Nephrotic syndrome

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Nephrotic syndrome
Classification and external resources

Histopathological image of diabetic glomerulosclerosis with nephrotic syndrome. H&E stain. ICD-10 ICD-9 DiseasesDB eMedicine MeSH N04. 581.9 8905 med/1612 ped/1564 D009404 Not to be confused with nephritic syndrome Nephrotic syndrome is a nonspecific disorder in which the kidneys are damaged, causing them to leak large amounts of protein[1] (proteinuria at least 3.5 grams per day per 1.73m2 body surface area)[2] from the blood into the urine. Kidneys affected by nephrotic syndrome have small pores in the podocytes, large enough to permit proteinuria (and subsequently hypoalbuminemia, because some of the protein albumin has gone from the blood to the urine) but not large enough to allow cells through (hence no hematuria). By contrast, in nephritic syndrome, RBCs pass through the pores, causing hematuria. According to NephCure, most often, Nephrotic Syndrome is defined by its primary diseases that attack the kidneys filtering system. Some of these cases are idiopathic.

Contents
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1 Signs and symptoms 2 Causes o 2.1 Primary o 2.2 Secondary 3 Diagnosis o 3.1 Classification o 3.2 Differential diagnosis 4 Treatment o 4.1 Supportive o 4.2 Specific o 4.3 Diet 5 Complications 6 Prognosis 7 See also 8 References 9 External links

[edit] Signs and symptoms

It is characterized by proteinuria (>3.5g/day), hypoalbuminemia, hyperlipidemia and edema which is generalized & also known as anasarca or dropsy. Common among 26 years old boys. The edema begins in the face. Lipiduria (lipids in urine) can also occur, but is not essential for the diagnosis of nephrotic syndrome. Hyperlipidemia is caused by two factors:
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Hypoproteinemia stimulates protein synthesis in the liver, resulting in the overproduction of lipoproteins. Lipid catabolism is decreased due to lower levels of lipoprotein lipase, the main enzyme involved in lipoprotein breakdown.[3]

A few other characteristics seen in nephrotic syndrome are:

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The most common sign is excess fluid in the body due to the serum hypoalbuminemia. Lower serum oncotic pressure causes fluid to accumulate in the interstitial tissues. Sodium and water retention aggravate the edema. This may take several forms: o Puffiness around the eyes, characteristically in the morning. o Pitting edema over the legs. o Fluid in the pleural cavity causing pleural effusion. More commonly associated with excess fluid is pulmonary edema.

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Fluid in the peritoneal cavity causing ascites. Generalized edema throughout the body known as anasarca. Most of the patients are normotensive but hypertension (rarely) may also occur. Anemia (iron resistant microcytic hypochromic type) maybe present due to transferrin loss. Dyspnea maybe present due to pleural effusion or due to diaphragmatic compression with ascites. Erythrocyte sedimentation rate is increased due to increased fibrinogen & other plasma contents. Some patients may notice foamy or frothy urine, due to a lowering of the surface tension by the severe proteinuria. Actual urinary complaints such as hematuria or oliguria are uncommon, though these are seen commonly in nephritic syndrome. May have features of the underlying cause, such as the rash associated with systemic lupus erythematosus, or the neuropathy associated with diabetes. Examination should also exclude other causes of gross edemaespecially the cardiovascular and hepatic system.
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[edit] Causes
Nephrotic syndrome has many causes and may either be the result of a disease limited to the kidney, called primary nephrotic syndrome, or a condition that affects the kidney and other parts of the body, called secondary nephrotic syndrome.

[edit] Primary
Primary causes of nephrotic syndrome are usually described by the histology, i.e. minimal change disease (MCD) like minimal change nephropathy which is the most common cause of nephrotic syndrome in children, focal segmental glomerulosclerosis (FSGS) and membranous nephropathy (MN) like membranous glomerulonephritis which is the main cause of nephrotic syndrome in adult. They are considered to be "diagnoses of exclusion", i.e. they are diagnosed only after secondary causes have been excluded.

