamin D2 and is used to prevent and treat vitamin D deficiency. However, it is the
inactive form of vitamin D and therefore alphacalcidol should be used instead in this case.
Calcitonin
Calcitonin is used for treatment of hypercalcaemia or osteoporosis. Calcitonin acts on the osteoclasts,
resulting in inhibition of bone resorption and attenuation of subchondral bone turnover. It also acts
directly on the chronocytes, attenuating cartilage degradation and stimulating cartilage formation.
Cinacalcet
Cinacalcet is a PTH antagonist, but is only recommended by NICE for the management of patients with
hyperparathyroidism who are on dialysis and not fit for surgery
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A.57.A 63-year-old woman attends the Emergency Department with reduced mobility. She says that her
muscles and joints have become increasingly more painful over the last two months and the skin
surrounding her joints has become thickened and tight. She reports that the mobility in her joints is
significantly reduced to the point that she needs to use a wheelchair to go to the shops. Any minor daily
activity makes her feel extremely fatigued and she is sleeping for most of the day. Her past medical
history includes chronic kidney disease (CKD) stage 5, for which she receives peritoneal dialysis and
diagnosed tuberculous meningitis for which she underwent MRI brain with contrast 2 months ago.
On examination, her chest sounds are clear, with normal heart sounds. She has a soft and non-tender
abdomen. Her skin is diffusely thickened and appears tightened and shiny around her knees and ankles.
Her knees have a significantly reduced range of movement and are partially locked in the flexed
position.
AA amyloidosis
AL amyloidosis
Beta-2 microglobulin amyloidosis
Scleroderma
Explanation
85
Nephrogenic systemic fibrosis is the rapid process of the skin and soft tissue thickening and fibrosis. In
more advanced disease, the skin can be appear hardened and indurated and, if present around a joint,
can lead to painful contractures and reduced mobility, with some patients becoming wheelchair-bound.
Skin discolouration, such as reddening, may be seen. The disease process arises secondary to gadolinium
contrast exposure in patients with impaired renal function, usually those with end-stage renal disease or
those already on dialysis. This patient had a contrast enhanced MRI two months ago, which is likely the
triggering event. The mainstay of treatment is to maintain an adequate renal function to prevent further
progression of the disease.
AA amyloidosis
AA amyloidosis is an inflammatory process seen in patients with an existing autoimmune or

inflammatory disease such as rheumatoid arthritis or systemic lupus erythematosus (SLE). Serum
amyloid A protein is an acute inflammatory protein that is raised in inflammatory disease. It is most
commonly deposited in the liver, spleen and kidney, with a common presentation of AA amyloidosis
being nephrotic syndrome and progression of CKD. It does not cause the dermatological symptoms
described.
AL amyloidosis
AL amyloidosis is another subtype of amyloidosis that is seen in patients with an underlying malignancy
such as multiple myeloma. Abnormal antibody products known as ‘light chains’ can form amyloid
deposits in various organs, most commonly the kidney causing renal failure. There is nothing in this
patient’s history to suggest an underlying haematological malignancy, making AL amyloidosis an
incorrect answer.
Beta-2 microglobulin amyloidosis
Beta-2 microglobulin amyloidosis is a type of amyloidosis seen in patients with CKD on long-term
haemodialysis. Over time, there is a gradual accumulation of beta-2 microglobulin, as it is unable to
cross the dialysis filter. It tends to present after five years of dialysis use and typically affects joints.
However, the disease process described in this question is much more acute and progressive. Beta-2
microglobulin does not account for the skin changes seen here and given the recent magnetic resonance
imaging, an alternative diagnosis should be considered.
Scleroderma
Scleroderma is an important differential to consider. Scleroderma is a chronic autoimmune disease that

causes widespread fibrosis and vascular abnormalities affecting multiple organs. Typically, the skin
becomes hardened and sclerotic, similarly to that seen here. The disease has two subtypes: diffuse and
limited cutaneous (also known as CREST syndrome) that is associated with calcium deposition,
Raynaud’s disease, oesophageal dysmotility, skin tightening and telangiectasia. Although the
musculoskeletal findings in scleroderma have similarities to this patient’s presentation, the absence of
cardiopulmonary, renal or gastrointestinal symptoms and the onset of symptoms shortly after a contrast
MRI procedure suggest nephrogenic systemic fibrosis to be the more likely diagnosis.
************************************************************************************
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A.58.A 27-year-old normotensive type I diabetic presents to the Diabetes Clinic. He has pre-proliferative
retinopathy but no other known diabetic complications. He has good glycaemic control. A spot urine test
in the clinic reveals protein +. This has not been noted before.
What is the most appropriate initial management strategy?
Commence a low-protein diet
Confirm microalbuminuria by sending an early-morning urine sample for albumin-to-creatinine ratio
Start an angiotensin-converting enzyme inhibitor
Start an angiotensin II inhibitor
Start a calcium channel antagonist
Explanation
Confirm microalbuminuria by sending an early-morning urine sample for albumin-to-creatinine ratio
Microalbuminuria is a reliable predictive marker of progression to nephropathy in type I diabetes

mellitus. Early urinary albumin excretion rates of 30–300 mg/24 hours are too small to be detected on
conventional urine dipsticks, and either special dipsticks or radioimmunoassay are used.
The next stages are macroalbuminuria (over 300 mg/24 hours), followed by persistent proteinuria, by
which time chronic kidney disease is usually 5–10 years away.
The optimal treatment for diabetic albuminuria is an angiotensin-converting enzyme inhibitor (ACE)
inhibitor (or an angiotensin II inhibitor), in combination with meticulous glycaemic and BP control. When
the initial detection of proteinuria is between 3 and 70 mg/mmol (microalbuminuria), then this should
be confirmed with early-morning samples of urine sent for albumin to creatinine ratio.
Start an angiotensin II inhibitor
The optimal treatment for diabetic albuminuria is an angiotensin II inhibitor in combination with
meticulous glycaemic and BP control. Following confirmation and quantification of microalbuminuria,
The NICE guidelines recommend either an ACE inhibitor or an angiotensin II inhibitor. Some studies have
suggested the role of combination therapy, although the NICE guidelines recommend single-agent
therapy in the first instance.
Commence a low-protein diet
There is no evidence that a low-protein diet can cause a long-term reduction in proteinuria. In fact, a
low-protein diet may be harmful due to the risk of malnutrition.
Start an angiotensin-converting enzyme inhibitor
The optimal treatment for diabetic albuminuria is an ACE inhibitor in combination with meticulous
glycaemic and BP control. The National Institute of Healthcare Excellence (NICE) guidelines recommend
87
that early-morning urine samples for albumin-to-creatinine ratio are required to detect proteinuria as
urinalysis is not able to detect low levels of microalbuminuria or quantify it.
Start a calcium channel antagonist
There is some evidence that calcium channel antagonists may exacerbate proteinuria. Therefore, they
would not be the agent of choice
*****************************************************************************
A.59.A 17-year-old patient attends a Follow-up Clinic appointment. She has a family history of
autosomal dominant polycystic kidney disease. Her older brother was recently diagnosed with the
condition and she was referred by her general practitioner for screening. She has remained
asymptomatic and was seen in clinic previously by a colleague.
On physical examination, she has a blood pressure of 135/80 mmHg, with chest and abdominal
examination unremarkable.
Investigations:
Renal ultrasound No renal cysts detected
What is the next best management step in this case?
Cerebral magnetic resonance angiogram
Computerised tomography of the abdomen
Discharge from clinic
Repeat renal ultrasound after 1 year
Repeat renal ultrasound after 3 years
Explanation
Repeat renal ultrasound after 3 years
Autosomal polycystic kidney disease (ADPKD) genes are located on chromosomes 16 (majority) and 4
(minority). The best screening tool is ultrasound. In patients below the age of 20 years you can get false-
negative test results, and it is recommended to screen patients after the age of 20 years.
Cerebral magnetic resonance angiogram
88
There is an association with intra-cranial aneurysms and ADPKD, but screening for these is not indicated
in this patient. Routine screening for intracranial aneurysms is recommended only for high-risk patients,
such as those with a previous rupture, a positive family history of an intra-cerebral bleed, warning
symptoms, and prior surgery that is likely to be associated with hypertension.
Computerised tomography of the abdomen
A computerised tomography scan, although more sensitive than ultrasound, involves a high radiation
dose to this young girl.
Discharge from clinic
This girl is may have ADPKD, which would have future implications. Therefore, it is essential to follow up
and not simply discharge from the clinic.
Repeat renal ultrasound after 1 year
1 year would be too early - this girl would not be 20 years old at this point. As explained above, there
would be false-negative test results when screening too early.
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A.60.A 25–year-old mechanic presents to his general practitioner (GP) with a 3-week history of
worsening leg swelling. The rest of the history is unremarkable. He is on no medication.
Examination confirms pitting oedema to the thighs. Blood pressure is 125/63 mmHg with no postural
drop.
Blood results are shown below (the sample was noted to be lipaemic):
Plasma osmolality 280 mOsmol/kg
24 hour urinary protein 6.2 g
What investigation would help explain this patient’s hyponatraemia?
Plasma antidiuretic hormone
Plasma lipids
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Spot urinary sodium
Short synacthen test
Urine osmolality
Explanation
Plasma lipids
This patient has pseudohyponatraemia due to hyperlipidaemia secondary to the nephrotic syndrome.
The major clue to this is the normal measured plasma osmolality. The mechanism of
pseudohyponatraemia can be explained by the electrolyte exclusion effect. This is the exclusion of
electrolytes from the fraction of total plasma volume that is occupied by solids. The volume of total
solids (primarily protein and lipid) in an aliquot of plasma is normally approximately 7%. This will be
increased in conditions that cause a rise in protein or lipid. A spuriously low sodium result occurs
because the measured sodium is confined to the aqueous phase, but has its concentration expressed in
terms of the total volume of plasma. It is important to diagnose pseudohyponatraemia, because as the
plasma osmolality is normal no treatment is required.
Urine osmolality
Urine osmolality would be high in cases of pre-renal kidney disease as the urine is more concentrated. It
would not help in this case.
Plasma antidiuretic hormone
The antidiuretic hormone level would be normal which would not discern the cause of hyponatraemia.
Spot urinary sodium
Spot urinary sodium may be useful to differentiate the cause of acute kidney disease. In cases of acute
kidney disease with a low urinary sodium would suggest a pre-renal cause.
Short synacthen test
In this case the Short synacthen test would be normal. It would be abnormal in Addison's disease.
*******************************************************************************
A.61.A 19-year-old woman of African descent is referred to your Nephrology Clinic by her General
Practitioner (GP) with a history of dipstick-positive proteinuria and lower limb oedema. She has no past
medical history of note and is on no regular medication. Other than a moderate ache in her elbows and
knees, for which she takes paracetamol intermittently, she has no ongoing concerns.
On examination, her blood pressure is 118/60 mmHg.
Investigations:
90
Beta-human chorionic
gonadotrophin
(BHCG) Negative
A urine dipstick is +++ for protein, and + for blood. Her urine protein : creatinine (PCR) ratio is measured
as 200 mg/mmol (normal range: < 5 mg/mmol).
What is the most appropriate test to identify this patient’s underlying diagnosis?
Anti-glomerular basement membrane (GBM)
Antinuclear antibody (ANA)
Antineutrophil cytoplasmic autoantibody (ANCA)
Protein electrophoresis
Ultrasound (US) renal tract
Explanation
Antinuclear antibody (ANA)
In a young woman of African descent, with vague joint aches and a history of nephrotic syndrome, the
likely diagnosis is systemic lupus erythematosus (SLE). A strongly positive antinuclear antibody (ANA)
level is most likely to point to a diagnosis of SLE, which fits with the clinical picture above. Subsequent
investigations which will be required include anti-double-stranded (ds) deoxyribonucleic acid (DNA) and
a kidney biopsy. A biopsy of the renal cortex will clinch the diagnosis of lupus nephritis, classify it and
provide prognostic information. It is generally recognised that lupus nephritis comes in six different
classes (I–VI). These are identified on biopsy and carry different prognostic weights and require different
treatments.
Class Findings Treatment
Class I Normal microscopy, but immune deposits Monitor
Class II Mesangial proliferation Monitor
Class III Focal disease Steroids + mycophenolate mofetil (MMF) ± cyclophosphamide
Class IV Diffuse disease Steroids + MMF ± cyclophosphamide
91
Class V Membranous Angiotensin-converting enzyme inhibitor (ACEi) ± tacrolimus
Class VI Advanced sclerosis ACEi
Ultrasound (US) renal tract
The value of an ultrasound (US) is threefold. It would identify obstruction, assess the cortical reflectivity
of the kidneys (which implies chronic kidney disease) and allow quantification of the size of the kidneys
(which, if small, may point to either chronic kidney disease or chronic vascular insufficiency). It is unlikely
that this young woman has obstructive pathology, and as such, an US here has limited diagnostic
potential.
Anti-glomerular basement membrane (GBM)
Anti-glomerular basement membrane (GBM; Goodpasture’s) disease is a crescent-forming

pulmonary/renal syndrome that usually presents with a dramatic inflammatory disorder of the kidneys
and/or lungs. It would be unusual for anti-GBM to present with vague joint aches and pains and heavy
proteinuria, which is present in this case.
Antineutrophil cytoplasmic autoantibody (ANCA)
Other than vague joint aches, this woman has no history to support an underlying cause of vasculitis.
She has no history of rash or constitutional symptoms. Furthermore, there are no airway or upper
airway symptoms which may point in the direction of a vasculitic process, for which testing her
antineutrophil cytoplasmic autoantibody (ANCA) level would provide diagnostic evidence.
Protein electrophoresis
Multiple myeloma would be a more likely diagnosis in an older patient with a history of bony aches and
pains, hypercalcaemia and renal failure. Protein electrophoresis would then be useful in identifying and
quantifying the presence of a paraprotein. However, considering this patient’s young age, this is an
unlikely diagnosis.
*************************************************************************************
62.A 27-year old African gentleman is admitted with symptoms of generalised muscle weakness. He has
a family history of sickle cell anaemia and has needed admission to hospital in the past with renal colic.
On admission, his blood pressure is 120/70 mmHg.
Venous blood results reveal the following :
Bicarbonate (HCO3-) 13 mmol/l 24–30 mmol/l
Chloride (Cl-) 117 mmol/l 98-106 mmol/l
Arterial blood gas reveals the following:
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pH 7.26 7.35–7.45
p(CO2) 3.0 kPa 4.6–6.0 kPa
The most likely diagnosis and cause for the above blood results is:
Distal renal tubular acidosis
Proximal renal tubular acidosis
Bartter’s syndrome
Diabetic ketoacidosis
***********************************************************************************
A.63.A 29-year-old woman with renal disease secondary to systemic lupus erythematosis is seen in the
Rheumatology Clinic. She has had three urinary tract infections in the past year and was recently
admitted to the Emergency Department with acute severe unilateral abdominal pain which settled
spontaneously after several hours.
On physical examination, her blood pressure is 130/80 mmHg and chest and abdominal examination is
unremarkable.
On her last outpatient visit a number of investigations were requested, the results of which are now
available:
Urea 9.0 mmol/ 2.5 - 6.5 mmol/l
Urine pH 7.0 4.6 - 8.0
What is the most likely underlying cause of these results and possibly for some of her recent
presentations?
Bartter syndrome
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Staghorn calculus leading to recurrent urinary sepsis
Explanation
This lady has a compensated metabolic acidosis with relatively high urinary pH and low potassium.
These are features of type 1 (distal) renal tubular acidosis (RTA), and are probably secondary to her
lupus-associated renal impairment. The suggestion of urinary calculi and recurrent urinary infections
support this as these are recognised complications of the inability to acidify the urine. A staghorn
calculus with recurrent sepsis would not in itself explain her biochemical picture, although
nephrocalcinosis can also be associated with RTA. Nephrocalcinosis and recurrent urinary sepsis are also
associated with type 1 (distal) RTA, and in the context of her rheumatological disease it is more likely
that this is the underlying cause. It would also be highly unusual to find this condition coexisting with
lupus renal disease.
Proximal renal tubular acidosis is due to impaired retention of bicarbonate in the proximal tubule
leading to bicarbonate wasting and a systemic acidosis. It presents as a hyperchloraemic metabolic
acidosis, usually with other features of proximal tubular dysfunction. Common causes include myeloma,
amyloidosis, cystinosis and Wilson’s disease.
Bartter syndrome
Barrter syndrome is an inherited or sometimes sporadic disorder leading to hypokalaemia; however, this
would not cause the urinary abnormalities seen here and, if inherited, would have presented earlier.
This is less common than type 1 and 2 renal tubular acidosis. It is due to aldosterone resistance.
Staghorn calculus leading to recurrent urinary sepsis
Staghorn calculus is caused by infection with Proteus, Klebsiella or Serratia. Typically, these stones are
seen on KUB. It is not associated with SLE.
***********************************************************************************
A.64.A 45-year-old man is referred to the Nephrology Clinic. He has type II diabetes mellitus, for which
he currently takes metformin 500 mg twice a day. He also takes atorvastatin 10 mg at night and ramipril
5 mg once a day. He is on no other regular medication.
Clinical examination reveals a middle-aged man, with a body mass index (BMI) of 30 kg/m2. His blood
pressure (BP) is 150/90 mmHg.
Investigations:
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Estimated glomerular filtration rate (eGFR) 41 ml/min/1.73 m2 > 90 ml/min/1.73 m2
Urine dipstick protein ++
Glycated haemoglobin (HbA1c) 7.1 % < 6.0%
What is the most appropriate alteration to the patient’s current management plan?
Add amlodipine 5 mg once a day
Increase atorvastatin to 40 mg at night
Increase metformin to 1 g twice a day
Increase ramipril to 10 mg
Replace metformin with gliclazide 40 mg at night
Explanation
Proteinuria is clearly associated with a deterioration in renal function among diabetic patients and
indeed is an important determinant/negative prognostic indicator of disease progression among all
forms of proteinuric chronic kidney disease (CKD) – the diagnosis here. Angiotensin-converting enzyme
(ACE) inhibitors, such as ramipril, function by inhibiting arteriolar vasoconstriction, reducing glomerular
pressure and reducing proteinuria. They also improve BP parameters and are, therefore, ideal
interventions for proteinuric CKD diabetic patients.
Add amlodipine 5 mg once a day
While amlodipine will improve the BP parameters in this patient, it will not significantly affect
proteinuria and will, therefore, have a less significant impact on the progression of renal disease, in
comparison to ACE inhibitors such as lisinopril.
Increase atorvastatin to 40 mg at night
The role of statins in CKD is contentious. There is evidence that in end-stage renal failure, statins have
no positive impact on cardiovascular health. In this patient, the main ongoing problems are that he has
diabetes, with evidence of diabetic nephropathy, characterised by proteinuria and chronic renal
95
impairment. Initiation of statins will not arrest the proteinuric process or slow down the rate of decline
in renal function.
Increase metformin to 1 g twice a day
This patient has CKD secondary to diabetic nephropathy. He is proteinuric and hypertensive. The
mainstay of preventing deterioration in renal function is threefold – firstly, arresting the process of
proteinuria; secondly, improving BP parameters; and thirdly, ensuring good diabetes control. In this
patient, given his eGFR is < 45 ml/min/1.73 m2, the risk of lactic acidosis is increased with metformin.
Ideally, metformin should only be prescribed with caution and ceased if the eGFR falls below 30.
Regardless, the most important step would be treatment to reduce proteinuria, which will not be
affected by metformin.
Replace metformin with gliclazide 40 mg at night
Improving glycaemic control is an important step in affecting the trajectory of disease and slowing
progression. However, there are some downsides to this option that make it the incorrect answer.
Firstly, gliclazide is associated with an increased risk of hypoglycaemia in CKD. Secondly, this change will
not reduce proteinuria, a primary determinant of disease progression; and finally, this change from high-
dose metformin to starting-dose gliclazide is unlikely to result in a significant change in glycaemic
parameters.
***********************************************************************************
A.65.A 61-year-old man who came to your clinic complains of weakness and loss of weight. His voice is
hoarse. He has no past medical history of note.
On examination, he has pedal oedema up to the tibial tuberosity, enlarged tongue and liver edge
palpable up to three finger breadths below the right costal margin.
Investigations
Investigation Results Normal Values
Urine dipstick 3+++ protein
What is the most appropriate test that will lead to the correct diagnosis?
Liver spleen scan
96
24 h urinary protein
Subcutaneous fat biopsy
Urine protein electrophoresis
Urine protein immunophoresis
Explanation
Subcutaneous fat biopsy
The clinical features of the patient are suggestive of amyloidosis. It is characterised by extracellular
deposition of fibrous protein in various tissues and organs. It may be primary or associated with other
chronic diseases like myeloma or rheumatoid arthritis. Diagnosis is made by typical findings and
demonstration of amyloid fibrils by Congo red staining under polarised light. Abdominal subcutaneous
fat pad aspirate or rectal submucosal biopsy are often performed to reach a final diagnosis.
Liver spleen scan
In amyloid there may be an enlarged spleen and liver. This is not specific for amyloidosis.
Lymphoproliferative diseases may also cause hepatosplenomegaly. Therefore, this would not be the
investigation of choice for diagnosis.
24 h urinary protein
Heavy proteinuria is a feature of amyloidosis. However, proteinuria may be found in other glomerular
diseases such as minimal-change glomerulopathy and membranous glomerulopathy. Therefore,
quantification of protein would not give the definitive diagnosis.
Urine protein electrophoresis
Urine protein electrophoresis would be abnormal in myeloma.
Urine protein immunophoresis
Similarly, in myeloma the urine immunophoresis would be abnormal.
**********************************************************************************
A.66.A 32-year-old woman presents with dizziness. Symptoms have been coming on for the last few
months, and she now needs help withstanding. She is treated with bendroflumethiazide for recently
diagnosed hypertension, smokes 10 cigarettes daily and drinks around 30 units per week of alcohol. She
is one of four children, a brother having died in the past of renal cancer.
On direct questioning, she admits to cough productive of phlegm, present most mornings for the last
few years, but has normal exercise tolerance.
On examination, she is noticeably plethoric. There is an audible expiratory wheeze throughout the chest.
Oxygen saturations are normal. There is nystagmus present looking left or right. Fundoscopy reveals
dilated retinal veins. She has a broad-based gait, and is unsteady with eyes open or closed.
97
Platelets (PLT) 169 × 109/l 150–400 × 109/l
Haematocrit 0.61 0.35–0.55
Red cell mass 2312 ml 1312–2187 ml
Plasma volume 2392 ml 2362–3938 ml
Erythroid culture in the absence of erythropoietin No endogenous erythroid colony formation
Which of the following is the most likely diagnosis?
Primary polycythaemia rubra vera
Von Hippel–Lindau syndrome
Renal cell carcinoma with endogenous erythropoietin production
Gaisbock syndrome
Alcohol abuse
Explanation
Von Hippel–Lindau syndrome
This patient has a high haematocrit; with an increased red cell mass and a low–normal plasma volume,
confirming true polycythaemia. Von Hippel–Lindau (VHL) syndrome is an autosomal dominant condition
that leads to retinal and central nervous system tumours, as well as phaeochromocytomas. Signs of VHL
syndrome include balance disturbance, hypertension, headaches and dizziness. Death is often due to
renal cell carcinoma.
Criteria are the following:
More than one hemangioblastoma in the CNS (brain, spinal cord) or eye.
A single hemangioblastoma in the CNS or retina, plus a visceral manifestation (multiple renal, pancreatic
or hepatic cysts; pheochromocytoma; renal cancer).
Positive family history plus any one of the above clinical manifestations.
Elucidation of a deleterious mutation in the VHLgene.
Management depends on the site of individual tumours, but close ophthalmic, renal and radiographical
follow-up is recommended for all patients. There is no ultimate cure for VHL. A proportion of cerebellar
haemangiomas secrete erythropoietin-like substances, leading to a secondary polycythaemia.
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Erythroid colony studies suggest that her red cells do not show autonomous growth; autonomous
growth of erythroid colonies is taken as a sign of primary polycythaemia, where erythropoiesis has
escaped the control of erythropoietin; erythroid colony studies are thought to have high specificity for
detecting primary versus secondary polycythaemia, but the technique is poorly standardized so remains
an experimental tool.
Renal cell carcinoma with endogenous erythropoietin production
Rare cases of renal cell carcinoma proliferate with endogenous erythropoietin production. There was no
endogenous erythroid colony found on testing.
Primary polycythaemia rubra vera
This is caused by bone marrow overproduction of red blood cells (and sometimes white cells and
platelets). Patients maybe asymptomatic, presenting with blood clots, itching and skin changes.
Gaisbock syndrome
This is a form of pseudo-polycythaemia, usually found in obese men with hypertension causing a
reduction in plasma volume.
Alcohol abuse
It is not uncommon to see borderline plasma volumes in patients with excessive alcohol intake (or on
diuretics).
**********************************************************************************
A.67.A 72-year-old woman was reviewed in Outpatients having recently been treated with furosemide
and amiloride combination tablets for heart failure. You understand that the GP increased the number
of combination diuretic tablets six weeks ago, rather than just increasing the furosemide component.
She also has a history of angina, for which she takes a slow-release nitrate preparation.
On examination, her blood pressure is 130/80 mmHg, chest examination was unremarkable and heart
sounds were normal.
Her amiloride has been stopped that day.
An electrocardiogram (ECG) is shown below.
What immediate further urgent action needs to be taken with respect to the potassium?
99
Intravenous calcium gluconate 10%
Intravenous insulin 10 U and dextrose 5 %
None
Nebulised salbutamol 5 mg
Oral calcium resonium
Explanation
None
This woman has renal impairment and slightly elevated potassium. In the absence of ECG changes
suggestive of hyperkalaemia (peaked T waves, widened QRS), no further action is required immediately.
The poor R wave progression and Q wave in V2 on the ECG is suggestive of a previous anterior
myocardial infarction. Where peaked T waves and widened QRS exist in conjunction with
hyperkalaemia, calcium gluconate and intravenous insulin and dextrose would be initial therapies of
choice. In the mid to long term this patient's medications, especially her diuretics, should be reviewed
with the aim of reducing the potassium level without compromising the renal function.
Image source: https://commons.wikimedia.org/wiki/File:E000716_(CardioNetworks_ECGpedia).jpg
Intravenous calcium gluconate 10%
Calcium prevents cardiac arrythmias that occur as a result of hyperkalaemia. Caution must be exercised
when administering intravenously because extravasation may lead to localised tissue necrosis.
Intravenous insulin 10 U and dextrose 5 %
Insulin moves the potassium from extracellular to intracellular compartment thereby reducing serum
potassium levels.
Nebulised salbutamol 5 mg
Salbutamol, similar to insulin, moves potassium from extracellular to intracellular compartment. A side
effect of nebulised salbutamol is tachycardia therefore care must be taken in patients who are already
tachycardic.
Oral calcium resonium
Calcium resonium acts by preventing absorption of potassium by the gut. As a consequence, it is slower
acting. A common side effect is constipation.
***********************************************************************************
A.68.A 60-year-old woman presents with refractory hypertension first picked up on a routine health
check. She is a long-term smoker. She has mild asthma and eczema but no other significant medical
history.
100
She has recently complained to her general practitioner of pains in her calves which have occurred while
she is walking her dog. Her blood pressure remains 150/95 mmHg in spite of multiple medical therapy
trials.
Her blood results are as follows:
She has had a test of her renin and aldosterone activity both of which were high.
The most likely diagnosis is:
Bilateral adrenal hyperplasia
Cushing’s disease
Primary hyperaldosteronism (Conn’s syndrome)
Renovascular hypertension secondary to fibromuscular dysplasia
Renovascular hypertension secondary to renal arterial sclerosis
Explanation
Renovascular hypertension secondary to renal arterial sclerosis
This woman has evidence of secondary hyperaldosteronism with high normal sodium and low potassium
levels in the presence of high renin and aldosterone activity. This is likely to be a result of renal artery
stenosis from atherosclerosis. She has a risk factor for arteriosclerosis and has symptoms that could
reflect peripheral vascular disease which is recognised to occur with atherosclerotic renal artery
sclerosis. Limb arteries can be affected by fibromuscular dysplasia, but this occurs only in about 5% of
cases.
Primary hyperaldosteronism (Conn’s syndrome)
In Conn’s syndrome the renin activity would be suppressed.
Bilateral adrenal hyperplasia
Adrenal hyperplasia may lead to increased secretion of aldosterone. This is primary hyperaldosteronism,
and therefore the renin would be suppressed.
Cushing’s disease
101
Potassium is normal in Cushing's disease because the underlying disease is due to an excess of cortisol
production.
Renovascular hypertension secondary to fibromuscular dysplasia
Her age makes fibromuscular dysplasia less likely as it usually presents between the ages of 30 and 40
years. In addition, arteriosclerosis is a more common cause of renal artery stenosis than fibromuscular
dysplasia.
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A.69.A 60-year-old man presents to the Nephrology Clinic for a routine follow-up appointment. He
reports changes to his skin over the last four weeks, with his skin becoming progressively thicker and
hardened. Initially, he noticed these changes over his forearms, but now the skin surrounding his fingers
has become tightened, with increased difficulty in moving his fingers. His past medical history includes
chronic kidney disease stage 5, for which he receives dialysis three days per week, and prostate
carcinoma that was treated following surgical resection and radiotherapy four years ago. He was
admitted to the hospital two months ago with a suspected transient ischaemic attack and was
discharged following a normal magnetic resonance angiogram (MRA). He denies smoking but consumes
10–12 units of alcohol per week.
On examination, the skin over his arms and legs is thickened, with scattered erythematous papules
bilaterally. His facial skin is spared. Multiple excoriations are noted over his body. His little and ring
fingers are in a fixed flexion deformity.
Alcohol excess
Beta-2 microglobulin deposition
Gadolinium exposure
Previous radiotherapy
Uraemia
Explanation
Gadolinium exposure
This patient has nephrogenic systemic fibrosis, a progressive disease characterised by rapid skin and soft
tissue thickening and fibrosis. In more advanced disease, the skin can appear hardened and indurated. If
present around a joint, it can lead to painful contractures and reduced mobility, with some patients
becoming wheelchair-bound. Skin discolouration such as reddening may be seen. The disease process
arises secondary to gadolinium contrast exposure in patients with impaired renal function, usually those
with end-stage renal disease or those already on dialysis. The mainstay of treatment is to maintain an
adequate renal function to prevent further progression of the disease.
102
Alcohol excess
This patient’s skin changes after a recent MRA scan suggests nephrogenic systemic fibrosis, a
progressive fibrotic disease of the skin and soft tissues. Alcohol can cause liver impairment which may
cause some skin changes such as Dupuytren’s contracture, which has a similar appearance to this
patient’s flexion deformity of his hands. Alcohol excess can also cause pruritus secondary to jaundice.
However, alcohol does not cause diffuse skin fibrosis.
Beta-2 microglobulin deposition
Beta-2 microglobulin amyloidosis is a type of amyloidosis seen in patients with chronic kidney disease on
long-term haemodialysis. Over time, there is a gradual accumulation of beta-2 microglobulin, as it is
unable to cross the dialysis filter. It tends to present after five years of dialysis use and typically affects
the joints. However, the disease process described in this patient is much more acute and progressive.
Beta-2 microglobulin does not account for the skin changes seen here and given the recent MRA, an
alternative diagnosis should be considered.
Radiotherapy is a risk factor for multiple illnesses, including secondary malignancy and retroperitoneal
fibrosis. Local effects of radiotherapy can be seen, including local skin irritation and soft tissue
inflammation. However, radiotherapy would not cause diffuse skin fibrosis and joint contractures.
Uraemia
Uraemia causes a number of symptoms, including nausea, vomiting, polyneuritis, headache, fatigue,
seizures and coma. Symptoms of skin disease secondary to uraemia include itching and calciphylaxis.
However, the pattern of symptoms described in this patient, such as joint contractures and progressive
fibrotic skin changes, points towards a diagnosis of nephrogenic systemic fibrosis, a condition related to
gadolinium exposure.
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A.70.A 40-year-old woman with end-stage kidney disease and undergoing regular haemodialysis is due
to undergo renal transplantation from her sister the following day. The primary cause of her kidney
disease is hypertension and she has no other past medical history.
On examination, her blood pressure is 145/70 mmHg. Her chest and abdominal examination is
unremarkable.
Investigations:
103
Platelets (PLT) 243 × 109 /l 150–400 × 109/l
Which of the following represents the best treatment plan?
Give calcium resonium
Consider dextrose and insulin infusion
Do nothing
Arrange an extra haemodialysis session
Give regular salbutamol nebulisers
Explanation
Arrange an extra haemodialysis session
Raised serum potassium in a patient with end-stage kidney disease is an indication for delaying routine
surgery. As this patient is not due to have her transplant until the following day however, there is an
opportunity to intervene with an extra haemodialysis session. Serum potassium above 5.5 mmol/l is the
level at which intervention should be considered. Calcium resonium, insulin and dextrose and
salbutamol nebulisers are all treatments for acute hyperkalaemia.
Give calcium resonium
Calcium resonium prevents the absorption of potassium from the gut. It is a slow treatment and would
not be used acutely in this situation.
Consider dextrose and insulin infusion
Insulin will push potassium from extracellular to intracellular compartment, but will not alter the total
body potassium content. It is a temporary treatment.
Do nothing
Hyperkalaemia pre-renal transplant will increase anaesthetic risk. The patient would be at risk of
arrythmias, therefore high potassium needs to be treated.
Give regular salbutamol nebulisers
Salbutamol forces potassium from extracellular to intracelluar compartment. It is used in the treatment
of acute hyperkalaemia.
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A.71.A 29-year-old man with a history of polycystic kidney disease presents to the Emergency
Department with 24 h of left loin pain. He is managed with ramipril and amlodipine for his blood
pressure (BP) and has suffered a progressive rise in his creatinine over the past few years.
On examination, his BP is 142/82 mmHg. The pain gradually resolves in the Emergency Department with
simple analgesia.
Investigations:
Hb 114 g/l
WCC 5.2 × 109/l
PLT 201 × 109/l
Na+ 141 mmol/l
K+ 4.9 mmol/l
Creatinine 156 μmol/l (153 3 months earlier in the clinic)
CRP 12 mg/l
Urine blood +, protein –
Admit for observation
Discharge with trimethoprim
Arrange an abdominal CT scan
Arrange an abdominal ultrasound scan
Arrange review in 3 days for urine culture results
Explanation
Arrange an abdominal ultrasound scan
The most likely diagnosis is haemorrhage into a renal cyst. Haematuria is usually self-limiting and, as the
pain has resolved, it seems likely that there is no further bleeding. Given that the renal function has
remained stable, he can be reassured. That being said, given the easy availability of ultrasound scanning
it would seem sensible to arrange a scan to rule out other causes of haematuria/proteinuria.
Arrange an abdominal CT scan
An abdominal CT will show haemorrhage into the renal cyst. However, the treatment would still be
conservative, ie analgesia for any pain and rest. Therefore, there is no indication at present to subject
the patient to high doses of radiation from a CT scan.
Admit for observation
105
Haematuria is usually self-limiting, and as the pain has resolved it seems likely that there is no further
bleeding. Therefore, there is no need to keep the patient in for observation. Any investigations can be
carried out as an outpatient.
Discharge with trimethoprim
As explained above, this man is unlikely to have a urinary tract infection as a cause of the pain because
he did not have any urinary symptoms such as dysuria and frequency of urination. You may expect some
white cells in the urine if there is a urinary tract infection. As a result, treatment with trimethoprim is
unlikely to be helpful.
Arrange review in 3 days for urine culture results
This patient did not have any urinary symptoms such as dysuria and frequency of urination. You may
expect some white cells in the urine if there is a urinary tract infection. Therefore, this man is more likely
to have haemorrhage into a renal cyst for which a review with urine cultures will not help.
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A.72.A 38-year-old man with a history of hypertension is referred to the endocrine clinic. The GP has not
yet commenced anti-hypertensive therapy, but is worried as three measured BPs over the past 2
months have been > 150 mmHg systolic. On examination he looks well and his BP is 157/92 mmHg. His
BMI is 29.
Platelets (PLT) 190 × 109/l 150–400 × 109/l
Fasting glucose 5.2 nmol/l < 7 mmol/l
Urinary free cortisol 320 nmol/24 hours < 280 nmol/24 hours
Renin
Supine: 3.0 pmol/ml/hour
Upright: 5.0 pmol/ml/hour after 30 minutes
1.1–2.7 pmol/ml/hour
3.0–4.3 pmol/ml/hour
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Aldosterone
Supine: 600 pmol/ml after 30 minutes
Upright: 1200 pmol/ml after 4 hours
135–400 pmol/ml
330–830 pmol/ml
Primary hyperaldosteronism
Cushing syndrome
Phaeochromocytoma
Coarctation of the aorta
Explanation
Both this patient’s renin and aldosterone levels are raised, which rules out primary hyperaldosteronism.
The fact that this is secondary hyperaldosteronism raises the possibility of intrinsic renal disease, which
given the options makes renal artery stenosis the most likely cause. The next diagnostic steps would be
to consider a renal ultrasound scan and magnetic resonance angiogram.
Coarctation of the aorta
Symptomatic coarctation of the aorta without other presenting features, such as shortness of breath on
exercise, differential blood pressure in both arms and radio-femoral delay, is an unlikely alternative
diagnosis.
This is effectively ruled out because of the elevation in renin levels seen here.
Cushing syndrome
The urinary free cortisol is only slightly above the upper limit of normal and is therefore very unlikely to
account for the hypertension seen here.
Phaeochromocytoma
The raised renin and aldosterone levels are a pointer towards renal artery stenosis, and the
hypertension seen with phaeochromocytoma is often episodic in nature.
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107
A.73.A 65-year-old man presents to the Emergency Department with severe chest pain. He is a heavy
smoker with poorly controlled type II diabetes and is a known vasculopath.
He is poorly compliant with his medication. He had a coronary artery bypass graft a year ago. A
computerised tomography (CT) pulmonary angiogram was performed on admission to rule out a
pulmonary embolism. Three days later, you are asked to review him, given the development of an acute
kidney injury.
His current medications include metformin 500 mg twice a day, atorvastatin 80 mg at night, aspirin 75
mg once a day, bisoprolol 5 mg once a day and ramipril 10 mg once a day.
Investigations:
Investigation On day of admission Three days later Normal value
White cell count (WCC) 7.0 × 109/l 9 × 109/l 4–11 × 109/l
Eosinophils 0.07 × 109/l 0.9 × 109/l 0.04–0.4 × 109/l
Contrast nephropathy
Ramipril-induced acute kidney injury
Rhabdomyolysis
Explanation
Contrast agents (particularly iodine-based) are important nephrotoxins. They belong to a long list of
drugs that can impair renal function by causing an acute interstitial inflammatory response. Importantly,
the peak incidence of contrast nephropathy is 48–72 hours post-contrast, as is seen here. The incidence
of contrast nephropathy is increased by the co-prescription of angiotensin-converting enzyme (ACE)
inhibitors (here, ramipril) and can be reduced by prehydration. The presence of a subtle eosinophilia can
indicate the presence of an acute interstitial inflammatory process.
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Cholesterol embolism is an important, common cause of acute kidney injury with eosinophilia, often
accompanied by a distal limb rash of livedo reticularis. It often occurs secondary to procedures and
drugs which result in cholesterol becoming dislodged. These include angiography with stenting,
procedures to AAAs, carotid endarterectomies and the initiation of warfarin.

progressive renal failure, which often coexists with other diseases, such as hypertension. It is unlikely to
cause acute kidney injury.
Ramipril-induced acute kidney injury
Initiation of ACE inhibitors results in inhibition of efferent arteriolar vasoconstriction. Consequently, an

increase of Cr by 20% is expected and within normal limits following the initiation of ramipril. It would
be highly unlikely for this to occur de novo in someone stably established on the drug.
Rhabdomyolysis
Rhabdomyolysis can occur secondary to underlying genetic disorders like McArdle’s disease, or
secondary to polymyositis or a direct muscle injury following a long lie. It results in a sharp uptrend in
creatine kinase level and is associated with myoglobinuria, myoglobin cast formation and subsequent
interstitial nephritis. It is treated with early, aggressive hydration. If this fails, patients may require an
interval of haemodialysis, but the injury usually resolves.
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A.74.A 72-year-old woman who has undergone haemodialysis for 8 years presents with numbness and
tingling in both hands, which wakes her from sleep in the early hours. In addition, she has had trouble
holding items between her fingers and thumb recently. She also complains of dysphagia.
On examination, her BP is 155/82 mmHg, pulse is 75 bpm and BMI is 23. There is wasting of the thenar
eminence on both hands and numbness over the palmar aspect of the thumb index and middle fingers.
Investigations:
Haemoglobin 124 g/l 115–155 g/l
Platelets 500 × 109/l 150–400 × 109/l
109
Hand X-ray Some blood vessel calcification
Which of the following measures is most appropriate in this patient?
Ciclosporin therapy
Corticosteroid therapy
Incision of the flexor retinaculae
Non-steroidal anti-inflammatory drugs
Switch to low-flux dialysis membranes
Explanation
Incision of the flexor retinaculae
This patient has dialysis amyloidosis due to excess beta-2 microglobulin. Most of the manifestations of
dialysis amyloidosis are musculoskeletal and include carpal tunnel syndrome, flexor tenosynovitis,
subchondral bone cysts and even pathological fractures. Treatment in this case involves surgical incision
of the flexor retinaculae, as this patient has demonstrable distal nerve loss. In addition, switching to
high-flux dialysis membranes consisting of polyacrylonitrile and polysulphone membranes reduces
amyloid deposition. Renal transplantation halts progression of the disease. It is common for patients
with kidney disease to have calcification of blood vessels due to high calcium.
Ciclosporin therapy
Ciclosporin therapy would not be used in amyloidosis. It may be useful as an immunosuppressant in

many autoimmune conditions, and for prevention of rejection in renal transplants.
Corticosteroid therapy
There is no evidence that steroids are useful for amyloidosis, although steroid injections may be useful
for some types of inflammatory rheumatoid arthritis.
Non-steroidal anti-inflammatory drugs
Although non-steroidal anti-inflammatory drugs (NSAIDs) may help the pain in arthritic conditions, this
would not cure the condition of amyloidosis in this case. NSAIDS in combination with paracetamol may
be prescribed for pain relief.
Switch to low-flux dialysis membranes
It is high-flux rather than low-flux dialysis membranes which are useful for amyloidosis in dialysis. In fact,
several studies have demonstrated that the serum levels of beta-2 microglobulin are lower in patients
treated with high-flux biocompatible membranes.
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110
A.75.A 22-year-old student presents with dark urine with the appearance of ‘black tea’, followed by
swelling of the eyelids associated with malaise, anorexia and weakness.
She has no relevant medical history, although she reports having had an infection at the site of a skin
piercing approximately 4 weeks previously, for which she was prescribed some antibiotics. She took
these for 2 days but stopped because the infection cleared and she developed some abdominal pain
while taking them.
On physical examination, her blood pressure is 125/70 mmHg, with chest and abdominal examination
unremarkable.
Urinalysis:
Protein 3+
Blood 3+
Nitrites Negative
Leukocytes Negative
Acute intermittent porphyria
Acute variegate porphyria
Alkaptonuria
Mesangioproliferative glomerulonephritis (immunoglobulin A [IgA] nephropathy)
Post-streptococcal glomerulonephritis
Explanation
This young woman has presented with a nephritic syndrome with frank (probably haemolysed) blood in
her urine. The length of time after her skin infection makes the likely diagnosis a post-streptococcal
glomerulonephritis (Option E).
Alkaptonuria
Alkaptonuria is an inborn error of the metabolism of tyrosine, resulting again in urine which turns black
on standing due to excretion of homogentisic acid, which is oxidised on contact with air.
Acute intermittent porphyria
Acute intermittent porphyria can cause urinary discoloration when the urine is left to stand in sunlight,
but this lady has dark urine when passed.
Acute variegate porphyria
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The clinical features in any case are not those of this form of porphyria, which typically presents with
neuropathy, severe episodes of abdominal pain and neuropsychiatric disturbance. When comparing
acute intermittent porphyria (AIP) and variegate porphyria, the latter rarely presents with acute
episodes (unlike the former), and often the only reported symptom is excessive skin sensitivity to
sunlight.
Immunoglobulin A (IgA) nephropathy usually occurs with upper respiratory tract infections and usually
either at the same time or within 2–3 days of these.
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A.76.A 65-year-old woman presents for review. She is using acetazolamide and timolol for chronic
glaucoma. She has a past medical history of asthma.
On physical examination, her blood pressure is 130/80 mmHg and chest and abdominal examination is
unremarkable.
Investigations:
Sodium 145 mmol/l 135–145 mmol/l
Potassium 2.6 mmol/l 3.5–5.0 mmol/l
Creatinine 128 μmol/l 50–120 µmol/l
Corrected calcium 2.1 mmol/l 2.20–2.60 mmol/l
Chloride 118 mmol/l 98-106 mmol/l
Phosphate 0.65 mmol/l 0.70–1.40 mmol/l
What is the cause of the metabolic abnormality?
Lactic acidosis
Renal tubular acidosis type 1 (distal)
Renal tubular acidosis type 2 (proximal)
Explanation
112
Renal tubular acidosis type 2 (proximal)
Renal tubular acidosis is a disorder of the renal tubules’ ability to maintain acid–base balance, leading to
chronic hyperchloraemic metabolic acidosis.
The proximal tubule is the major site for reabsorption of filtered bicarbonate. In proximal RTA (type 2
RTA, pRTA), bicarbonate reabsorption is defective. pRTA rarely occurs as an isolated defect of
bicarbonate transport and is usually associated with multiple proximal tubule transport defects;
therefore, urinary loss of glucose, amino acids, phosphate, uric acid and other organic anions such as
citrate can also occur (Fanconi syndrome).
Plasma bicarbonate is <21 mmol/l, and urine pH may fall below 5.5 with acidification.
Features of RTA 2 are:
acidosis
hypokalaemia: due to inability of renal tubules to secrete hydrogen ions, potassium is preferentially
excreted
glycosuria
aminoaciduria
hyperchloraemia: chloride ions are mobilised to replace lost bicarbonate ions
osteomalacia: excess hydrogen ions are buffered by calcuim, resulting in depletion of calcium from
bone.
Causes and associations of RTA 2 are:
Fanconi syndrome, Wilson's disease, cystinosis and hereditary fructose intolerance and acetazolamide,
which leads to increased proximal tubule bicarbonate loss.
Renal tubular acidosis type 1 (distal)
Renal tubular acidosis type 1 will include a normal anion gap acidosis. There is failure to reabsorb
bicarbonate ions or insufficient excretion of hydrogen ions. There is hypokalaemia, and other associated
features such as urinary stone formation from the alkaline urine, hypercalciuria and low urinary citrate,
nephrocalcinosis and bone demineralisation.
Bartter syndrome is an autosomal recessive inheritance. It is usually diagnosed in children or

adolescents with failure to thrive, polydipsia, polyuria and cramps. There is hypokalaemia, mild
metabolic alkalosis, increased urinary sodium, potassium and calcium with increased serum renin and
aldosterone.
Lactic acidosis
There would be a high anion gap with a raised lactate level in lactic acidosis. Lactic acidosis would occur
in sepsis or shock. It may also occur as a complication of metformin use in kidney disease.
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Nephrogenic diabetes insipidus would not cause a metabolic acidosis. This condition may be caused by
drugs, and symptoms include excessive thirst and polyuria.
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A.77.A 22-year-old man with spina bifida who practises intermittent self-catheterisation comes to the
Emergency Department for review. He has no symptoms of urinary tract infection (UTI) although reports
that his past two catheterisations have produced cloudy malodourous urine.
On examination, he is apyrexial, his blood pressure is 122/82 mmHg; pulse is 72 bpm and regular. There
is no tenderness on abdominal palpation.
Investigations:
Platelets (PLT) 194 × 109/l 150–400 × 109/l
C-reactive protein (CRP) 12 mg/l 0–10 mg/l
Urine Protein +, Nitrite +
Bladder washout
Increase oral fluids and catheter change frequency
Intravenous (IV) co-amoxiclav
Oral co-amoxiclav
Supra-pubic catheterisation
Explanation
Increase oral fluids and catheter change frequency
Approximately 70% of patients who self-catheterise may develop bacteriuria. There are no signs of
sepsis and malodourous urine can simply be treated with increasing oral fluids and changing of catheter
more frequently.
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Bladder washout
Bacteriuria is common in self-catheterisation and there are no signs of sepsis.
Intravenous (IV) co-amoxiclav
There are no clinical signs of sepsis and bacteriuria is not an indication for IV antibiotics.
Oral co-amoxiclav
Only 5% of patients who self-catheterise will develop a symptomatic UTI. There are no clinical symptoms
or signs of sepsis in this case and therefore oral antibiotics is not appropriate.
Supra-pubic catheterisation
Supra-pubic catheterisation has the same risks as urethral catheterisation and therefore is not an
appropriate next step.
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A.78.A 52-year-old man presents with several months’ history of generalised swelling, fatigue, cough,
dyspnoea and several episodes of haemoptysis. There is no significant past medical history and he did
not take any regular medication. He smokes 20 cigarettes per day and drinks 14 units of alcohol per
week.
On examination, he is grossly oedematous and has ascites. Cardiorespiratory examination is

unremarkable and there are no neurological signs or rashes.
Platelets (PLT) 380 × 109/l 150 - 400 × 109/l
Bilirubin 16 μmol/l 1 - 22 μmol/l
Aspartate aminotransferase (AST) 32 u/l 10 - 40 u/l
Alkaline phosphatase (ALP) 120 u/l 30 - 150 u/l
115
Urinalysis Protein +++
24-hour urinary protein excretion 5g < 150 mg
Echocardiogram Mild left ventricular impairment, no valve lesion
Abdominal ultrasound scan Normal-sized kidneys, no abnormality seen
A renal biopsy was performed.
His chest X-ray is shown below.
What is it most likely to show?
Crescent formation
Kimmelstiel–Wilson lesions
Fusion of podocyte foot processes on electron microscopy
Necrotising granulomata
Thickened glomerular basement membrane with deposits of IgG and C3
Explanation
Thickened glomerular basement membrane with deposits of IgG and C3
This patient has developed nephrotic syndrome as a result of membranous glomerulonephritis which is
associated with an underlying bronchial carcinoma (seen as a right hilar mass invading the trachea on
the chest x-ray). Membranous glomerulonephritis accounts for 20–30% of adult nephrotic syndromes,
and there will be underlying malignancy in 10% of cases. Other causes of membranous nephropathy are
drugs (eg gold and penicillamine), autoimmune diseases (eg systemic lupus erythematosus (SLE)) and
infections (eg hepatitis). Approximately one-third of patients will progress to end-stage kidney disease
within 10–20 years of diagnosis. Typical renal biopsy features of membranous nephropathy is thickened
glomerular basement membrane with deposits of IgG and C3. Treatment may include chlorambucil and
cyclophosphamide.
Image source: https://radiopaedia.org/cases/bronchial-carcinoma?lang=gb
Fusion of podocyte foot processes on electron microscopy
This feature is found in minimal change disease. Although minimal change disease can present with
nephrotic syndrome, it is more common in young children. The prognosis is generally good and steroid
responsive.
Crescent formation
Crescents are found in rapidly progressive glomerulonephritis. The presentation would be a quickly
deteriorating renal function with blood and protein in the urine. Crescentric glomerulonephritis may be
found in anti-glomerular basement membrane disease and lupus nephritis.
116
Kimmelstiel–Wilson lesions
These lesions are pathognomonic of diabetic nephropathy. In this scenario there is no mention of
history of diabetes and there is no sugar in the urine. The first sign of diabetic nephropathy is
microalbuminuria.
Necrotising granulomata
Granulomas are an organised collection of macrophages. There are a variety of conditions which can
cause necrotising granulomata, of which the most classic is tuberculosis. In this scenario there is no
history that is indicative of tuberculosis such as fever, weight loss or any contact history.
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A.79.A 51-year-old man visits his General Practitioner with a 1-month history of generalised muscle pain
and fatigue. He has also noticed the onset of progressive skin changes. He reports that the skin around
his ankles and wrists has become thickened and the joints feel tight and restricted. It has spread to his
knees and his mobility is deteriorating. His past medical history includes chronic kidney disease stage 5,
for which he receives renal replacement therapy and a pituitary macroadenoma that was surgically
removed following by a brief period of stereotactic radiotherapy. He denies any recent illnesses and his
surveillance magnetic resonance imaging (MRI) brain two months ago showed no recurrence of disease.
He regularly smokes ten cigarettes per day.
His observations are recorded to be within the normal range. On examination, his skin is thickened and
indurated, particularly over his joints. There are multiple erythematous patches of skin over his
extremities. His face is spared.
Which of the following is the biggest risk factor for the underlying diagnosis?
Age > 50 years
Patients on regular Haemodialysis
Male sex
Smoking
Explanation
Patients on regular Haemodialysis
This patient has nephrogenic systemic fibrosis, the rapid development of skin and soft tissue thickening
and fibrosis. The disease process arises secondary to gadolinium contrast exposure, eg following this
patient’s MRI scan. The increased risk is related to the reduced ability of the kidneys to remove the
contrast agent from the bloodstream. Therefore, patients with poor renal function and particularly
those with end-stage renal disease requiring dialysis are at highest risk of the disease.
Age > 50 years
117
The biggest risk factor for nephrogenic systemic fibrosis is poor renal function. Any patients with poor
renal function are susceptible to the disease and age is not a discriminating factor.
Male sex
Nephrogenic systemic fibrosis causes progressive skin and soft tissue fibrosis in patients with end-stage
renal disease following exposure to gadolinium. It can affect each gender equally.
Radiotherapy is a risk factor for multiple illnesses, including secondary malignancy and retroperitoneal
fibrosis. However, it is not a risk factor for nephrogenic systemic fibrosis.
Smoking
The biggest risk factor for nephrogenic systemic fibrosis is poor renal function. Smoking is not a risk
factor for the onset of the disease.
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A.80.A 56-year-old woman with chronic kidney disease secondary to chronic pyelonephritis was
reviewed in the Renal Clinic. She was complaining of some exertional dyspnoea and fatigue but no other
symptoms. She had a history of hypertension that was difficult to control, despite being on multiple
agents. She has taken oral iron sulphate.
On examination, she was pale, blood pressure was 190/110 mmHg, jugular venous pressure not raised.
She had bi-basal crepitations in her chest and mild pitting ankle oedema. Her abdomen was soft, non-
tender and not distended.
Platelets (PLT) 220 × 109/l 150–400 × 109/l
Ferritin 90 μg/l 20–260 μg/l
Folate (serum) 10 μg/l 2–11µg/l
Vitamin B12 (serum) 240 ng/l 150–675 ng/l
What is the most appropriate management of the anaemia?
Blood transfusion Intravenous iron infusion Subcutaneous erythropoietin Oral ferrous gluconate
Immediate commencement of haemodialysis
118
Explanation
Intravenous iron infusion
Anaemia is present in the majority of patients with chronic kidney disease and is due to a number of
factors, including erythropoietin and haematinic deficiency, increased bleeding tendency due to reduced
platelet function, and shortened red cell survival time. The target haemoglobin in these patients is
usually 100–120 g/l. This lady's iron is low despite taking oral iron supplements; this may be due to poor
oral absorption which commonly occurs. Therefore, it is important to administer the iron intravenously
which will bypass the gut. Once iron levels are restored, if haemoglobin is still low then erythropoietin
treatment may be considered.
Subcutaneous erythropoietin
Erythropoietin is usually very effective and is given subcutaneously, usually three times per week.
Disadvantages are that it is expensive and can accelerate hypertension and occasionally cause
encephalopathy. It should therefore be avoided in this patient until her blood pressure is better
controlled (below 170/110) and her ferritin is restored to the mid range (ferritin >100 μg/l), with a
normal vitamin B12 and folate.
Blood transfusion
Blood transfusion should be avoided if possible as she is fluid overloaded and it may precipitate
pulmonary oedema.
Oral ferrous gluconate
Oral iron therapy is usually ineffective, and ferrous gluconate is unlikely to be any more successful than
oral ferrous sulphate.
Immediate commencement of haemodialysis
Haemodialysis will not improve anaemia. Dialysis treatment may remove fluid, if any blood transfusion is
given.
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A.81.A 30-year-old woman was admitted yesterday with sudden onset breathlessness while eating
dinner and associated cough with frothy pink sputum. She was treated in the Emergency Department
and has been transferred to the ward. A similar episode prompted admission to hospital 2 months ago.
She has a background medical history of hay fever.
She now has bibasal crackles on auscultation only and she feels a lot better. Her blood pressure is
160/90 mmHg. The remainder of the examination is unremarkable.
Investigations on admission reveal the following:
Electrocardiogram (ECG) Sinus rhythm at 120 beats per minute (bpm)
119
Her chest X-ray is shown below.
What is the most likely underlying diagnosis?
Conn’s syndrome
Fibromuscular dysplasia
Goodpasture’s syndrome
Aortic stenosis
Explanation
Fibromuscular dysplasia
The patient is presenting with ‘flash’ pulmonary oedema. In pulmonary oedema, patients can cough up
frothy pink sputum. The association of ‘flash’ pulmonary oedema and hypertension in a young patient
(<50 years) with no obvious cardiovascular risk factors points towards fibromuscular dysplasia. In an
older patient with vascular pathology, renal artery stenosis is most likely.
Image source: https://commons.wikimedia.org/wiki/File:Pulmonary_oedema.jpg
GPA it is a rare disease in which blood vessels become inflamed (a condition called vasculitis) and
localised, nodular collections of abnormal inflammatory cells, known as granulomas, are found in
affected tissues.
Conn’s syndrome
Conn’s syndrome presents with hypokalaemia and hypertension.
Also known as antiglomerular basement antibody disease, or anti-GBM disease, it is a rare autoimmune
disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding
from the lungs and kidney failure. Flash pulmonary oedema is not a feature, pulmonary haemorrhage
may be present.
Aortic stenosis
120
Pulmonary oedema may be present in patients with severe critical aortic stenosis. However, it is not
common in young adults. There is no history of ischaemic heart disease or murmur to suggest aortic
stenosis in this case.
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A.82.A 24-year-old woman was admitted to hospital with a 3-day history of feeling generally unwell,
with fatigue, arthralgia and pruritis.
She had recently finished a 5-day course of antibiotics for a urinary tract infection but there was no
other significant past medical history. She had no significant findings on clinical examination except for a
widespread erythematous rash.
White cell count (WCC) 13.0 × 109/l (eosinophilia) 4.0–11.0 × 109/l
Platelets (PLT) 390 × 109/l 150–400 × 109/l
Creatinine (Cr) 720 μmol/l 50–120 μmol/l
Urinalysis Protein ++ blood +
What is the most important investigation to establish the diagnosis?
Autoimmune profile Renal tract ultrasound scan Renal biopsy Urine microscopy and culture
Antistreptolysin-O titre
Explanation
Renal biopsy
The diagnosis is acute tubulo-interstitial nephritis. Renal biopsy will show interstitial oedema with a
heavy infiltrate of inflammatory cells and variable tubular necrosis. Eosinophilia and eosinophiluria are
often seen. The most common cause is a hypersensitivity drug reaction to certain antibiotics (penicillins,
cephalosporins), non-steroidal anti-inflammatory drugs (NSAIDS) and allopurinol. The condition
generally has a good prognosis with the majority of patients recovering fully on withdrawal of the
offending drug. The acute kidney disease can often be managed conservatively unless the patient is
uraemic. There is some evidence that a short course of corticosteroids may accelerate recovery and
prevent long-term renal damage. Interstitial nephritis may also occur in a chronic form.
121
Autoimmune profile
An autoimmune profile might aid in the diagnosis of systemic lupus erythematosus (SLE) particularly as
there is arthralgia and rash, but there is no eosinophilia in SLE.
Renal tract ultrasound scan
In acute tubulo-interstitial nephritis, the kidney sizes would be normal. Renal ultrasound would be
useful in cases to exclude hydronephrosis.
Urine microscopy and culture
This scenario is not a urinary tract infection in view of the eosinophilia. If there were a urinary infection,
we may expect white cells in the urine.
Antistreptolysin-O titre
Streptococcal infection generally presents with a sore throat. Acute glomerulonephritis following
streptococcal infection is characterised by the sudden appearance of haematuria, proteinuria, red blood
cell casts in the urine, oedema, and hypertension with or without oliguria; this occurs 10–14 days after
streptococcal sore throat.
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A.83.A 38-year-old man with a 12-year history of human immunodeficiency virus (HIV), with a good
response to therapy, was admitted with a 12-day history of weakness, nausea, dysuria, myalgia and
decreased urine output. His last CD4 lymphocyte count was 368 × 106/l (normal range 500-900). The
current medications included didanosine, stavudine, atorvastatin, and trimethoprim-sulfamethoxazole.
He had been commenced on Indinavir for the last 5 months.
On examination, he was afebrile, heart rate100/min and regular, BP 176/99 mmHg, the JVP was not
elevated; the chest and abdominal examinations were normal.
Investigations:
White cell count (WCC) 6.7 × 109/l 4.0 – 11.0 × 109/l
Platelets (PLT) 200 × 109/l 150 - 400 × 109/l
Chloride (Cl–) 102 mmol/l 98 - 106 mmol/l
Bicarbonate 16 mmol/l 22 - 29 mmol/l
122
Bone profile Normal
Creatine kinase 100 u/l 22 - 198 u/l
Antineutrophil cytoplasmic antibodies (ANCA) Negative
Anti-GBM Negative
Hep B/Hep C Negative
Antistreptolysin O titre (ASOT) Negative
Cultures of blood/urine/sputum were negative.
Which one of the following investigations will be most helpful in making the diagnosis?
Native renal biopsy
Serum immunoglobulins
Urine for crystals
X-ray studies of kidneys, ureters, bladder (KUB)
Explanation
Urine for crystals
Testing the urine for crystals would confirm nephrolithiasis as the cause of AKI and specifically point to
indinavir as the causative agent. Drugs used in patients with HIV may cause AKI. This occurs most
commonly with protease inhibitors, in particular indinavir; therefore, it is the causative agent in this case
by causing intratubular crystal obstruction. It has also been reported with ritonavir.
Native renal biopsy
Nephrolithiasis may not be detected and the presence of acute tubular necrosis would not allow you to
differentiate the offending agent.
Renal ultrasound scan would be useful if obstruction was suspected or to detect shrunken kidneys from
chronic kidney disease. Although the blood pressure is high, there is no sign of fluid overload and the
acute kidney injury (AKI) is related to medication changes.
Serum immunoglobulins
There are no clinical or biochemical parameters to suggest myeloma or light chain deposition disease as
the cause of AKI. There is no anaemia and bone profile is normal.
X-ray studies of kidneys, ureters, bladder (KUB)
123
The radiological imaging procedures typically used in the diagnosis of ureteral stones appear to be
unreliable in the diagnosis of non-opaque stones due to indinavir.
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A.84.A 24-year-old man presents with facial swelling. He has recently recovered from an upper
respiratory tract infection, for which his general practitioner (GP) had prescribed a course of amoxicillin
three days earlier. He was diagnosed with minimal change nephropathy 2 years ago, which was
confirmed on biopsy. He is not currently taking corticosteroids.
On examination, his blood pressure (BP) is 150/88 mmHg. Chest and abdominal examination is
unremarkable, except he has pitting ankle oedema.
Investigations:
Platelets (PLT) 195 × 109/l 150 - 400 × 109/l
Albumin (Alb) 25 g/l 35 - 55 g/l
Urine protein +++
Immunoglobulin A (IgA) nephropathy
Relapse of minimal change disease
Explanation
Relapse of minimal change disease
Given the previous diagnosis, a relapse of minimal change disease is the most likely underlying diagnosis
here. The interval between streptococcal infection and appearance of symptoms is too short to indicate
that this is post-streptococcal glomerulonephritis. Minimal change disease is usually very responsive to
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corticosteroids, and hypertension should be managed aggressively, with angiotensin-converting enzyme
(ACE) inhibitors as the first-choice therapy.
IgA nephropathy is the most common cause of glomerulonephritis in developed countries. There would
be blood in the urine, instead of heavy proteinuria as in this case. Many patients are asymptomatic and
the haematuria is detected incidentally.
Focal segmental glomerulosclerosis may be a cause of proteinuria, but it would be unusual for a new
diagnosis of another cause of nephrotic syndrome, considering the previous diagnosis of minimal change
disease.
Presentation of focal segmental glomerulosclerosis includes proteinuria, often heavy, microscopic

haematuria and hypertension. An impaired renal function is common. Young black men are more
commonly affected.
Membranous nephropathy may be a cause of proteinuria, but it would be unusual for a new diagnosis of
another cause of nephrotic syndrome, considering the previous diagnosis of minimal change disease.
Findings in membranous nephropathy include nephrotic syndrome, and reduced immunoglobulins are
common, with IgG > IgA. Hepatitis B and C may be associated with membranous nephropathy.
Classically, post-streptococcal glomerulonephritis occurs in childhood (< seven years old), with a trigger
of streptococcal sore throat 10–21 days before nephritis. There is gross haematuria, oliguria, oedema
and hypertension. Bilateral loin pain or renal angle tenderness secondary to renal engorgement may be
present. Nephrotic syndrome is not a feature of post-streptococcal glomerulonephritis.
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A.85.A 54-year-old woman attends the Renal Clinic for a follow-up appointment. She has a history of
chronic kidney disease but has never required renal replacement therapy. Her creatinine level has
increased steadily from 230 to 320 over the past three years. Most recently, she has developed
increased shortness of breath and reduced exercise tolerance.
On examination, her BP is 142/82 mmHg and her heart rate is 82 bpm and regular. Her chest is clear and
her abdomen is soft and non-tender.
Investigations:
White cell count (WCC) 7.2 × 109/l 4.0–11.0× 109/l
Platelets (PLT) 201 × 109/l 150–400× 109/l
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Haemoglobin A1c(HbA1c) 52 mmol/l < 48 mmol/l
Ferritin 72 µg/l 24–355 µg/l
Which of the following is the most appropriate initial management according to National Institute for
Health and Care Excellence (NICE) guidelines?
Blood transfusion
IV iron replacement
Oral iron replacement
Subcutaneous monthly erythropoietin analogue
Subcutaneous weekly erythropoietin analogue
Explanation
Oral iron replacement
In the context of non-dialysis-dependent chronic renal impairment, a ferritin level of less than 100 ng/ml
is considered deficient. The NICE guidelines recommend a trial of oral iron for patients who are not
receiving haemodialysis and switching to IV iron if patients are intolerant of oral supplementation or do
not reach their target haemoglobin levels within three months.
Blood transfusion
Blood transfusion is not recommended in this situation because it can drive antibody formation, which
may sensitise against a future renal transplant; it also does not address the underlying iron deficiency.
IV iron replacement
In a patient with a ferritin level of less than 100 µg/l, absolute iron deficiency is suspected; where the
ferritin level is between 100 and 200 µg/l, functional iron deficiency may be present. As such, in this
situation, iron should be replaced to achieve a ferritin level of more than 200 µg/l. Although absorption
of oral iron is often poor, NICE guidelines recommend a trial of oral therapy before moving to IV
replacement.
Subcutaneous monthly erythropoietin analogue
Where the ferritin level is between 200 and 500 μg/l, erythropoietin is indicated for the treatment of
anaemia. However, a weekly preparation is usually instigated until the haemoglobin level is maintained
within an appropriate range.
Subcutaneous weekly erythropoietin analogue
126
Subcutaneous weekly erythropoietin is indicated after iron transfusion in this situation, in the event that
anaemia persists.
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A.86.A 50-year-old man presents to the Emergency Department with a 4-day history of abdominal pain
and increased urinary frequency. He also complains of intermittent burning on micturition. His past
medical history includes alcoholic liver disease, and he smokes 20 cigarettes per day.
On examination, he has a temperature of 39.2 °C with a heart rate of 120 bpm and a BP of 100/60
mmHg. His abdomen is soft with suprapubic tenderness. There are multiple spider naevi across his chest
wall.
He is catheterised with 200 ml residual volume in his bladder.
Investigations:
Investigation Results Normal value
Platelets (PLT) 390 × 109/l 150–400 × 109/l
Urinary sodium 60 mmol/l 40–220 mmol/day
Urinary osmolality 320 mOsm/kg 50 to 1200 mOsm/kg
Urinalysis Brown granular casts in urine
A CT of the kidneys, ureter and bladder confirm a diagnosis of right-sided pyelonephritis without
hydronephrosis.
What is the most likely cause of this patient’s acute kidney injury?
Acute tubulointerstitial nephritis
Hepatorenal syndrome
Papillary necrosis
127
Prerenal acute kidney injury
Explanation
Taking a look at this patient’s blood results, it is clear that they have an acute kidney injury with
significantly deranged urea and creatinine levels. In the assessment of an acute kidney injury, it is
important to establish whether the cause is prerenal (eg hypoperfusion secondary to sepsis), renal (eg
acute tubular necrosis) or post-renal (eg urinary obstruction). Given the information provided from the
additional tests such as urinary sodium and osmolality, it is most likely that this patient has acute tubular
necrosis (ATN).
ATN is an intrinsic renal injury commonly caused by ischaemia, sepsis or nephrotoxins, including drugs
such as radiocontrast agents, non-steroidal anti-inflammatory drugs and antibiotics, including
aminoglycosides. The history of significant sepsis (fever, tachycardia and hypotension) is supportive of a
diagnosis of ATN.
In patients with ATN, due to the intrinsic damage to the renal tubular epithelium, the kidneys cannot
hold on to electrolytes causing an elevated urinary sodium level (ie over 40 mmol/l). There is also
reduced reabsorption of water, electrolytes and urea, causing a fairly dilute urine with a normal-to-low
urine osmolality (below 450 mOsmol/kg) and a urea-to-creatinine ratio of below 40 : 1 (calculated as
plasma urea / (plasma creatinine / 1000). This patient’s urea-to-creatinine ratio is 39. In contrast,
Additionally, the urinalysis of a patient with ATN frequently shows tubular epithelial cells or muddy
brown casts, as seen here.
Papillary necrosis
Papillary necrosis is a less common cause of acute kidney injury, and common contributors include
analgesia use (analgesic nephropathy) where damage to the renal papilla causes cells to slough off into
the renal tubules leading to an obstructive uropathy. However, obstructive uropathy can be ruled out,
given the lack of hydronephrosis on the CT scan.
Acute tubulointerstitial nephritis
Acute tubulointerstitial nephritis is classically caused by a hypersensitivity reaction to drugs such as non-
steroidal anti-inflammatory drugs and antibiotics. However, autoimmune diseases and infections are
also possible (albeit less common) causes. The condition manifests with an acute kidney injury in
combination with rash, fever and eosinophilia with a sterile pyuria and white cell casts in the urine. This
does not completely fit this patient's presentation, making acute tubulointerstitial nephritis unlikely.
Hepatorenal syndrome
Hepatorenal syndrome (HRS) is a reasonable differential diagnosis given the history of alcoholic liver
disease. HRS is a diagnosis of exclusion after ruling out other causes of renal injury and a liver transplant
is the only definitive treatment. HRS manifests secondary to portal hypertension and the production of
128
vasoactive mediators that, in turn, cause splanchnic vasodilatation, leading to renal hypoperfusion and a
prerenal acute kidney injury.
Since the liver is involved in the synthesis of urea, urea levels are variable and may frequently be low.
Furthermore, urinalysis is unlikely to show brown casts as seen in this case. In fact, a key distinguishing
factor between HRS and ATN is the urinary sodium and urine osmolality. In HRS, there is preserved
tubular function and sodium retention, meaning that the urinary sodium would be low in HRS and the
urine osmolality would be raised.
Prerenal acute kidney injury is classically caused by hypoperfusion. This should be considered as the
cause of this patient’s renal impairment, given the presentation of urinary sepsis. However, patients
with a prerenal acute kidney injury will have concentrated urine with low urinary sodium levels as the
kidneys are trying to preserve volume.
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A.87.A 50-year-old woman, who has a 10-year history of chronic kidney disease and is on haemodialysis,
is admitted with a pathological fracture of her left femur. She is taking ramipril 10 mg, amlodipine 10
mg, atorvastatin 10 mg, calcium and vitamin D.
On examination, she has hepatosplenomegaly. Her father had passed away due to a kidney disease. Her
blood pressure is 155/92 mmHg and she has peripheral oedema. Previously, when she was still passing
reasonable amounts of urine, her protein excretion was measured at 3.2 g/24 hours.
Investigations:
Platelets 178 × 109/l 150–400 × 109/l
Corrected Ca2+ 2.61 mmol/l 2.20–2.60 mmol/l
Alkaline phosphatase 295 IU/l30–130 IU/l
Parathyroid hormone (PTH) 64 pmol/l 1-6 pmol/l
X-ray left femur lucency in mid-shaft
T score -3.0
129
Which of the following is the most likely cause of the fracture?
Dialysis related amyloid Multiple myeloma Hyperparathyroidism Osteomalacia Malabsorption
Explanation
Hyperparathyroidism
This person has familial renal amyloidosis, as evidenced by hepatosplenomegaly, nephrotic syndrome
and progressive kidney disease. The fracture is most likely to be related to hyperparathyroidism and loss
of bone density. The raised alkaline phosphatase is likely to be as a result of the recent fracture, and the
calcium level to slight over-replacement with calcium and vitamin D. Cinacalcet would be the obvious
next therapy for this patient.
Dialysis related amyloid
Features of amyloid arthropathy on radiography will include juxta-articular soft tissue swelling, mild
periarticular osteoporosis, subchondral cystic lesions with well-defined sclerotic margins and normal
joint space. These lesions were not apparent in the radiograph in this case.
Multiple myeloma
Fractures and skeletal complications are found in up to 80% of patients with myeloma due to the plasma
cell dyscrasias. Radiographically there are multiple destructive lytic lesions in the skeleton, due to severe
demineralization that characterises multiple myeloma. This is not the case in this scenario.
Osteomalacia
Osteomalacia is due to insufficient mineralisation of the osteoid due to vitamin D deficiency or defects in
phosphate metabolism. The radiograph shows diffuse demineralisation with the characteristic smudgy,
‘erased’ type of demineralisation with pseudo-fractures (Looser zones).
Malabsorption
Malabsorption may lead to pathological fractures because of reduced absorption of vitamin D and
calcium essential for bone formation. There is no history of malabsorption, such as diarrhoea or weight
loss, in this case. We may expect the calcium level to be low.
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A.88.A 24-year-old man presents to the Emergency Department with visible haematuria. He has no past
medical history of note, other than a sore throat, which quickly resolved. He has no history of kidney
stones and no history of weight loss, fatigue or fevers.
There is no pain or tenderness on examination of his abdomen, and his urine is noted to be stained pink-
red. An abdominal computerised tomography (CT) is performed which is reported as normal.
His blood pressure is 165/95 mmHg.
Investigation:
130
Compliment C3 100 mg/dl 80–160 mg/dl
Compliment C4 35 mg/dl 20–50 mg/dl
Antinuclear antibody (ANA) Negative
Antineutrophil cytoplasmic antibodies (ANCA) Negative
Glomerular basement membrane (GBM) Negative
Electrophoresis No paraprotein
ANCA vasculitis
Anti-GBM disease
Transitional cell carcinoma (TCC) of the bladder
Explanation
IgA nephropathy is a common cause of haematuria, which can be microscopic or visible. It can present
de novo but is also stimulated by mucosal infection. It is associated with progression to renal failure,
hypertension and recurrence in kidney transplants. There are no highly evidenced treatment strategies
against IgA, but smoking and hypertension are known to accelerate its progression. The time frame from
infection to presentation is usually short, compared to, for example, PSGN, which takes weeks to
develop.
ANCA vasculitis
131
ANCA-associated vasculitis is a crescent-forming inflammatory disease. A crescent is an inflammatory
destructive process of the glomeruli which presents with acute, rapidly progressive renal failure and
symptoms of vasculitis (which this man lacks). It is treated with immunosuppression, initially with
steroids.
Anti-GBM disease
Goodpasture’s (or anti-GBM) disease is an autoimmune disorder characterised by antibodies against the
alpha-3 subunit of type IV collagen found in the basement membrane of the lungs and kidney. Its
presentation is heterogeneous and may present with either alveolar haemorrhage or rapidly progressive
glomerulonephritis, or both. It is treated with steroids and plasma exchange. In this case, the patient has
a negative anti-GBM titre, which would make Goodpasture’s disease unlikely.
Post-streptococcal glomerulonephritis (PSGN) is an immune complex-forming illness following

streptococcal infection, which results in hypocomplementaemia (absent in this case), and classically
occurs weeks after infection, rather than soon after. It would be unusual for it to present with this
degree of hypertension.
Transitional cell carcinoma (TCC) of the bladder
Transitional cell carcinoma (TCC) of the bladder can present with visible haematuria and obstructive
renal failure. It would, however, be unlikely to present in such a young patient with no significant
history. Similarly, given the normality of the CT scan, this degree of kidney disease cannot be explained
by obstruction. TCC does not fit with the clinical picture.
A.89.A 55-year-old man is referred to the Ambulatory Care Unit by his General Practitioner (GP). He
presents with a 4-week history of lower back and abdominal pain. The pain occasionally radiates down
to his scrotum. He reports passing less urine than normal but denies any additional lower urinary tract
symptoms. There is no history of recent illness. His past medical history includes angina for which he
takes bisoprolol, diltiazem, aspirin, atorvastatin and glyceryl trinitrate as required.
His observations are within normal limits. On examination, chest sounds are clear and heart sounds are
normal. His abdomen is soft with no palpable organomegaly. There is bilateral lower limb oedema
extending up to his mid-thighs.
Investigations:
Neutrophils 6.1 × 109/l 2.5–7.5 × 109/l
Lymphocytes 3.7 × 109/l 0.8–5.0 × 109/l
132
Platelets (PLT) 213 × 109/l 150–400 × 109/l
Intravenous urography: there is distal narrowing and proximal dilatation of the ureters and renal pelvis
bilaterally
Which of the following medications is most likely to be associated with the underlying diagnosis?
Pantoprazole
Atorvastatin
Bisoprolol
Diltiazem
Glyceryl trinitrate
Explanation
Bisoprolol
combined with deranged kidney function, and compression of the arterial supply to and venous return
from the lower limbs can cause intermittent claudication and bilateral limb swelling, respectively.
Chronic normocytic anaemia is also common on routine laboratory testing, in addition to raised
inflammatory markers. Retroperitoneal fibrosis is exceedingly rare and most cases are idiopathic,
although the disease is associated with a number of other illnesses such as malignancy, sarcoidosis,
radiotherapy, surgery and drugs. Drug causes include ergot-derived drugs and beta-blockers.
Diltiazem
Diltiazem is a calcium channel blocker used in the treatment of angina. Side-effects include abdominal
pain and peripheral oedema. Malaise and congestive heart failure may also be seen. However, diltiazem
does not explain this patient’s full presentation and laboratory findings.
133
Pantoprazole
It is unlikely that pantoprazole would be responsible for this patient’s presentation. An alternative
diagnosis should be considered. Pantoprazole has no role in the pathogenesis of retroperitoneal fibrosis.
Atorvastatin
Common side-effects of atorvastatin include myalgia and gastrointestinal upset. In some cases,
myopathy, myositis and rhabdomyolysis may be seen. However, atorvastatin does not cause
retroperitoneal fibrosis and cannot explain this patient’s symptoms.
Glyceryl trinitrate
Glyceryl trinitrate is used to provide symptomatic relief for patients with angina. Common side-effects
include headache, flushing and hypotension. It is not a cause of retroperitoneal fibrosis and would not
be responsible for this patient’s current clinical presentation.
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A.90.A 22-year-old man is admitted to the ward with an infective exacerbation of his asthma.
While on the ward, he develops pain in his left flank that radiates down to his groin with associated
dysuria.
Urinalysis Hexagonal crystals
Kidney–ureter–bladder (KUB) X-ray Area suspicious of a renal stone calculus seen in the region of
the left vesico-ureteric junction
The patient passes the stone and is diagnosed with cystinuria. He is advised on diet, alkalinization of the
urine and adequate hydration therapy. He is discharged but returns a few weeks later with similar
symptoms.
What is the next best medical therapy?
Allopurinol
Furosemide
Penicillamine
134
Bendroflumethiazide
Desferrioxamine
Explanation
Penicillamine
Penicillamine is effective at treating cystinuria, although it is usually reserved for patients who suffer
persistent stones despite urinary alkalinisation because of issues around tolerability.
Bendroflumethiazide
Bendroflumethiazide reduces urinary calcium excretion and therefore is of most value in patients with
recurrent calcium containing kidney stones.
Allopurinol
Allopurinol is useful in reducing symptoms of urate nephropathy.
Furosemide
Furosemide inhibits calcium reabsorption and therefore increases the risk of calcium containing renal
stones.
Desferrioxamine
Desferrioxamine is used as an iron chelator rather than in the treatment of cystinuria.
**********************************************************************************
A.91.A 65-year-old man, a retired factory worker, presents with some troublesome urinary symptoms
over the last two months. He reports feeling the need to empty his bladder frequently and often feels he
still needs to void immediately after having been to the toilet. He admits to some terminal dribbling. He
denies any systemic symptoms.
He was seen by his General Practitioner (GP) who advised that he stops drinking tea and coffee,
although this has had little effect on his symptoms. His GP referred him because he found blood on
urinalysis.
He has a history of hypertension and diabetes mellitus, for which he takes bendroflumethiazide and
metformin. He is a smoker who has smoked around ten cigarettes per day since he was a teenager.
On examination, he appears well; his blood pressure is 160/95 mmHg, with a normal abdominal
examination. Rectal examination reveals a smooth, moderately enlarged prostate, with preservation of
the median sulcus. A urine sample appears normal on visual inspection.
Blood results are as follows:
135
Alanine aminotransferase (ALT) 33 IU/l 5–30 IU/l
Alkaline phosphatase (ALP) 98 IU/l 30–130 IU/l
Bilirubin 24 µmol/l 2–17 µmol/l
Prostate-specific antigen (PSA) 4 ng/l 4 ng/l
Urinalysis reveals:
Blood ++
Leukocytes +
Protein +
Nitrites Negative
Urine microscopy demonstrates erythrocytes at > 5 per high-power field (HPF) (microscopic haematuria
defined as > 3 per HPF). No casts or crystals are seen. No micro-organisms are found on Gram staining.
Benign prostatic hypertrophy
Non-infectious haemorrhagic cystitis
Prostatic carcinoma
Transitional cell carcinoma of the bladder
Explanation
Transitional cell carcinoma of the bladder
The likely diagnosis is transitional cell carcinoma of the bladder. This man is a life-long smoker, and his
work in a factory may have exposed him to recognised carcinogens associated with urothelial
malignancy.
The combination of symptoms suggesting bladder irritation and persistent, albeit microscopic,
haematuria makes this a concern. Persistent microscopic haematuria needs to be taken seriously and
investigated appropriately.
136
His symptoms are not suggestive of prostatic hypertrophy, although mild prostatic enlargement is
common and might be found coincidentally. Benign prostatic hypertrophy would not fit with the urine
findings of haematuria seen here, which are a stronger pointer towards transitional cell bladder cancer.
Non-infectious haemorrhagic cystitis
Non-infectious haemorrhagic cystitis is commonly associated with a specific insult to the bladder (such
as metabolites of cyclophosphamide or radiotherapy of the pelvis and lower abdomen), none of which
are reported in this case.
Prostatic carcinoma
The normal rectal examination and low PSA make a diagnosis of prostatic carcinoma less likely, although
it would still be on the differential and would need to be excluded. A transrectal biopsy would be one
option for further investigation.
Transitional cell carcinoma is much more common than squamous cell carcinoma. Squamous cell
carcinoma is characteristically seen in patients with long-term bladder infection/inflammation, such as
those with schistosomiasis.
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A.92.A 26-year-old man is referred to you by his general practitioner (GP) with persistent microscopic
haematuria. He has recently been diagnosed with coeliac disease and commenced on a gluten-free diet.
The dermatologists have also started oral dapsone. The patient otherwise has remained asymptomatic.
Initial investigations show:
Investigation
Result
Normal Values
Blood pressure
115/65 mmHg
90/60 mmHg - 120/80 mmHg
Haemoglobin (Hb)
135 g/l
135 - 175 g/l
8 × 109/l
4.0 – 11.0 × 109/l
137
Platelets (PLT)
320 × 109/l
150 - 400 × 109/l
Immunoglobulin G (IgG)
7.3 g/l
6.0 - 13.0 g/l
Immunoglobulin A (IgA)
2.8 g/l
0.8 - 3.0 g/l
Immunoglobulin M (IgM)
0.6 g/l
0.4 - 2.5 g/l
Sodium (Na+)
143 mmol/l
135 - 145 mmol/l
Potassium (K+)
3.9 mmol/l
3.5 - 5.0 mmol/l
Urea
8.5 mmol/l
2.5 - 6.5 mmol/l
Creatinine (Cr)
115 μmol/l
50 - 120 µmol/l
Bilirubin
20 μmol/l
1.71 - 20.5 μmol/l
Aspartate aminotransferase (AST)
10 u/l
138
10 - 40 u/l
Alkaline phosphatase (ALP)
60 u/l
20 - 140 u/l
Albumin
43 g/l
35 - 55 g/l
24-h urinary protein collection
1.8 g/24 h
< 0.2g/24h
Urine microscopy
Red cell casts present
Renal biopsy light microscopy
Mesangial proliferation
Amyloidosis
Cryoglobulinaemia
Dapsone side-effect
Immunoglobulin (IgA) nephropathy
Explanation
Immunoglobulin (IgA) nephropathy
IgA nephropathy (Berger’s disease) is a mesangioproliferative glomerulonephritis. It usually presents

with persistent microscopic haematuria or recurrent macroscopic haematuria, usually associated with
an upper respiratory tract infection. IgA levels are raised in 50% of cases. There is an association with
cirrhosis and coeliac disease. Most cases of IgA nephropathy, if it does not affect renal function, do not
require any treatment, apart from angiotensin-converting enzyme (ACE) inhibitor to control blood
pressure. There is a lack of large-scale randomised controlled trials in support of immunosuppressive
regimens.
Dapsone side-effect
139
Dapsone causes haemolytic anaemia. Side-effects of dapsone can present with an allergic reaction with
difficulty in breathing, angio-oedema and swelling of the lips. There may be a bluish skin colour, muscle
weakness, numbness or tingling and abdominal pain.
Amyloidosis
Amyloid gives rise to proteinuria and the nephrotic syndrome.
Cryoglobulinaemia
Cryoglobulinaemia usually presents with more cutaneous and articular manifestations. It is associated
with haematological malignancies and connective tissue diseases.
Renal cell carcinoma arises from the tubular epithelium. Typically, renal cell carcinoma may present with
painless haematuria, with a palpable abdominal mass
For further reference:
Lai K N et al. 2015. The treatment of IgA nephropathy. Kidney Disease (Basel), 1, 19–26.
For further reference: Lai K N et al. 2015. The treatment of IgA nephropathy. Kidney Disease (Basel), 1,
19–26.
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A.93.A 48-year-old woman is referred to the Endocrine Clinic after routine blood testing at the GP
surgery. She has no significant previous medical history; her only recent attendance at the doctor’s was
after fainting at a wedding when the weather was noted to be rather hot.
There was no other past history of note and examination revealed blood pressure of 112/82 mmHg. No
previous history of hypertension was noted, nor any episodes of hypovolaemia.
Investigations:
Platelets (PLT) 197 × 109/l 150 - 400 × 109/l
Which of the following investigations would be most useful in pointing to the diagnosis?
140
Urinary chloride excretion
Aldosterone level
Renal ultrasound scan Renal biopsy Renal angiography
Explanation
Urinary chloride excretion
This patient has hypokalaemic alkalosis without evidence of hypovolaemia or hypertension. The
presentation is typical of Gitelman syndrome presenting at older age, with no symptoms of
hypovolaemia. Gitelman is caused by mutation in the thiazide-sensitive NaCl transporter, and resultant
salt wasting leads to activation of the renin-angiotensin system also leading to raised aldosterone levels.
The easiest way to diagnose Gitelman’s is to measure urinary chloride excretion, levels > 40mmol/l are
consistent with a diagnosis of Gitelman’s or Barrter’s syndrome.
It is the most common congenital renal tubular disorder in white people, with a prevalence of 1 in
40,000, and is associated with hypocalciuria and hypomagnesaemia. Treatment is with potassium and
magnesium replacement. Where patients fail to respond to supplementation, potassium-sparing
diuretics may be required to restore serum potassium levels.
Aldosterone level
Although primary hyperaldosteronism presents with hypokalaemia and metabolic alkalosis,

hypertension is absent here, meaning it’s unlikely to be the diagnosis. Measurement of aldosterone is
therefore less useful than urinary chloride measurement.
Renal ultrasound scanning is most useful in identifying kidney diameter as a pointer to the underlying
cause of hypertension, rather than pointing to the cause of hypokalaemia when blood pressure is
normal.
Renal biopsy
Although not required for diagnosis, renal biopsy in Gitelman reveals hyperplasia of the juxtaglomerular
apparatus and prominence of medullary interstitial cells. It is most useful in identifying the pathology
underlying other causes of tubular dysfunction.
Renal angiography
Renal angiography is considered post-renal ultrasound in the event that there is hypertension,
differential kidney sizing, hypokalaemia, and hyper-reninaemic hyperaldosteronism, because of
increased likelihood of renal artery stenosis as the underlying diagnosis.
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A.94.A 58-year-old man presents to the Emergency Department with a 4-month history of worsening
lower back and abdominal pain. He feels generally fatigued with a reduced appetite and intermittent
nausea and vomiting. In total, he reports having lost 1–2 kg in weight. His abdominal pain occasionally
141
extends down to his scrotum and testicles and he has noticed increased urinary frequency for the last
two weeks. He has been married for 20 years and denies new sexual partners. His past medical history
includes colorectal carcinoma which was surgically resected 18 months ago. He remains in remission.
On examination, his temperature is 37.7 °C. He has a heart rate of 96 bpm and regular, with a blood
pressure of 130/70 mmHg. On auscultation, his chest is clear, with saturations of 95% on room air. There
is palpable tenderness over his right flank, but no signs of peritonism or organomegaly. There is
moderate pitting oedema to his knees.
Investigations:
Neutrophils 5.2 × 109/l 2.5–7.5 × 109/l
Lymphocytes 3.4 × 109/l 0.8–5.0 × 109/l
Platelets (PLT) 399 × 109/l 150–400 × 109/l
Urinalysis: + blood
Benign prostatic hyperplasia (BPH)
Polycystic kidney disease (PKD)
Renal metastases
142
Ureteric calculi
Explanation
combined with deranged kidney function, and compression of the arterial supply to and venous return
from the lower limbs can cause intermittent claudication and bilateral limb swelling, respectively.
Chronic normocytic anaemia is also common on routine laboratory testing, in addition to raised
inflammatory markers. Retroperitoneal fibrosis is rare and most cases are idiopathic, although the
disease is associated with a number of other illnesses such as malignancy, sarcoidosis, radiotherapy,
surgery and infection, including Mycobacterium tuberculosis and Treponema pallidum. This patient has
a history of colorectal carcinoma and surgical resection, which have both likely been risk factors for
retroperitoneal fibrosis.
Benign prostatic hyperplasia (BPH)
Benign prostatic hyperplasia (BPH) is a benign growth of the prostate gland that is naturally seen with
age. Lower urinary tract symptoms (LUTS) are common in BPH and include increased frequency, poor
stream and post-void dribbling. Although increased urinary frequency is seen in this patient’s
presentation, the lack of any other LUTS, along with the presence of weight loss, anaemia, deranged
kidney function and leg swelling, points away from a diagnosis of BPH.
Polycystic kidney disease (PKD)
Polycystic kidney disease (PKD) is an inherited disease characterised by multiple cystic lesions
developing within the kidneys that cause the kidney to slowly enlarge and ultimately become non-
functional. The growing cysts can cause flank pain, particularly following rupture, also causing
haematuria. This patient has previously been diagnosed with colorectal cancer and likely underwent
imaging such as computed tomography (CT) chest, abdomen and pelvis to exclude metastatic spread.
Cysts begin to develop at an earlier age and by 58 years, cystic disease should be well established. CT
abdomen is very capable of detecting PKD and is a frequently used investigation for diagnosing PKD.
Furthermore, the presence of weight loss and pitting oedema make PKD less likely to be the underlying
diagnosis.
Renal metastases
Renal metastases is a reasonable differential, given the previous history of malignancy. However, rather
than renal metastases, colorectal cancer most commonly metastasises to the liver, although lung, bone
and central nervous system (CNS) metastases are also possible. There should always be a long index of
suspicion for metastases in patients with previous malignancy. However, this patient has additional
symptoms that seem unlikely to be secondary to renal metastases, including lower limb oedema.
Ureteric calculi
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Ureteric calculi present as acute-onset loin-to-groin pain that is often severe and intermittent in nature.
Haematuria is often seen from microtrauma to the ureters. However, the duration of symptoms tends
to be much shorter and a 4-month history of abdominal pain, in combination with additional clinical
features such as weight loss and pitting oedema, makes an alternative diagnosis, such as retroperitoneal
fibrosis, more likely.
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A.95.A 28-year-old man with Alport’s syndrome received a pre-emptive living related renal transplant
from his mother. He has excellent primary graft function and the baseline Creatinine was 102 µmol/l.
Three months later, he presented with a 1-week history of decreased urine output with a raised
creatinine of 188 µmol/l.
The biopsy did not show evidence of acute rejection. Urine microscopy showed dysmorphic red blood
cells. He became progressively oligo-anuric and became dialysis dependent.
Anti-glomerular basement membrane (Anti-GBM) disease
Aspergillus pneumonia
Post-transplant lymphoproliferative disorder (PTLD)
Mycoplasma pneumonia Granulomatosis with polyangiitis
Explanation
Anti-glomerular basement membrane (Anti-GBM) disease
Alport’s is an inherited disorder resulting in microscopic haematuria, progressive nephritis with renal
impairment, sensorineural deafness and ocular abnormalities. It is due to mutations of tissue-specific
type IV collagen chains, leading to formation of autoantibodies similar to those on Goodpasture’s
syndrome. Therefore, a minority of Alport’s patients with a renal transplant develop a rapidly
progressive glomerulonephritis indistinguishable from Goodpasture’s syndrome but without pulmonary
haemorrhage. Treatment is similar to that of de novo anti-GBM disease but treatment efficacy is limited.
Aspergillus pneumonia
Opportunistic infections are common in transplant recipients, especially with over-immunosuppression,

but would not explain the graft loss. Other common infections in transplant patients include
cytomegalovirus, varicella zoster virus and fungal diseases.
Post-transplant lymphoproliferative disorder (PTLD)
Post-transplant lymphoproliferative disorder is an uncommon but serious manifestation of

immunosuppression. The early onset of disease in this patient with likely minimal immunosuppression
from a live donor transplant makes this answer incorrect. The majority of PTLD are B-cell clonality
144
associated with Epstein-Barr virus. Treatment is often by a reduction of the immunosuppression and
chemotherapy. Graft loss is common.
Mycoplasma pneumonia
Opportunistic infections are common in transplant recipients, especially with over-immunosuppression,

but would not explain the graft loss.
The short duration of illness and known association of anti-GBM in patients with Alport’s syndrome.
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A.96.A 66-year-old woman presents to the Emergency Department with a 5-day history of nausea,
vomiting and reduced oral intake. Today, she reports that her symptoms have worsened, and she now
feels increasingly fatigued and dizzy on exertion. Her past medical history includes diabetes mellitus, for
which she takes metformin and insulin Lantus twice daily.
On examination, she has a temperature of 38.4 °C with a heart rate of 110 bpm and BP of 99/60 mmHg.
Auscultation of the chest is clear with normal heart sounds and her abdomen is soft with mild,
generalised tenderness.
Investigations:
Investigation Results Normal value
Platelets (PLT) 390 × 109/l 150–400 × 109/l
Urinalysis Brown granular casts in urine
Which of the following findings are the most in keeping with the underlying diagnosis?
Decreased urine output
Elevated eosinophil count
Elevated blood urea-nitrogen-to-serum-creatinine ratio
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Elevated urine sodium concentration
Elevated urine osmolality
Explanation
Elevated urine sodium concentration
This patient’s blood results show that the patient has an acute kidney injury with significantly deranged
urea and creatinine levels. In the assessment of an acute kidney injury, it is important to establish
whether the cause is prerenal (eg hypoperfusion secondary to sepsis), renal (eg acute tubular necrosis)
or post-renal (eg urinary obstruction). Given the urinalysis showing brown casts in the urine and the
history of sepsis, acute tubular necrosis (ATN) is the likely diagnosis.
ATN is an intrinsic renal injury commonly caused by ischaemia, sepsis or nephrotoxins, including drugs
such as radiocontrast agents, non-steroidal anti-inflammatory drugs and antibiotics, including
aminoglycosides.
In patients with ATN, due to the intrinsic damage to the renal tubular epithelium, the kidneys are less
able to hold onto electrolytes, causing an elevated urinary sodium level (ie over 40 mmol/l). In contrast,
Elevated eosinophil count
An elevated eosinophil count is more commonly associated with acute tubulointerstitial nephritis. Acute
tubulointerstitial nephritis presents with an acute kidney injury in combination with rash, fever and
eosinophilia with a sterile pyuria and white cell casts in the urine. This is not seen in this patient.
Decreased urine output
A decreased urine output can be a feature of prerenal, renal or post-renal causes of an acute kidney
injury and is not specific. ATN typically follows the phases of oliguria, polyuria to recovery. However, not
all patients may present with oliguria, necessitating the need for additional investigations.
Elevated blood urea-nitrogen-to-serum-creatinine ratio
Due to the renal tubules being less able to reabsorb urea after it is filtered out from the glomerulus, the
urea-to-creatinine ratio is reduced in cases of ATN. A urea-to-creatinine ratio of below 40 : 1 (calculated
as plasma urea / (plasma creatinine / 1000) is indicative of ATN.
Elevated urine osmolality
In ATN, there is reduced reabsorption of water, electrolytes and urea, causing a dilute urine with a
normal-to-low urine osmolality (below 450 mOsmol/kg). In contrast, patients with a prerenal acute
kidney injury will have concentrated urine with low urinary sodium levels, as the kidneys are trying to
preserve volume.
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A.97.A 28-year-old woman who is 14 weeks’ pregnant with her first pregnancy attends for a routine
antenatal check. Her only past history of note includes two admissions to hospital with pyelonephritis as
a child. She takes no regular medication.
On examination, her blood pressure is 142/92 mmHg. It is rechecked an hour later and found to be
138/90 mmHg. There was no other significant abnormality found.
Investigations:
Urinalysis NAD
Ultrasound scan, kidneys:
Right kidney BPD 10.8 cm
Left kidney BPD 5.5 cm
Left kidney Irregular outline
What is the likely diagnosis?
Renal scarring due to recurrent pyelonephritis
Congenital renal atrophy
Glomerulonephritis
Pre-eclampsia
Explanation
Renal scarring due to recurrent pyelonephritis
This woman has hypertension in pregnancy, of which there are many causes. Her ultrasound scan
showing a damaged left kidney is suggestive of renal scarring recurrent. Chronic long-term renal damage
related to pyelonephritis may predispose to the development of hypertension. Enalapril,
bendrofluazide, hydralazine and atenolol are not recommended for the treatment of hypertension at
this stage of pregnancy, but methyldopa would be an acceptable choice. Long-term management of the
condition involves identification of any predisposing risk factors, such as abnormal ureteric implantation
or other structural problem that might be suitable for surgical repair.
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Congenital renal atrophy
Congenital renal atrophy may be an incidental finding where one kidney is smaller than the other. There
are no complications and she does not need any special treatment. The history of pyelonephritis as a
child suggests that chronic pyelonephritis is more likely to be the cause rather than congenital renal
atrophy.
Glomerulonephritis
Glomerulonephritis would not present with one smaller kidney compared with the other unless it is a
very chronic process, in which case there would be long-standing renal impairment and hypertension.
You would expect blood and protein in the urine in glomerulonephritis.
Pre-eclampsia
Pre-eclampsia would not present with one smaller kidney. There would be hypertension and a
significant amount of proteinuria. The condition begins after 20 weeks of pregnancy. In severe disease
there is microangiopathic anaemia, low platelet count, impaired liver function tests, dyspnoea and
oedema.
Renal artery stenosis will present with hypertension that may be resistant to treatment. Although the
renal size may be smaller on the side of the renal artery stenosis, we would expect the patient to have
long-standing renal impairment and hypertension. Also, renal artery stenosis is very uncommon in
young patients unless due to rare conditions such as fibromuscular dysplasia or Takayasu’s arteritis.
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A.98.A 62-year-old man presents to the Emergency Department with severe left loin pain and fevers
which have increased over the past 48 h. He has a history of chronic tophaceous gout, for which he
takes allopurinol and benzbromarone, and hypertension, for which he takes amlodipine 5 mg once daily.
Examination reveals a BP of 100/60 mmHg, pulse is 95/min and regular. He is pyrexial 38.5 °C.
Abdominal examination reveals severe left loin pain. His body mass index is 36 kg/m2.
Investigations:
Platelets (PLT) 201 × 109/l 150 - 400 × 109/l
Creatinine (Cr) 187 µmol/l (up from 121 µmol/l at last clinic appointment) 50 - 120 µmol/l
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Urine Red cells++, white cells++, nitrites+
Which of the following is the most useful next investigation?
Cystoscopy
Intravenous pyelogram (IVP)
Kidney, ureter and bladder X-ray (KUB)
Non-contrast enhanced computed tomography (CT)
Ultrasound abdomen
Explanation
Non-contrast enhanced computed tomography (CT)
The potential diagnosis here is obstructed pyelonephritis due to a urate-containing stone within the left
ureter. Benzbromarone, which the patient takes, increases urate excretion, thus leading to increased
risk of stones. In this situation, with a significant rise in creatinine, contrast-enhanced imaging carries
risk. Out of the other remaining options, non-contrast enhanced CT is thought to be more sensitive in
detecting obstruction versus ultrasound scanning.
In the presence of significant infection, a conservative approach is not appropriate. Intravenous

antibiotics plus cystoscopy and potential JJ stent insertion is therefore the management of choice.
Intravenous pyelogram (IVP)
An IVP will involve injection of contrast, therefore this will carry risk of worsening renal impairment and
also there may be added fluid load of contrast. Extra fluid from contrast is particularly important to
avoid in patients who are already fluid overloaded if they are renally impaired as well.
Cystoscopy
Cystoscopy will need to be performed later on after the diagnosis with potential JJ stent insertion;
however, to first obtain the diagnosis, the next investigation of choice would be non-contrast enhanced
CT.
Kidney, ureter and bladder X-ray (KUB)
A KUB is only sensitive in identifying calcium containing renal stones. Urate stones are radiolucent and
will not be identified on KUB.
Ultrasound abdomen
Ultrasound scanning is not as sensitive in detecting obstruction compared with non-contrast enhanced
CT. Ultrasound is operator-dependent, and also a large patient body habitus may make obtaining an
adequate image difficult.
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A.99.A 64-year-old man on the Cardiology Ward complains of a rash on his lower limbs and chest, which
started overnight. Two days previously, he underwent a primary percutaneous coronary intervention
after presenting with an ST-segment elevation myocardial infarction and an occlusion of the left anterior
descending artery was stented.
On examination, his BP is 140/84 mmHg and his heart rate is 86 bpm. The patient’s leg is shown below:
Investigations:
Platelets (PLT) 220 × 109/l 150–400 × 109/l
Neutrophils 6.5 × 109/l 2.5–7.58 × 109/l
Lymphocytes 2.2 × 109/l 1.5–3.5 × 109/l
Eosinophils 1.2 × 109/l 0–0.4 × 109/l
Acute kidney injury due to rhabdomyolysis
Contrast-induced nephropathy
Drug-induced tubulointerstitial nephritis
Explanation
The combination of a recent invasive vascular intervention and the presentation with livedo reticularis
and blue toe syndrome, along with eosinophilia and acute kidney injury, is suggestive of cholesterol
embolism. Treatment is primarily aimed at managing risk factors, such as with the use of statins. While
there is a role in select cases for surgical intervention with stenting or removal of emboli,
anticoagulation and antiplatelet therapy are not indicated.
Image credit: CC BY-SA 3.0 https://commons.wikimedia.org/wiki/File:Livedo_reticularis_of_left_leg.jpg
Acute kidney injury due to rhabdomyolysis
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While rhabdomyolysis can result in acute kidney injury, skin changes are seen in less than 10% of
patients and are typically blistering rashes. Additionally, the lack of trauma or prolonged immobilisation
makes rhabdomyolysis unlikely.
Contrast-induced nephropathy
In a patient who recently had coronary angiography and developed acute kidney injury, the two most
important differentials are cholesterol embolism and contrast-induced nephropathy. The presence of
eosinophilia and livedo reticularis differentiates cholesterol embolism from contrast-induced
nephropathy. The risk of contrast-induced nephropathy is higher in individuals with dehydration,
congestive cardiac failure and diabetes mellitus.
Drug-induced tubulointerstitial nephritis
Peripheral eosinophilia with acute kidney injury can occur in drug-induced tubulointerstitial nephritis.
However, the presence of livedo reticularis, particularly in a distribution which indicates an anatomical
location for the emboli, is likely due to cholesterol embolism.
While prerenal acute kidney injury may explain the raised creatinine level post-procedure, it would not
explain the rash or eosinophilia.
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A.100.A 45-year-old woman who was admitted with pyrexia and left renal colic is reviewed on the ward
some 12hrs after admission. An ultrasound conducted on the evening of admission revealed evidence of
left hydronephrosis with a suspected left ureteric stone estimated at 1.5cm in diameter, obstruction
appears to be in the lower third of the ureter. Her pyrexia has failed to settle despite IV rehydration and
IV Co-amoxiclav, and she still has significant pain. On examination her BP is 122/82 mmHg, pulse is
88/min and regular, her temperature is 38.0°C. There is left loin pain on abdominal palpation.
Which of the following is the most appropriate intervention?
Continued rehydration Lithotripsy Nephrostomy Ureteric stenting Ureteroscopy and stone removal
Explanation
Ureteroscopy and stone removal
The answer is Ureteroscopy and stone removal -
In this situation there is significant risk from continued proximal renal tract infection, therefore
intervention to relieve the obstruction is required. Out of the options for intervention, ureteroscopy and
stone removal carries the lowest potential burden of morbidity. Ureteroscopy without stent placement
is however only considered for selected patients.
Continued rehydration
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Continued rehydration is incorrect, it is inappropriate given continued symptoms of infection and
obstruction. Intervention to relieve obstruction is required.
Lithotripsy
Lithotripsy is incorrect, it is inappropriate because the procedure may worsen sepsis in the short term by
leading to disseminated bacteraemia.
Nephrostomy
Nephrostomy is incorrect, it is inappropriate because it carries a greater burden of morbidity versus

ureteroscopy. Where ureteroscopy is unavailable, percutaneous nephrostomy is the usual intervention.
Ureteric stenting
Ureteric stenting is incorrect, it is suboptimal because although it is effective in the short term, stents
may become obstructed or dislodged, leading to subsequent re-presentation with symptoms of ureteric
colic.
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A.101.A 21-year-old woman, who works as a receptionist in a doctor’s surgery, is admitted to the
Emergency Department after fainting at work. She puts it down to it being a particularly hot day but, on
further questioning, admits to extreme fatigue and muscle cramps when she takes on relatively minor
exercise, for as long as she can remember.
Her mother, who has come to the Emergency Department to collect her, confirms she was always
missing school sports because of lethargy and muscle pains.
Examination reveals a blood pressure (BP) of 100/70 mmHg; pulse is 75/min and regular. She is of
normal height and her BMI is 22.
Investigations:
Platelets (PLT) 189 × 109/l 150 - 400 × 109/l
24 hour urinary calcium 2.2 mmol 2.50 - 7.50 mmol
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Bartter syndrome
Conn syndrome
Cushing syndrome
Diuretic abuse
Gitelman syndrome
Explanation
Gitelman syndrome
This patient, in all likelihood, has suffered from hypokalaemia for many years, leading in conjunction
with hypomagnesaemia to severe fatigue and muscle cramps.
Gitelman syndrome presents with hypokalaemic metabolic alkalosis in the absence of hypertension.
Gitelman syndrome may be associated with milder disease and presents later than Bartter syndrome.
Work-up to confirm the diagnosis includes a diuretic screen and urinary calcium excretion. Gitelman
syndrome is associated with reduced calcium excretion, whereas Bartter syndrome is not, and this helps
to differentiate between the two conditions.
Bartter syndrome
Bartter syndrome usually presents in childhood because of failure to thrive. If urinary calcium is elevated
to levels > 6.9 mmol over a 24-hour period, this suggests Bartter syndrome.
Conn syndrome
Conn syndrome is associated with hypertension. Conn syndrome also presents with hypokalaemic
metabolic alkalosis. There may be other symptoms such as myalgia, muscle spasms, paraesthesiae and
polyuria. Complications may include stroke, myocardial infarction and kidney disease.
Cushing syndrome
Cushing syndrome is associated with hypertension, weight gain, abdominal striae, round facies, fat pad
between shoulder blades, muscle wasting and poor wound healing.
Diuretic abuse
Given the long history, although she works in a doctor’s surgery, access to or abuse of diuretics seems
unlikely.
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A.102.A 23-year-old man presents to the Emergency Department with bilateral leg swelling. He takes no
regular medications. He reports that at the age of eight, his parents took him to hospital with leg
swelling and he was given a short course of steroids which resolved it. He tells you that he has been well
and woke up with leg swelling.
153
On examination, he has bilateral pitting oedema up to his thighs. There are no other clinical findings of
note.
Investigations:
Platelets (PLT) 250 × 109/l 150–400 × 109/l
Urine protein : creatinine ratio (uPCR) 300 mg/mmol < 5 mg/mmol
Glycated haemoglobin (HbA1c) 4.5% 4.0–6.5%
A kidney biopsy is performed and he is initiated on a course of steroids; his oedema completely resolves
within three weeks.
What is the most likely outcome of histology expected on light microscopy if performed?
Effacement of podocyte foot processes
Kimmelstiel–Wilson nodules
Normal light microscopy
Segmental sclerotic lesions
Spikes on the basement membrane
Explanation
Normal light microscopy
Here is a 23-year-old man with heavy proteinuria. Of note, the characteristic history of minimal change
nephropathy is one of feeling generally well and waking to find the sudden onset of lower limb oedema,
or gross anasarca, with heavy albuminuria, which is present in this case. The classical light microscopy
findings of this pathology are of normal glomeruli.
Effacement of podocyte foot processes
Minimal change nephropathy is a predominantly childhood disorder characterised by heavy proteinuria

which resolves when treated with immunosuppression. Effacement of podocyte foot processes is seen
in minimal change nephropathy but is an electron microscopy finding. This question asks for the light
microscopy features expected in minimal change nephropathy.
154
Kimmelstiel–Wilson nodules
Nodular sclerosis of glomeruli is characteristic in only a handful of conditions, including amyloidosis and
cigarette smoking and is pathognomonic of diabetic nephropathy. In this case, this young man has an
Hba1c of 4.5% and no prior history to suggest diabetes.
Segmental sclerotic lesions
Segmental sclerotic lesions are likely to indicate a diagnosis of focal segmental glomerulosclerosis
(FSGS). FSGS is characterised by heavy proteinuria and can be either a primary autoimmune
phenomenon, treated with immunosuppression such as with tacrolimus, or secondary. Common causes
of secondary FSGS include hypertension and certain infections, including human immunodeficiency
virus, which causes a ‘collapsing’ variant with a very poor prognosis. Of note, primary FSGS often has
‘skip lesions’ and as such, many patients with a diagnosis of treatment-resistant minimal change do, in
fact, have FSGS.
Spikes on the basement membrane
There are two different kinds of membranous nephropathy – primary and secondary. Primary
membranous nephropathy is a protein-losing disorder that is characteristically driven by phospholipase
A2 receptor (PLA2R) antibodies. It is treated by aggressive immunosuppression. Secondary membranous
nephropathy commonly occurs as a result of an underlying malignancy. It is also characterised by heavy
protein loss and nephrotic syndrome. The classical pattern of histopathology in membranous
nephropathy is ‘spike’ formation of the basement membrane.
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A.103.A 48-year-old man presents to the Emergency Department with complaints of severe flank pain,
fever and dysuria for the past five days. The fever is high grade and associated with chills and rigours. On
examination, his blood pressure is 100/60 mmHg and his heart rate is 112 bpm. Severe flank tenderness
is present on the right side.
He is immediately started on intravenous rehydration with crystalloids and empirical antibiotic therapy.
A plain computed tomography (CT) abdomen is performed, which shows evidence of a ureteral calculus
measuring 11 mm, with hydroureteronephrosis. In addition, there is evidence of perinephric fat
stranding of the right kidney.
Which of the following is the optimal management for this patient?
Continue antibiotics and hydration
Elective surgery for ureteral calculus with stenting
Emergency surgery for ureteral calculus with stenting
Intensive Care Unit (ICU) admission for vasopressor support
Right partial nephrectomy
155
Explanation
Emergency surgery for ureteral calculus with stenting
In this patient with acute pyelonephritis due to obstructive uropathy, emergency surgical intervention
should be made to decompress the urinary tract. Indications for emergency surgery are:
confirmed complicated urinary tract infection due to obstructing stones
bilateral obstruction and acute kidney injury (AKI)
unilateral obstruction with AKI in a solitary functioning kidney.
Right partial nephrectomy
A nephrectomy can be performed in patients with a renal abscess in a small, scarred, chronically
pyelonephritic and poorly functioning kidney. It is not indicated in this patient.
Continue antibiotics and hydration
In this patient with clinically and radiologically confirmed acute pyelonephritis due to obstructive
uropathy, it is mandatory for the obstruction to be relieved by surgical intervention. Conservative
management is not the ideal line of management in this patient.
Elective surgery for ureteral calculus with stenting
Elective surgery for ureteral calculi can be performed when there is no associated pyelonephritis.
Indications for elective surgery are:
ureteral stones measuring > 10 mm
uncomplicated distal ureteral stones measuring at least 10 mm that have not passed after 4–6 weeks of
observation
symptomatic stones in patients without any other aetiology for the pain
persistent hydroureteronephrosis related to stones
recurrent uncomplicated urinary tract infections related to stones.
Intensive Care Unit (ICU) admission for vasopressor support
This patient’s blood pressure is 100/60 mmHg and so does not require any vasopressor support.
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A.104.A 67-year-old woman is referred to the General Nephrology Clinic for investigation and diagnosis
after her General Practitioner (GP) identified abnormal kidney function. She has type II diabetes and
takes linagliptin 5 mg once daily.
Clinical examination identifies a blood pressure of 130/75 mmHg and bilateral lower limb oedema
extending to the thighs. She is wearing orthotics and complains of progressive loss of sensation in her
feet.
156
A kidney biopsy is performed, which reveals diffuse mesangial expansion, glomerular basement
membrane (GBM) thickening and widespread Kimmelstiel–Wilson nodules.
Her blood test results are as follows:
White cell count
(WCC) 7.0 × 109/l 4.0–11.0 × 109/l
Platelets (PLT) 357 × 109/l 200–400 × 109/l
Glycated haemoglobin
(HbA1c)9.5% 4.0–6.5%
Focal segmental glomerulosclerosis (FSGS)
Hypertensive nephropathy
Explanation
Diabetic nephropathy is the most common secondary cause of nephrotic syndrome. In this case, this
patient has a history consistent with poorly controlled type II diabetes, evidenced by loss of sensation in
her peripheries. Similarly, her kidney biopsy is positive for Kimmelstiel–Wilson nodules. Nodular
sclerosis of glomeruli is characteristic in only a handful of conditions, including amyloidosis, cigarette
smoking and diabetes.
Focal segmental glomerulosclerosis (FSGS)
Focal segmental glomerulosclerosis (FSGS) is a common primary cause of nephrotic syndrome. It is

characterised by heavy proteinuria and can be either a primary autoimmune phenomenon, treated by
immunosuppression such as with tacrolimus, or secondary. Common causes of secondary FSGS include
hypertension and certain infections, including human immunodeficiency virus, which causes a
‘collapsing’ variant with a very poor prognosis.
157
Goodpasture’s (or antiglomerular basement membrane antibody) disease is an autoimmune disorder

characterised by antibodies against the alpha-3 subunit of type IV collagen found in the basement
membrane of the lungs and kidneys. It may present with alveolar haemorrhage or rapidly progressive
glomerulonephritis. Goodpasture’s disease is treated with steroids and plasma exchange. It would be
highly unusual for Goodpasture’s disease to present with nephrotic syndrome.
Hypertensive nephropathy refers to a process of kidney injury, the result of either longitudinal exposure
to poorly controlled hypertension or an acutely accelerated process of malignant hypertension and
tissue injury. It is characterised by moderate protein loss, oedema and sclerosis of the glomeruli. It is not
characterised by nodular sclerosis, as is seen here.
There are two different kinds of membranous nephropathy: primary and secondary. Primary
membranous nephropathy is a protein-losing disorder that is driven characteristically by phospholipase
A2 receptor (PLA2R) antibodies. It is treated by aggressive immunosuppression. Secondary membranous
nephropathy commonly occurs as a result of an underlying malignancy. It is also characterised by heavy
protein loss and nephrotic syndrome. The classical pattern of histopathology in membranous
nephropathy is a ‘spike’ formation of the basement membrane.
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A.105.A 32-year-old man is admitted from the Emergency Department after suffering a collapse with
severe headache while chasing a man who had stolen alcohol from his shop. He has no other past
medical history of note, although you understand his father and an aunt died at a young age of strokes.
On examination, he has a blood pressure (BP) of 185/105 mmHg and retinal changes consistent with
hypertensive retinopathy.
Investigations:
Platelets (PLT) 202 × 109/l 150–400 × 109/l
Abdominal ultrasound scan Right adrenal mass,
multiple bilateral renal cysts
158
Urine dipstick Protein +, blood +
An magnetic resonance imaging brain is shown below.
Which of the following is the most appropriate next investigation?
Adrenal biopsy
24-h urinary catecholamine
24-h urinary 5-hydroxyindoleacetic acid (5-HIAA)
Computed tomography brain
Cystoscopy
Explanation
24-h urinary catecholamine
The suspicion from the history and the investigation findings given above is that he is suffering from a
phaeochromocytoma. His cerebellar mass may be a cerebellar haemangioblastoma. Both central
nervous system (CNS) haemangioblastomas and retinal lesions are commonly encountered in patients
with von Hippel–Lindau syndrome. Multiple renal cysts, which may be subject to malignant
transformation, are another common finding.
Most importantly of all, however, is appropriate confirmation of the possible phaeochromocytoma, so

that appropriate antihypertensive therapy can be instigated before this man undergoes a number of
operative procedures and investigations.
Image source:
https://upload.wikimedia.org/wikipedia/commons/f/f5/Hemangioblastoma_cerebellum.jpg
24-h urinary 5-hydroxyindoleacetic acid (5-HIAA)
24-h urinary 5-hydroxyindoleacetic acid (5-HIAA) is the most useful test in diagnosing carcinoid
syndrome. Typically, carcinoid syndrome presents with flushing and diarrhoea. It occurs due to the
excess production of serotonin by carcinoid tumours in amounts that overwhelm the liver’s metabolic
capabilities.
Adrenal biopsy
Adrenal biopsy is not indicated in the diagnosis of phaeochromocytoma. Any surgical procedures or
invasive investigation should only be undertaken after appropriate antihypertensive therapy has been
instituted, to avoid precipitating hypertensive crises.
Computed tomography brain
Magnetic resonance imaging brain may be of help in the diagnosis of a cerebellar tumour but would not
aid in the diagnosis of the phaeochromocytoma, which is the pressing problem here.
159
Cystoscopy
The presence of a one-off positive dipstick for microscopic haematuria in this patient would not warrant
a cystoscopy.
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A.106.A 45-year-old man with a history of Immunoglobin A (IgA) nephropathy comes to the Renal Clinic.
He has a history of microscopic haematuria and proteinuria which has been under follow-up for 10
years, and a recent renal biopsy has suggested IgA nephropathy with extensive sclerosis.
On examination, he has a blood pressure (BP) of 158/92 mmHg and has bilateral ankle oedema.
White Cell Count (WCC) 6.1 × 109/l 4.0 - 11.0 × 109/l
Platelets (PLT) 201 × 109/l 150 - 400 × 109/l
Creatinine (Cr) 280 μmol/l 50 - 120 μmol/l
Parathyroid hormone (PTH) 10 pmol/l 0/9 - 5.4 pmol/l
24 hour urinary protein 4.7 g < 0.2 g
Which of the following is the most important line of management for him?
Vitamin D
Sevelamer
Ramipril
Atenolol
Prednisolone and azathioprine
Explanation
Ramipril
This man has a well-established glomerulosclerosis related to his IgA nephropathy with impaired renal
function; as such, aggressive immunosuppressive therapy is less likely than improved blood pressure
control to have a significant impact on his prognosis. Among available blood pressure agents, ACE
160
inhibitors are thought to have the greatest effect in reducing progression of proteinuria and worsening
of creatinine.
Vitamin D
Vitamin D would not be a treatment in IgA nephropathy. Many patients with renal disease are vitamin D
deficient, and therefore it would be useful for measurement and replacement with vitamin D if levels
are low. This is especially if patients have symptoms of lethargy.
Sevelamer
Sevelamer is a phosphate binder, and we aren’t given information on calcium/phosphate status here,
but the PTH is within twice the upper limit of normal. Guidelines only recommend treating PTH when it
is above twice the upper limit of normal because of the risk of adynamic bone disease.
Atenolol
Atenolol would be useful to control blood pressure. However, the evidence is for use of ACE inhibitors
for the reduction of proteinuria and worsening of creatinine in IgA nephropathy.
Prednisolone and azathioprine
Because glomerulosclerosis is established, aggressive immunosuppressive therapy is less likely than

improved blood pressure control to have a significant impact on his prognosis. Prednisolone and
azathioprine may be considered if there are crescents in the biopsy or if there is active disease with
rapidly worsening renal impairment.
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A.107.A 53-year-old woman who has type II diabetes mellitus presents to the Emergency Department
(ED). She has vomiting and malaise. She is known to have stage 4 chronic kidney disease (CKD). Her
estimated glomerular filtration rate (eGFR) at her last General Practitioner (GP) appointment was 28
ml/min/1.73 m2. She is monitored annually by her GP to assess disease progression.
On examination, she has a body mass index (BMI) of 41 kg/m2. Respiratory examination is normal. You
note mild bilateral, symmetrical ankle oedema. Her blood pressure (BP) is 150/95 mmHg.
She is currently taking lisinopril 5 mg once daily, along with amlodipine 5 mg once daily, metformin 1000
mg twice daily, atorvastatin 10 mg at night and aspirin 75 mg once daily. The remainder of her
An arterial blood gas reveals the following:
pH 7.21 7.35–7.45
p(CO2) 3.9 kPa 4.6 – 6.4 kPa
p(O2) 11.5 mmHg 11.0 - 14.5 kPa
Bicarbonate (HCO3−) 15 mU/l 22–28 mU/l
161
Chloride (Cl−) 98 mmol/l 96–106 mmol/l
Glucose 9.1 mmol/l < 7.1 mmol/l
Base balance −7 (base deficit)
Decompensated respiratory acidosis
Diabetic ketoacidosis (DKA)
Lactic acidosis
Pyloric stenosis
Renal tubular acidosis (RTA)
Explanation
Lactic acidosis
This patient demonstrates raised anion gap metabolic acidosis; therefore, elevated lactate is an
important differential diagnosis. She is at risk of lactic acidosis by virtue of her metformin in the context
of CKD stage 3b. It is important to stop metformin in patients with an eGFR of < 30 ml/min/1.73 m2.
In this patient with raised anion gap metabolic acidosis, DKA is an important differential. It is made less
likely, however, by the absence of starvation or medications, such as dapagliflozin or other sodium–
glucose co-transporter-2 (SGLT2) inhibitors.
Decompensated respiratory acidosis
This patient is acidotic. Her p(CO2) is low and p(O2) elevated, indicating that she is likely
hyperventilating in response to metabolic acidosis. Respiratory acidosis with an elevated p(CO2) would
likely present with hypoventilation and carbon dioxide narcosis, which is treated with bilevel positive
airway pressure (BiPAP).
Pyloric stenosis
Pyloric stenosis is typically an illness of childhood but can occur in adults. It is characterised by
hypochloremic metabolic alkalosis as a result of loss of hydrogen ions from persistent vomiting. This
patient is acidotic, making this an inaccurate diagnosis. Her vomiting and malaise are likely a secondary
response to her metabolic acidosis, rather than to the primary disorder.
Renal tubular acidosis (RTA)
162
In this patient, the anion gap is elevated: (Na+ + K+) − (HCO3− + Cl−) = 37.9. In RTA, the expected anion
gap is normal. There are three main types of RTA: distal (type 1 – hypokalaemic), proximal (type 2 –
hypokalaemic) and type 4 (hyperkalaemic). In distal RTA, nephrocalcinosis occurs and the pathology is
characterised by an inability to secrete hydrogen ions. In proximal RTA, there is failure to reabsorb
HCO3−. Proximal tubular cellular failure can also present with loss of phosphate and is known as Fanconi
syndrome.
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A.108.A 23-year-old woman presents to the surgery after an upper respiratory tract infection and sore
throat complaining of passing brown urine, she became ill with the chest cold about 5 days ago. She tells
you that she has suffered from blood in the urine on a previous occasion after suffering from a sore
throat.
On examination, her BP is slightly elevated at 139/87 mmHg. Otherwise, physical examination is

unremarkable.
Platelets (PLT) 145 × 109/l 150–400 × 109/l
Urine blood ++, protein +
Which of the following investigations is most likely to confirm the diagnosis?
Urine culture
Serum IgA levels
ASOT
Renal biopsy
Cystoscopy
Explanation
Renal biopsy
163
Recurrent haematuria, occurring in close proximity to upper respiratory tract infection, is highly
suggestive of IgA nephropathy. Renal biopsy is the investigation of choice to confirm the diagnosis, with
usual findings including diffuse mesangial proliferation and extracellular matrix expansion.
Immunofluorescence is used to visualise IgA deposits, and C3 is also often seen. Aggressive control of
hypertension, with ACE inhibition as the main therapeutic option and management of rising creatinine
or proteinuria with the use of corticosteroids, form the backbone of therapy.
Serum IgA levels
It would seem reasonable to measure IgA levels, as these are usually elevated in IgA nephropathy, but
raised IgA levels are not specific for the condition of IgA nephropathy.
Urine culture
Urine culture would diagnose urinary tract infection. However there is no history of dysuria, frequency
or hesitancy of urination. The history of sore throat would not be consistent with a diagnosis of urinary
tract infection.
ASOT
ASOT would be more likely to be positive in post-streptococcal glomerulonephritis. This would also give
haematuria. The disorder develops 1-2 weeks after an untreated throat or skin infection. It most often
occurs in children aged 6-10 years old. Treatment is symptomatic relief, which would include antibiotics
and anti-hypertensives to control blood pressure. Corticosteroids and anti-inflammatories are generally
not effective.
Cystoscopy
Cystoscopy would be useful in excluding bladder cancer. However, in young women without any other
significant medical history such as occupational exposure to dye, etc. it is unlikely that haematuria is due
to bladder cancer.
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A.109.An 18-year-old man is referred to the Nephrology Clinic by his General Practitioner (GP) with high
blood pressure (BP). He tells you that he has been having frequent headaches for the last two years,
which he has been managing with oral paracetamol. He lives at home with his mother who is on
treatment with two agents for hypertension – amlodipine and lisinopril. His GP has initiated lisinopril but
is concerned that there is an underlying cause for his hypertension and has referred him for a second
opinion.
On examination, he appears to be a healthy young man. His BP is recorded at 160/110 mmHg.
Investigations:
164
Sodium (Na+) 145 mmol/l 135–145 mmol/l`
Renin 5.4 mU/l Low
Aldosterone 120 pmol/l Low/normal
pH 7.51 7.35–7.45
What is the most likely underlying pathological process?
Epithelial sodium channel (ENaC) loss-of-function mutation
ENaC gain-of-function mutation
Sodium–chloride (Na-Cl) symporter mutation
Sodium–potassium–chloride (Na-K-2Cl) channel mutation
Explanation
ENaC gain-of-function mutation
This channel acts to increase the uptake of Na+ from the collecting tubules in exchange for K+. A
mutation in the ubiquitin gene results in loss of ability to degrade this channel naturally and as such, a
gain-of-function mutation results in overactivity. Consequently, these patients present with metabolic
alkalosis, hypoaldosteronism, hypokalaemia and hypertension. It is called Liddle syndrome and is
treated with amiloride.
Sodium–potassium–chloride (Na-K-2Cl) channel mutation
Disorders of this channel occur in the thick ascending limb of the loop of Henle. Genetic mutations
which affect this channel result in hypotension, failure to thrive, metabolic alkalosis and hypokalaemia
with hypotension. It is often diagnosed in children and is known as Bartter syndrome.
Epithelial sodium channel (ENaC) loss-of-function mutation
The ENaC functions by receiving Na+ from the lumen of the collecting tubule. It works in tandem with a
sodium/potassium (Na/K) ATPase enzyme to move Na+ from the lumen into the interstitial space. A loss-
of-function mutation would result in loss of Na+ in the urine and the retaining of K+ rather the changes
seen in Liddle syndrome here.
165
Renal artery stenosis can occur in the older population as a consequence of atherosclerotic disease and
among young people secondary to disorders such as fibromuscular dysplasia, which appears as ‘beaded’
narrowing of the renal arteries. Classically, this results in secondary hyperaldosteronism, hypertension
and occasionally hypocalcaemic metabolic alkalosis. Evidence suggests that stenting these stenotic renal
arteries does not improve outcome secondary to standard medical care. However, this practice is still
performed in patients with flash pulmonary oedema, as this group was not included in the initial trial
(ASTRAL).
Sodium–chloride (Na-Cl) symporter mutation
Disorders of this channel that occur in the distal convoluted tubule result in hypotension, metabolic
alkalosis, hypokalaemia and classically hypomagnesaemia and hypercalcaemia. It is often milder than
Bartter syndrome and is diagnosed later in life. It is known as Gitelman syndrome.
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A.110.A 38-year-old man is referred to the Acute Medical Unit by his General Practitioner after
complaining of reduced frequency and volume of urine. Two days earlier, when he had attended with a
sore throat, cough, lethargy and fever, he had been prescribed cefalexin for a respiratory tract infection.
On examination, his urine output in the hospital is only 5–10 ml in the past two hours, and his
temperature is 38.3 °C. He has an erythematous oropharynx with tonsillar exudates.
White cell count (WCC) 14 × 109/l 4.0–11.0 × 109/l
Platelets (PLT) 220 × 109/l 150-400 × 109/l
Antineutrophil cytoplasmic antibodies (ANCA) Peripheral ANCA (p-ANCA) positive
Streptococcal antigen Negative
Urinalysis Red cell casts and dysmorphic red blood cells
What is the most appropriate next step?
Administer broad-spectrum antibiotics
Administer IV penicillin
Give a low dose of IV methylprednisolone
Give a high dose of IV methylprednisolone and cyclophosphamide
Prescribe a high dose of ciclosporin
166
Explanation
Give a high dose of IV methylprednisolone and cyclophosphamide
This is rapidly progressive glomerulonephritis (RPGN), and the blood picture is suggestive of microscopic
polyangiitis with granulomatosis. In adults, in whom the outcome is much worse, early initiation of high-
dose steroids has been associated with improved mortality and a more rapid return of kidney function.
RPGN is a disease of the kidney that results in a rapid decrease in the glomerular filtration rate of at
least 50% over a short period (from a few days to three months). The main pathological finding is
fibrinoid necrosis (over 90% of biopsy specimens); extensive crescent formation is present in at least
50% of glomeruli. This type of glomerulonephritis is also known as crescentic glomerulonephritis, after
the ‘crescents’ seen on light microscopy. These crescents are collections of epithelial cells and
macrophages within Bowman’s space.
The presence of crescents indicates that glomerular damage is rapid and progressive (from onset to
chronic kidney disease within weeks to months). Immunofluorescence detects deposits of
immunoglobulin G (IgG) or complement factor C3 in the glomerular basement membrane. There are
three distributions of immunofluorescence in crescentic glomerulonephritis:
absent
granular
linear.
In the UK, the frequency is estimated at two cases per 100 000 persons. In Sweden, the frequency is
estimated at one case per 100 000 persons. Massive pulmonary haemorrhage is the most common
cause of death in patients presenting with antineutrophil cytoplasmic antibodies (ANCA)-associated
disease. However, once immunosuppressive therapy has begun, infection is more common. The two
major patterns of ANCA are:
proteinase 3 – PR3-ANCA (formerly cytoplasmic or c-ANCA): positive in 90% of cases of granulomatosis

with polyangiitis
myeloperoxidase – MPO-ANCA (formerly p-ANCA): positive in Churg–Strauss syndrome and rarely

reported as positive in polyarteritis nodosa (PAN).
With respect to symptoms and signs of kidney disease, there may be loin pain, haematuria and systemic
symptoms (fever, malaise, myalgia, weight loss). Renal biopsy specimens show a diffuse, proliferative,
necrotising glomerulonephritis with crescent formation. High-dose corticosteroids and
cyclophosphamide and/or plasma exchange/renal transplantation are the treatments. The prognosis is
poor if initial creatinine levels are higher than 600 µmol/l.
Give a low dose of IV methylprednisolone
While methylprednisolone is part of the treatment, cyclophosphamide is required as part of the

immunosuppressive regimen.
Administer broad-spectrum antibiotics
167
Broad-spectrum antibiotics are only needed if there is an active infection. They do not treat
glomerulonephritis, which is suggested by the urinalysis revealing dysmorphic red blood cells. If this was
pyelonephritis, then broad-spectrum antibiotics would be appropriate, although we would expect to
find abdominal pain and potentially nitrates on urinalysis.
Administer IV penicillin
Penicillin would be indicated in pyelonephritis, where the presentation would include blood and protein
in the urine, fever and deranged renal function. Here, the absence of dysmorphic red blood cells in the
urine makes progressive glomerulonephritis (RPGN) secondary to microscopic polyangiitis with
granulomatosis more likely.
Prescribe a high dose of ciclosporin
Ciclosporin is not the immunosuppressant of choice initially. Ciclosporin may be used as maintenance
immunotherapy for membranous or minimal change glomerulopathy.
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A.111.A 45-year-old woman presents to the Renal Clinic with increasingly severe peripheral oedema,
which has developed over the past few months. She was diagnosed around a year earlier with arthritis
affecting her hips, knees, ankles and elbows, but has no other past medical history of note.
Examination reveals a blood pressure of 149/92 mmHg and a pulse of 80 bpm and regular. There is
pitting oedema affecting both lower limbs.
Investigations:
White cell count (WCC) 3.4 × 109/l (lymphocytes 0.7 x109/l) 4–11 × 109/l
Platelets (PLT) 167 × 109/l 150–400 × 109/l
Urine: Protein +++
You notice a white covering over her tongue.
Chronic interstitial nephritis
168
Human immunodeficiency virus (HIV) associated nephropathy
Membranous nephropathy Minimal change disease Renal amyloidosis
Explanation
Human immunodeficiency virus (HIV) associated nephropathy
There a number of clues that point to HIV-associated nephropathy as the underlying diagnosis. These
include oral candida, the presence of lymphopenia, the history of arthritis (joint pain, arthralgia or frank
arthritis may be present in up to 25% of patients with HIV) and peripheral oedema and low albumin
which is suggestive of clinically significant proteinuria. The key next step is to confirm the diagnosis of
HIV and consider intervening with highly active antiretroviral therapy (HAART), which has been shown to
significantly reduce progression of HIV nephropathy.
Image source: https://commons.wikimedia.org/wiki/File:CandidiasisFromCDCinJPEG03-18-06.JPG
Renal amyloidosis
Inflammatory disease associated with the underlying arthritis here could lead to renal amyloidosis,
although the short duration of symptoms makes this an unlikely cause of the renal disease seen here.
Chronic interstitial nephritis
Chronic interstitial nephritis is associated with haematuria, not seen here. A precipitant, such as long-
term non-steroidal anti-inflammatory drug (NSAID) use, is also usually identified and is not reported
here as part of the scenario.
This would be a reasonable alternative cause of marked proteinuria, although the evidence of
immunosuppression counts against idiopathic membranous nephropathy as the diagnosis and there is
no evidence of underlying malignancy as a cause of secondary membranous disease.
Minimal change disease is associated with episodes of proteinuria and oedema, usually presenting in
childhood or teenage years. It responds well to intervention with corticosteroids.
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A.112.A 40-year-old woman attended Accident and Emergency Department. She is on established
peritoneal dialysis (PD) and has presented with generalised abdominal pain, vomiting and pyrexia. She
has a past medical history of hypertension.
On questioning, she says her menstruation is erratic and she has not had a menstrual period for several
months now. Furthermore, she says she has not passed any urine for a few days.
On physical examination, she has a temperature of 38 °C, heart rate 100 bpm and a blood pressure of
140/80 mmHg. Her abdomen is distended and there is generalised tenderness with no guarding or
rigidity.
169
Platelets (PLT) 560 × 109/l 150 - 400 × 109/l
What is the likely cause of her abdominal pain?
Appendicitis
Ectopic pregnancy
Menstruation
Peritonitis
UTI
Explanation
Peritonitis
Patients on peritoneal dialysis (PD) are at risk of peritonitis. Enquiry should be made about the colour of
the dialysate. A cloudy dialysate would suggest that the possibility of an underlying infection.
Microscopy, culture and sensitivity (MC+S) of the fluid should be requested, in addition to the standard
septic screen. Many protocols exist for treatment of PD peritonitis; it largely depends on local
experience and prevalence of different strains of bacterium. The International Society of Peritoneal
Dialysis recommends the following, as a guide: for Gram-positive cover use vancomycin or a
cephalosporin, and for Gram-negative cover use an aminoglycoside or third-generation cephalosporin.
For example, vancomycin 2 g intraperitoneally on day 1 with a further dose a week later depending on
vancomycin levels. In addition, gentamicin 0.6 mg/kg intraperitoneally with adjustment of levels on days
3–5.
Appendicitis
Appendicitis would give localised right lower quadrant pain.
Ectopic pregnancy
170
Patients in chronic kidney disease are unlikely to either pass urine or menstruate. The rate of conception
for pregnancy is lower in patients with end-stage renal disease compared with the general population.
Pain associated with ectopic pregnancy would be unilateral rather than generalised abdominal pain.
Menstruation
Menstruation is commonly erratic in patients with renal disease – irregular or cessation of menstruation
is common.
UTI
Patients who have chronic kidney disease may not pass any urine if there is lack of residual renal
function.
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A.113.An 18-year-old woman presents with ankle swelling and facial puffiness. Two weeks previously,
she had a flu-like illness with a productive cough. Her father reports that she had suffered a similar
episode of swelling two years ago, but that things had returned to normal after a period of treatment.
At this time, urea and electrolytes (U&E) had been normal and her father does not recall her having had
a rash.
On examination, there was no rash or fever. She had pitting oedema to the waist.
Initial investigations were as follows:
Creatinine (Cr) 60 μmol/l 50 - 120 µmol/
24h urine collection 3.4 g protein
Given the likely diagnosis, what is the best initial treatment?
Antibiotics
Corticosteroids (high dose, 1 mg/kg)
Cyclophosphamide
Loop diuretics and an albumin infusion
171
Nothing, observation only
Explanation
Corticosteroids (high dose, 1 mg/kg)
Nephrotic syndrome is defined by urinary protein loss of > 3.5 g of protein/24 h (0.05 g/kg per 24 h in
children), accompanied by hypoalbuminaemia (75% of cases).
Upper respiratory tract infection (URTI) commonly precedes attacks, although a definite causal
relationship is debated – the most common bacterial antigens implicated are streptococcal.
Minimal change disease has a good prognosis in childhood; a third have just one episode and a third
have infrequent relapses, while a third have frequent relapses throughout childhood that stop in adult
life.
Steroids are an effective treatment for minimal change disease, and as this is, by far, the most common
cause of childhood nephrotic syndrome, a trial of high-dose steroids is accepted practice, with only
those patients who do not respond being investigated for other renal disease.
Antibiotics
There is no role for prophylactic antibiotics; however, treatment may be required for infections.
Cyclophosphamide
There is no evidence for cyclophosphamide as primary treatment for minimal change disease.
Cyclophosphamide is used as one of the standard treatments for vasculitis, as part of the CYCLOPS
regimen, which includes methylprednisolone and monthly cyclophosphamide.
Loop diuretics and an albumin infusion
Loop diuretics may be required for fluid overload; however, it is not the treatment for the underlying
disorder. Loop diuretics are useful for fluid management and may also be used to lower blood pressure.
However, they are not a curative treatment.
Nothing, observation only
Minimal change disease responds well in general to steroid treatment; therefore, it is not appropriate to
do nothing and observe.
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A.114.A 42-year-old man presents with generalised oedema. He has a background history of intravenous
drug use and excess alcohol intake. He smokes 20 cigarettes per day. He suffers from depression and
mild asthma and is currently on fluoxetine, and has progressively lost weight and energy over the past
few weeks and months.
On physical examination, his blood pressure (BP) is 150/70 mmHg, chest and abdominal examination is
unremarkable. There is bilateral peripheral leg oedema. BMI is 25.
Initial investigations reveal:
172
Investigations Result Normal Values
24-hour urinary protein collection 3.6 g/24 h < 0.2 g/24 h
Renal biopsy Focal segmental glomerulosclerosis
In this patient what is the most likely association with this disease?
Alcohol abuse
Cigarette smoking
Hepatitis A
Hepatitis C
HIV
Explanation
HIV
FSGS can present with nephrotic syndrome. It has been associated with HIV, hepatitis B, intravenous
heroin use and massive obesity. The outcome varies and some patients will progress to end-stage renal
disease.
With nephrotic syndrome, he is at risk of thromboembolism, infection (especially pneumococcus) and

hyperlipidaemia. In HIV-related FSGS, highly active retroviral therapy (HAART) is associated with a
reduction in proteinuria.
Other measures include following a low-salt diet and use of ACE inhibitors. Where heroin addiction
persists, discontinuation of heroin may also result in a reduction in proteinuria.
Hepatitis C
There is a strong and likely causal association between chronic hepatitis C virus (HCV) infection and
glomerular disease. Several types of renal disease have been recognised, including mixed
cryoglobulinaemia, membranoproliferative glomerulonephritis (MPGN), membranous nephropathy, and
polyarteritis nodosa (PAN). Less commonly, other glomerular lesions have been reported in HCV-
infected patients, including proliferative glomerulonephritis, and fibrillary and immunotactoid
glomerulopathies.
173
Alcohol abuse
Chronic alcohol abuse increases the risk of acute kidney disease in unobstructed acute pyelonephritis
and is a rare cause of renal papillary necrosis. However, alcohol abuse is not associated with focal
segmental glomerulosclerosis (FSGS).
Cigarette smoking
Cigarette smoking is not associated with FSGS, but studies have shown that cigarette smoking is a risk
factor for the development and progression of chronic kidney disease in the community. Many studies
also indicate a relationship between smoking and renal function deterioration in lupus patients,
polycystic kidney disease, Goodpasture syndrome, renal artery stenosis, glomerulonephritis and
proximal tubular dysfunction. However, the mechanism is not always clear.
Hepatitis A
Hepatitis A runs a benign course in children, but may have atypical presentations in adults. Very rarely,
acute kidney disease complicates non-fulminant hepatitis A. Hepatitis A is associated with acute kidney
disease, but not FSGS.
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A.115.A 45-year-old man from Ghana underwent a live-related renal transplant three weeks ago for
chronic kidney disease due to hypertensive nephropathy.
His current medications are ciclosporin, mycophenolate mofetil, prednisolone, cotrimoxazole, ranitidine,
amlodipine and atenolol.
There were no complications after the procedure and his creatinine normalised a few days after surgery.
He has no symptoms. Two days ago, his serum creatinine was 155 µmol/l.
Investigations:
What definitive investigation will confirm that acute rejection is the problem?
24 h urine with creatinine estimation
Ciclosporin level
Renal biopsy
174
Serum immunoglobulin
Explanation
Renal biopsy
Acute rejection develops in 30–50% of renal transplant recipients. The risk is greater in the first three
weeks after transplant. In patients on ciclosporin there are often no symptoms. Patients with
deteriorating renal function with normal ciclosporin level should be investigated by percutaneous renal
biopsy.
24 h urine with creatinine estimation
Creatinine estimation would not help in this situation because a rise in serum creatinine is a good
enough indication that there may be abnormality in renal function. Urinary creatinine would only be
helpful in conditions such as alcoholism or extremes in weight.
Ciclosporin level
A high ciclosporin level may affect creatinine levels by causing acute toxicity. However, this will not
determine whether there is any acute rejection.
Renal ultrasound scan will identify any obstruction or possible urinary leak post-transplant. It cannot
diagnose acute rejection.
Serum immunoglobulin
There are no characteristic changes in serum immunoglobulins that are associated with rejection.
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A.116.A 71-year-old woman is admitted with general deterioration and ‘off-legs’. She has no significant
past medical history and lives alone.
On examination in the Emergency Department, she looks dry and her skin has a yellow tinge. Blood
pressure is 125/50 mmHg, chest examination has a few scattered bibasal crepitations at bases and heart
sounds are normal. Abdominal examination is unremarkable. There are peaked T waves on the 12-lead
ECG.
She is immediately put on a cardiac monitor.
175
Which one of the following measures is next most important in her immediate management?
Calcium resonium
Intravenous insulin and dextrose infusion
Intravenous saline infusion
Salbutamol nebuliser
Explanation
This patient has markedly raised serum potassium and is likely to have significant cardiotoxicity. Signs
include peaked T waves, PR interval prolongation and QRS widening. Calcium gluconate stabilises nerve
and muscle membrane excitation and should be considered the first-line therapy in this situation.
Next, immediate measures likely to result in reduction of serum potassium include nebulised salbutamol
and insulin and dextrose infusion. After this, it is important to establish fluid status via catheterisation
and measurement of urine output, and consideration of line placement to measure central venous
pressure. Urgent renal ultrasound may also be useful at this stage to establish pointers towards the
aetiology of kidney disease.
Calcium resonium
Calcium resonium acts by inhibiting absorption of potassium by the gut. The side-effect includes
constipation. It is slow acting and therefore would not be the immediate first treatment choice.
Intravenous insulin and dextrose infusion
Intravenous (iv) insulin and dextrose infusion would be the next treatment step after iv calcium
gluconate. The insulin and dextrose act to push potassium into the intracellular compartment thereby
reducing serum potassium levels. This is a temporary procedure as the potassium needs to be excreted
or removed via dialysis.
Intravenous saline infusion
Saline will not affect the potassium level, although it may help uraemia and dehydration to improve fluid
status. This would not be the immediate first treatment.
Salbutamol nebuliser
After intravenous calcium gluconate, the next immediate measures will include nebulised salbutamol
which will act to push potassium intracellularly. It is important to note that this is a short-term measure
if used on its own.
*****************************************************************************
176
A.117.A 69-year-old man with hypertension comes to the Emergency Department complaining of
dysuria, flank pain, fever and chills for 2 days. He takes metoprolol and hydrochlorothiazide for his
hypertension.
Vital signs are temperature 39.3 °C, blood pressure 100/60 mmHg, pulse 120/min, respiration 28/min
and oxygen saturation 98% on room air. He has mild tenderness at the left costovertebral angle. The
rest of the physical examination is normal.
The chest X-ray is normal and urine dipstick positive for blood, protein and nitrites. Blood and urine are
sent for analysis and cultures.
What is the most appropriate next step in management?
Call for a urological consultation
Insert a Foley catheter
Obtain an ultrasound of the kidneys
Start a dopamine infusion
Start intravenous antibiotic therapy
Explanation
Start intravenous antibiotic therapy
The patient is experiencing signs and symptoms of Gram-negative sepsis stemming from a urinary tract
infection. The most important intervention is early antibiotic therapy. The choice of antibiotic depends
on history and Gram stain of the urine. The most common pathogens are Gram-negative rods (E. coli),
so initial antibiotic coverage should include Gram-negative coverage and be adjusted depending on the
culture sensitivity. Urinary tract infection in men is less common than in women, due to the anatomical
differences, and is usually caused by obstruction because of benign prostatic hyperplasia (BPH),
especially in elderly men.
In today's office practice, the dipstick test for nitrite is used as a surrogate marker for bacteriuria. It
should be noted that not all uropathogens reduce nitrates to nitrite. For example, enterococci, S.
saprophyticus and Acinetobacter species do not and therefore give false-negative results.
Call for a urological consultation
Urological consultation may be useful in recurrent urinary tract infections or obstructive uropathy, but
urological consult would depend on what ultrasound shows so is not the most appropriate next step.
Insert a Foley catheter
Insertion of a Foley catheter is only useful if there is suspicion of obstructive uropathy, such as that
caused by benign prostatic hypertrophy.
Obtain an ultrasound of the kidneys
177
In this scenario, antibiotics need to be started first and an ultrasound would be needed, but that is not
the initial step. It would be useful for detecting hydronephrosis.
Start a dopamine infusion
Dopamine infusion has no role to play in urinary tract infection treatment. Dopamine may be used to
support renal blood flow in patients with persistent hypotension and poor urine output.
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A.118.A 42-year-old man has completed 2 years of haemodialysis and is suffering increasing symptoms
of tiredness and lethargy. His past medical history includes hypertension and type 2 diabetes mellitus.
He is frustrated with waiting for a cadaveric transplant and wants to discuss options for securing a new
kidney.
On physical examination, his blood pressure is 140/80 mmHg; chest and abdominal examination is
unremarkable.
Platelets (PLT) 167 × 109/l 150–400 × 109/l
Ejection fraction 48%.
Which of the following is the best advice?
Convince him to take his willing 13-year-old son's kidney
Suggest that he will have to wait his turn on cadaveric transplant list
His wife is highly likely to be a match and should be approached
Refer for urgent cadaveric transplant
Urge him to contact an overseas supplier to source a kidney
Explanation
Suggest that he will have to wait his turn on cadaveric transplant list
178
Currently he should continue to wait his turn on the cadaveric list, although it should be possible to
review the possibility of a transplant from his son in 2 or 3 years time. Clearly should his medical status
to deteriorate, then he could be moved to the urgent cadaveric transplant list.
Convince him to take his willing 13-year-old son's kidney
It’s inappropriate for this man to receive a kidney from his 13-year-old son, and his symptoms do not
currently suggest that he requires urgent transplantation. Potential indications for urgent
transplantation will include lack of dialysis access, severe psychological problems, severe complications
of haemodialysis (severe hypotension during treatment) and uraemic polyneuropathy.
His wife is highly likely to be a match and should be approached
A genetically related first-degree relative is more likely to be a match compared to his wife.
Refer for urgent cadaveric transplant
As explained previously, this gentleman has no indication at present for urgent renal transplantation.
Potential indications for urgent transplantation will include lack of dialysis access, severe psychological
problems, severe complications of haemodialysis (severe hypotension during treatment) and uraemic
polyneuropathy.
Urge him to contact an overseas supplier to source a kidney
This man does not have any urgent need for renal transplantation. It is preferable for a living donation
from a closely related first-degree relative because they are more likely to share the same tissue type
and blood group as the recipient.
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A.119.A 23-year-old woman presents with a 3-week history of increasing swelling of her ankles. There
are no preceding symptoms and there is no past medical or drug history of note. She then develops
hand and facial swelling and finds her trousers increasingly tight around the waist. She has no rash, joint
pains or other symptoms.
On examination, her blood pressure is 160/70 mmHg, she has gross pitting oedema of both legs, ascites
and facial, hand and sacral oedema.
White Cell Count (WCC) 5.1 × 109/l 4.0–11.0 × 109/l
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Platelets (PLT) 243 × 109 /l 150–400 × 109/l
Glucose 4 mmol/l 3.5–5.5 mmol/l
Cholesterol 10 mmol/l < 5.2 mmol/l
24-hour urine collection 4.2 g/24 hours proteinuria
Chest X-ray (CXR) Small bilateral pleural effusions
Urine dipstick 4+ proteinuria and no blood
Post-infectious glomerulonephritis
IgA nephropathy
Explanation
This woman has nephrotic syndrome (oedema, hypoalbuminemia, hypercholesterolaemia and more
than 3.5g/24-hours proteinuria). The commonest cause in young people is minimal change disease. A
renal biopsy would be required to confirm the diagnosis of minimal change disease and the treatment
would be steroids, although in practice response to steroids may obviate the need for biopsy.
Post-infectious glomerulonephritis
This is more likely to present as a nephritic illness with hypertension and haematuria, as well as
proteinuria, oedema and renal impairment.
This will more likely present with nephritic syndrome with associated haematuria with proteinuria. By
definition there will also be pulmonary haemorrhage.
IgA nephropathy
IgA nephropathy rarely causes nephrotic syndrome and usually has haematuria as well as proteinuria.
Diabetic nephropathy can present as nephrotic syndrome but only in long-standing diabetics and this girl
has no past medical history and normal serum glucose.
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A.120.A 19-year-old man presents to the clinic with raised BP of 152/82 mmHg. He has been previously
fit and well as a child, with no developmental problems.
On examination, apart from the raised blood pressure, his chest and abdominal examination is
unremarkable. You are concerned that he may have serious underlying pathology and arrange a series
of tests.
Platelets (PLT) 204 × 109/l 150–400 × 109/l
Aldosterone erect 150 pmol/l 200-1000 pmol/l
Renin erect 2.2 pmol/ml/hr 2.8-4.5 pmol/ml/hr
Bartter syndrome
Gitelman syndrome
Conn syndrome
Liddle syndrome
Explanation
Liddle syndrome
This man has hypertension presenting at a young age with profound hypokalaemia, alkalosis and
suppressed renin and aldosterone. This fits with the picture of Liddle syndrome, a rare autosomal
dominant condition causing a mutation in the highly selective epithelial sodium channel in the distal
nephron. This results in sodium retention independent of mineralocorticoid activity, and hence the
clinical picture seen here.
181
Renal artery stenosis would be associated with elevated renin and aldosterone levels. There may also be
a renal bruit on abdominal examination.
Bartter syndrome
Bartter syndrome is associated with normo-tension or slightly reduced blood pressure. Bartter
syndrome is an autosomal recessive inheritance, usually diagnosed in children or adolescents with
failure to thrive, polydipsia, polyuria and cramps. There is hypokalaemia, mild metabolic alkalosis,
increased urinary sodium, potassium and calcium with increased serum renin and aldosterone.
Gitelman syndrome
Gitelman syndrome is associated with normo-tension or slightly reduced blood pressure. This is an
autosomal recessive inheritance. It is usually diagnosed in young adults who are asymptomatic with low
serum potassium (2.0-3.0 mmol/l), reduced magnesium with mild metabolic alkalosis and increased
renin and aldosterone. Urinary calcium is low, unlike Barrter syndrome.
Conn syndrome
Conn syndrome is associated with elevated aldosterone. Patients with Conn syndrome often have few or
no symptoms, but some may get occasional muscle weakness, muscle spasms, paraesthesiae or
polyuria. High blood pressure and muscle cramps occur due to low serum potassium.
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A.121.A 39-year-old man, with a history of treated hypertension treated with ramipril 10 mg daily,
comes to see the GP complaining of tiredness and lethargy. He runs some routine bloods, which are
given below:
Platelets (PLT) 170 × 109/l 150–400 × 109/l
Renal tubular acidosis type I
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Renal tubular acidosis type II
Renal tubular acidosis type IV
Addison’s disease
Conn syndrome
Explanation
Type IV RTA, also known as hyperkalaemic RTA occurs when the kidney fails to respond to aldosterone.
This leads to hyperkalaemic metabolic acidosis. Pharmacological causes of type IV RTA include
spironolactone, ACE inhibitors and ARBS, trimethoprim, heparin, pentamidine and NSAIDs. Structural
kidney diseases such as SLE and amyloidosis can also affect response to aldosterone and lead to type IV
RTA. Where there is a causative agent that can be discontinued, alternative therapy should be provided,
otherwise patients may be offered bicarbonate supplements and agents to lower serum potassium.
Conn syndrome
Conn syndrome is associated with elevated aldosterone and therefore the serum potassium would be
low rather than high in this case. Patients with Conn syndrome often have few or no symptoms, but
some may get occasional muscle weakness, muscle spasms, paraesthesiae or polyuria. High blood
pressure and muscle cramps occur due to low serum calcium.
There is disordered excretion of acid (H+) in this condition from the collecting ducts, resulting in
acidosis. Therefore the clinical picture will be a hyperchloraemic, hypokalaemic metabolic acidosis with
hypophosphataemic metabolic bone disease, renal stones or diffuse nephrocalcinosis. It is rarely
inherited, or it may be secondary to conditions such as chronic tubular-interstitial disease and
dysproteinaemias.
There is impaired bicarbonate retention in the proximal tubule leading to bicarbonate wasting and a
systemic acidosis. This will present with a hyperchloraemic metabolic acidosis, with other features of
proximal tubular dysfunction such as phosphate wasting and reduced serum urate. There would be
glycosuria and proteinuria with loss of amino acids.
Addison’s disease
Patients with Addison’s disease have low blood pressure rather than hypertension. There may be
hyperpigmentation of the skin, even in areas that are not sun-exposed. Characteristic areas of darkening
include skin creases, nipple and buccal mucosa. This is because melanocyte-stimulating hormone and
ACTH share the same precursor (pro-opiomelanocortin). Investigations review a low sodium, high
potassium, high calcium and low glucose.
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A.122.A 19-year-old student from England is diagnosed with malaria following a 2-week history of
headaches, cyclical fever, fatigue and muscle pains whilst on a gap year break in Zambia. He has no
previous medical history to note and is usually fit and well.
The patient is admitted to hospital started on oral chloroquine, however his condition deteriorates over
the next day. It is noted by nursing staff that his urine output has dropped significantly despite IV fluid
therapy.
His blood results are as follows:
Platelets (PLT) 213 × 109 /l 150 - 400 × 109/l
Blood film +++ for ring forms
The patient is catheterised and only 50 ml of dark urine is drained. He is given treatment to address his
hyperkalaemia.
What of the following is the most appropriate next step in the management of this patient?
Arrange a blood transfusion
Peritoneal dialysis
Repeat the blood film
Start intravenous artesunate
Explanation
Start intravenous artesunate
This patient has severe malaria with an associated acute kidney injury (AKI). Renal impairment is
common among adults with severe falciparum infection; Renal impairment is secondary to disease in
glomeruli, tubules and in the interstitial region. Kidney disease in malaria is usually secondary to
abnormality of erythrocytes
184
Clinical manifestations of malaria-associated AKI include oligo-anuria, severe metabolic acidosis and a
hypercatabolic state.
Severe malaria with AKI is associated with a high mortality rate unless treatment is rapidly commenced.
The mainstay of treatment involves administering the appropriate antimalarial drugs parenterally,
alongside supportive measures such as IV fluids and dialysis to treat AKI. The antimalarial drug of choice
in severe malaria is artesunate, however quinine may be used if artesunate is unavailable. This patient’s
hyperkalaemia should also be addressed, for example with IV calcium.
Arrange a blood transfusion
There is no role for blood transfusion in malaria treatment unless there is co-existent symptomatic
anaemia.
Peritoneal dialysis
This patient has an acute kidney injury secondary to malaria, which can be reversed with malaria
treatment and supportive care including intravenous fluids. Acute dialysis may be required if there is
pulmonary oedema resistant to medical treatment, life-threatening hyperkalaemia, flash pulmonary
oedema, metabolic acidosis resistant to medical treatment, uraemic pericarditis or encephalopathy. If
dialysis is indicated, the preferred method would be haemodialysis not peritoneal dialysis. However, the
next most important step in this patient’s management would be administering antimalarial medication
via the parenteral route.
Repeat the blood film
A repeat blood film will confirm the diagnosis, but the next best step here is to initiate treatment.
In this scenario, the patient has not passed urine because he has an acute kidney injury secondary to
malaria infection; therefore, waiting to obtain a urine sample would not be the next most important
step in his management.
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A.123.A 54-year-old man presents with a 4-month history of intermittent dysuria, some pain on
ejaculation and intermittent problems passing urine. He was previously fit and well and has no
significant past medical history.
On examination, his blood pressure (BP) is 135/75 mmHg and general physical examination is
unremarkable. PR reveals a normal-sized prostate which is mildly tender and nodular to palpation.
Investigations:
Invetsigation Result Normal value
Platelet (PLT) 201 × 109/l 150–400 × 109/l
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Prostate specific antigen (PSA) 6 μg/l < 4 μg/l
Prostatic carcinoma Benign prostatic hypertrophy
Acute prostatitis
Chronic intermittent prostatitis Non-specific urethritis
Explanation
Chronic intermittent prostatitis
The nodularity with mild diffuse tenderness is consistent with chronic infection. Mild elevation in the
PSA is also consistent with a chronic prostatitis. A trial of antibiotics is required ± an alpha antagonist if
symptoms of obstruction are present.
Acute prostatitis
This does not usually cause intermittent symptoms over 4 months and there are usually more systemic
symptoms such as fever. Ejaculatory pain is more typical with chronic prostatitis rather than in the acute
presentation. Furthermore, one would expect the prostate to be acutely tender (rather than mildly
tender), hot, nodular or boggy on digital examination.
Prostatic carcinoma
This is unlikely given the intermittent (rather than progressive) nature of symptoms and only very mildly
elevated PSA. Prostate carcinoma should remain within the differential given the mild nodularity, but a
trial of antibiotics remains a priority followed by further investigations and repeat prostate assessment
at the end of treatment.
This is unlikely to be the diagnosis given the normal-sized prostate.
Non-specific urethritis
This does not usually present over a 4-month period. There is no mention of urethral discharge and the
mildly tender, nodular prostate is more consistent with prostatitis.
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A.124.A 34-year-old man with chronic kidney disease is seen in the Nephrology Clinic. He is
asymptomatic.
186
His general practioner prescribed a course of ferrous sulphate which he has taken diligently when his
haemoglobin was noted to be 86g/l three months ago. His current other medications include atenolol
100 mg once daily, ramipril 10 mg once daily, alfacalcidol 0.25 µg once daily, Calcichew® 1 tablet with
meals and a multivitamin preparation
Clinical Investigations:
Haemocrit (HCT) 0.3 0.45 - 0.54 (male)
Parathyroid hormone (PTH) 8.5 pmol/l 0.9 - 5.4 pmol/l
Ferritin 24 µg/l 24 - 355 ug/l (male)
What is the most appropriate next intervention for his anaemia?
Continue oral ferrous sulphate
Intravenous iron Intravenous erythropoietin Red cell transfusion Subcutaneous erythropoietin
Explanation
Intravenous iron
Anaemia is almost universal in patients with chronic kidney disease; it is characteristically normochromic
and normocytic and is associated with erythropoietin (Epo) deficiency and shortening of red cell survival.
This patient has failed a trial of oral iron (the target range for haemoglobin improvement is 100g/l to
120g/l) and intravenous iron is therefore the most appropriate next intervention. Oral iron absorption is
often defective in chronic kidney disease and NICE guidelines (see link below) recommend offering a
trial of oral iron to asymptomatic patients with non-dialysis dependent chronic kidney disease who are
not taking erythropoietin stimulating agents but stipulate this should be switched to intravenous iron if
there is a lack of sufficient response after 3 months.
Continue oral ferrous sulphate
This patient’s ferritin, and haemoglobin levels, have failed to correct with an appropriate trial of oral
iron therapy. This is likely due to defective oral iron absorption, continuing oral iron replacement is
highly unlikely to be effective for this patient. NICE guidelines (see link below) recommend offering a
trial of oral iron to asymptomatic patients with non-dialysis dependent chronic kidney disease who are
not taking erythropoietin stimulating agents but stipulate this should be switched to intravenous iron if
187
there is a lack of sufficient response after 3 months. The ideal response range for such a trial is a
haemoglobin of 100g/l to 120g/l.
Intravenous erythropoietin
This is only used in dialysis patients and given via the dialysis circuit.
Red cell transfusion
Red cell transfusions should be avoided unless in an emergency, as this patient will develop circulating
antibodies which would make future transplantation more difficult.
Subcutaneous erythropoietin
Eventually, patients are commenced on erythropoietin. Iron stores should be optimised before
beginning erythropoietin.
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A.125.A 45-year-old contract painter presents for review. He consulted the GP complaining of tiredness
and lethargy, abdominal pain and headaches. He works regularly on renovation jobs in old houses.
Only past history of note includes atenolol for hypertension and gout, which has become a recent
problem over the past few months.
On examination, his BP is 138/80 mmHg, with pulse 72 and regular. He looks pale and has a peripheral
neuropathy.
Investigations:
Haemoglobin (Hb) 91 g/l (hypochromic, microcytic anaemia) 135 - 175 g/l
Platelets (PLT) 173 × 109/l 150 - 400 × 109/l
Creatinine (Cr) 122 μmol/l 50 - 120 μmol/l
Uric acid 680 µmol/l
What diagnosis fits best with this clinical picture?
Acute interstitial nephritis
Lead poisoning Hypertensive nephropathy Pseudohypoaldosteronism Atenolol use
Explanation
188
Lead poisoning
The picture here, with hypochromic, microcytic anaemia and gout fits best with lead poisoning. Lead
nephropathy leads to proximal tubular defects including phosphate, amino acid and glucose loss in the
urine. Pencillamine is one effective treatment for lead poisoning, promoting increased lead excretion;
other options include dimercaptosuccinic acid (DMSA) and calcium disodium edetate. Prolonged low
level of lead exposure can also result in lead related nephrotoxicity which results in CKD and
hyporeninemic hypoaldosteronism which in turn can cause hyperkalaemia.
Typically, acute interstitial nephritis begins abruptly, manifesting as acute kidney injury. In most
instances, acute interstitial nephritis occurs within days of exposure to the offending drug. In some
instances (particularly with nonsteroidal anti-inflammatory drugs [NSAIDs]), acute interstitial nephritis
begins after several months of exposure. Almost all acute interstitial nephritides are caused by
hypersensitivity reactions to drugs and are not mediated by direct toxicity. Although any drug can
potentially cause a hypersensitivity reaction involving the kidney, the following agents are the most
frequently implicated:
Antibiotics (e.g. penicillins, cephalosporins, sulfa drugs, quinolones)
NSAIDs
Diuretics (e.g. thiazides, furosemide)
Allopurinol
Phenytoin
Rifampin
Interferon alfa
Proton pump inhibitors.
Lead poisoning is a cause of chronic interstitial nephritis.
Hypertension is almost always present in lead nephropathy, and, in the absence of appropriate testing
or careful exposure history, lead nephropathy is often misdiagnosed as so-called hypertensive kidney
disease. Patients with lead nephropathy tend to have disproportionately worse hyperuricaemia
compared to patients with other kidney diseases, because of the unique effects of lead on urate
metabolism, and, consequently, gout is common.
Pseudohypoaldosteronism
Pseudohypoaldosteronism comprises a heterogeneous group of disorders of electrolyte metabolism

characterised by an apparent state of renal tubular unresponsiveness or resistance to the action of
aldosterone. It is manifested by hyperkalaemia, metabolic acidosis and a normal glomerular filtration
rate (GFR). In our case, there is mild hypokalaemia and reduced
189
GFR; therefore, the diagnosis of pseudohypoaldosteronism is unlikely.
Atenolol use
Atenolol is unlikely to have caused the kidney disease; however, dose adjustment is required according
to the patient’s creatinine clearance.
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A.126.A 32-year-old vegan woman is reviewed in the General Medical Clinic. She presented to the acute
take with renal colic and passed grit/renal stone material a few hours after admission. Analysis of the
material suggested it comprised calcium oxalate. This latest episode was the third episode of renal colic
that had occurred over the past year. Her diet is rich in fruit, nuts and berries. Examination in the clinic
reveals a blood pressure of 108/72 mmHg and a pulse of 67 bpm and regular. Her body mass index
(BMI) is 22 kg/m2. Her abdomen is soft and non-tender and there are no abnormal masses.
Investigations;
Investigations Results Normal values
Platelets (PLT) 198 × 109/l 150–400 × 109/l
Glucose 5.0 mmol/l 3.5–5.5 mmol/l*
Which of the following is the most appropriate initial intervention?
Increased fluid intake Increased green vegetable intake Pyridoxine Thiamine Vitamin C
Explanation
Increased fluid intake
Urinary oxalate stones can be related to excess consumption of foods containing large amounts of
oxalate. Examples include spinach, rhubarb, nuts, beetroot, chocolate, wheat, bran and tea. Excessive
meat consumption can also drive increased oxalate absorption, but given this woman is a vegan, it
would not be considered a problem here. The initial management for the treatment of urinary oxalate
stones is to increase fluid.
Vitamin C
Vitamin C can increase urinary oxalate and should therefore be avoided.
190
Increased green vegetable intake
Given that this patient is a vegan, it is likely that her diet is already high in green vegetables.
Pyridoxine
Pyridoxine, magnesium and phosphate supplementation may all reduce the risk for stone formation but
should be trialled as a follow-on intervention to increasing fluid intake.
Thiamine
Thiamine can help to reduce the formation of oxalate-based kidney stones but should not be
recommended as a first-line therapy.
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A.127.A 62-year-old man with a known history of type 2 diabetes comes to the Emergency Department
having taken an overdose of an unknown quantity of tablets some 3 hours earlier.
He refuses to discuss what he took, but his last discharge suggests he is taking a number of medications
including metformin, gliclazide, ramipril, aspirin and atorvastatin.
You review his arterial blood gas results, which are shown below:
PO2 10.4 kPa > 10.5 kPa
PCO2 3.9 kPa 4.7 - 6.0 kPa
pH 7.34 7.35 - 7.45
Which of the following is the most likely explanation for the blood gas results?
Compensated metabolic acidosis Compensated respiratory alkalosis Compensated respiratory acidosis

Compensated metabolic alkalosis Normal blood gas result
Explanation
Compensated metabolic acidosis
This man’s bicarbonate is below the lower limit of normal, and he is blowing off CO2 to allow him to
maintain a normal pH. In this context, given that he is taking metformin, he may have overdosed on it
leading to his presentation with raised anion gap acidosis. His other results, including his chloride, could
be scrutinised to confirm this suspicion.
Compensated respiratory alkalosis
This is seen in the first few hours after aspirin overdose, and in this case is associated with vomiting and
tinnitus.
191
Compensated respiratory acidosis
This occurs in patients with chronic obstructive pulmonary disease (COPD), and is associated with an
elevated pCO2 and bicarbonate.
Compensated metabolic alkalosis
This is seen in persistent vomiting, although respiratory compensation is limited by a compulsion to

breathe and maintain adequate oxygenation.
Normal blood gas result
The bicarbonate is below the lower limit of normal, as is the PCO2.
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A.128.A 42-year-old man with severe acute left-sided flank pain is referred by his GP to Accident and
Emergency. This is the first time he has had such severe sudden pain. He has no past medical history of
cardiovascular or renal disease, his brother died of a subarachnoid haemorrhage.
On examination, his blood pressure is 170/100 mmHg, pulse 78 bpm and regular. His jugular venous
pressure (JVP) is not raised, heart sounds are normal and his chest is clear. His liver is not palpable but
he has a mass in the left flank. The mass is bimanually palpable and you can get above it.
Platelets (PLT) 243 × 109 /l 150 - 400 × 109/l
Erythrocyte sedimentation rate (ESR) 8 mm/h1 - 20 mm/h
Urine dipstick Blood +++, protein +
Which is the most appropriate investigation to request?
CT scan of the abdomen
192
MRI scan of the abdomen
Ultrasound of the abdomen
Urine microscopy
Explanation
Ultrasound of the abdomen
Ultrasound of the abdomen is easily performed, non-invasive and plays a critical diagnostic role. The
suspicion here would be one of polycystic kidney disease. There is an association with Berry aneurysm
which may well have resulted in the death of his brother. Aggressive management of hypertension is the
most appropriate way to prevent or at least slow the accelerated deterioration in renal function
associated with this condition.
CT scan of the abdomen
CT scan of the abdomen is incorrect. Although a CT scan of the abdomen would also show polycysts in
the kidneys, it would not be as easily performed compared with ultrasound. It would also expose the
patient to radiation.
Intravenous urogram (IVU) is incorrect. IVU may be normal in polycystic kidney disease. There would be
associated unnecessary contrast exposure.
MRI scan of the abdomen
MRI scan of the abdomen is incorrect. MRI compared with ultrasound abdomen is more difficult to
perform. There is nephrogenic systemic fibrosis with the use of contrast agent in MRI scans in patients
with kidney disease.
Urine microscopy
Urine microscopy is incorrect. The urine microscopy would typically be normal in polycystic kidney
disease unless there are associated complications such as bleeding in the cysts where there may be
haematuria.
A.129.A 62-year-old man with a long history of type II diabetes and chronic kidney disease comes to the
clinic complaining of tiredness. Current therapy includes Mixtard insulin, ramipril, amlodipine,
atorvastatin and aspirin.
On examination, his blood pressure is 130/80 mmHg, he looks pale and his chest and abdominal
193
Platelets (PLT) 201 × 109/l 150–400 × 109/l
Ferritin 260 μg/l 20–250 µg/l
B12and Folate within the normal range TSAT 23%
Which of the following is the most appropriate management for him?
Commence oral ferrous sulphate
Treat with EPO to a target Hb of 135 g/l
Arrange IV iron replacement
No current intervention, but plan for review in three months
Explanation
Further iron replacement will not improve his Hb levels further. A Cochrane meta-analysis suggested
that treating to the higher Hb target of 135 g/l may result in an increased risk of hypertensive crises.
European renal guidelines, therefore, recommend a lower target haemoglobin of 110 g/l.
Commence oral ferrous sulphate
This patient is iron-replete. The recommendation from the UK Renal Association is for the ferritin to be >
100mg/l and TSAT > 20% (or < 10% hypochromic red cells). Therefore, there is no need for further iron
replacement.
As explained above, a Cochrane meta-analysis suggested that treating to the higher Hb target of 135 g/l
may result in an increased risk of hypertensive crises. European renal guidelines, therefore, recommend
lower target haemoglobin of 110 g/l.
Arrange IV iron replacement
The recommendation from the UK Renal Association is for the ferritin to be > 100 mg/l and TSAT > 20%
(or < 10% hypochromic red cells). Therefore, this patient is iron-replete and there is no need for
additional iron replacement.
No current intervention, but plan for review in three months
194
Given his symptoms of lethargy, it would be unacceptable to leave his Hb at 97 g/l. It is important to
ensure that he is iron-replete, which he is, then to increase haemoglobin by administering
erythropoietin.
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A.130.A 21-year-old Nigerian woman was referred by her GP with a progressive history of polydipsia and
polyuria of 6 months' duration.
She has a history of sickle cell disease and had been admitted on two previous occasions to hospital with
chest pain precipitated by crisis. Fasting blood glucose is 4.5 mmol/l. She is not on any medication.
On examination, her blood pressure is 120/60 mmHg and heart rate is 90 bpm. A water deprivation test
was performed.
The water deprivation phase reveals the following:
Plasma osmolality 298 mosm/kg 278–305 mosm/kg
Urine osmolality 300 mosm/kg 350-1000 mosm/kg
The DDAVP phase reveals the following:
Plasma osmolality 295 mosm/kg 278–305 mosm/kg
Urine osmolality 325 mosm/kg 350-1000 mosm/kg
What diagnosis would best fit the clinical picture and investigation result?
Analgesic nephropathy
Cranial diabetes insipidus
Lithium-induced nephrotoxicity
Psychogenic polydipsia
Explanation
Nephrogenic diabetes insipidus (NDI) is characterised by the inability to concentrate urine due to the
lack of response of renal tubules to antidiuretic hormone (ADH). Normally the kidneys concentrate urine
by increasing water resorption by the collecting duct in the presence of ADH; this mechanism helps to
maintain plasma osmolality and extracellular volume. Without ADH, a large amount of dilute urine is
excreted. The water deprivation test after an overnight fast assesses the kidneys’ urine-concentrating
195
ability and response to ADH. The result shows abnormally low (<350 mosmol/kg) urine osmolality, which
increases only slightly after exogenous ADH (1-deamino-8-d-arginine-vasopressin (DDAVP), vasopressin).
Healthy individuals show a 2- to 4-fold increase in urinary osmolality over plasma osmolality.
NDI is X-linked recessive.Homozygous affected people (all males) are completely unresponsive to ADH
while heterozygous females show normal or slight impairment. Acquired NDI occurs in disorders that
disrupt the medulla or distal nephron and impair concentrating ability. Causes are: sickle cell
nephropathy, polycystic kidney disease, pyelonephritis, amyloidosis, and certain nephrotoxins such as
lithium and demeclocycline.
Psychogenic polydipsia
Psychogenic polydipsia is characterised by excessive fluid intake despite no physiological stimuli to drink,
therefore plasma sodium is low and urinary osmolality is low. However, water deprivation test is
normal.
The injuries that may be caused by analgesia include renal papillary necrosis and chronic interstitial
nephritis. There is also an increased risk of cancers of the urinary system. Water deprivation test is
classically normal in these conditions in contrast to the above scenario.
Cranial diabetes insipidus
Although there is polyuria and polydipsia associated with cranial diabetes insipidus, the water
deprivation test is normal and serum ADH levels low.
Lithium-induced nephrotoxicity
When lithium induces acute kidney disease, there is associated severe dehydration, natriuresis and
water diuresis. Other symptoms include altered mental status and poor oral intake. Water deprivation
test is normal. Nephrogenic DI is also associated with lithium treatment.
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A.131.A 40-year-old woman is admitted to the medical assessment unit with a history of fatigue,
headache, and leg oedema. She also complains of pruritus and heartburn.
On examination, she has slight oedema of the fingers and some perioral telangiectasia. Her mouth is dry
and her jugular venous pressure (JVP) is slightly elevated. Auscultation of the lungs reveals fine bibasal
end inspiratory crepitations. Her pulse is 105 beats per minute (bpm), respiratory rate 20 breaths per
minute, and her blood pressure 195/95 mmHg. There is thickening of the skin on the fingers with
peripheral calcinosis. Urine dipstick shows proteinuria and blood film shows helmet cells.
Given the likely diagnosis, what treatment is likely to have the most favourable outcome?
Beta-blocker
Nitroprusside
196
Glucocorticoid
Angiotensin-converting enzyme (ACE) inhibitor
Cyclophosphamide
Explanation
The history and examination give features of scleroderma renal crisis. Histologically interlobular arteries
are affected with intimal thickening. Features include malignant hypertension, oliguria, fluid retention,
and microangiopathic haemolytic anaemia. ACE inhibitors will significantly reduce progression to kidney
disease and increase the chance of recovery if kidney disease has already developed.
Beta-blocker
A beta-blocker may help control blood pressure, but it will not help control renal scleroderma renal
crisis. It may make any Raynaud's phenomenon worse.
Nitroprusside
An excessive reduction in pressure and hypovolaemia should be avoided in renal crisis because both can
further diminish renal perfusion and superimpose acute tubular necrosis upon the lesions of
scleroderma. Thus, parenteral antihypertensive agents (such as intravenous nitroprusside or labetalol)
should be avoided, if possible, as should other nephrotoxins such as nonsteroidal anti-inflammatory
drugs (NSAIDs) and radiocontrast agents.
Glucocorticoid
Similarly, glucocorticoids are effective immunosuppressants; however, they are not used for the
treatment of scleroderma. A side-effect of steroids is dyspepsia and may make heartburn symptoms
worse.
Cyclophosphamide
Cyclophosphamide is an immunosuppressant. It has not been shown to be an effective treatment for

scleroderma renal crisis.
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A.132.A 78-year old white man presents to the Emergency Department with a 1-day history of
haemoptysis and a 2-week history of general malaise, shortness of breath, cough and intermittent
fevers. He was prescribed clarithromycin for a presumed chest infection 10 days ago, but he has failed to
respond to treatment.
On examination, he has a temperature of 37.8 °C; he is tachypnoeic and has evidence of bilateral lung
consolidation.
197
C-reactive protein (CRP) 120 mg/l <10 mg/l
Erythrocyte sedimentation rate (ESR) 90 mm/h 1- 20 mm/h
Antibodies to nuclear antigens (ANA) Positive
Antineutrophil cytoplasmic antigens (ANCA) Positive
Antiproteinase 3 Positive
Antiglomerular basement membrane antibodies Negative
Urine dipstick 3+ blood and 2+ protein
Urine microscopy Red cell casts
His chest X-ray is shown below.
What is the most appropriate treatment for this patient?
High-dose prednisolone alone
High-dose prednisolone and cyclophosphamide
Immunoglobulin IV
Plasmapheresis
Supportive care and IV antibiotics
Explanation
High-dose prednisolone and cyclophosphamide
The patient has evidence of a rapidly progressive glomerulonephritis. The differential diagnosis includes
pauci-immune small vessel vasculitides (Granulomatosis with polyangiitis (GPA), polyarteritis nodosa
(PAN) and microscopic polyangiitis), Goodpasture’s syndrome, systemic lupus erythematosus (SLE) and
IgA nephropathy. The presence of a pulmonary–renal syndrome (note the respiratory symptoms and
198
bilateral infiltrates on the chest x-ray) coupled with the pattern of autoantibodies points to a diagnosis
of GPA. The most appropriate treatment for this is high-dose steroids with cyclophosphamide, as this
can induce remission in 80–90% of patients.
Image source: https://radiopaedia.org/cases/eosinophilic-granulomatosis-with-polyangiitis-3?lang=gb
High-dose prednisolone alone
High-dose prednisolone on its own would not be enough immunosuppression for rapidly progressive
glomerulonephritis.
Immunoglobulin IV
There is no evidence that there is any benefit for IV immunoglobulins in this scenario. In fact, it may be
important to remove the antigenic immunoglobulins for treatment, which is the concept for
plasmapheresis.
Plasmapheresis
When there is significant pulmonary haemorrhage associated with Granulomatosis with polyangiitis and
a rapid response to immune intervention isn’t seen, then plasmapheresis would be indicated.
Supportive care and IV antibiotics
It is important to stop progression of the inflammation and damage; therefore supportive care will not
be the appropriate treatment. Appropriate immunosuppression in the form of prednisolone and
cyclophosphamide is crucial.
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A.133.A 62-year-old man, who has been managed with long-term haemodialysis for end-stage kidney
disease as a result of type 1 diabetes, presents to the clinic complaining of pain and tingling in his hands,
particularly in the early hours of the morning. He also has trouble with dysphagia and indigestion, and
echocardiography performed for decreased LV function revealed a suspicion of early constrictive
pericarditis.
On further questioning, he also admits to drinking a glass of whisky each evening.
On examination, he has weakness of thumb abduction, apposition and flexion, and there is suspicion of
some sensory loss.
Investigations:
Platelets (PLT) 192 × 109/l 150 - 400 × 109/l
199
Urate 0.54 mmol/l 0.2 - 0.4 mmol/l (male)
Glucose 9.2 mmol/l 3.9 - 7.1 mmol/l
Which of the following is the most likely cause of his upper limb neurological symptoms?
Alcoholic neuropathy
B12 deficiency
Carpal tunnel syndrome
Diabetic neuropathy
Uraemic neuropathy
Explanation
Carpal tunnel syndrome
Evidence of GI involvement, constrictive pericarditis and thenar weakness with sensory loss is suggestive
of beta-2 microglobulin deposition. Indigestion and symptoms of reflux occur due to disordered gut
motility as a result of amyloid deposition in the GI tract. In the normally functioning kidney, beta-2
microglobulin is cleared by glomerular filtration and catabolised in the proximal tubules. In patients with
end-stage kidney disease, particularly those who have undergone long-term dialysis, accumulation
occurs and the symptoms of systemic amyloidosis develop. High-flux biocompatible dialysis membranes
may reduce accumulation of beta-2 microglobulin, and renal transplantation can lead to a gradual
reduction in symptoms, but unfortunately those with significant multi-system disease may be
considered unfit for surgical intervention. The carpal tunnel syndrome seen here is associated with
peripheral beta-2 microglobulin deposition.
Uraemic neuropathy
Uraemic neuropathy is a distal sensorimotor polyneuropathy caused by uraemic toxins. There is

evidence that the severity of the kidney disease is related to the severity of the neuropathy.
Alcoholic neuropathy
The other symptoms, such as constrictive pericarditis, do not fit with alcoholic neuropathy.
B12 deficiency
Vitamin B12 deficiency may result in sensory or motor deficiencies (absent reflexes, diminished vibration
or soft touch sensation), subacute combined degeneration of the spinal cord, seizures and dementia.
Diabetic neuropathy
Diabetic neuropathy occurs after many years of diabetes and is mainly a sensory neuropathy.
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A.134.A 66-year-old man on peritoneal dialysis for end-stage kidney disease presents with a 3-month
history of tiredness secondary to anaemia, in spite of increased subcutaneous erythropoietin therapy.
Investigations:
Differential WCC normal
Platelets (PLT) 200 × 109/l 150 - 400 × 109/l
Haematocrit 0.2 0.45 - 0.54 (male)
Reticulocyte count 0% 0.5 - 2.5 %
Faecal occult bloods negative
Colonoscopy normal
Upper GI endoscopy normal
Haptoglobins normal
What is the most likely cause of his anaemia?
Hypothyroidism Myelodysplasia Myelofibrosis Pure red cell aplasia Chronic lymphocytic leukaemia
Explanation
Pure red cell aplasia
Pure red cell aplasia (PRCA) is a rare condition defined by the absence of erythroblasts in the bone
marrow, leading to profound anaemia with normal leucocyte and platelet counts, and characterised by
low or absent circulating reticulocytes. Serum iron and ferritin rise sharply as iron cannot be
incorporated into the erythrocytes.
PRCA occurs due to neutralising antibodies to erythropoietin. There has been a recent increase in PRCA
in patients with chronic kidney disease on subcutaneous erythropoietin alpha. Treatment includes
discontinuation of the EPO preparation and repeated transfusions.
Hypothyroidism
The normal MCV and WCC make hypothyroidism unlikely.
Myelodysplasia
201
The normal MCV and WCC make myelodysplasia unlikely. The myelodysplastic syndromes comprise a
heterogeneous group of malignant hematopoietic stem cell disorders characterised by dysplastic and
ineffective blood cell production and a variable risk of transformation to acute leukaemia.
Myelofibrosis
In myelofibrosis the blood film shows a leucoerythroblastic picture with an increased reticulocyte count.
Chronic lymphocytic leukaemia
The normal MCV and WCC make chronic lymphocytic leukaemia unlikely.
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A.135.A 58-year-old man, previously in good health, suddenly develops severe abdominal pain radiating
from the left loin to the groin and associated with nausea, perspiration and frequent urination.
There is a history of diverticular disease but nil else of note. He is restless, tossing and turning in bed.
His BP is elevated at 180/90 mmHg, his pulse is 95/min and he is in obvious pain. He has 3+ of
haematuria on urine dipstick.
Herpes zoster involving the left lower rib dermatome
Left ureteric calculus
Retroperitoneal haemorrhage
Sigmoid diverticulitis
Torsion of the left testicle
Explanation
Left ureteric calculus
This is a typical case of ureteric colic. It is sudden in onset, radiating from loin to groin, and is associated
with urge to urinate frequently. It is the pain radiating to the groin that points more towards ureteric
colic versus diverticulitis, the major differential here. The usual causes are blood clots and calculi. Urine
examination reveals macroscopic or microscopic haematuria.
Herpes zoster involving the left lower rib dermatome
The left lower rib dermatome would not be radiating pain from loin to groin.
Retroperitoneal haemorrhage
We would expect a drop in blood pressure with retroperitoneal haemorrhage and back pain.
Sigmoid diverticulitis
202
Sigmoid diverticulitis would not reveal haematuria; it would present with acute abdominal pain, fever,
nausea and vomiting. Frequently diagnosed on CT abdominal scan.
Torsion of the left testicle
Torsion of the left testicle would not cause loin pain typically. Testicular torsion would present with
severe testicular pain and tachycardia. There may be difficulty in passing urine due to the pain. The
testicles would be swollen, red and tender.
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A.136.A 22-year-old woman is admitted with a third episode of syncope over the past six months. She
says that she tries her best in hot weather to maintain her fluid intake, but often feels light-headed. She
does not take any regular medication apart from the combined oral contraceptive pill.
On examination, her blood pressure (BP) is 110/80 mmHg, pulse is 75/min and regular. Body mass index
(BMI) is 23. There are no abnormal findings.
Investigations:
Platelets (PLT) 183 × 109/l 150 - 400 × 109/l
Bicarbonate 33 mmol/l 22 – 29 mmol/l
Bartter syndrome
Conn syndrome
Cushing syndrome
Gitelman syndrome
Vasovagal syncope
Explanation
Gitelman syndrome
203
Gitelman’s presents with hypokalaemic metabolic alkalosis in the absence of hypertension. Gitelman’s
may be associated with milder disease and presents later than Bartter syndrome. Gitelman syndrome is
associated with reduced calcium excretion, whereas Bartter’s is not, and this helps to differentiate
between the two conditions.
Bartter syndrome
Bartter syndrome presents with hypokalaemic metabolic alkalosis in the absence of hypertension.
Bartter’s normally presents in childhood with constipation, growth failure, muscle cramps and
weakness. Renin and aldosterone are both elevated.
Conn syndrome
Conn syndrome presents with hypokalaemic metabolic alkalosis but with hypertension. There may be
other symptoms such as myalgia, muscle spasms, paraesthesiae and polyuria. Complications may
include stroke, myocardial infarction and kidney disease.
Cushing syndrome
Cushing syndrome is associated with obesity. Other symptoms and signs include hypertension,
abdominal striae, round facies, fat pad between shoulder blades, muscle wasting and poor wound
healing.
Vasovagal syncope
Vasovagal syncope will present with low blood pressure; however, there would not be any metabolic
disturbance such as hypokalaemic metabolic alkalosis which would suggest a different diagnosis. In
vasovagal syncope, when the patient lies flat and circulation returns to the brain, consciousness is
restored.
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A.137.A general practitioner (GP) has referred a 20-year-old patient to the Renal Clinic with haematuria.
The patient recalls having coryzal symptoms two weeks ago and then developing frank haematuria for a
few days. He says the symptoms have resolved. The patient recalls his maternal grandfather had ‘kidney’
problems at a young age.
He has a past history of hearing difficulties and wears hearing aids, and is under the ophthalmologists
for treatment of corneal ulcers.
Blood pressure 145/80 mmHg
Urine dipstick ++protein, ++blood
Fundoscopy Bilateral yellow dots around the macula
Evidence of what underlying diagnosis is likely to be seen on the renal biopsy?
204
Rapidly progressive glomerulonephritis
Mesangiocapillary glomerulonephritis (MPGN)
Primary focal segmental glomerulosclerosis (FSGS)
Alport’s syndrome
Explanation
Alport’s syndrome
Alport’s syndrome consists of:
nephritis - causing microscopic haematuria and progressive kidney disease with episodic frank
haematuria
ocular pathology – corneal ulcerations, bilateral anterior lenticonus, bilateral dot-fleck marks around the
fovea
progressive high-frequency sensorineuronal deafness.
A light microscope examination can be unremarkable and electron microscope examination is needed.
Occasionally focal and segmental glomerulosclerosis, interstitial fibrosis, tubular atrophy, and infiltration
of lymphocytes and plasma cells may be seen on light microscopy. The syndrome is inherited mainly as
X-linked.
Rapidly progressive glomerulonephritis
This is a syndrome consisting of a rapid loss in renal function (usually a 50% decline in glomerular
filtration rate within 3 months) with glomerular crescent formation in at least 50–75% of glomeruli on
renal biopsy. If left untreated it rapidly progresses into acute kidney disease.
Mesangiocapillary glomerulonephritis (MPGN)
MPGN is an uncommon cause of chronic nephritis in children and young adults. There is a thickening of
the peripheral capillary walls by subendothelial immune deposits and or intramembranous dense
deposits.
Primary focal segmental glomerulosclerosis (FSGS)
FSGS is a common cause of nephrotic range proteinuria in adults. There would not be haematuria on
dipstick.
This is the commonest cause of nephrotic syndrome (proteinuria, oedema and hypoalbuminaemia) in
adults. There would not be blood in membranous glomerulonephritis on dipstick.
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205
A.138.A 19-year-old boy presents to his general practitioner (GP) complaining of macroscopic
haematuria which began 48 h after he started with a recent respiratory tract infection. He has a past
medical history of asthma and eczema. He takes salbutamol inhalers, as required, on occasions.
On examination, his blood pressure is 142/86 mmHg, chest and abdominal examination is
unremarkable. He has no peripheral oedema.
Investigations reveal:
Creatinine (Cr) 155µmol/l 50 - 120 µmol/l
Urine Culture negative, red cells 3+, protein 1+
Urine microscopy Leukocytes and red cell casts
Renal biopsy Diffuse mesangial proliferation and extracellular matrix expansion. A few necrotising
lesions with crescent formation are noted.
Which is the next best treatment for his condition?
Calcium channel antagonists
Ciclosporin
Low-protein diet
Prednisolone therapy
Doxazocin
Explanation
Prednisolone therapy
This young man has immunoglobulin A (IgA) nephropathy as illustrated by the presentation with
haematuria post-respiratory tract infection and the histological findings on renal biopsy. Gross
proteinuria is rare, but haematuria is the commonest form of presentation. The disease is commoner in
males, and presents most often in the second and third decades. Management includes control of
hypertension, with angiotensin-converting enzyme (ACE) inhibitors the preferred agent, and use of
corticosteroids. Where there is crescenteric nephritis on biopsy, intravenous pulsed corticosteroids are
employed, with the addition of cyclophosphamide. Thankfully, progression to end-stage renal disease is
uncommon in this condition. Where end-stage renal disease does occur, renal transplantation carries a
high chance of success.
206
Calcium channel antagonists
Angiotensin-converting enzyme (ACE) inhibitors are the preferred agent rather than calcium channel
antagonists because ACE inhibitors will help reduce proteinuria while calcium channel antagonists may
even increase proteinuria.
Ciclosporin
It is cyclosphosphamide which would be added early to the steroid regime rather than ciclosporin.
Ciclosporin may be a considered agent later on down the line for disease control.
Low-protein diet
A low-protein diet may reduce proteinuria. However, it would not be used on its own to control
symptoms. In fact, there is no evidence that low-protein diets alter disease progression.
Doxazocin
Angiotensin-converting enzyme (ACE) inhibitors are the preferred agents rather than doxazocin to
control blood pressure and proteinuria.
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A.139.A 67-year-old man is referred to the Hypertension Clinic with difficult-to-control blood pressure.
He is currently taking atenolol 50 mg daily, ramipril 10 mg daily and indapamide 2.5 mg daily. There is a
past history of an inferior myocardial infarction (MI) some 3 years earlier and he continues to smoke ten
cigarettes per day.
On examination, his BP is 155/95 mmHg, pulse is 62/min and regular. His chest and abdominal
examination is unremarkable. You note left carotid and left femoral bruits and poor capillary return in
his feet.
Investigations:
Platelets (PLT) 212 × 109/l 150 - 400 × 109/l
Ultrasound renal tract Differential kidney size with small left kidney
207
Essential hypertension
Phaeochromocytoma
Reflux nephropathy
Explanation
There are multiple clues to the fact that this patient is a significant vasculopathy, with the previous
inferior MI, poor peripheral circulation and multiple bruits. This, coupled with a differential kidney size
on ultrasound, is suggestive of renal artery stenosis, resultant secondary hyperaldosteronism leading to
the hypokalaemia seen here. Angiography is the definitive investigation to confirm the diagnosis;
although, in practice, recent large studies have suggested that medical management of hypertension is
as effective as vascular intervention.
Essential hypertension
Essential hypertension can give rise to renal impairment and difficult-to-treat hypertension. However,
there would not be a differential kidney size. Both kidneys would be small in chronic hypertensive
nephropathy.
Phaeochromocytoma
Phaeochromocytoma would not have different sized kidneys. There may be other symptoms such as
flushing and tachycardia. The hypertension is often episodic rather than permanent.
Conn syndrome is an excess production of aldosterone and there is a low renin level. Often, there are
few symptoms except high blood pressure. There may be myalgia, paraesthesiae and polyuria. There is
no differential kidney size in primary hyperaldosteronism.
Reflux nephropathy
Reflux nephropathy is due to reflux of urine from bladder back towards the kidneys – vesicoureteral
reflux. Longstanding vesicoureteric reflux can result in small and scarred kidneys during the first five
years in affected children. The end result is hypertension, proteinuria and may lead to end-stage kidney
disease. In this scenario, there is mainly a vascular history rather than a history of recurrent urinary tract
and vesicoureteric reflux; therefore, renal artery stenosis is a likely diagnosis.
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A.140.You are asked to urgently review a 62-year-old man who is admitted to the Emergency
Department. There is a past history of chronic kidney disease. On this occasion, he complains of
increasing shortness of breath.
208
On examination, he has a blood pressure of 90/60 mmHg, with a heart rate of 100 bpm. He has a
pericardial rub and there are bibasal crackles consistent with heart failure. Both legs are swollen up to
the thighs.
Platelets (PLT) 243 × 109 /l 150–400 × 109/l
Which of the following represents the best treatment option in this patient?
Blood transfusion
Erythropoietin
Urgent haemodialysis
Intravenous furosemide
Intravenous iron replacement
Explanation
This man has uraemic pericarditis, an indication for urgent haemodialysis. Haemorrhagic pericardial
effusion and atrial fibrillation are often associated. Anticoagulation is not advised because of the risk of
precipitating cardiac tamponade. Uraemic pericarditis usually resolves after a period of intensive
dialysis. Uraemic neuropathy is also an indication for urgent dialysis, as is evidence of fluid overload
coupled with kidney disease which is non-responsive to diuretics.
Blood transfusion
Giving blood transfusion in this scenario would exacerbate the problem as the patient is already fluid
overloaded.
Erythropoietin
209
Erythropoietin will increase the haemoglobin, but will need time to act. It may be given subcutaneously
or intravenously. However, it would not be a treatment of uraemic pericarditis and has no effect on
uraemia.
Intravenous furosemide
Furosemide may be useful to reduce fluid overload if the patient is still passing urine. However, it would
potentially exacerbate uraemia and would not be the definitive treatment for uraemic pericarditis.
Intravenous iron replacement
Since giving intravenous iron adds to fluid load, it would especially not be recommended in this case.
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A.141.A 70-year-old man was admitted with anuria and lower back pain of 24 hour duration.
His only medication is bendroflumethiazide 2.5 mg for hypertension. He is awaiting an appointment for
urinary frequency, urgency and dribbling; otherwise, he is fit and well.
Investigations:
Platelets (PLT) 160 × 109/l 150–400 × 109/l
Total protein 80 g/l 60-80 g/l
You have given him intravenous (iv) insulin and dextrose to reduce his potassium. A follow-up potassium
level is measured at 5.4 mmol/l, and you catheterise him.
Which of the following is the next most appropriate management?
Central venous line for monitoring intravascular volume
210
Restrict oral fluid
Urgent intravenous urography (IVU)
Urgent renal tract ultrasound
Explanation
Urgent renal tract ultrasound
The symptom of urinary frequency and the age of the patient suggest probable prostatic hypertrophy.
Prostatic obstruction is the most common cause of obstructive uropathy in men.
In community studies, urinary tract obstruction is responsible for 25% of cases of acute kidney disease. It
is easily treatable if the diagnosis is made accurately and early. The cause of the acute presentation in
this case is most likely due to obstructive uropathy.
The first investigation should be renal and pelvic ultrasound to exclude hydronephrosis which, if present,
warrants urgent nephrostomy for ureteric obstruction, or transurethral/suprapubic catheterisation, as
appropriate, for bladder obstruction. Renal function often normalises with relief of the obstruction.
Urgent intravenous urography (IVU)
IVU would be more invasive than ultrasound of the urinary tract. Ultrasound would be less invasive, easy
to carry out and contrast-free.
There are no acute indications in this scenario for urgent haemodialysis such as uraemic symptoms,
resistant hyperkalaemia or resistant acidosis and pulmonary oedema not amenable to medical
treatment.
Central venous line for monitoring intravascular volume
Central venous line may be useful to discern the volume status of the patient, but in this scenario of
obstruction, it would not be helpful.
Restrict oral fluid
Restriction of oral fluid would make the patient dehydrated and will not resolve the obstruction.
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A.142.An 18-year-old man presents with longstanding polyuria, polydipsia and occasional abdominal and
calf cramping pains, along with the following biochemical abnormalities. His parents are first cousins.
He has a normal stature and has been noted to have a low-normal blood pressure at around 95/50
mmHg.
211
Magnesium (Mg2+) 0.59 mmol/l 0.75–1.00 mmol/l
Urinary calcium excretion: Low (urinary calcium/creatinine ratio 0.1)
What is the likely diagnosis?
Alport syndrome
Bartter syndrome
Gitelman syndrome
Liddle syndrome
Severe Cushing syndrome
Explanation
Gitelman syndrome
The patient has features of Gitelman syndrome with hypokalaemic metabolic alkalosis,
hypomagnesaemia and hypocalciuria. His symptoms are relatively mild, and his age at presentation and
the low to normal blood pressure all support the diagnosis of Gitelman syndrome. This can occur either
as a sporadic or as an autosomal recessive condition. The consanguinity in this case suggests that this
may be an autosomal recessive case. The prognosis of Gitelman syndrome is very good, and treatment is
with potassium and magnesium supplements.
Alport syndrome
Alport syndrome is an inherited condition, in which an abnormal form of the glomerular basement
membrane is produced. It is associated with bilateral sensorineural deafness, kidney disease and
haematuria.
Bartter syndrome
Bartter syndrome shares some features (including hypokalaemia and hypotension) with Gitelman
syndrome but is more severe and patients therefore present earlier with profound hypokalaemia and
hypotension in early childhood.
In Gitelman syndrome, urinary calcium excretion is low, and this can also be used as a differentiator,
although calcium excretion is not the best differentiator between the two conditions.
Liddle syndrome
212
Liddle syndrome is associated with hypokalaemic metabolic alkalosis, but usually in the context of
hypertension which develops in adolescence. It is inconsistent with the low to normal blood pressure
seen here.
Severe Cushing syndrome
Cushing syndrome would be expected to produce other characteristic features, as well as hypertension,
without the magnesium and calcium abnormalities seen here. Usual features of Cushing syndrome
include significant weight gain, abdominal striae and impaired glucose tolerance.
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A.143.A 25-year-old woman comes to the clinic with a 3-day history of lower abdominal pain that is now
beginning to radiate to her right flank. She denies nausea, vomiting, or diarrhoea but states that she
feels feverish and is worried that she may have the flu. She has noticed an increase in her urinary
frequency.
Vital signs are temperature 39.2 °C, blood pressure 120/70 mmHg, pulse 95 beats per minute (bpm) and
respiration of 16 breaths per minute.
Physical examination shows tenderness in the suprapubic region, hyperactive bowel sounds, and no
signs of peritoneal irritation or masses. Her right flank is tender to light percussion. The remainder of the
physical examination is within normal limits.
Urinalysis reveals the following:
Leukocytes +++
Proteinuria +
Gram stain Gram-negative bacilli
Acute appendicitis
Ovarian torsion
Perinephric abscess
Pelvic inflammatory disease
Explanation
This is a typical picture of pyelonephritis. Additional testing, such as renal ultrasonography or

intravenous pyelography, is indicated only if there is suspicion of a complication such as a stone stricture
213
or tumour causing the pyelonephritis. These tests are not necessarily part of the routine management of
a simple uncomplicated pyelonephritis. If symptoms persist beyond 3–5 days of effective treatment, an
ultrasound or computed tomography (CT) scan of the kidney should be obtained to exclude a
perinephric abscess or other drainable collection of fluid.
Pelvic inflammatory disease
Pelvic inflammatory disease would not explain the Gram-negative bacilli in the urine.
Acute appendicitis
Acute appendicitis would not explain the gram-negative bacilli in the urine. It would present as right iliac
fossa pain, with nausea or vomiting accompanied by fever. There may be blood and leucocytes in the
urine.
Ovarian torsion
Ovarian torsion would not explain the gram negative bacilli in the urine. Ovarian torsion will present
with acute onset iliac fossa pain - it may be so severe as to cause hypotension and tachycardia. The
patient can be very unwell.
Perinephric abscess
If symptoms in this scenario persist beyond 3–5 days of effective treatment, an ultrasound or computed
tomography (CT) scan of the kidney should be obtained to exclude a perinephric abscess or other
drainable collection of fluid.
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A.144.A 25-year-old woman presents to Medical Clinic complaining of back pain. Past medical history of
note includes hypertension which is currently monitored by the GP. Apparently, one of her family died
suddenly of a stroke at the age of 41.
On physical examination, her blood pressure is 175/80 mmHg, BMI was 27 kg/m2, her chest was clear
and abdomen examination was unremarkable.
214
Platelets (PLT) 560 × 109/l 150 - 400 × 109/l
Erythropoietin level Raised
What investigation should be performed next?
Arterial blood gases (ABGs)
Bone marrow examination to exclude myeloproliferative disorder
Chest X-ray
Lactate dehydrogenase (LDH) levels
Renal USS
Explanation
Renal USS
The combination of back pain and raised Hb should raise the suspicion of renal cystic disease. One cause
may be autosomal-dominant polycystic kidney disease (ADPKD) accounting for the death of a relative at
an early age. Because this lady is 25 years old, the kidneys would not have been so enlarged to have
been detectable on physical examination, therefore the abdominal examination is unremarkable. APKD
is a common autosomal dominant disease with a gene defect on the short arm of chromosome 16 and
usually presents in the fourth and fifth decades. APKD may present at any age from the second decade
with acute loin pain or haematuria due to cyst haemorrhage or infection, loin pain due to increased
renal size, subarachnoid haemorrhage due to berry aneurysm rupture, complications of hypertension,
complications of liver cyst formation or symptoms of chronic kidney disease.
Arterial blood gases (ABGs)
The ABG result will be normal in ADKPD and therefore would not help in discerning any differentials.
Bone marrow examination to exclude myeloproliferative disorder
The bone marrow in ADPKD may be normal providing there is no associated renal bone disease
associated with kidney disease. There would be an increased production of erythropoietin from the
kidneys though.
Chest X-ray
In ADPKD, the chest X-ray is typically normal.
Lactate dehydrogenase (LDH) levels
LDH may be abnormal in myeloproliferative and lymphoproliferative disorders. Haemolytic anaemia

would be one example where LDH would be elevated. In ADPKD, the LDH level would be normal.
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215
A.145.A 58-year-old woman presents with lethargy, confusion and vomiting. She has not presented to
health services for many years, although she has been known to use paracetamol/dextropropoxyphene
(co-proxamol) tablets for arthritis in the past and takes a diuretic for management of hypertension.
On examination, her blood pressure is 165/85 mmHg and she is confused, with evidence of nystagmus,
hyper-reflexia and clonus. General examination reveals evidence of vaginal bleeding.
Blood tests reveal:
Which one of the following diagnoses fit best with this clinical picture?
Hypertensive encephalopathy
Intracranial metastases
Opiate intoxication
Subdural haematoma
Explanation
The evidence of vaginal bleeding is suggestive of uterine or cervical cancer. Cervical cancer is much more
likely to lead to ureteric obstruction and this type of presentation with kidney disease. The neurological
findings are suggestive of uraemic encephalopathy, which progresses to coma, seizures and death if left
untreated. The standard therapy for uraemic encephalopathy is initiation of dialysis, although a long-
term commitment to renal replacement therapy should be considered carefully in the light of possible
underlying advanced cervical carcinoma. Conventionally, in this case, urgent imaging would be
considered to elucidate the extent of any underlying tumour and consequent obstruction before
necessarily committing to dialysis.
Hypertensive encephalopathy
Hypertensive encephalopathy is a syndrome consisting of a sudden elevation of arterial pressure usually

preceded by severe headache and followed by convulsions, coma or a variety of transitory cerebral
phenomena. The syndrome may complicate acute glomerulonephritis, toxaemia of pregnancy and
essential or malignant hypertension. In this scenario, the history of vaginal bleeding would not be
consistent with hypertensive encephalopathy.
Intracranial metastases
216
Malignancy with intracranial metastases can present with headaches and vomiting, but the kidney
disease would not be in keeping with this diagnosis as the best explanation.
Opiate intoxication
Opiates may give rise to vomiting, nausea and confusion. Intoxication is more likely in renal impairment
because opioids are renally excreted. The hyper-reflexia and clonus would go against this diagnosis
because with opiate poisoning there would not be increased reflexes. We would expect small pupils on
examination.
Subdural haematoma
Any head injury may predispose to subdural haematoma especially in the elderly or patients consuming
alcohol in excess. There are no risk factors in this case for subdural haematoma. The presentation of a
subdural bleed can include hyper-reflexia and upward going plantars.
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A.146.A 23-year-old man attends his GP complaining of reduced exercise tolerance, nocturia and
frequent hiccups over the course of the past four months. He also has generalised bony pain.
In his past medical history, he has known rheumatoid arthritis and has had a contrast angiography 1
week ago for investigation of chest pain.
Upon examination, his blood pressure is 165/90 mmHg and his BMI is stable at 25. There are a few
scattered crepitations at the bases of his lung fields. His abdomen is distended with dullness in both
flanks. There are two palpable masses in the abdomen which is palpable above and rises with
respiration. No tenderness, rigidity or guarding is detected.
Corrected Calcium (Ca2+) 2.1 mmol/l 2.20–2.60 mmol/l
What is the most likely cause of these results?
Adult polycystic kidney disease
Diabetes
Diffuse proliferative glomerulonephritis
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Explanation
These results are a picture of chronic kidney disease (CKD) as there is a raised phosphate and creatinine.
This has probably resulted in hypovitaminosis D and secondary hyperparathyroidism. Adult polycystic
kidney disease (APKD) is correct because it can cause chronic kidney disease with a maintained
haemoglobin level due to excess erythropoietin secretion. Hiccups are seen as a result of uraemia.
It would cause an acute kidney disease with normal phosphate, calcium and haemoglobin levels.
Contrast nephropathy accounts for about 10% of all in-hospital acute kidney disease. The clinical
features include a drop in renal function immediately after contrast exposure, though creatinine may be
initially unchanged. The earliest sign may be oliguria. Unlike other causes of acute tubular necrosis,
fractional excretion of sodium is < 1%.
Diabetes
Although this can cause CKD you would expect to see a chronic anaemia picture. Diabetic nephropathy
results from diabetes and is due to microvascular and macrovascular glycosylation. The renal
impairment is generally progressive. One of the first signs of disease is microalbuminuria. Important
treatment methods to prevent kidney disease progression would be good blood pressure control,
diabetic control and, once proteinuria occurs, to commence an angiotensin-converting enzyme (ACE)
inhibitor.
Diffuse proliferative glomerulonephritis
Similar to contrast nephropathy, this would cause an acute kidney disease rather than chronic kidney
disease which is the biochemical picture in this case. Typical presentations of diffuse proliferative
glomerulonephritis would be a rapidly progressive acute kidney disease (oliguria, pulmonary oedema,
peripheral oedema, rising creatinine levels) with haematoproteinuria. Depending on the underlying
cause of the glomerulonephritis there may be other signs such as pulmonary haemorrhage and ear/nose
and throat symptoms especially with vasculitis.
In hypertensive nephropathy there would be a long-standing history of hypertension associated with a

progressive renal impairment. There may be other signs of chronic hypertensive damage such as left
ventricular hypertrophy, fundoscopy showing haemorrhages, exudates or papilloedema, and urine
dipstick may show blood and protein. There are often other cardiovascular diseases such as previous
stroke and cardiac events.
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A.147.A 69-year-old woman is found on the floor, having slipped in the bathroom at home. She has been
lying on the floor for up to 8 hours before being found by her carer.
There is a past history of ischaemic heart disease, for which she is treated with ramipril, bisoprolol,
aspirin and atorvastatin.
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Her blood pressure (BP) is 122/72 mmHg, pulse is 80/min, sinus rhythm. She has signs of left lower lobe
consolidation and there is extensive bruising over the left hip and pelvis.
Platelets (PLT) 183 × 109/l 150 - 400 × 109/l
Urine dipstick 3+ blood
You are concerned about the possibility of rhabdomyolysis.
What is the most appropriate investigation to evaluate the risk of AKI?
Albumin Creatinine
Creatine kinase
Lactate dehydrogenase (LDH)
Urine microscopy
Explanation
Creatine kinase
The options for diagnosing rhabdomyolysis include serum creatine kinase (CK) and urine microscopy.
The presence of blood on urine dipstick and an absence of cells on microscopy would suggest that it is
myoglobinuria rather than haematuria which is responsible for the positive test. However, CK is the
preferred answer, as the level is predictive of possible renal impairment.
Urine microscopy
The presence of blood in urine dipstick and an absence of cells on microscopy would suggest that it is
myoglobinuria rather than haematuria which is responsible for the positive test. However, creatine
kinase is the preferred answer, as the level is predictive of possible renal impairment.
Albumin
In rhabdomyolysis, at an early stage, dehydration causes hyperalbuminaemia, whereas later

malnutrition, inflammation, capillary leak and fluid overload cause hypoalbuminaemia. Changes in
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serum albumin may result in the misinterpretation of total plasma calcium concentrations. Albumin is
not specific for rhabdomyolysis.
Creatinine
While elevated creatinine may be seen, it does not reveal the underlying cause of renal impairment.
Creatinine is raised in renal impairment of any cause.
Lactate dehydrogenase (LDH)
A rise in LDH is seen in muscle necrosis but it occurs later in the process. LDH may be raised in
myocardial infarction due to muscle necrosis. It is not specific to rhabdomyolysis.
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A.148.A 48-year-old man presents with painless microscopic haematuria discovered by his GP at a new
patient medical. He also has vague right iliac fossa pain. There is a past history of tuberculosis and chest
X-ray is suggestive of right apical fibrosis. His father died of a brain haemorrhage.
On examination, his blood pressure is 160/l00 mmHg and there is appears to be a palpable mass on
balloting the right kidney.
Platelets (PLT) 243 × 109 /l 150–400 × 109/l
Plasma viscosity Normal
Which of the following stems fits best with this diagnosis?
Autosomal dominant polycystic kidney disease (APKD)
Wilms' tumour
Transitional cell carcinoma
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Explanation
Autosomal dominant polycystic kidney disease (APKD)
APKD may present at any age from the second decade with acute loin pain or haematuria due to cyst
haemorrhage or infection, loin pain due to increased renal size, subarachnoid haemorrhage due to berry
aneurysm rupture, complications of hypertension, complications of liver cyst formation or symptoms of
chronic kidney disease. The picture seen here with mild anaemia, hypertension and a palpable right
kidney points towards the diagnosis; the normal viscosity counts against renal cell carcinoma.
The diagnosis of medullary sponge kidney is based on the finding of dilated collecting ducts (precalyceal
ducts) that may or may not contain calculi and/or nephrocalcinosis. The classic radiological appearance
is described as 'papillary blush' or 'paint brush' on early and delayed films during intravenous urography.
Wilms' tumour
Wilms' tumour (nephroblastoma) is a rare kidney cancer and it affects mainly children. In fact, it is the
most common cancer in children.
Renal cell carcinoma is the commonest type of kidney cancer in adults, accounting for between 90% and
95% of cases. The classical triad of symptoms includes haematuria, flank pain and abdominal mass,
although this triad of symptoms occurs in only 10–15% of patients and is usually associated with
advanced disease. Mostly, nowadays, renal cell carcinoma is asymptomatic and detected incidentally
when investigating for other conditions.
This is also known as urothelial carcinoma; it is the most common type of bladder cancer and cancer of
the ureter and urethra. This condition may asymptomatic or present with haematuria, back pain, weight
loss, or painful or frequent urination. The normal viscosity, anaemia and hypertension are not due to
transitional cell carcinoma.
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A.149.A 42-year-old man presents for review. He admits to unprotected sex with a number of men and
has suffered a dry cough, shortness of breath and weight loss over the past few months.
His major current complaint is that has had progressive lower limb swelling which has worsened over
the past few weeks. Blood pressure on examination is 135/75 mmHg and there is peripheral oedema.
His BMI is 21.
24-h urinary protein excretion 5.2 g
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CD4 count 175 cells 500-1500 cells
Albumin 27 g 35–55 g/l
Which of the following is the initial treatment of choice for this patient’s renal dysfunction?
Antiretroviral drugs
Corticosteroids
Ciclosporin
Cyclophosphamide
Furosemide
Explanation
Antiretroviral drugs
This patient has HIV-associated nephropathy (HIVAN), characterised by nephrotic range proteinuria,
normal blood pressure, normal or increased kidney size on ultrasound scan and focal segmental
glomerulosclerosis on renal biopsy. Treatment includes aggressive antiretroviral therapy and
angiotensin-converting enzyme (ACE) inhibitor therapy for patients where this is not contraindicated
due to hyperkalaemia.
Corticosteroids
No completed randomised controlled trials exist for the use of second-line agents such as
corticosteroids for HIVAN, although observational studies suggest that a benefit may exist.
Ciclosporin
No completed randomised controlled trials exist for the use of second-line agents such as ciclosporin for
HIVAN, although observational studies suggest that a benefit may exist.
Cyclophosphamide
There is no evidence for cyclophosphamide in the treatment of HIVAN. It may even cause harm because
cyclophosphamide is an immunosuppressant and would predispose to patients with HIV to opportunistic
infections.
Furosemide
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Furosemide would help with volume overload and hypertension in HIVAN. It may help with relieving the
peripheral oedema. However, caution must be used because the blood pressure is not too high and
furosemide may lower the blood pressure further. Furosemide may be used in this scenario for the
symptoms, but it is not definitive treatment for the condition.
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A.150.A 65-year-old man with no significant previous medical history presents to his GP with symptoms
of an upper respiratory tract infection, for which he is prescribed a 10-day course of amoxicillin.
Towards the end of the course of antibiotics, he comes back to the GP complaining of increasing nausea,
anorexia and shortness of breath. He has a BP of 145/95 mmHg and a diffuse rash over his groin and
thighs.
Investigations:
White cell count (WCC) 9.2 × 109/l (elevated eosinophil count) 4.0 - 11.0 × 109/l
Platelets (PLT) 187 × 109/l 150 - 400 × 109/l
Urine Blood +, protein +, WBC +
Which of the following is the most likely cause?
Goodpasture syndrome
Henoch–Schonlein purpura
IgA nephropathy
Explanation
AIN is an important cause of kidney disease and may be the cause of kidney disease in up to 15% of
patients undergoing renal biopsy evaluation. Sulphonamides, penicillins, cephalosporins, diuretics and
NSAIDS are all recognised causes. Discontinuation of the possible causative agent is the treatment of
choice, and slow recovery is possible over the course of around six weeks. It is possible, however, that
223
up to 40% of patients may progress to chronic kidney disease. High-dose prednisolone may improve the
outcome when used in the acute situation. The findings of elevated creatinine, peripheral blood
eosinophilia and red cells, protein and white cells in the urine all support the underlying diagnosis.
Acute tubular necrosis is associated with a significant period of renal hypoperfusion, which may for
example be related to sepsis or haemorrhage.
Goodpasture syndrome
Goodpasture is associated with pulmonary haemorrhage. In Goodpasture syndrome the anti-GBM

antibody is positive.
Henoch–Schonlein purpura
Henoch–Schonlein purpura does not fit with the eosinophilia seen here. Henoch–Schonlein purpura may
have a raised IgA present in the blood.
IgA nephropathy
IgA nephropathy usually follows a benign course, with no significant rise in BP or creatinine in the
majority of cases, out of keeping with what is seen here. In IgA nephropathy there is frequently an
elevated IgA level in the blood.
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A.151.A 50-year-old woman with a diagnosis of systemic sclerosis is seen in Rheumatology Outpatient
Clinic. She has recently had severe Raynaud’s phenomenon, with some infarction of the pulps of two of
her fingertips. This is being treated with prostacyclin infusions, with some success. However, she is now
reporting excessive weakness, fatigue and malaise. She has started high dose corticosteroids a week
earlier.
Besides pallor, the only features on examination are longstanding and relate to her rheumatological
diagnosis. Her blood pressure is 170/90 mmHg.
White cell count (WCC) 8.4 × 109/l-normal differential 4.0 - 11.0 × 109/l
Platelets (PLT) 99 × 109/l 150 - 400 × 109/l
Blood film Schistocytes, increased reticulocytes and helmet cells
Results from today:
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Results from 6 weeks ago:
Investigation Result Normal Range
International normalised ratio (INR) 1.2 <1.1
Activated partial thromboplastin time (APTT)
45 s 35 - 45 s
What is the most beneficial treatment likely to be over the longer term?
Cyclophosphamide
High-dose corticosteroids
Plasma exchange
Stopping the prostacyclin infusions
Explanation
This patient has evidence of a scleroderma renal crisis, with acute kidney disease, significant
hypertension and microangiopathic haemolytic anaemia, evidenced by schistocytes and helmet cells
(red blood cell fragments caused by intravascular shredding of the cells by abnormal fibrin strands).
The treatment most likely to benefit her at this stage is the commencement of ACE inhibitors, which will
improve hypertension and slow further renal impairment.
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The prognosis of this complication used to be abysmal, and it was the main cause of death in systemic
sclerosis. Since the introduction of ACE inhibitors, prognosis is better. Unfortunately, however, many
patients do ultimately progress to end-stage kidney disease and the prognosis remains poor due to
multiple organ involvement.
Cyclophosphamide
Immunosuppressive regimes can be helpful in autoimmune forms of thrombotic thrombocytopenic

purpura (TTP) but are not helpful in systemic sclerosis.
High-dose corticosteroids
Immunosuppressive regimes can be helpful in autoimmune forms of TTP but are not helpful in systemic
sclerosis. High-dose corticosteroids may even worsen a scleroderma crisis.
Plasma exchange
Plasma exchange is sometimes used, along with fresh frozen plasma, in treating TTP to remove causative
antibodies. Plasma exchange may be used for treatment of antiglomerular basement membrane (GBM)
disease and vasculitis. This is to remove autoantibodies that are causative for the condition.
Stopping the prostacyclin infusions
Prostacyclin may actually improve the situation by preserving renal blood flow and can be beneficial in
treating TTP.
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A.152.A 50-year -old Turkish male with a 3-year history of liver cirrhosis secondary to hepatitis C was
admitted with a right gangreneous leg ulcer of 2 months’ duration with pain in his joints. He has
developed pleuritic chest pain with dyspnoea over the last few days. He is on interferon for his liver
disease. He is a non-smoker and does not drink.
On examination, he is dyspnoeic and slightly jaundiced, with widespread spider naevi. He also has
multiple purpuric rashes on his legs with a reticulated pattern to the skin. The leg ulcer is below the right
knee with large ulceration and necrosis.
PH 7.45 7.35–7.45
Arterial pa(O2) 7.2 kPa 10.5–13.5 kPa
Arterial pa(CO2) 3.5 kPa 4.6–6.0 kPa
Chest X-ray (CXR) normal
Sodium (Na+) 135 mmo/l 135–145 mmol/l
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Aspartate transaminase (AST) 200 IU/l10–40 IU/l
Alanine aminotransferase (ALT) 289 IU/l5–30 IU/l
Platelets (PLT) 200 × 109/l 150–400 × 109/l
Urinalysis Protein +++, blood +++
What is the underlying diagnosis?
Multiple myeloma
Rheumatoid arthritis
Waldestrom’s macroglobulinaemia
Cryoglobulinaemia
Hepatocellular carcinoma
Explanation
Cryoglobulinaemia
Cryoglobulins are immunoglobulins that precipitate when blood is cooled while flowing through the skin
and subcutaneous tissues. This leads to small vessel damage in the limbs and deposition on the wall of
small vessels resulting in generalised vasculitis, which presents with a reticulated skin pattern of
microthrombosis and areas of gangrene. Pulmonary embolism, and arterial and venous thrombosis are
common. Vasculitis results in glomerulonephritis, polyneuropathy, Raynaud’s phenomenon and leg
ulcers. Recognised associations are chronic infections such as hepatitis C, systemic lupus erythematosus,
rheumatoid arthritis and Sjögren’s syndrome. Management is with plasma exchange, chemotherapy and
treatment of the underlying condition.
Multiple myeloma
Multiple myeloma is a haematological malignancy. It is characterised by an aberrant clone of plasma

cells producing immunoglobulin (IgG or IgA) or immunoglobulin light chain. It generally presents in the
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over 60s with a male preponderance. Median survival is 3 years. The usual presentation is unexplained
renal impairment with normal-sized kidneys and a bland urine deposit. Bone pain, weakness, fatigue,
easy bruising, pallor and hepatomegaly (20%) are other features.
Rheumatoid arthritis
This history includes large ulcerations which are not a usual feature of rheumatoid arthritis per se,
although patients with rheumatoid arthritis are at increased risk of ulcers.
Waldestrom’s macroglobulinaemia
Waldestrom’s macroglobulinaemia is a type of non-Hodgkin lymphoma. There is a large amount of

macroglobulin produced. Features include weakness, fatigue, weight loss, and chronic oozing of blood
from the nose and gums. Peripheral neuropathy occurs in 10% of patients. Lymphadenopathy and
hepatosplenomegaly are present in 30–40% of cases. Other possible signs and symptoms include
blurring or loss of vision, headache, and (rarely) stroke or coma.
Hepatocellular carcinoma
Although hepatitis C with chronic liver disease is associated with hepatocellular carcinoma; ulceration
and skin necrosis is not a feature of hepatocellular carcinoma.
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A.153.A 68-year-old man with a long history of hypertension and chronic kidney disease (creatinine 190
μmol/l on admission) is admitted for angiography. He is kept in over the weekend and you are asked to
review him three days post procedure. Nursing staff have noticed a purpuric rash over his feet and think
the blood supply to his lower limbs is impaired.
Blood tests reveal erythrocyte sedimentation rate (ESR) of 86, eosinophilia and a creatinine level of 450
μmol/l.
Which of the following stems represents the most likely diagnosis?
Acute vasculitis
Renal artery thrombosis
Renal vein thrombosis
Explanation
This patient has cholesterol embolism, which is most commonly seen in patients with existing arterial
disease who have undergone arterial manipulation. Pre-existing kidney disease, diabetes mellitus and
hypovolaemia at time of procedure are all associated with increased risk. It may be silent or patients can
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present with a peripheral purpuric rash and dusky lower limbs. Associated laboratory tests include
eosinophilia, worsening kidney disease raised erythrocyte sedimentation rate (ESR) and low levels of
complement. Unfortunately, there is no specific treatment shown to be of benefit.
Contrast nephropathy can occur after a contrast load in patients with renal impairment, particularly if
there is co-existent diabetes mellitus. It would not account for the eosinophilia in this case.
Acute vasculitis
Vasculitic rash is classically a palpable purpura that occurs in crops. Vasculitis is a systemic illness and
there would be other associated symptoms such as fever, weight loss, fatigue and joint pains. This
scenario is not a presentation of vasculitis.
Renal artery thrombosis can cause renal impairment due to complete occusion of the renal artery from
thromboemboli, atherosclerosis or fibromuscular disease. However, it would not cause an eosinophilia.
Although renal vein thrombosis can cause renal impairment, it does not present like this scenario. It
occurs most commonly in patients with nephrotic syndrome with heavy proteinuria, hypoalbuminemia,
hypercholesterolemia and peripheral oedema. This is due to a hypercoagulable state.
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A.154.A 56-year-old woman is admitted to the ward. She developed a sore throat a week ago and the
general practitioner commenced her on penicillin. She now feels more unwell and has developed
generalised joint pains and shivering attacks with a rash. For bipolar disorder she has also been taking
lithium for the past two years.
On physical examination, her blood pressure is 140/80 mmHg, and chest and abdominal examination is
unremarkable.
Investigations:
Platelets (PLT) 282 x 109/l 150 - 400 × 109/l
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Urine microscopy Eosinophils, red blood cells
Acute tubulo-interstitial nephritis
Lithium toxicity
Systemic lupus erythematosus
Explanation
Acute tubulo-interstitial nephritis
Acute tubule-interstitial nephritis presents with arthralgia, skin rashes, fever and kidney disease. It is
mainly caused by drug reactions, commonly non-steroidal anti-inflammatory agents, antibiotics
(penicillin, sulphonamides, cephalosporins, rifampicin, quinolones), allopurinol and phenytoin.
Investigations can reveal eosinophilia, urinary eosinophils and raised immunoglobulin E (IgE) levels.
There would not be eosinophils in the urine. There would be haematuria. IgA nephropathy is the most
common primary glomerulonephritis in the world.
Lithium toxicity
Lithium toxicity presents with neurological symptoms of tremor, weakness, and dysarthria. It can also
cause nephrogenic diabetes insipidus.
Although blood and protein in the urine after an episode of sore throat may suggest post-streptococcal
glomerulonephritis, there would not be any eosinophils in the urine.
Systemic lupus erythematosus
Systemic lupus erythematosus can cause a chronic tubule-interstitial nephritis. Apart from joint pains
there are no other symptoms of SLE, such as malar rash, alopecia or weakness.
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A.155.A 42-year-old man, who has received long-term haemodialysis for chronic kidney disease due to
type I diabetes, presents for review. He has been suffering pain and tingling in both hands, which
particularly comes on during the early hours of the morning. He has also noticed shoulder pain over the
past few months. He is dialysed using a conventional cellulose acetate dialysis membrane.
On physical examination, there is no obvious structural deformity in the hands. An X-ray of both hands is
ordered and it shows a destructive arthropathy with periarticular cystic bone radiolucencies.
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Which of the following diagnoses fits best with this clinical picture?
Uraemic neuropathy
AA amyloidosis
AL amyloidosis
Beta 2 microglobulin amyloidosis
Explanation
Beta 2 microglobulin amyloidosis
Conventional dialysis membranes cannot clear substances with molecular weights of > 200 daltons.
These allow accumulation of beta-2 microglobulin, which has a molecular weight of 11,800 daltons. A
normal functioning kidney would filter this in the glomerulus and it is catalysed in the proximal tubule. In
some patients dialysed for more than 5 years on a conventional membrane, accumulation of beta-2
microglobulin amyloid leads to carpal tunnel syndrome, tenosynovitis, scapulohumeral arthropathy,
bony cysts and even pathological fractures. GI or cardiovascular manifestations may occur. The
mainstays of management are moving towards polyacrylnitrile or polysulphone dialysis membranes,
which are higher flux and allow beta-2 microglobulin to be removed, or moving to transplant if a kidney
is available. Other forms of renal amyloidosis occur in association with systemic inflammation, such as in
rheumatoid arthritis, or haematological malignancies, such as myeloma. Aggressive use of secondary
agents may slow deposition of amyloid in inflammatory conditions, as may chemotherapy in conditions
such as myeloma.
AL amyloidosis
AL amyloidosis is due to immunoglobulin light chain production. The musculoskeletal manifestations

may include muscle weakness and myopathy with arthropathy and osteopathy. There is no history in
this case to suggest that this patient has myeloma, such as light chains in the urine or immunoparesis.
Uraemic neuropathy
Similar to diabetic neuropathy, uraemia may lead to neuropathy and hence a neuropathic arthropathy
may result in dialysis patients. This is rare and there would be joint deformity with X-ray findings of joint
destruction rather than bony cystic changes.
Diabetic nephropathy is likely to be the underlying renal disease in this case. However, it would not
produce the arthropathy symptoms and signs. Diabetic patients may have diabetic neuropathy that
would cause loss of sensation of peripheral nerves, which may then lead to Charcot joints. This would be
more common in the feet and also there would be joint deformity, because the joint and bony
structures are destroyed.
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AA amyloidosis
AA amyloidosis may be secondary to causes such rheumatoid arthritis, ankylosing spondylitis, psoriatic
arthritis and juvenile arthritis. This is due to the chronic inflammation. These rheumatic conditions may
be a cause of joint pain. However, there is no history to suggest rheumatic disease in this scenario. Also,
there may be characteristic X-ray signs for these rheumatoid conditions; for instance, X-ray of the hands
may show soft tissue swelling and early erosions in the proximal interphalangeal joints. It would not be
the bony cystic destruction as seen here.
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A.156.A 40-year-old woman, who has suffered from Crohn’s disease for some 18 years, comes to the
Emergency Department complaining of left loin pain. Her Crohn’s disease is currently quiescent. She is
currently taking daily azathioprine and has undergone three previous small bowel resections over the
past five years.
On examination, her blood pressure is 118/72 mmHg; pulse is 95 bpm and regular, temperature is 37.6
°C. You confirm left loin pain on abdominal palpation, and she has a midline laparotomy scar.
Investigations:
Platelets (PLT) 191 × 109/l 150–400 × 109/l
C-reactive protein (CRP) 17 mg/l 0–10 mg/l
Urine Protein –, Blood +++
Which of the following is the most likely cause of her presentation?
Cystine renal stone Oxalate renal stone Struvite renal stone Urate renal stone Urinary tract infection
Explanation
Oxalate renal stone
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Oxalate renal stones are common in patients with short bowel syndrome. Oxalate is absorbed through
the small bowel. When this is resected, there is an increase in colon absorption of oxalate due to
increased colonic permeability. Oxalate is neither stored or metabolised and therefore urinary excreted.
The excess urinary oxalate supersaturates within the kidneys to form kidney stones. Treatment options
include increasing fluid intake, reducing oxalate intake and supplementation with calcium citrate.
Cystine renal stone
Cystine renal stones are seen in patients that have an inherited increase in urinary citrate excretion. The
clinical scenario is more in keeping with short bowel syndrome and therefore is more likely to be
associated with oxalate stones.
Struvite renal stone
Struvite renal stones are often seen in patients with proteus urine tract infections. This is not consistent
with the history in this case.
Urate renal stone
Urate stones are seen in patients with gout. This is not consistent with the history given in the clinical
scenario.
Urinary tract infection
There is no history of urinary symptoms in the clinical scenario and the urinalysis is not consistent with a
urinary tract infection.
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A.157.An 18-year-old A-level student comes to the clinic, complaining of amenorrhoea. There is no
history of weight loss. She has been recently referred to the dentist for assessment of badly eroded
teeth.
On examination, her parotid glands are enlarged, and scratch marks are noticed over the dorsum of the
knuckles. The rest of the examination is normal.
She is an active girl, who runs 5 miles daily. Her body mass index (BMI) is 17. Her blood pressure (BP) is
105/70 mmHg.
Her blood results showed:
pH 7.5 7.35 - 7.45
233
Which of the following conditions is the most likely diagnosis?
Addison’s disease
Conn syndrome
Cushing syndrome
Eating disorder
Long-term diuretic abuse
Explanation
Eating disorder
The acid–base disturbance shown is a metabolic alkalosis. Recurrent vomiting ± laxative abuse may well
have led to the hypokalaemia and raised bicarbonate levels seen here, with the knuckle callusing also
supporting the diagnosis.
Addison’s disease
Hypokalaemia is not a feature of Addison’s disease. Addison’s disease is a primary adrenal insufficiency
with low cortisol. Symptoms include abdominal pain, weakness and weight loss. Darkening of the skin
due to increased pigmentation may occur in non-sun-exposed areas. Blood results may show high
calcium, low glucose, low sodium, high potassium and eosinophilia.
Conn syndrome
Conn syndrome is associated with hypertension. This syndrome is caused by an excess production of
aldosterone by the adrenal glands, with low renin. There may be few symptoms. High blood pressure,
with poor vision and headaches, may be another presentation. Occasionally, there may be muscle
weakness, spasms and paraesthesiae. Low potassium is classically described; however, it is only present
in about a quarter of patients.
Cushing syndrome
Cushing syndrome is effectively ruled out by her BMI. It presents with hypertension, abdominal obesity,
thin arms, stretch marks, a rotund face and fragile skin with poor wound healing. Women may also
complain of hirsutism and irregular periods.
Long-term diuretic abuse
Long-term diuretic abuse may coexist with an eating disorder, but given her BMI is only 17, it is vomiting
that is the likely predominant factor.
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A.158.A 35-year-old man with a history of mild asthma comes to your clinic with 10 days’ history of
malaise, fever and tightness in the chest. On further inquiry, he also mentions shortness of breath,
weight loss and swelling in the legs and scrotum. There is no significant past medical history.
234
On examination, his temperature is 38 °C, blood pressure is 175/90 mmHg, pulse is 77 beats/min and
respiratory rate 14 breaths/min. Physical examination confirms bilateral pedal and scrotal oedema.
There are also scattered bilateral wheezes. Chest X-ray is unremarkable.
Chloride (Cl-) 99 mmol/l 98-106 mmol/l
Eosinophils Mildly elevated
Urinalysis reveals the following:
Specific gravity 1.020
Protein 2+
Blood 4+
Red cell casts Many
24 hour protein 1.9 g
Which laboratory finding would support the most likely diagnosis?
Anti-glomerular basement membrane antibody
Anti-streptolysin titre
c-ANCA (anti-neutrophil cytoplasmic antibodies)
p-ANCA
Serum immunoglobulin A (IgA) levels
Explanation
p-ANCA
This patient has eosinophilic granulomatosis with polyangiitis – a nephritic syndrome associated with
eosinophilia and asthma. It is associated with positive p-ANCA. The patient has nephritic syndrome as
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evidenced by hypertension, oedema and haematuria. Asthma is suggested by his chest tightness and
shortness of breath.The American College of Rheumatology has defined the criteria for a diagnosis of
eosinophilic granulomatosis with polyangiitis:
Asthma
High numbers of eosinophils
Sensory neuropathy
Lung abnormalities
Sinus problems such as abnormal growths (polyps)
A tissue sample showing a blood vessel with eosinophils around it.
A person with eosinophilic granulomatosis with polyangiitis may have some or all of these symptoms.
Besides the criteria for diagnosis listed above, the person with eosinophilic granulomatosis with
polyangiitis may have:
A nodular or purpuric skin rash
Renal, intestinal or cardiac involvement
Malaise
Loss of appetite (anorexia) and weight loss
Other symptoms specific to the part of the body affected.
Internationally: incidence is approximately 2.5 cases per 100,000 adults per year. The principal causes of
morbidity and mortality are myocarditis and myocardial infarction secondary to coronary arteritis. With
treatment, the 1-year survival rate is 90% and the 5-year survival rate is 62%. Overall, without
treatment, the 5-year survival rate is about 25%. Glucocorticoids alone usually are adequate for
treatment of eosinophilic granulomatosis with polyangiitis. Cytotoxic drugs are necessary in less than
20% of patients. Major life-threatening organ involvement may require treatment with pulse doses of
intravenous (IV) corticosteroids as well as other cytotoxic agents. Other treatments include intravenous
immune globulin, interferon-alpha and plasma exchange. Plasma exchange has not improved the course
of the disease. Complications of eosinophilic granulomatosis with polyangiitis depend on the specific
organ system involvement.
Serum immunoglobulin A (IgA) levels
IgA may be elevated in IgA glomerulopathy, which is a common cause of glomerulonephritis presenting
with haematuria.
Anti-glomerular basement membrane antibody
Anti-glomerular basement membrane antibody is found in anti-GBM disease. This would present with
haemoptysis and rapidly progressive glomerulonephritis.
Anti-streptolysin titre
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Anti-streptolysin titre would be useful in post-streptococcal glomerulonephritis. This would be a cause
of haematuria.
c-ANCA (anti-neutrophil cytoplasmic antibodies)
C-ANCA is present in granulomatosis with polyangiitis (GPA).
GPA is a rare small and medium vessel vasculititis with a predominance of upper and lower respiratory
tract symptoms with chronic sinusitis being the most common initial complaint and is classically
associated with nose bleeds. It can affect multiple systems.
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A.159.A 54-year-old man with chronic kidney disease is managed by peritoneal dialysis. He has been on
dialysis for the past three years and has a history of long-standing type 1 diabetes. He has suffered an
anterior MI some 3 years earlier and is considered unfit for parathyroid surgery.
On examination in the clinic, his BP is 152/84 mmHg, with pulse 70/min and regular. There are no
unexpected findings.
Investigations:
Platelets (PLT) 180 × 109/l 150 - 400 × 109/l
Parathyroid hormone (PTH) 118.2 pmol/l 0.9 - 5.4 pmol/l
Which of the following best treats the range of abnormalities associated with tertiary
hyperparathyroidism?
Calcium and vitamin D
Cinacalcet
Low-phosphate diet
Sevelamer
237
Vitamin D
Explanation
Cinacalcet
Vitamin D (1-alpha-calcidol) is used in the early stages of hyperparathyroidism related to kidney disease
when calcium levels are low–normal and PTH is above twice the upper limit of the normal range.
In this case, we have both a raised serum phosphate with hypercalcemia and marked elevated PTH
suggestive of tertiary hyperparathyroidism. In this respect the calcimimetic cinacalcet may be used in
the treatment of tertiary hyperparathyroidism, in conjunction with other measures such as a low-
phosphate diet and a suitable phosphate binder such as sevelamer, but only if the patient is unable to
undergo surgery.
NICE guidelines recommend that cinacalcet is used only in patients who have refractory secondary
hyperparathyroidism and who are resistant to medical therapy for high parathyroid hormone levels (>85
pmol/l) with a normal or high adjusted serum calcium level and where surgical parathyroidectomy is
contraindicated. The parathyroid hormone level is checked four months later and, if there is <30%
reduction, then cinacalcet should not be continued. There is no evidence from large clinical trials that
cincalcet reduced cardiovascular endpoints compared to placebo.
Calcium and vitamin D
Vitamin D (1-alpha-calcidol) is used in the early stages of hyperparathyroidism related to kidney disease,
Low-phosphate diet
This would be used in conjunction with cinacalcet. It is unlikely to have an effect on its own in this
scenario.
Sevelamer
This would be used in conjunction with cinacalcet. It is unlikely to have an effect on its own in this
scenario.
Vitamin D
Vitamin D (1-alpha-calcidol) is used in the early stages of hyperparathyroidism related to kidney disease,
************************************************************************************
A.160A 32-year-old man attends the clinic as an urgent referral from his GP, he has been suffering from
a cough, fevers and increasing lethargy over the past few days, but most recently has begun to suffer
from haemoptysis.
When he makes it to the clinic, he looks quite unwell, with a raised respiratory rate of 31/min. His BP is
155/82 mmHg and he has crackles at both lung bases on auscultation.
Investigations:
238
haemoglobin 109 g/l 135—175 g/l
White cell count 12.1 × 109/l 4–11 × 109/l
Platelets 185 × 109/l 150–400 × 109/l
Na+ 141 mmol/l 135–145 mmol/l
K+ 5.9 mmol/l 3.5–5.0 mmol/l
c-ANCA Negative
Erythrocyte sedimentation rate 12 mm/h 0–10 mm in the 1st hour
Chest X-ray Patchy perihilar and basal infiltration
PFTS Transfer factor increased
Which of the following tests would you do next?
Anti-nuclear antibodies
Anti-smooth muscle antibodies
P-ANCA
Anti-GBM antibodies
Anti-Ro antibodies
Explanation
Anti-GBM antibodies
The presentation with worsening haemoptysis due to pulmonary haemorrhage, and acute kidney
disease, is typical of Goodpasture syndrome.
P-ANCA
P-ANCA shows a perinuclear staining pattern and the most common p-ANCA target is myeloperoxidase,
a neutrophil granule protein. P-ANCA is associated with ulcerative colitis, eosinophilic granulomatosis
with polyangiitis, microscopic polyangiitis and focal necrotising and crescentic glomerulonephritis.
Anti-nuclear antibodies
The anti-nuclear antibodies would be negative in this case. This test is used to identify connective tissue
diseases such as rheumatoid arthritis, systemic lupus erythematosus (SLE), polymyositis and
scleroderma. It is an auto-antibody that binds to contents of the cell nucleus.
239
Anti-smooth muscle antibodies
Anti-smooth muscle antibodies are antibodies that are formed against smooth muscle. They are directed
against actin, troponin and tropomyosin. These antibodies are found in autoimmune hepatitis.
Anti-Ro antibodies
Anti-Ro antibodies are associated with many autoimmune diseases such as SLE, neonatal lupus and
primary biliary cholangitis. The presence of anti-Ro antibodies in pregnant women with SLE is associated
with heart block in the fetus..
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A.161.A 38-year-old woman, who underwent a live related renal transplant for chronic kidney disease
secondary to chronic pyelonephritis 12 weeks previously, attends the clinic for routine follow up. She is
taking tacrolimus and mycophenolate mofetil (MMF).
Her urea and electrolytes are below:
She had last been seen in clinic 2 weeks previously, when her urea was 4.2 mmol/l and creatinine 98
µmol/l. She is clinically well and asymptomatic.
On examination, she was apyrexial and normotensive. Her transplant site was non-tender with no
swelling and there were no other signs to be found.
Which of the following is the next radiological investigation of choice?
CT abdomen Doppler ultrasound scan of the transplant site Intravenous urography
Plain abdominal film
Renal angiography
Explanation
Doppler ultrasound scan of the transplant site
Graft dysfunction is an early complication of renal transplantation and can present asymptomatically
with a rising serum creatinine. It is important to exclude sepsis in these patients, even if they are
asymptomatic and apyrexial, and cultures should always be checked. Tacrolimus levels should also be
measured, as this drug can be directly nephrotoxic. Mycophenolate mofetil (MMF) does not need levels
240
monitoring. Doppler ultrasound of the transplant site should be arranged to look for any obstruction,
arterial or venous occlusion and is the initial radiological investigation of choice.
CT abdomen
CT abdomen will be normal in graft dysfunction and will not show any abnormality in this case. In
addition, you would not want to expose the patient to unnecessary radiation.
Intravenous urography
Intravenous urography will show any obstruction in the urinary tracts. It would not be helpful in this
situation as the first step is to carry out a simple Doppler ultrasound of the transplant site. If there was a
ureteric obstruction you would expect some abdominal pain which is absent in this case.
Plain abdominal film
A plain abdominal film would not be helpful in graft dysfunction. It may be used to diagnose intestinal
obstruction. This patient has no abdominal pain which would indicate the need for a plain abdominal
film to be carried out.
Renal angiography
Renal angiography or biopsy may be needed in due course, but it is not a first-line investigation. This
investigation may be used to diagnose any thrombosis causing renal graft dysfunction.
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A.162.A 22-year-old man, with a history of nephrotic syndrome due to minimal change disease, comes
to the clinic because he feels more poorly than usual. He has noticed that despite his usual dose of
furosemide, his leg oedema is worsening and for the past few days he has suffered from some vague left
flank pain.
On examination, his BP is 155/92 mmHg and you confirm the left flank pain.
Platelets (PLT) 232 × 109/l 150–400 × 109/l
Creatinine (Cr) 280 μmol/l (190 μmol/l at the clinic a month earlier) 50–120 µmol/l
24 hour urinary protein 3 g (up from 1.6 g last time it was assessed)
Cholesterol 6.9 mmol/l < 5.2 mmol/l
241
Nephrolithiasis
Left sided renal cysts
Explanation
Patients with nephrotic syndrome are known to suffer from a hypercoagulable state and, as such, are
more prone to suffering from renal vein thrombosis. This picture of vague flank pain, coupled with
worsening creatinine and proteinuria, is fairly typical. Initial investigation with ultrasound is likely to
reveal a swollen, oedematous kidney on the left when compared to the other side. Long-term
anticoagulation may be considered in this patient for prophylaxis against further thromboembolic
events.
Renal artery thrombosis causes complete blockage to the blood flow through the main arteries. This is a
rare cause of acute kidney injury. The clinical manifestation depends on the amount of renal tissue being
supplied by the renal artery being blocked by the embolism. It may range from mild increase in serum
creatinine to complete anuria. Most patients experience a sudden onset of abdominal, flank or back
pain. Renal artery thrombosis is not associated with minimal change disease/nephritic syndrome. The
major causes of renal artery thrombosis include thromboemboli (systemic clot emboli) and
atheroemboli (cholesterol emboli).
Pyelonephritis may give left flank pain, but there would be other symptoms such as fever and chills or
rigors. You would expect the white cell count to be raised. There is no other history to suggest acute
pyelonephritis.
Nephrolithiasis
Nephrolithiasis typically causes renal colic when passed. Patients are usually male and in their 4th
decade. There would be renal angle tenderness and haematuria, which is often macroscopic with
urinary symptoms such as dysuria and frequency of urination. Predisposing factors include dehydration,
exercise and high protein load. There is no history to support renal stones in this case.
Left sided renal cysts
242
Renal cysts may give rise to left flank pain, especially with haemorrhage or rupture of the cyst. There is
no increase in incidence of renal cysts in minimal change disease and there is no history in this case to
suggest that renal cysts are present.
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A.163.A 44-year-old man has been referred to the nephrologists with a serum creatinine of 210 µmol/l.
He has a 15-year history of osteoarthritis.
One year ago, he was diagnosed with diabetes mellitus and essential hypertension.
His current medications include ramipril 10 mg, aspirin 75 mg, diclofenac 150 mg and atorvastatin 10 mg
daily.
On examination, his BP was 110/70 mmHg. Systemic examination was normal.
Investigations:
Platelets (PLT) 210 × 109/l 150 - 400 × 109/l
HbA1c 37 mmol/mol (5.5 %) 9.29 - 53.0 mmol/mol (3 - 7 %)
Echocardiogram normal
24-h urinary protein collection 0.9 g < 0.2 g
Renal ultrasound scan reveals unobstructed echogenic kidneys, right kidney 9.4 cm, left kidney 8.8 cm
What is the most likely cause of his renal impairment?
Renovascular disease Hypertensive nephrosclerosis Analgesic nephropathy Herbal nephropathy

243
Explanation
The patient in question has chronic kidney disease due to the long-term use of non-steroidal anti-
inflammatory drugs (NSAIDs). Chronic NSAID abuse leads to renal injury due to renal papillary necrosis
and chronic interstitial nephritis. The degree of renal injury correlates with the duration of use and
analgesic load. There is no specific treatment apart from avoidance of NSAIDs. In this situation, the
proteinuria raises the possibility of a membranous nephritis pattern of disease.
Renovascular disease
As he is on ACE inhibitors, the absence of bruits and symmetrical kidneys make renovascular disease
unlikely.
Hypertensive nephrosclerosis
The duration of diabetes mellitus and the normal blood pressure with no evidence of other end-organ
damage (normal fundi, no LVH on voltage criteria in the ECG) excludes hypertensive nephrosclerosis.
Herbal nephropathy
There is no clue in the history that he has been taking over-the-counter herbal medications. Some
Chinese herbs have been implicated in the development of rapidly progressive kidney disease due to
interstitial nephritis and progressive tubulo-interstitial fibrosis.
The duration of diabetes mellitus and the normal blood pressure with no evidence of other end-organ
damage (normal fundi, no LVH on voltage criteria in the ECG) excludes diabetic nephropathy.
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A164.A 50-year-old woman received a cadaveric renal transplant 8 weeks ago for diabetic nephropathy.
Upon discharge, her serum creatinine was normal. She attended the Transplant Follow-up Clinic.
You understand that her GP prescribed an antibiotic for a respiratory tract infection during the last 2
weeks, and she is penicillin allergic. Her BP is 135/72 mmHg, and her transplant scar is healing well.
Investigations:
White cell count (WCC) 9 × 109 /l 4–11 × 109/l
Platelets 350 × 109 /l 150–400 × 109/l
244
Which one of the following is most likely to explain the rise in creatinine?
Acute ciclosporin toxicity
Acute graft rejection
Chronic graft rejection
Vascular thrombosis of the transplant vessels
Explanation
Acute ciclosporin toxicity
Ciclosporin toxicity can occur either as acute or chronic in otherwise well patients. Given the recent
history of antibiotic use for respiratory tract infection in a patient who is penicillin allergic, it’s likely she
was exposed to erythromycin or clarithromycin. These two macrolides are potent inhibitors of CYP3A4,
and as such lead to elevated levels of ciclosporin. Given its narrow therapeutic window, a relatively
small elevation in ciclosporin can result in renal toxicity. Erythromycin and clarithromycin should be
avoided in patients treated with ciclosporin if at all possible. Azithromycin is associated with a lesser
degree of 3A4 inhibition.
Transplant recipients are immunosuppressed, and therefore they are susceptible to a range of
infections. Patients who are at risk of CMV are generally started on prophylaxis such as
valganciclovir/ganciclovir – and therefore these infections are less likely. There may be a low white cell
count associated with CMV infection, which is not demonstrated in this case.
Acute graft rejection
Acute graft rejection is a sudden deterioration in renal function associated with specific
immunopathological changes, as categorised according to histological criteria called the Banff
classification. The risk is greater up to 2 weeks postoperatively, and it occurs in about 30–50% of all
transplants. Classically the presentation includes fever, painful graft and oligo-anuria, but this is now
rare. It now usually presents with asymptomatic rise in creatinine. The recent infection with antibiotic
makes ciclosporin toxicity more likely in this case.
Chronic graft rejection
Both immunological and non-immunological factors play a role in graft dysfunction and loss beyond 3
months. It is used to describe the appearances found with a progressively declining graft function. At 2
years post-transplant, 70–90% of grafts will demonstrate features of chronic allograft nephropathy. In
this scenario it is too early to be chronic graft rejection.
Vascular thrombosis of the transplant vessels
245
Transplant renal artery thrombosis may occur, and would present with reduction in renal function,
hypertension and salt and water retention. Doppler ultrasound and angiography will diagnose any
thrombosis. The infection history is not relevant in vascular thrombosis, so makes this diagnosis less
likely in this case.
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A.165.A 62-year-old patient who has type I diabetes and end-stage renal failure on haemodialysis comes
to the Rheumatology Clinic for review. He has been suffering from increasing joint pain affecting his
shoulders, bilateral carpal tunnel syndrome and flexor tenosynovitis affecting both hands. He takes a
number of medications for blood pressure and calcium homeostasis, and insulin for his diabetes.
On examination, his blood pressure is 144/82 mmHg and pulse 80 bpm and regular. His body mass index
(BMI) is 23 kg/m2. You confirm changes in the hands consistent with carpal tunnel syndrome.
Investigations:
Platelets (PLT) 191 × 109/l 150–400 × 109/l
HbA1c 54.10 mmol/mol (7.1%) (9.29-53.0 mmol/mol, 3–7%) < 53 mmol/mol (< 7.0%)
Ferritin 210µg/l 20–250 µg/l
Calcium (Ca2+) 2.3 mmol/l 2.20–2.60 mmol/l
Aluminium overload
Beta-2 microglobulin (B-2m) amyloidosis
Haemochromatosis Hypovitaminosis D Secondary hyperparathyroidism
Explanation
Beta-2 microglobulin (B-2m) amyloidosis
B-2m amyloidosis is seen after five years or more of haemodialysis and most commonly affects the axial
skeleton. It is also associated with tenosynovitis, carpal tunnel syndrome and shoulder pain. Visceral
246
involvement in B-2m amyloidosis is very rarely seen. The disease can be prevented using high-flux
dialysers, and renal transplantation halts progression of the disease.
Aluminium overload
Aluminium overload causes osteomalacia, which is not the clinical picture here, given that the calcium
level is in the normal range. Symptoms of bone pain and proximal muscle weakness are also absent, as is
iron replacement-resistant microcytic anaemia.
Haemochromatosis
Haemochromatosis leads to iron overload and is usually associated with a significant rise in ferritin level
(which is normal here). Other features such as increased skin pigmentation, diabetes and symptoms
suggestive of pseudogout are also not reported in the scenario.
Hypovitaminosis D
Low vitamin D leads to the development of osteomalacia, which is associated with proximal muscle
weakness and long bone pain. Although low vitamin D is a feature of renal failure, the normal calcium
level counts against that here.
Secondary hyperparathyroidism
Secondary hyperparathyroidism is a feature of renal impairment and it does lead to bone pain, although
the other features seen here such as carpal tunnel syndrome are not suggestive of the disorder.
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A.166.A 24-year-old man presents with macroscopic haematuria. He is seen a few days following an
upper respiratory tract infection.
Urine is positive for blood and protein, but no growth is seen after urine culture. Blood pressure is
normal. Renal biopsy reveals focal proliferative glomerulonephritis with mesangial IgA deposition.
Which of the following diagnoses fits best with the clinical picture?
Anti-GBM disease
IgA nephropathy
Minimal-change disease
Explanation
IgA nephropathy
IgA nephropathy is the most common form of glomerulonephritis seen worldwide, and consists of focal
proliferative glomerulonephritis with mesangial deposits of IgA. It is thought to occur due to an
247
exaggerated immune response to viral or other antigens, and may follow respiratory or gastrointestinal
infection. They may present with asymptomatic microscopic haematuria or macroscopic haematuria,
proteinuria occurs and 5% may be nephrotic. Good prognosis is associated with normal blood pressure,
renal function and absence of proteinuria at presentation.
Anti-GBM disease
Anti-GBM disease would present with haemoptysis and rapidly progressive kidney disease. Anti-GBM
antibodies would be present. There would be protein and blood in the urine.
The main distinguishing feature here from post-streptococcal glomerulonephritis is the presence of
mesangial IgA. Although small amounts of IgA may be present in post-streptococcal GN, significant
quantities suggest a different diagnosis. Post-streptococcal glomerulonephritis also tends to have a
longer lead time than that seen here.
cANCA is positive in granulomatosis with polyangiitis, and there is an association with ear nose and
throat symptoms in addition to rapidly progressive kidney disease.
Minimal-change disease
Minimal-change disease is the most common cause of nephrotic syndrome in children. Haematuria is
not a feature.
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A.167.A 65-year-old woman is admitted as an emergency following her renal function having been
assessed 1 month after starting ramipril therapy for hypertension.
Examination in the clinic reveals abdominal and carotid bruits; she is clinically euvolaemic. Her blood
pressure (BP) is 156/88 mmHg. Electrocardiogram (ECG) on admission is unremarkable.
On admission:
One month previously:
248
Which one of the following represents the most appropriate initial step in the management of this
patient?
Add bendroflumethiazide 2.5 mg po daily
Advise a low-potassium diet
Observe renal function over the next week
Start calcium resonium
Stop ramipril therapy
Explanation
Stop ramipril therapy
This clinical scenario is highly suggestive of underlying renal artery stenosis. ACE inhibitors result in a
decrease in efferent renal arteriolar blood pressure and a deterioration in serum creatinine in patients
with renal artery stenosis. Magnetic resonance angiography is the definitive investigation.
The key intervention for this patient is to stop the ramipril and substitute another antihypertensive
drug, such as a calcium channel antagonist if required. Depending on local protocol, most physicians
check renal function one week and one month after initiating ACE inhibitors or angiotensin II receptor
blockade.
Her potassium is likely to fall within a day or two of stopping the ramipril therapy; given the fact that her
ECG on admission is unremarkable, no acute intervention for her potassium is warranted here.
Add bendroflumethiazide 2.5 mg po daily
She is clinically euvolaemic; therefore, there is no indication to start a diuretic. Furthermore, as the
angiotensin-converting enzyme (ACE) inhibitor is contributing to the hyperkalaemia, it is more important
to stop this medication.
Advise a low-potassium diet
A low-potassium diet will not correct the underlying cause, which is deterioration in renal function due
to the ACE inhibitor. A low-potassium diet will help with some control.
Observe renal function over the next week
The renal function will stay the same or worsen if the patient is continued on the ACE inhibitor, so there
is no rationale in observing the renal function. The renal function will not be altered unless the offending
agent is stopped.
Start calcium resonium
249
Calcium resonium acts to prevent potassium absorption by the gut. It has side-effects of constipation.
Although calcium resonium may control any potassium rise, the underlying cause is not resolved;
therefore, the potassium will not be entirely corrected. Calcium resonium alone will be unpredictable in
the level of control of potassium and is not fast-acting.
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A.168.A 22-year-old saleswoman is admitted to the emergency department with a 2-day history of
generalised headache, vomiting, unsteadiness, dizziness and blurred vision.
The unsteadiness has been progressive over a number of weeks, and colleagues have mentioned to her
that her speech has become slurred. She has also lost 1 stone in weight within the last month, although
she has attributed this to pressure at work, as she feels increasingly nauseated and tired.
She has a past medical history of epilepsy diagnosed at the age of 8 years and has had photocoagulation
of her left eye for multiple angioma. Her current medication includes phenytoin and the oral
contraceptive pill.
On examination she is lethargic and has marked dysarthria. Her blood pressure is 143/89 mmHg and
pulse 82/min. Fundoscopy examination reveals bilateral papilloedema and evidence of previous
photocoagulation. She has reduced visual acuity of 6/9 in both eyes. Pupils are equal and reactive to
light. The rest of the cranial nerves are normal. On examination of the upper and lower limbs, tone,
power and reflexes all appear normal; however, there is a marked intention tremor bilaterally and her
gait is broad based and ataxic. Sensory examination appears normal.
Chest examination appears normal, but on abdominal examination there is a palpable right flank mass.
Her magnetic resonance scan of the brain reveals bilateral haemangioma in both cerebellar hemispheres
and enlarged lateral and third ventricles.
Red blood cells (RBC) 7.0 × 1012 3.9–5.6 × 1012
Platelets (PLT) 399 × 109/l 150–400 × 109/l
250
Phenytoin 81 µmol/l 40–80 µmol/l
Urine Blood+++, protein++
Given the above findings, what is the likely underlying diagnosis in this patient?
Phenytoin toxicity Renal carcinoma with cerebellar metastases Von Hippel–Lindau disease (VHL)
Neurofibromatosis type II Tuberous sclerosis
Explanation
Von Hippel–Lindau disease (VHL)
This patient has features in keeping with a diagnosis of VHL. Diagnosis is based on haemangioblastomas
of the central nervous system or retina and the presence of one associated VHL tumour (renal cell
carcinoma, islet cell tumour or adenoma).
This patient has bilateral cerebellar haemangioma, which have led to compression of the fourth
ventricle, and hydrocephalus manifest by headache, papilloedema and vomiting.
There is a history given of retinal angioma, and it appears she has developed a renal cell carcinoma with
secondary polycythaemia as evidenced by right flank mass, haematuria and deranged biochemistry.
Phenytoin toxicity
This is a cause of ataxia, headache and vomiting but is not the cause in this case and cannot account for
all the features seen on examination and brain imaging.
Renal carcinoma with cerebellar metastases
The patient likely has renal cell carcinoma with polycythaemia (ectopic EPO production), but there are
features here to suggest this is just part of the broader syndrome of VHL.
Neurofibromatosis type II
NF type II causes acoustic schwannomas, which may be bilateral, and meningiomas with fewer skin
stigmata compared to type I.
Tuberous sclerosis
This presents with seizures, retinal hamartomas and calcified tubers on imaging. In some cases patients
can develop cerebellar astrocytomas. Renal cell carcinomas are very rare, although patients may
develop renal angiomyolipomas. Almost all patients have skin manifestations such as facial
angiofibromas, periungal fibromas and hypomelanotic macules (ash leaf spots).
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A.169.A 50-year-old woman with a 4-year history of type 2 diabetes was referred to the Renal Clinic. She
was commenced on 5 mg of lisinopril five weeks ago for hypertension. Otherwise, she is well, without
any symptoms. Her diabetes is controlled with twice-daily (bd) mixed insulin.
251
On examination, her blood pressure (BP) is 153/81 mmHg and her pulse rate is 75 bpm and regular.
There is minor loss of sensation affecting both feet.
Electrolytes five weeks ago:
Electrolyte results in clinic today are as follows:
HbA1c 54.10 mmol/mol (7.1%) 9.29 - 53.0 mmol/mol (3 - 7%)
What is the most likely cause for the electrolyte change?
Progression of diabetic nephropathy
Starting angiotensin-converting enzyme (ACE) inhibitor treatment
Hypertension
Explanation
Starting angiotensin-converting enzyme (ACE) inhibitor treatment
When starting an ACE inhibitor, a non-progressive rise in Cr levels of 20–30% is normal. This is observed
in almost all intervention trials, with respect to the renin–angiotensin system, and is associated with an
initial reduction in BP; it has no prognostic significance. It is due to the ACE inhibitor affecting the
glomerular perfusion. It is reversed once the ACE inhibitor is stopped.
Guidelines generally suggest checking serum creatinine levels one week, one month and then
periodically, after starting an ACE inhibitor.
252
Progression of diabetic nephropathy
Due to it being five weeks, this is too short to be progression. Diabetic nephropathy is not generally a
rapidly progressive disease; it progresses generally over years.
A progressive rise of > 30% in Cr levels should prompt consideration of renal artery stenosis. In this case,
the rise is smaller than this. Renal artery stenosis can be diagnosed with renal Doppler or renal magnetic
resonance angiography (MRA).
DKA would present with vomiting and abdominal pain. It would not cause a rise in Cr levels, unless there
was dehydration or hypoperfusion.
Hypertension
There are no signs of malignant hypertension such as headache, vomiting or visual disturbances. The BP
is not particularly high in this case.
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A.170.A 28-year-old lawyer, originally from Singapore and who has recently returned from the Far East,
presents complaining of passing bright red urine, facial puffiness and malaise. He also has a severe sore
throat, which began 2 or 3 days before he noticed the red urine. His past medical history includes hay
fever.
On physical examination, his blood pressure is 130/80 mmHg, and chest and abdominal examination is
unremarkable.
Urinalysis:
Protein 2+
Blood 3+
Nitrites Negative
Leucocytes Negative
The likely diagnosis is?
Focal segmental glomerulonephritis
Schistosomiasis
253
Explanation
Mesangioproliferative glomerulonephritis (immunoglobulin A [IgA] nephropathy) is primarily a disease

of young adults who develop ‘synpharyngitic’ episodes of glomerulonephritis, i.e. episodes which either
coincide or occur within 2–3 days of an upper respiratory tract infection. It is the commonest
glomerulonephritis (GN) in adults in the West. It is particularly common in the Far East, which is thought
to be associated with a high incidence of the human leucocyte antigen (HLA) DQW7 haplotype in the
local population.
Post-streptococcal GN usually occurs several weeks after a streptococcal infection of either the upper
respiratory tract or the skin.
Focal segmental glomerulonephritis
We would expect significant proteinuria with focal segmental glomerulonephritis. The history of sore
throat is not typical.
Membranous nephropathy is the most common cause of nephrotic syndrome (oedema,

hypoalbuminaemia, nephrotic-range proteinuria) in adult patients. There is no haematuria, unlike in this
scenario.
Schistosomiasis
Schistosomiasis, also known as bilharzia, is a disease caused by parasitic flatworms called schisotosomes.
The urinary tract or the intestines may be infected. Signs and symptoms may include abdominal pain,
diarrhoea, bloody stool or blood in the urine. Those who have been infected for a long time may
experience liver damage, kidney failure, infertility or bladder cancer. In children, it may cause poor
growth and learning difficulties.
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A.171.A 74-year-old woman is admitted by ambulance having been found by her daughter on her hall
floor. It appears that she had been there for some hours. She has a past history of hypertension, which
is treated with ramipril 10 mg daily, and a TIA for which she is treated with aspirin.
On examination in the Emergency Department, her blood pressure is 120/70 mmHg, with a pulse of
105/min; she is pyrexial (38.2 °C) and has evidence of right-sided pneumonia. There is a fractured left
femoral neck in addition.
254
Platelets (PLT) 235 × 109/l 150–400 × 109/l
Creatine kinase (CK) 3890 IU/l 23–175 IU/l
Urine output 25ml over 2 No significant residual on catheterisation
Urine Positive to blood on dipstick, but no cells
seen on microscopy
Chest X-ray (CXR) Right-sided pneumonia
Electrocardiogram (ECG) Lateral ST depression
Which of the following apart from pneumonia would best explain some of the other results seen here?
Anterior myocardial infarction
Rhabdomyolysis
Fat embolus
Subendocardial myocardial infarction
Explanation
Rhabdomyolysis
This patient has a raised CK and urine testing, which is suggestive of myoglobinuria (positive dipstick but
no cells seen). It is likely she has been lying on the floor for some time, which has precipitated muscle
breakdown and the picture consistent with rhabdomyolysis seen here. Her creatinine of 199 μmol/l is
likely to represent a degree of acute on chronic renal impairment. Vigorous rehydration with isotonic
crystalloid is the mainstay of therapy for rhabdomyolysis. Severe or refractory cases may require
haemodialysis.
Anterior myocardial infarction
An anterior myocardial infarction would result from an occlusion of the left anterior descending artery.
This carries the worst prognosis of all infarct locations, mainly because it affects a large area. The ECG
finding would be ST segment elevation with Q wave formation in the precordial leads (V1-6) and
possible the high lateral leads (I and aVL). There would be reciprocal ST depression in the inferior leads
(III and aVF). In this scenario there is lateral ST depression, which is not consistent with anterior
myocardial infarction.
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Although urinary tract infection may result in blood and protein in the urine with fever, this does not
produce a raised creatinine kinase which, in this case, is the key to diagnosis.
Fat embolus
A fat embolism is a clot that consists of fatty material. This may result from physical trauma such as a
fracture of a long bone, soft tissue trauma or burns. If fat embolism is severe it may cause respiratory
failure or myocardial infarction. There may be purpura that appears on the upper anterior body
including chest, neck and upper arm – this is pathognomonic sign of fat embolism syndrome. This is not
present in this case.
Subendocardial myocardial infarction
Subendocardial myocardial infarction involves infarction of the subendocardial wall of the left ventricle,
ventricular septum or papillary muscles. In established infarction you would expect to see Q waves. In
this case there is subendocardial ischaemia because of myocardial strain from anaemia and pneumonia.
Myocardial strain occurs with inadequate oxygenation to the heart. This is evidenced by the lateral ST
depression.
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A.172.A 70-year-old man presents with tiredness and lethargy, accompanied by weight loss of 1 stone
over the past 4 months, and severe lower back pain. He has a history of hypertension which is managed
with ramipril and amlodipine and an inferior myocardial infarction some 7 years earlier.
On examination, his blood pressure (BP) is 142/72 mmHg, pulse is 82/min and regular, his BMI is 21. He
looks pale and emaciated.
Haemoglobin (Hb) 89 g/l (normocytic) 135 - 175 g/l
Platelet (PLT) 125 × 109/l 150 - 400 × 109/l
Calcium (Ca2+) 2.95 mmol/l 2.2 - 2.7 mmol/l
Renal biopsy Cast nephropathy
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Interferon alpha
Interferon gamma
Haematopoietic stem cell transplant (HCT)
Methotrexate
Non-steroidal anti-inflammatory agents
Explanation
Haematopoietic stem cell transplant (HCT)
This man has a clinical picture which is highly suggestive of myeloma with renal involvement. Diagnosis
of myeloma relies on plasma electrophoresis and bone marrow biopsy to assess plasma cell infiltration.
Cast nephropathy is classically associated with myeloma of the kidney and is characterised by
eosinophilic, dense, homogeneous casts that are often fractured or laminated and are partially
surrounded by multinucleated foreign body-type giant cells. Congo-red-positive casts have been
reported in a few cases. Intratubular light chains apparently may undergo alteration in situ, resulting in
amyloid formation.
Autologous HCT is now the mainstay of treatment in eligible patients. Induction regimens consist of
melphalan, the dose of which can be reduced in patients with poor renal function. Patients ineligible for
HCT may be offered a bortezomib based triple therapy.
Interferon alpha
Interferons are soluble proteins produced naturally by cells in response to viruses. Interferon alpha has
both antiproliferative and immunomodulating properties and is one of the first examples of a biological
response modifier used to treat multiple myeloma. Despite considerable efforts, numerous clinical trials
and two large meta-analysis, its exact role in the management of multiple myeloma still remains
unclear.
Interferon as a single induction agent or co-induction agent with other chemotherapy agents appears to
have only minimal benefit in myeloma. Its role as maintenance therapy in the plateau phase of myeloma
also remains uncertain.
Interferon gamma
IInterferon gamma (IFN-gamma) showed the most powerful inhibiting activity on myeloma cell
proliferation in in-vitro studies. However, IFN-gamma has not been translated to be used in common
practice for multiple myeloma treatment.
Methotrexate
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Methotrexate is an immunosuppressant but it is not used commonly for treatment of multiple
myeloma. It is more frequently prescribed for rheumatological diseases such as rheumatoid arthritis for
disease control.
Non-steroidal anti-inflammatory agents
Non-steroidal anti-inflammatory agents would aid in pain relief; however, they are not a definitive
treatment for multiple myeloma. Furthermore, they may worsen renal function. Therefore, it should be
avoided in renal impairment.
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A.173.A 42-year-old woman with known systemic lupus erythematosus presents with a 3-month history
of fatigue, bilateral leg swelling and increasing abdominal distension.
On examination, she had periorbital oedema, ascites and pitting oedema to the groin. Her chest was
clear, the heart sounds were normal and her jugular venous pressure was not raised.
Haemoglobin (Hb) 90 g/l 115-155 g/l
Platelets (PLT) 120 × 109/l 150–400 × 109/l
Alkaline phosphatase (ALP) 80 IU/l 30–130 IU/l
Aspartate transaminase (AST) 22 IU/l 10–40 IU/l
24-hour urinary protein excretion 4.5 g
She was commenced on treatment with high-dose diuretics and immunosuppressant therapy. Several
weeks later, she was readmitted with abdominal pain. On examination, she had mild pyrexia and was
tender in both loins.
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Repeat investigation reveals the following:
Urea 14.6 mmol/l 2.5–6.5 mmol/
Urinalysis Protein +++ blood +++
What is the most likely cause for the acute deterioration in renal function?
Acute pyelonephritis Renal artery stenosis Renal infarction Renal vein thrombosis Renal calculus
Explanation
The initial diagnosis was nephrotic syndrome secondary to systemic lupus erythematosus, which was
then complicated by renal vein thrombosis. This occurs in 10–20% of patients with nephrotic syndrome
and is more common in those with membranous glomerulonephritis. Patients with nephrotic syndrome
are in a hypercoagulable state due to a combination of intravascular volume depletion (exacerbated by
diuretics) and the loss of clotting factors in the urine (particularly antithrombin III), and are therefore at
high risk of thromboses. Renal vein thrombosis may be silent, causing gradually worsening renal
impairment, or can present acutely as in this case. Diagnosis is with Doppler ultrasound of the renal
veins, computed tomography (CT)/magnetic resonance imaging (MRI) or venography. Management
includes mobilisation, avoidance of volume depletion and long-term anticoagulation. The imaging
results are not described here but the bilateral signs and symptoms do suggest involvement of both
kidneys.
Bilateral renal vein thrombosis, although less common than the unilateral form, can occur as an acute
phenomenon and is an indication for more aggressive management including, if necessary,
thrombolysis.
Pyelonephritis would tend to present with more severe pain, dysuria and haematuria; the clinical picture
of nephrotic syndrome would not be typical and is much better explained here by a diagnosis of renal
vein thrombosis.
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Renal artery stenosis is not associated with systemic lupus erythematosus (SLE), and it does not present
with abdominal pain and fever. The common presentations of renal artery stenosis would be medically
resistant hypertension, flash pulmonary oedema or a gradual worsening of renal impairment.
Renal infarction
Renal infarction may give rise to acute abdominal pain and haematuria. It would be unusual to have
renal infarction of both kidneys to give bilateral loin pain.
Renal calculus
Although renal calculus can present with abdominal pain and, if there is associated infection, there may
be fever with haematoproteinuria. However, there is no risk factor to suggest renal calculus such as
dehydration, hypercalcaemia or hyperuricaemia for example. Normally the pain would be on one side
rather than bilateral loin pain.
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A.174.A 55-year-old man presents to the clinic for review. He has suffered one episode of transient loss
of vision in his right eye, and most recently has suffered a mononeuritis affecting his left common
peroneal nerve. He also has a history of hypertension, for which he currently takes ramipril and
amlodipine.
On examination, his blood pressure (BP) is 155/85 mmHg, he has left foot drop consistent with his nerve
injury. You notice that he also has livedo reticularis.
Investigations:
Hb 122 g/l
WCC 12.3 × 109/l (increased neutrophils)
PLT 192 × 109/l
Na+ 139 mmol/l
K+ 4.8 mmol/l
Creatinine 194 µmol/l
P-ANCA Negative
ESR 61 mm/h
Renal angiogram Multiple microaneurysms
His arm has an abnormal appearance, as below.
Granulomatosis with polyangiitis (GPA)
260
SLE
Berger’s disease
Explanation
Both CNS and PNS symptoms are common presenting features of polyarteritis nodosa (PAN).
Angiography revealing small to medium-sized artery renal aneurysms is very typical of the condition.
Skin changes, including purpura, livedo reticularis, as here, and ulcers, may occur. Hepatitis B is
associated with an increased risk of PAN, and for this reason prevalence is increased in populations
where Hep B is endemic. Histology of lesions reveals microscopic polyangitis, which is also seen in GPA.
Corticosteroids and cyclophosphamide form the mainstay of therapy for idiopathic PAN. Multiple renal
microaneurysms are very typical of PAN, and this pushes us away from a diagnosis of GPA, SLE, etc.
Image source: https://commons.wikimedia.org/wiki/File:Livedo_reticularis_of_an_arm.png
Granulomatosis with polyangiitis (GPA)
GPA is a small vessel vasculitis characterised by the presence of anti-neutrophil cytoplasmic antibodies
(ANCA). The presentation includes fever, weight loss, malaise, myalgia and flitting polyarthralgia. There
are usually ear, nose and throat symptoms such as nasal discharge, epistaxis, sinusitis, oral or nasal
ulcers and otitis media.
SLE
SLE is a heterogenous multi-system disease characterised by auto-antibody production and impaired

immune complex clearance. It is more common in Afro-Caribbean, Hispanic or Asian females in youth or
young adulthood. The American College of Rheumatology has established clinical criteria for diagnosis.
These include malar rash, photosensitivity, oral ulcers, arthritis, serositis and kidney disease.
Berger’s disease
Berger’s disease is otherwise known as IgA nephropathy. It is the most common cause of haematuria in
the developed world. The clinical presentation is asymptomatic, with patients being investigated for
incidental haematuria on dipstick. The haematuria may occasionally be classically timed with the onset
of upper respiratory illnesses (synpharyngetic haematuria). Associated proteinuria is common and it
may present with rapidly progressive glomerulonephritis with acute kidney disease.
Goodpasture, also known as anti-GBM disease, occurs because of anti-basement membrane

autoantibodies directed against type-IV collagen. HLA-DR2 and HLA-B7 are thought to occur with
increased frequency in patients who have Goodpasture. Renal biopsy demonstrates features of
crescenteric necrotising glomerulonephritis. Worsening haemoptysis due to pulmonary haemorrhage,
and acute kidney disease, is typical of Goodpasture syndrome.
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A.175.A 78-year-old woman was admitted to the Accident & Emergency Department with lethargy and
lower limb swelling.
On examination, the ST2 noticed that her left leg was markedly more swollen, consistent with a deep
vein thrombosis (DVT). Her blood pressure was 140/60 mmHg, chest and abdominal examination was
unremarkable.
Sodium (Na+) 135mmol/l 135 - 145 mmol/l
Urinalysis Blood none, protein +++
24-hour protein >3 g < 0.2 g
Renal biopsy Evidence of membranous disease
What is the most direct cause of DVT in patients with marked proteinuria?
Antithrombin deficiency
Chronic inflammation
Hypoalbuminaemia
Hypercholesterolaemia
Volume depletion
Explanation
Antithrombin deficiency
Nephrotic syndrome is not only associated with albumin loss, but also with loss of a number of other
plasma proteins. Relative deficiency of antithrombin is known to occur in nephrotic syndrome due to
loss in the urine and this is thought to be one risk factor in increased development of DVT in these
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patients. Antithrombin deficiency may also be inherited in autosomal dominant fashion and occur in
association with trauma, major surgery or use of the oral contraceptive pill.
Chronic inflammation
Although chronic inflammation may be associated with hypoalbuminaemia, this is not the mechanism
for DVT. Studies have shown that chronic inflammation is a result of DVT rather than a cause of the DVT.
Hypoalbuminaemia
Nephrotic syndrome is associated with loss of albumin however this is not the mechanism by which DVT
is formed. It is loss of other factors such as antithrombin that causes the DVT. Low albumin may be
found in other conditions such as liver failure or malnutrition.
Hypercholesterolaemia
A high cholesterol is found in nephrotic syndrome because there is both an increased in synthesis and
decreased clearance of lipoproteinemia. There is an increased level of total and low-density lipoprotein
(LDL) cholesterol as the most characteristic abnormality. This does not relate to DVT in any way.
Volume depletion
Although colloid osmotic pressure is low as a result of hypoalbuminaemia, it is less likely to be a cause of
DVT than excretion of antithrombin III as a result of marked proteinuria.
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A.176.A 70-year-old man is admitted to the Emergency Department acutely unwell. He has a 2-day
history of disorientation reported by a neighbour. He lives alone and was found collapsed on the floor. It
is suspected that he was on the floor for many hours and probably overnight. He is now drowsy and
incoherent.
His pulse is 120/min and his blood pressure 80/40 mmHg. His respiratory rate is 20/min with saturations
of 95% on air. He is febrile, with a temperature of 38.5 °C. He is stabilised in the resuscitation room and
given IV calcium with IV glucose and insulin to reduce his potassium over the first hour of his treatment
and, in spite of catheterisation, he does not pass any urine.
Urgent investigations:
White cell count (WCC) 19.0 × 109/l (predominantly neutrophils) 4.0 - 11.0 × 109/l
Platelets (PLT) 480 × 109/l 150 - 400 × 109/l
International normalised ratio (INR) 1.1 < 1.1
Activated partial thromboplastin time (APTT) 44 s 25 - 35 s
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Corrected Calcium (Ca2+) 1.92 mmol/l 2.2 - 2.7 mmol/l
Creatine kinase 5999 u/l 22 - 198 u/l
He is fluid loaded in the hope of improving his blood pressure.
In his subsequent management, which one of the following steps is most likely to improve his outcome?
Continuous or intermittent renal replacement therapy (haemodialysis)
Further intravenous calcium supplementation
‘Renal dose’ dopamine infusion
Loop diuretic
Urinary acidification
Explanation
Continuous or intermittent renal replacement therapy (haemodialysis)
This man has rhabdomyolysis due to sepsis, and probably also due to prolonged lying on the floor before
being discovered. The steps most likely to improve his outcome are rigorous rehydration (with caution
to avoid cardiac failure), and definitive renal replacement therapy if this does not improve oliguria and
hyperkalaemia.
Further intravenous calcium supplementation
The calcium and phosphate abnormalities will probably self-correct if renal function can be maintained.
Therefore there is no need for supplementation.
‘Renal dose’ dopamine infusion
There is no conclusive evidence that dopamine or atrial natriuretic peptide is of benefit in the
management of oliguric renal impairment.
Loop diuretic
There is no conclusive evidence that loop diuretics are of benefit in the management of oliguric renal
impairment.
Urinary acidification
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Urinary alkalinisation is sometimes used in rhabdomyolysis, but urinary acidification would be very
dangerous in this case in view of the underlying metabolic acidosis (as suggested by the kidney disease
and low bicarbonate).
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A.177.A 50-year-old white woman presented to her GP with a 2-week history of nausea, vomiting, and
anorexia. She had mild epistaxis and scant haemoptysis for the last 6 to 8 months, with three episodes
of sinusitis, treated with antibiotics and inhaled steroids. Her serum creatinine on presentation was 144
µmol/l with protein ++ and blood +++. Urine microscopy revealed granular casts and dysmorphic red
blood cells.
She was referred to the hospital medical team the next day when her creatinine was 175 µmol/l. Her BP
was measured at 160/98 mmHg, with no rashes or other findings on physical examination.
Investigations:
Haematocrit (HCR) 0.36 0.37 - 0.48 (female)
Eosinophil 0.3 × 109/l 0.04–0.4 × 109/l
Platelets (PLT) 300 × 109/l 150 - 400 × 109/l
ANA negative
pANCA-positive vasculitis cANCA-positive vasculitis Lupus nephritis Post-streptococcal

glomerulonephritis Henoch-Schönlein purpura
Explanation
cANCA-positive vasculitis
This is a classic presentation of Granulomatosis with Polyangiitis. It is a necrotising granulomatous

vasculitis affecting the small vessels, associated with circulating anti-neutrophil cytoplasmic antibodies
(ANCA). It may manifest in a combination of upper and/or lower respiratory tract and/or renal disease.
On immunofluorescence, ANCA staining is cytoplasmic with specificity to proteinase-3.
pANCA-positive vasculitis
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pANCA-positive vasculitis is not classically associated with upper respiratory tract symptoms and the
eosinophil count would be expected to be raised.
Lupus nephritis
Lupus nephritis is associated with a positive ANA and hypocomplementaemia.
This does not explain the insidious upper respiratory tract symptoms. In addition, C3 would have likely
been low.
Henoch-Schönlein purpura
HSP is not classically associated with upper respiratory tract symptoms, and complement levels are
classically normal.
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A.178.A 19-year-old man comes to your office, referred by his rugby coach. He is usually in robust,
excellent health and is in the middle of 3 weeks of intensive training before a university tour to Australia
and New Zealand. He has been passing dark urine for the past few days, and he is worried that he might
have a serious illness. He describes the urine as cola-coloured. He denies fever and has no complaint or
discomfort other than the dark urine.
He is a large muscular young man who is in no obvious distress. His temperature is 37.4 °C, his blood
pressure 120/70 mmHg and pulse 65 bpm. His extremities are non-oedematous and non-tender. The
remainder of the physical examination is unremarkable.
White cell count (WCC) 10 × 109/l 4.0 – 11.0 × 109/l
Which test would be the most appropriate for initial work-up?
Computed tomography (CT) of the abdomen
Erythrocyte sedimentation rate (ESR)
Kidney, ureter and bladder (KUB) X-ray
Renal ultrasound
266
Urinalysis
Explanation
Urinalysis
Urinalysis is the most appropriate test for initial workup because it is simple to carry out in the
outpatients and will confirm that haematuria is causing the cola-coloured urine. The differential
diagnosis of haematuria includes stones, haematological disorders such as thrombocytopenia, infection,
tumours, trauma and treatment like cyclophosphamide.
In this case, the patient is at risk of rhabdomyolysis from the vigorous exercise he has been undergoing.
Rhabdomyolysis would give haemoglobin on stick urinalysis, but no red blood cells would be seen on
microscopy.
Other causes of haematuria in a young man without pain, who is fit and healthy, such as renal stones or
urological malignancy, are highly unlikely. Creatinine kinase may be raised. No specific treatment is
required, apart from maintaining adequate hydration.
Computed tomography (CT) of the abdomen
A CT scan of the abdomen would be normal in rhabdomyolysis. In addition, this would expose the
patient to unnecessary radiation exposure.
Erythrocyte sedimentation rate (ESR)
The ESR may be elevated in infectious or inflammatory causes of rhabdomyolysis. Therefore, ESR may be
raised in this situation; however, it would not discern the cause of the haematuria and is not specific for
rhabdomyolysis.
Kidney, ureter and bladder (KUB) X-ray
A KUB is useful for identifying any calcium-containing radio-opaque renal stones. This patient does not
present with any symptoms of renal colic or abdominal pain; therefore, a renal stone is unlikely.
Renal ultrasound
In rhabdomyolysis, the renal ultrasound is normal, so it would not be helpful. Renal ultrasounds are
useful to identify kidney sizes, which may be enlarged in certain conditions such as polycystic kidney
disease or small in chronic kidney disease. It is useful to exclude hydronephrosis as a cause of acute
kidney impairment.
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A.179.A 27-year-old dancer comes to the Emergency Department because of severe pain in her left side
and dark urine. The patient denies fever but states that the general practitioner (GP) had seen her
several days ago for urinary frequency and burning. She had been treated with trimethoprim at that
time, and the dysuria had resolved.
The pain now comes on very suddenly, and the paroxysms are so severe that walking is not possible.
There is some nausea and the patient had vomited twice. The pain radiates to her groin. The patient's
267
vital signs are a temperature of 37.2 °C, a blood pressure of 110/70 mmHg, a pulse rate of 95 bpm and a
respiratory rate of 14 breaths per min.
Physical examination of the chest and heart is normal. The abdomen is soft and non-tender, with no
guarding. Analgesia is given.
Corrected calcium 2.60 mmol/l 2.2 - 2.7 mmol/l
Which of the following is the most appropriate early treatment?
Hydration
Intravenous (iv) broad-spectrum antibiotics
iv furosemide
Lithotripsy
Percutaneous removal of stones
Explanation
Hydration
Kidney stones form when there is a high level of calcium (hypercalciuria), oxalate (hyperoxaluria) or uric
acid (hyperuricosuria) in the urine; a lack of citrate in the urine; or insufficient water in the kidneys to
dissolve waste products. The kidneys must maintain an adequate amount of water in the body to
remove waste products. If dehydration occurs, high levels of substances that do not dissolve completely
(eg calcium, oxalate, uric acid) may form crystals that slowly build up into kidney stones.
Urine normally contains chemicals (including citrate, magnesium and pyrophosphate) that prevent the
formation of crystals. Low levels of these inhibitors can contribute to the formation of kidney stones. Of
these, citrate is thought to be the most important. Approximately 80–85% of stones pass spontaneously.
Approximately 20% of patients require hospital admission because of unrelenting pain, inability to retain
enteral fluids, proximal urinary tract infection (UTI) or inability to pass the stone.
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A ureteral stone associated with obstruction and an upper UTI is a true urological emergency.
Complications include perinephric abscess, urosepsis and death. Immediate involvement of the urologist
is essential. Diagnosis includes blood tests, urinalysis, X-rays, iv urography, ultrasound scan and non-
contrast CT. Treatment depends on the type and cause of the stone. The usual initial treatment is
hydration (oral or intravenous, depending on patient circumstances) and analgesia, which allows
spontaneous passage of stones in the majority of patients. For those patients whose stones do not pass,
methods of stone removal, including percutaneous removal of stones, may be considered.
Intravenous (iv) broad-spectrum antibiotics
Broad-spectrum antibiotics would only be useful if there is an infection. In this scenario, there is no
history to suggest an infection – the patient is apyrexial, the WCC is normal and the ESR is not raised.
iv furosemide
iv furosemide would exacerbate the situation in renal stones. This is because there would be
hypercalciuria with furosemide if the stones are due to calcium; then this would increase the deposits.
Lithotripsy
For those patients whose stones do not pass, methods of stone removal including lithotripsy approaches
may be considered. However, in this case, the first treatment is fluids, and most small stones will pass
on their own.
Percutaneous removal of stones
For those patients whose stones do not pass, methods of stone removal including percutaneous
removal of stones may be considered. However, in this case, the first treatment is fluids, and most small
stones will pass on their own.
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A.180.A 35-year-old man attends the Renal Clinic for the first time. He has been referred by his GP
because of a progressive decline in renal function over several months. His past medical history includes
type 2 diabetes mellitus, repeated urinary tract infections as a child and recent admission to hospital
with renal colic.
On examination, he is apyrexial, his blood pressure is 180/70 mmHg, and chest and abdominal
examination are unremarkable.
Investigations:
269
HbA1C 6% (42 mmol/mol) 3 - 7% (9 - 53 mmol/mol)
Urine dipstick
No red blood cells
White blood cells (WBCs) ++
Urine microscopy WBC casts
Reflux nephropathy
Tuberculosis
Explanation
Reflux nephropathy
This man has reflux nephropathy, which is a progressive lesion caused by repeated urinary tract
infections in either or both kidneys. The clue is the urinary tract infections as a child, because reflux
nephropathy almost always develops in childhood in the setting of an abnormal renal tract. The long-
term consequences are scarring and insufficiency, accounting for 5-12% of end stage kidney disease in
the developed world. There are a variety of anatomical abnormalities that lead to the urinary tract
infections, which include vesico-ureteric reflux, incomplete bladder emptying and outflow tract
obstruction. The presentation of reflux nephropathy as an adult includes progressive renal impairment
and hypertension. On an intravenous urogram there would be renal scarring and ‘clubbed calyces’.
Although urinary tract infections may cause urinary white cell counts to be raised, there is no symptoms
that suggest active infection at present. This man does not complain of any symptoms such as urgency
or dysuria. The blood white cell count is normal. There is no mention of any growth of bacteria in the
urine.
Diabetic nephropathy presents with proteinuria but not white cells in the urine. Nephropathy occurs
due to microvascular glycosylation, renal hypertrophy and hyperfiltration. Two per cent of type 2
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diabetics develop microalbuminuria annually, and 2.3% have proteinuria with declining glomerular
filtration rate.
There would not be white cells in the urine in hypertensive nephropathy. We would expect proteinuria.
Hypertensive nephropathy may present with progressive renal impairment in the context of long-
standing hypertension. Associated signs may include retinal haemorrhages and exudates. There may be
a history of accompanying cardiovascular disease such as strokes and myocardial infarction.
Tuberculosis
Tuberculosis is a cause of sterile pyuria. However, in this scenario there are no associated symptoms to
suggest tuberculosis such as fever, weight loss, malaise or night sweats. In addition there is no mention
of any contact history or foreign travel to suggest exposure to tuberculosis.
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A.181.A 56-year-old woman with known chronic kidney disease (CKD) (usual creatinine 400 µmol/l),
secondary to adult polycystic kidney disease, presents with a 2-day history of dysuria, left loin pain,
fevers and vomiting.
On examination, she is pyrexial (temperature of 38.5 °C), with left loin tenderness and a pericardial rub.
Mucosal surfaces and skin turgor look normal. Her blood pressure is elevated at 162/90 mmHg. You
commence her on intravenous (iv) antibiotics.
Creatinine (Cr) 1200 µmol/l 50 - 120 µmol/l (Her normal: 400 µmol/l)
Electrocardiogram (ECG) Left bundle branch block. No other abnormality.
Which one of the following is the most appropriate intervention with respect to her renal impairment
and circulatory status?
Blood transfusion
Dialysis
Fluid restriction
Insulin and dextrose
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Rapid intravenous (iv) fluids
Explanation
Dialysis
This patient demonstrates a case of acute-on-chronic kidney disease, secondary to likely

pyelonephritis/infected cyst. She needs dialysis urgently, as she has a pericardial rub, which is most
likely due to uraemic pericarditis. She also has severe uraemia with urea and creatinine values as high as
these. Dialysis is required for severe renal impairment and uraemic pericarditis; it will result in the
lowering of the potassium level as well.
Blood transfusion
A blood transfusion is not needed, as many patients with CRF are anaemic, secondary to erythropoietin
deficiency, and this is usually worse in the setting of an acute infection. It is best to treat the kidney
disease and re-assess the anaemia once the infection is treated.
Fluid restriction
Fluid restriction will lead to further rise in urea levels and will potentially exacerbate the uraemic
pericarditis. In this situation, it would be better to give fluids instead.
Insulin and dextrose
Insulin and dextrose will lower the potassium level, but this is not dangerously elevated. Furthermore,
the combination of insulin and dextrose, when used to lower the potassium level, is only temporary
because it acts to push potassium into the cells. To remove total potassium, potassium needs to be
removed via the gut or kidneys. So if the kidneys are not functioning, then dialysis will be required to
remove potassium.
Rapid intravenous (iv) fluids
Although cautious iv fluids would not be inappropriate, the patient is only mildly dehydrated and they
will not correct this degree of kidney disease. Rapid iv fluids could result in pulmonary oedema if the
kidneys are not functioning.
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A.182.A 36-year-old man with spina bifida, who has a long-term indwelling catheter, comes to the
Emergency Department complaining that he feels lethargic and had a fever the previous evening.
On examination in the Emergency Department, he has a temperature of 38.1 °C. A catheter specimen of
urine sent by the General Practitioner the previous day shows a significant growth of coliforms. The
urine in the bag looks cloudy. His white count is normal, although the C-reactive protein (CRP) level is
raised to 85 mg/l.
Which of the following is the most appropriate intervention?
Admit for intravenous (IV) co-amoxiclav
272
Advise increased oral fluids and reassure
Change the catheter and give oral co-amoxiclav
Give a single dose of IV gentamicin
Give intravesical gentamicin via the catheter
Explanation
Change the catheter and give oral co-amoxiclav
There are symptoms of systemic infection, so a change of catheter, followed by a course of antibiotics, is
indicated. Given he appears to be relatively early with respect to his presentation and the WCC is
normal, oral antibiotics are indicated at this point. Treating without catheter change is associated with a
much lower chance of success in clearing the infection because of colonisation of the plastic which
makes up the catheter tubing.
Admit for intravenous (IV) co-amoxiclav
Although IV co-amoxiclav is effective in clearing Gram-negative pathogens, it is unlikely to completely

eradicate this patient’s infection without catheter change.
Advise increased oral fluids and reassure
Given there are features suggestive of systemic bacteraemia, including pyrexia and an elevated CRP
level, just increasing oral fluids is unlikely to clear any underlying infection.
Give a single dose of IV gentamicin
Although a single dose of IV gentamicin may reduce the risk of urine infection after catheter change, the
presence of systemic signs of infection here means a course of oral antibiotics is required.
Give intravesical gentamicin via the catheter
Intravesical gentamicin, like a single dose of IV gentamicin, is an option at catheter change, but it will not
eradicate the systemic bacteraemia which is evident here.
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A.183.A 20-year-old man of Japanese descent who is studying in the UK attends the clinic for advice. He
attended the optician for an eye test and was noted to have greenish-gold discoloration within the
limbus of both corneas.
Other past medical history of note includes polyarthritis, for which he takes regular analgesia.
On physical examination, his blood pressure is 130/80 mmHg; chest and abdominal examination is
unremarkable.
273
Alanine aminotransferase (ALT) 75 IU/l 5–30 IU/l
Urinary bicarbonate excretion Increased
Which of the following diagnoses fits best with the renal dysfunction seen here?
Papillary necrosis
Explanation
The findings from the optician are suggestive of Kayser-Fleischer rings, and Wilson’s disease. The raised
ALT and polyarthritic picture are supportive of this. Wilson’s is associated with renal tubular acidosis
Type 2. Other causes include Fanconi syndrome, hereditary fructose intolerance, Lowe syndrome, heavy
metal poisoning and certain chemotherapeutic agents. It is associated with an inability to reabsorb
sodium bicarbonate from urine, leading to increased urinary losses. Treatments for RTA type 2 include
bicarbonate replacement and the use of thiazide diuretics.
Type 4 is associated with a decreased response to aldosterone. This presents with hyperkalaemia,
reduced serum bicarbonate, normal anion gap with raised chloride, positive urinary anion gap and
reduced urinary citrate. It is caused by conditions such as diabetes mellitus, drugs such as NSAIDS and
ciclosporin and obstructive uropathy.
In contrast to Type II renal tubular acidosis, type 1 RTA is associated with an inability to acidify urine.
Type 1 RTA presents as a hyperchloraemic, hypokalaemic metabolic acidosis with hypophosphataemic
metabolic bone disease, renal stones or diffuse nephrocalcinosis. It is inherited or due to secondary
conditions such as Sjogren syndrome, rheumatoid arthritis or other auto-immune diseases.
274
Acute tubular necrosis may cause renal impairment. It may result from pre-renal injury such as
hypoperfusion from hypovolaemia, reduction in blood flow and sepsis, for example. There is no history
in this case to suggest injury that has resulted in acute tubular necrosis such as drop in blood pressure.
Papillary necrosis
Papillary necrosis is a disorder where there is death of part or some of the renal papillae. This would
present with haematuria and abdominal pain. It is a complication of analgesic nephropathy. In this
scenario, there is no mention of any abdominal pain. Furthermore, analgesic nephropathy is a result of
long-term analgesic abuse; in view of the fact he is only 20 years old, this makes the diagnosis unlikely.
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A.184.A 72-year-old man with a history of hypertension presents to the Emergency Department in
extremis. He awoke in the early hours of the morning with severe shortness of breath as if he was going
to gasp his last breath. You understand that his GP has commenced him on ramipril 5 mg daily a short
time ago.
On examination, he has a BP of 175/85 mmHg, a pulse of 100 bpm and is in left ventricular failure. You
manage him with a nitrate infusion and furosemide and he improves.
Platelets (PLT) 212 × 109/l 150–400 × 109/l
Creatinine (Cr) 204 μmol/l (134 μmol/l, 1 month earlier) 50–120 µmol/l
An echocardiogram (ECHO) shows mild LVH.
What is the most likely cause of his presentation with pulmonary oedema?
Coronary artery disease
Hypertensive heart disease
Hypertrophic cardiomyopathy
Aortic stenosis
Explanation
275
Patients with renal artery stenosis may present in this manner with rapidly worsening renal function, an
increase in blood pressure and so-called flash pulmonary oedema. In this case, the presentation has
almost certainly been accelerated by the starting of an ACE inhibitor. Investigations to confirm the
diagnosis include ultrasound, which may show differential kidney size, and magnetic resonance
angiography. Management centres on non-ACE inhibitor control of blood pressure. Large-scale studies
have suggested little or no benefit of angioplasty in renal artery stenosis, although it may be appropriate
in patients with recurrent episodes of pulmonary oedema.
Coronary artery disease
Myocardial infarction may be a cause of pulmonary oedema and renal impairment. However, the start
of ACEI recently would not result in coronary artery disease. The echo would show left ventricular failure
rather than just mild LVH.
Hypertensive heart disease
Hypertensive heart disease can cause left ventricular failure for patients who have hypertension over a
long period of time. There would be severe left ventricular hypertrophy and left ventricular failure on
the echo.
Hypertrophic cardiomyopathy
Hypertrophic cardiomyopathy is the leading cause of death in young athletes. This condition can
produce symptoms that mimic congestive heart failure, such as activity intolerance and dyspnoea. On
the echo there is systolic anterior motion of the anterior leaflet of the mitral valve. In this scenario, the
echo findings do not show this.
Aortic stenosis
Aortic stenosis may be a cause of flash pulmonary oedema. However, you would expect severe left
ventricular hypertrophy if there is sufficiently severe aortic stenosis to result in renal impairment and
pulmonary oedema. Furthermore ACEI should be helpful in aortic stenosis for cardiac remodelling and
blood pressure control. In this case, the ACEI resulting in acceleration of renal impairment suggests renal
artery stenosis.
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A.185.A 68-year-old man is admitted to the Emergency Department with an acute myocardial infarction.
He is hypotensive and sweating on admission, with a BP of 100/60 mmHg and pulse of 105/min. There is
anterior ST elevation. Past history of note is hypertension, for which he receives ramipril 5 mg,
indapamide 2.5 mg and amlodipine 5 mg.
Within a few minutes of admission, he suffers a cardiac arrest. In total he receives CPR for 6 min before
he regains his output. His BP is 95/60 mmHg, with pulse 100/min and regular. He later goes to the
catheter lab where he is stented. Creatinine on admission is 135 micromol/l.
What is the optimal initial intervention to prevent acute tubular necrosis?
276
Start IV furosemide
Start renal dose dopamine
Start dobutamine
Increase ramipril
Fluid replacement with CVP monitoring
Explanation
Fluid replacement with CVP monitoring
This patient is at significant risk of ischaemic acute tubular necrosis due to a prolonged period of renal
hypoperfusion associated with his anterior MI and subsequent cardiac arrest. Maintaining CVP in the
normal range with tailored fluid replacement is the best way to minimise deterioration in renal function.
Start IV furosemide
Although loop diuretics are used in this situation, there is little evidence that they have a positive impact
on renal outcomes.
Start renal dose dopamine
Although renal dose dopamine was historically used to prevent ATN, there is no evidence that it is any
more effective than placebo.
Start dobutamine
Dobutamine is used as a cardiac inotrope, and may increase the risk of arrhythmia and myocardial
ischaemia in this situation.
Increase ramipril
Ramipril increases the risk of renal hypoperfusion and has a potentially negative impact on the risk of
ATN.
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A.186.A 38-year-old Asian woman presents with several weeks of increased urinary frequency and
dysuria. She had already been treated with two courses of antibiotics from her general practitioner,
which had not helped. She had been feeling unusually lethargic recently and had lost a small amount of
weight, but had no other specific symptoms.
On examination, she was thin with a low grade fever of 37.6 °C. Her abdomen was soft with mild
suprapubic tenderness but no palpable masses. The rest of the clinical examination was normal.
277
Platelets (PLT) 390 × 109/l 150 - 400 × 109/l
Urinalysis Blood + protein +
Urine microscopy:
WCC 22/mm3
Red blood count 3/mm3
Few hyaline casts
No organisms seen
Urine culture No growth after 5 days
What is the next most appropriate investigation?
Micturating cystourethrography
Renal biopsy
Plain abdominal radiograph
Urine electrophoresis
Urine for mycobacterial culture
Explanation
Urine for mycobacterial culture
This woman has a sterile pyuria which could be due to a partially treated bacterial urinary tract
infection; however genitourinary tuberculosis has to be excluded, especially in the context of malaise
and weight loss and the patient’s ethnic origin. Genitourinary TB develops in approximately 5% of cases
of pulmonary TB and is usually due to haematogenous spread to the renal cortex during the primary
phase of infection. The cortical lesion may then ulcerate into the pelvis, ultimately involving the bladder,
278
seminal vesicles and prostate. It tends to present between 20 and 40 years of age. Other clinical features
include haematuria, urethral strictures, cold abscesses and chronic epididymo-orchitis. Kidney disease
may occur due to extensive destruction of the kidneys or by obstruction secondary to fibrosis. The
diagnosis can be made with intravenous urography, ultrasound or computed tomography (CT) scan, in
combination with several early morning urine samples for mycobacterial culture, in order to
demonstrate active infection. The treatment is the same as for pulmonary TB.
Micturating cystourethrography
This investigation visualises the urinary tract from bladder to urethra as the patient urinates. It is used
for diagnosis of vesicoureteral reflux (VUR) where there is reflux of urine from the bladder up the
ureters back into the kidneys. Radiologists may grade the degree/staging of VUR from the micturating
cystourethrography. Here, most likely diagnosis is renal tuberculosis where micturing
cystourethrography would be normal.
Renal biopsy
Although renal TB may reveal interstitial nephritis and caseating granulomata in the renal biopsy, there
are easier and non-invasive methods of diagnosing this from urine cultures and investigations described
above. Renal biopsy may have risks of bleeding, haematuria and, in severe cases if bleeding cannot be
stopped, the patient may need kidney removal. Therefore a renal biopsy should not be carried out when
renal TB is suspected unless absolutely indicated.
Plain abdominal radiograph
A plain abdominal radiography may show calcified lymph nodes from tuberculosis; however, it would
not provide a diagnosis. Calcified lymph nodes may be idiopathic and be due to causes other than TB.
Urine electrophoresis
The urine electrophoresis would be normal in TB. Electrophoresis would be helpful in diagnosis of
myeloma where there may be light chain excretion and Bence Jones proteins.
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A.187.A 29-year-old woman of Jamaican origin presented to her general practitioner with four months’
of amenorrhoea and increasing tiredness. She was adopted as a child and recalls that her immediate
brother died of an uncertain kidney disease a few years ago in Jamaica.
Routine examination reveals a blood pressure of 210/135 mmHg, a heart murmur and a palpable mass
in the abdomen. She was sent for a routine blood test and ultrasound of the abdomen and pelvis.
Investigations:
Ultrasound scan Enlarged kidneys with multiple cysts,
consistent with polycystic kidneys
Prolactin 1200 mU/l < 400 mU/l
279
Platelets 350 × 109/l 150–400 × 109/l
Chronic ureteric reflux Renal cell carcinoma Renovascular disease Transitional cell carcinoma
Explanation
Autosomal dominant polycystic kidney disease (APKD) presents with hypertension and haematuria, and
later in middle age with chronic kidney disease. Hyperprolactinaemia is common in chronic kidney
disease. Acute loin pain or haematuria due to haemorrhage into the cysts are common. Cysts may be
found in other organs such as the liver and ovaries, childhood autosomal recessive polycystic kidney
disease is also associated with liver fibrosis. Hypertension develops in over 75% of cases. Subarachnoid
haemorrhage from berry aneurysm is reported to occur in about 9% of patients, and 8% of patients with
APKD are thought to have asymptomatic intracranial aneurysms. Early aggressive management of blood
pressure is the cornerstone of therapy.
Chronic ureteric reflux
There is no mention of recurrent urinary tract infections. Chronic ureteric reflux generally presents with
chronic renal impairment as an adult, and may be due to recurrent urinary infections as a child. In
children, vesico-ureteric reflux, a congenital defect, is a cause of the urinary tract infections.
Renal cell carcinoma classically presents with haematuria, flank pain and abdominal mass. The lack of
flank pain and anaemia, with the relatively young age, would make renal cell carcinoma unlikely. By the
time there is the classical triad of symptoms, we would expect the renal cell carcinoma to be in an
advanced stage.
Renovascular disease
Patients with renovascular disease will have renal impairment, but there would not be an abdominal
mass. Furthermore, there are often signs of generalised atheroscelerosis such as a previous stroke,
myocardial infarction with a smoking history, and renal bruit may be present.
280
Transitional cell carcinoma is a typical cancer that occurs in the urinary system – kidneys, urinary
bladder and ureter, etc. It is the second most common type of kidney cancer (accounting for 5–10% of
primary malignant renal tumours). We would expect an anaemia with transitional cell carcinoma, rather
than a high red cell count as would be found in polycystic kidney disease.
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A.188.A 35-year-old woman presents to the Renal Clinic for review. She has difficult-to-manage
hypertension – her BP is still elevated despite therapy with ramipril 5 mg, amlodipine 5 mg and
indapamide 2.5 mg.
On examination in the clinic, her BP is 160/94 mmHg, with pulse 80 bpm and regular. Her BMI is 23.
Investigations:
Platelets (PLT) 198 × 109/l 150 - 400 × 109/l
Renal angiogram 'string of beads' appearance of left renal artery
Which of the following is the most appropriate next intervention?
Angioplasty
Add atenolol 50 mg
Add doxazosin 4 mg
Surgical renal artery reimplantation
Explanation
Angioplasty
Recent meta-analyses have suggested that atherosclerotic renal artery stenosis medical management is
as effective as angioplasty. This is in contrast to fibromuscular hyperplasia, which has the characteristic
angiography appearance seen here. In this case angioplasty has superior outcomes with respect to
control of blood pressure and long-term sequelae. As such, angioplasty is recommended as the most
281
appropriate intervention. Surgical arterial reimplantation is not recommended, particularly in the light
of modern angioplasty and stenting techniques.
Add atenolol 50 mg
Angioplasty has superior outcomes for blood pressure control compared to anti-hypertensives. Atenolol
is a non-cardioselective beta-blocker. It may have side effects of tiredness and impotence.
Add doxazosin 4 mg
Angioplasty has superior outcomes for blood pressure control compared to anti-hypertensives.
Doxazocin is an alpha-blocker that may be used as a treatment for hypertension, and also for
symptomatic relief for benign prostatic hyperplasia. For the latter condition it promotes smooth muscle
relaxation, improving urinary flow and reducing the problem of residual urine in the bladder post-
voiding.
Angioplasty has superior outcomes for blood pressure control compared to anti-hypertensives. Ramipril
is an angiotensin-converting enzyme inhibitor.
Surgical renal artery reimplantation
Surgical renal artery reimplantation, although a possible intervention for renal artery stenosis, has
inherent risks as with all surgical procedures and therefore would be an inferior option compared to
renal angioplasty.
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A.189.A 23-year-old adopted Jewish man is referred to the Renal Clinic by his GP because of proteinuria.
The GP also reports that he has suffered intermittent illnesses in the past, characterised by severe fevers
which were thought to be due to recurrent viral illnesses.
Other past history of note includes an appendicectomy, and arthritis affecting the hips and knees.
On physical examination, blood pressure is 130/80 mmHg, chest and abdominal examination was
unremarkable. ESR checked by the GP during one of the recent attacks of fever was raised at 65 mm/h.
Investigations on the day of clinic visit revealed:
Dipstick urine Protein 3 +
24-hour urine testing > 3 g protein
Which of the following diagnoses fits best with this clinical picture?
282
AL amyloidosis
Familial Mediterranean fever
Still’s disease
Explanation
Familial Mediterranean fever
The answer is familial Mediterranean fever (FMF). Populations having a high prevalence (1/200-1/1000)
of the disease are non-Ashkenazi Jews, Turks, Armenians and Arabs, but is also seen in Italy, Greece and
Spain.. The disease is characterised by intermittent paroxysms of fever, chronic arthritis,
appendicectomy related to paroxysmal abdominal pain and nephrotic syndrome due to renal
amyloidosis. Pleuritic involvement and pericarditis may also occur in around 25% of patients. Mortality is
related to deteriorating renal function. Colchicine therapy has been shown in a number of studies to
delay deterioration in renal function and is the mainstay of therapy. A large number of patients do still
progress and require renal transplantation.
AL amyloidosis
AL amyloid is due to mononclonal light chains usually lambda or light chain fragments produced by
plasma cell dyscrasia. It is more common in women who are over 50 years old. Although nephrotic range
proteinuria is a usual presentation, there may be other signs such as easy bleeding, cardiomegaly and
hepatomegaly. The young age and high fevers are unusual in AL amyloidosis.
Minimal change disease is the most common cause of nephrotic syndrome in the young, therefore the
age and nephrotic range proteinuria would fit with this diagnosis. However, there are no associated
symptoms of arthritis or recurrent fevers with minimal change disease.
Still’s disease
Still’s disease is a rare systemic inflammatory disease that is characterised by a triad of persistent high
spiking fevers, joint pains and a distinctive salmon-coloured raised rash. Nephrotic range proteinuria is
not a feature. In this case, the lack of a rash would also be against the diagnosis of Still’s disease.
SLE may present with rash, fatigue and arthralgia. It is an autoimmune condition that mostly occurs in
women of childbearing age. The condition is less common in people of European origin and more
common in people of African, Caribbean or Asian origin. The lack of clinical symptoms of SLE and the
patient being a Jewish man makes the diagnosis of SLE unlikely.
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283
A.190.A 44-year-old woman received a cadaveric renal transplant 8 weeks previously.
At a routine Transplant Clinic appointment, she is well and on the following medication: ciclosporin,
azathioprine, prednisolone, co-trimoxazole and lisinopril.
Electrocardiogram (ECG) is shown below.
What is the most appropriate initial therapy?
IV calcium gluconate
Immediate withdrawal of lisinopril
Inhaled salbutamol
Intravenous (IV) insulin with glucose
PO polystyrene sulphonate resin
Explanation
IV calcium gluconate
This patient has severe hyperkalaemia with typical changes on the electrocardiogram (ECG), of wide QRS
complexes and tall tented T waves, indicating that the hyperkalaemia is having an adverse effect on the
cardiac conduction system. Although some patients show a gradual progression of ECG findings, many
progress rapidly without warning. As such, patients with potassium levels >6.5 mmol/l and ECG changes
should be treated as a medical emergency. Protection of the heart is paramount. Calcium gluconate acts
within minutes and works by raising the depolarisation threshold for myocytes. The effect of bolus
calcium gluconate is transient, and repeated boluses may be necessary until the plasma potassium has
been lowered.
Image source: https://commons.wikimedia.org/wiki/File:118_(CardioNetworks_ECGpedia).jpg
Immediate withdrawal of lisinopril
Withdrawal of lisinopril is important, but will not lead to immediate decrease in potassium.
Inhaled salbutamol
284
Nebulised salbutamol is only an interim measure to lower potassium as it moves potassium into the
cells. Salbutamol will induce a tachycardia and may predispose to a tachyarrythmia.
Intravenous (IV) insulin with glucose
The fastest way to decrease the extracellular potassium is to shift it to the intracellular compartment.
This can be accomplished by insulin (along with glucose to prevent hypoglycaemia). However, it is not
directly cardioprotective.
PO polystyrene sulphonate resin
PO polystyrene sulphonate resin takes time to act, and this will be hours to days. Therefore it is not used
in the acute setting for rapid reduction in potassium.
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A.191.A 23-year-old man presents with a 4-day history of sore throat. He also mentions a change in
colour of urine and it looks blood-stained. There are no other associated urinary complaints.
His past medical history is unremarkable.
Upon examination, his temperature is 36 °C, blood pressure 130/80 mmHg, chest and abdominal
examination unremarkable. Chest X-ray is normal. Blood results are normal. Renal biopsy shows
mesangial proliferation with positive immunofluorescence for immunoglobulin A (IgA) and C3.
Henoch–Schönlein purpura
IgA nephropathy
Kawasaki disease
Explanation
IgA nephropathy
Immunoglobulin A (IgA) nephropathy, or Berger’s disease, is the commonest cause of

glomerulonephritis worldwide. A typical patient is a young male with asymptomatic microscopic
haematuria or episodic macroscopic haematuria, precipitated by infection (e.g. pharyngitis). Recovery is
usually rapid between attacks. The pathology consists of focal proliferative glomerulonephritis with
mesangial deposits of IgA. Prognosis is usually good in patients with normal blood pressure, normal
kidney function and absence of proteinuria at presentation. Approximately 20% of patients develop
kidney disease 20 years from the time of diagnosis.
285
Polycystic kidney disease is not associated with sore throat or any abnormality in immunofluoresence.
Diagnosis is with genetic studies or ultrasound identification of cysts.
Henoch–Schönlein purpura
There would be a history of skin rash with this condition.
Kawasaki disease
Kawasaki disease is associated with sore throat. However, it generally occurs in children and there may
be coronary artery aneurysms and peeling of the skin of the hands and feet.
Post-streptococcal glomerulonephritis is characterised by sudden onset of haematoproteinuria, oedema

and hypertension. It is not associated with predominantly IgA in the renal biopsy.
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A.192.A 68-year-old man comes to the renal clinic as a General Practitioner referral. He has a long
history of hypertension which is managed with ramipril 10 mg daily and amlodipine 10 mg, but most
recently he has complained of increasing tiredness.
On physical examination, his blood pressure was 140/80 mmHg, with chest and abdominal examination
unremarkable. The GP ran some blood tests and was very concerned about the results.
Investigations:
Platelets 184 × 109/l 150–400 × 109/l
Ferritin 90 μg/l 20–250 µg/l
You plan to treat him with EPO once his ferritin is maintained above 100.
Which of the following is the most appropriate Hb target to get above?
90 g/l
110 g/l
286
130 g/l
140 g/l
145 g/l
Explanation
110 g/l
Guidelines from the European Renal Association support a target for Hb of 100–120 g/l. Additionally,
they recommend reaching this target within four months of commencing treatment. Renal patients are
often additionally ferritin depleted, and assessment of ferritin level is crucial before starting EPO.
Patients who are ferritin depleted should first be given an IV infusion. Some controversy exists as to
whether a higher target would lead to lower CV events, but a study sponsored by Roche, which targeted
EPO use to achieving a Hb in the normal range, showed no CV benefit.
90 g/l
This target is too low and therefore, if patients were treated to this target, there may still be some who
experience anaemia symptoms, such as shortness of breath and lethargy.
Guidelines from the European Renal Association support a target for Hb of 100–120 g/l. This also the
midpoint of the NICE guidelines on Hb target.
130 g/l
This level of haemoglobin is a little too high, with some concern for risk.
140 g/l
The Correction of Haemoglobin and Outcomes in Renal Insufficiency (CHOIR) investigators concluded
that a haemoglobin target of 135 g/l was associated with significantly greater risk for the primary
composite outcome (death, MI, hospitalization for congestive heart failure without renal replacement
therapy, or stroke) compared with a lower target of 113 g/l. Therefore there should be caution in
haemoglobins > 135 g/l.
145 g/l
As above, the CHOIR investigators concluded that a haemoglobin target of 135 g/l was associated with
significantly greater risk for the primary composite outcome (death, MI, hospitalization for congestive
heart failure without renal replacement therapy, or stroke) compared with a lower target of 113 g/l.
Therefore there should be caution in haemoglobins greater than 135 g/l.
*****************************************************************************
A.193.A 63-year-old man was admitted to the hospital for an elective coronary angiogram. The
procedure went ahead without any obvious complications.
A day after the procedure, he complained of generalised aching abdominal pain, which improved
following the administration of lactulose, although it did not resolve entirely. A day later, he became
acutely unwell.
287
On examination, he appeared drowsy and confused. He had a pulse rate of 100 bpm, with a blood
pressure of 135/80 mmHg. He had peripheral oedema and coarse crackles in both lung bases. His
abdominal examination showed diffuse tenderness, but no rebound or guarding. There was a bluish
discolouration of his toes and signs of peripheral emboli.
Platelets (PLT) 349 × 109/l 150–400 × 109/l
Neutrophils 6.0 × 109/l 2.5-7.8 × 109/l
Lymphocytes 2.0 × 109/l 1.5-3.5 × 109/l
Eosinophils 2.1 × 109/l 0.04-0.40 × 109/l
Basophils 0.1 × 109/l < 0.1 × 109/l
Monocytes 0.2 × 109/l < 0.8 × 109/l
Acute kidney disease secondary to contrast-induced nephropathy
Acute kidney disease secondary to generalised sepsis
Acute kidney disease secondary to multiple cholesterol emboli
Acute kidney disease secondary to a catastrophic intra-abdominal event
Acute kidney disease secondary to exacerbation of congestive cardiac failure
Explanation
Acute kidney disease secondary to multiple cholesterol emboli
This patient has developed acute kidney disease and is severely unwell. The presentation with
abdominal pain and eosinophilia all support a diagnosis of multiple cholesterol emboli. Data about
cholesterol embolisation are largely based on postmortem examinations which are, by definition, a
288
selected group of poor outcomes. However, some studies suggest that as many as 25–30% of patients
undergoing angiography will have atherosclerotic emboli.
Acute kidney disease secondary to contrast-induced nephropathy
We are not told of any risk factors that would increase the chance of contrast-induced nephrotoxicity
(such as age over 70 years; coexisting nephrotoxicity, especially if diabetic; concomitant administration
of nephrotoxic drugs; dehydration before a procedure; or pre-existing congestive cardiac failure).
Acute kidney disease secondary to generalised sepsis
There are no convincing signs of severe sepsis.
Acute kidney disease secondary to a catastrophic intra-abdominal event
The abdominal pain is probably caused by bowel ischaemia, but the lack of signs of peritonism in this
case makes this a less likely primary cause for his deterioration.
Acute kidney disease secondary to exacerbation of congestive cardiac failure
The degree of cardiac insufficiency is unlikely to have caused such a significant deterioration.
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A.194.A 34-year-old man with a history of autosomal dominant polycystic kidney disease (APKD) comes
to the Emergency Department complaining of sudden-onset, severe right loin pain. He has a history of
hypertension and chronic renal failure and takes ramipril and amlodipine for control of his blood
pressure.
On examination, his blood pressure is 155/95 mmHg; pulse is 90 bpm and regular. His temperature is
37.3 °C. Abdominal palpation reveals bilateral renal masses and severe right loin tenderness on renal
palpation.
Investigations:
Investigation
Result
Normal value
Haemoglobin (Hb)
130 g/l
135–175 g/l
10.0 × 109/l
4–11 × 109/l
Platelets (PLT)
289
203 × 109/l
150–400 × 109/l
Sodium (Na+)
138 mmol/l
135–145 mmol/l
Potassium (K+)
5.0 mmol/l
3.5–5.0 mmol/l
Creatinine (Cr)
231 µmol/l
50–120 µmol/l
Glucose
5.4 mmol/l
3.5–5.9 mmol/l
C-reactive protein (CRP)
19 mg/l
0–10 mg/l
Urine
Protein +, Blood +++
Haemorrhage into a renal cyst Oxalate renal stone Renal abscess Transitional cell carcinoma Urinary
tract infection
Explanation
Haemorrhage into a renal cyst
Haemorrhage into a renal cyst is the correct answer. Spontaneous haemorrhage of renal cysts is a well-
known complication of patients with APKD. Ultrasound scanning is key to the diagnosis and sometimes
percutaneous drainage during ultrasound can be attempted depending on the clinical indication.
Analgesia is often the mainstay of treatment.
Oxalate renal stone
290
Although renal stones are a possibility given sudden-onset loin pain and microscopic haematuria,
spontaneous haemorrhage is more likely in the case of patient with a history of APKD, than oxalate renal
stones. Oxalate stones are most commonly seen in patients with short bowel syndrome.
Renal abscess
Renal abscess would be unlikely to present with sudden-onset loin pain and microscopic haematuria.
Given the CRP is only mildly raised, and the WBC is within the normal range, it would unlikely account
for the clinical presentation.
Transitional cell carcinoma is no more likely to be seen in patients with APKD than in the normal
population. Therefore, it is unlikely to be the explanation for the clinical presentation.
Although the patient presents with sudden-onset loin pain, there is no evidence of nitrites or leukocytes
in the urinalysis and inflammatory markers are only mildly raised. This makes the diagnosis less likely.
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A.195.A 60-year-old woman presents with a short history of sinus congestion, epistaxis and joint pains in
her hands. She becomes increasingly short of breath and develops haemoptysis precipitating admission
to hospital. On direct questioning, she admits to passing less urine in the preceding few days.
On examination, she has splinter haemorrhages in both hands and feet. Her blood pressure is 160/80
mmHg. Heart sounds are normal with no murmurs. She has bibasilar fine crackles on auscultation of the
lungs but her jugular venous pressure is not elevated and she has no pedal oedema.
Platelets (PLT) 243 × 109/l 150–400 × 109/l
Urine microscopy Red blood cells and red blood cell casts
Her chest X-ray is shown below.
291
Goodpasture's syndrome
Cryoglobulinaemia
Antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis
IgA nephropathy
Uric acid nephropathy
Explanation
Antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis
This is a classic history for Granulomatosis with polyangiitis. She would need treatment with dialysis and
immunosuppression and most centres would advocate plasma exchange with a creatinine above 400
μmol/l. None of the other diagnoses would classically cause sinus or joint involvement, and pulmonary
haemorrhage is typically seen only in Goodpasture's syndrome and ANCA-positive vasculitis. Note left
ventricular failure can lead to pink sputum and may be confused with pulmonary haemorrhage. To
confirm the diagnosis of pulmonary haemorrhage, a raised KCO would be seen on lung function tests.
The low haemoglobin in the setting of acute kidney disease and nodular changes with cavitation on
chest x-ray with the clinical findings suggest pulmonary haemorrhage rather than infection or fluid to
explain the chest X-ray findings.
Image source: https://commons.wikimedia.org/wiki/File:Wegeners_Granulomatosis_CXR.jpg
Goodpasture's syndrome
Goodpasture's syndrome would present with rapidly progressive glomerulonephritis and pulmonary
haemorrhage. Severe pulmonary haemorrhage may require intubation. Sinus congestion and upper ear,
nose and throat symptoms suggest Granulomatosis with polyangiitis rather than Goodpasture's
syndrome.
Cryoglobulinaemia
Cryoglobulins deposited in medium and small vessels in the glomeruli lead to complement fixation and
inflammation injury which leads to glomerulonephritis. This may be related to lymphoproliferative
diseases such as myeloma and Wasldenstom's macroglobulinaemia or infection such as hepatitis C.
IgA nephropathy
There are no ear, nose and throat symptoms nor pulmonary haemorrhage with IgA nephropathy.
Furthermore ANCA would be negative.
Uric acid nephropathy
Uric acid nephropathy may present with renal colic due to uric acid stones. There are no ear, nose and
throat symptoms as in this case.
*********************************************************************************
292
A.196.A 54-year-old man, who is on continuous peritoneal ambulatory dialysis, presents to the
Emergency Department with dull abdominal pain.
He has a history of hypertension for which he takes a number of agents, and type II diabetes for which
he takes BD insulin.
On examination, he is pyrexial, 37.8 °C; the dialysate fluid is cloudy (with 160 white blood count and 70%
PMNs) but the site of the catheter looks clean.
Investigations:
Platelets 204 × 109/l 150–400 × 109/l
Dialysate fluid 120 white cells/ml
Which of the following is the most appropriate initial treatment?
IV ciprofloxacin
IV gentamicin and vancomycin
Intraperitoneal vancomycin and gentamicin
Oral ciprofloxacin
Oral vancomycin
Explanation
Intraperitoneal vancomycin and gentamicin
This man has symptoms and signs consistent with spontaneous bacterial peritonitis associated with
continuous ambulatory peritoneal dialysis (CAPD). Standard therapy involves instilling IP vancomycin
and gentamicin, as coagulase-negative Staphylococcus is a very common pathogen. Once culture results
are available, antibiotics can then be tailored appropriately. Persistent or relapsing peritonitis, erosion
or protrusion of the cuff around the dialysis catheter may unfortunately necessitate removal.
IV ciprofloxacin
293
Generally IP antibiotics are recommended as first line. Ciprofloxacin may be used in resistant peritonitis,
however quinolone treatment is no longer recommended wherever an effective alternative is available
due to a small but significant risk of debilitating adverse effects.
IV gentamicin and vancomycin
Generally IP antibiotics are recommended as first line. This regimen of antibiotics is, however,
commonly used for tunnel line infections.
Oral ciprofloxacin
Generally IP antibiotics are recommended as first line. Oral ciprofloxacin may be useful in exit site
infections.
Oral vancomycin
Generally IP antibiotics are recommended as first line. Oral vancomycin is used in the treatment of
Clostridium difficile diarrhoea, but it is not helpful in CAPD peritonitis.
294

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A.1.

The rash shown below is seen on his right leg:

Investigation Result Normal Values

Haemoglobin (Hb) 120 g/l 115–155 g/l

White cell count (WCC) 8.0 × 109/l 4.0–11.0 × 109/l

C-reactive protein (CRP) 4.0 mg/l 0–10 mg/l

Corrected calcium (Ca2+) 3.0 mmol/l 2.0–2.6 mmol/l

Creatinine (Cr) 145 µmol/l 50–120 µmol/l

Urea 8.0 mmol/l 2.5–6.5 mmol/l

He is referred for a kidney biopsy.

Your answer was correct

‘Basket weave’ pattern of basement membrane

Granulomatous tubulointerstitial nephritis

Immune complex deposits

Normal kidney biopsy

Granulomatous tubulointerstitial nephritis

Image source: Erythema Nodosum, provided by Wiki Commons

‘Basket weave’ pattern of basement membrane

Crescentic glomerulonephritis refers to a group of illnesses, including antiglomerular basement

Immune complex deposits

Normal kidney biopsy

Investigation Result Normal value

White cell count (WCC) 11.0 × 109/l 4.0–11.0 × 109/l

Creatinine (Cr) 150 µmol/l 50–120 µmol/l

Urea 9.0 mmol/l 2.5–6.5 mmol/l

Sodium (Na+) 139 mmol/l 135–145 mmol/l

Potassium (K+) 3.7 mmol/l 3.5–5.0 mmol/l

Urine dipstick protein Positive

Urine dipstick blood Positive

Antinuclear antibody (ANA) 1/40

Complement C32.2 g/l 0.8–2.1 g/l

Complement C40.49 g/l 0.15–0.5 g/l

Antineutrophil cytoplasm antibodies (ANCA) Negative

Antiglomerular basement membrane (anti-GBM) antibodies Negative

Creatine kinase (CK) 250 U/l 33–211 U/l

Corrected calcium (Ca2+) 2.30 mmol/l 2.2–2.6 mmol/l

An abdominal ultrasound (US) is conducted which is demonstrated below.

What is the most likely diagnosis?

Your answer was incorrect

Drug-induced tubulointerstitial nephritis (TIN)

Multiple myeloma (MM)

Prerenal acute kidney injury (AKI)

Drug-induced tubulointerstitial nephritis (TIN)

Normal kidney ultrasound, courtesy of Wiki Commons

Multiple myeloma (MM)

Prerenal acute kidney injury (AKI)

A computerised tomography (CT) scan is performed:

What is the most likely diagnosis?

Your answer was incorrect

Benign prostatic hypertrophy (BPH)

Abdominal Aortic Aneurysm, courtesy Wiki Commons,

Benign prostatic hypertrophy (BPH)

--He is indicated by the black arrow.

Investigation Result Normal values

White cell count (WCC) 7.0 × 109/l 4.0–11.0 × 109/l

Haemoglobin (Hb) 125 g/l 135–175 g/l

Creatinine (Cr) 80 µmol/l 50–120 µmol/l

Urea 5.0 mmol/l 2.5–6.5 mmol/l

Sodium (Na+) 139 mmol/l 135–145 mmol/l

Potassium (K+) 3.2 mmol/l 3.5–5.0 mmol/l