[edit] Secondary
Secondary causes of nephrotic syndrome have the same histologic patterns as the primary causes, though may exhibit some differences suggesting a secondary cause, such as inclusion bodies. They are usually described by the underlying cause. Secondary causes by histologic pattern: Membranous nephropathy (MN):
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Hepatitis B & Hepatitis C

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Sjgren's syndrome Systemic lupus erythematosus(SLE) Diabetes mellitus Sarcoidosis Drugs (such as corticosteroids, gold, intravenous heroin) Malignancy (cancer) Bacterial infections, e.g. leprosy & syphilis Protozoal infections, e.g. malaria

Focal segmental glomerulosclerosis (FSGS)[4]

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Hypertensive nephrosclerosis HIV[citation needed] Obesity[citation needed] Kidney loss

Minimal change disease (MCD)[4]

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Drugs, especially NSAIDs in the elderly Malignancy, especially Hodgkin's lymphoma Leukemia

Allergy
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Bee sting

[edit] Diagnosis
The gold standard in diagnosis of nephrotic syndrome is 24 hour urine protein measurement. Aiding in diagnosis are blood tests and sometimes imaging of the kidneys (for structure and presence of two kidneys), and/or a biopsy of the kidneys. The following are baseline, essential investigations:
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24 hour bedside urinary total protein estimation.

Urine sample shows proteinuria (>3.5 g per 1.73 m2 per 24 hours). It is also examined for urinary casts, which are more a feature of active nephritis.
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Comprehensive metabolic panel (CMP) shows hypoalbuminemia: albumin level 2.5 g/dL (normal=3.5-5 g/dL). Lipid profile.

High levels of cholesterol (hypercholesterolemia), specifically elevated LDL, usually with concomitantly elevated VLDL is typical.

Electrolytes, urea and creatinine (EUCs): to evaluate renal function.

Further investigations are indicated if the cause is not clear:

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Biopsy of kidney (in case of adult patients only). Auto-immune markers (ANA, ASOT, C3, cryoglobulins, serum electrophoresis). Ultrasound of the whole abdomen.

[edit] Classification
A broad classification of nephrotic syndrome based on underlying cause: Nephrotic syndrome Primary Nephrotic syndrome is often classified histologically: Nephrotic syndrome MCD FSGS MN MPGN Secondary

[edit] Differential diagnosis

When someone presents with generalized edema, the following causes should be excluded: 1. Heart failure: The patient is older, with a history of heart disease. Jugular venous pressure is elevated on examination, might hear heart murmurs. An echocardiogram is the gold standard investigation. 2. Liver failure: History suggestive of hepatitis/ cirrhosis: alcoholism, IV drug use, some hereditary causes. Signs of liver disease are seen: jaundice (yellow skin and eyes), dilated veins over umbilicus (caput medusae), scratch marks (due to widespread itching, known as pruritus), enlarged spleen, spider angiomata, encephalopathy, bruising, nodular liver. 3. Acute fluid overload in someone with kidney failure: These people are known to have kidney failure, and have either drunk too much or missed their dialysis. 4. Metastatic cancer: when cancer spreads to the lungs or abdomen it causes effusions and fluid accumulation due to obstruction of lymphatics and veins, as well as serous exudation.

[edit] Treatment

This section does not cite any references or sources. Please help improve this section by adding citations to reliable sources. Unsourced material may be challenged and removed. (August 2008) Treatment includes:

[edit] Supportive
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Monitoring and maintaining euvolemia (the correct amount of fluid in the body): o Monitoring urine output, BP regularly. o Fluid restrict to 1 L. o Diuretics (IV furosemide). Monitoring kidney function: o do EUCs daily and calculating GFR. Treat hyperlipidemia to prevent further atherosclerosis. Prevent and treat any complications [see below] Albumin infusions are generally not used because their effect lasts only transiently.

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Prophylactic anticoagulation may be appropriate in some circumstances.[5]

[edit] Specific
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Immunosuppression for the glomerulonephritides (corticosteroids,[6] ciclosporin). Standard ISKDC regime for first episode: prednisolone -60 mg/m2/day in 3 divided doses for 4 weeks followed by 40 mg/m2/day in a single dose on every alternate day for 4 weeks. Relapses by prednisolone 2 mg/kg/day till urine becomes negative for protein. Then, 1.5 mg/kg/day for 4 weeks. Frequent relapses treated by: cyclophosphamide or nitrogen mustard or ciclosporin or levamisole. Achieving better blood glucose level control if the patient is diabetic. Blood pressure control. ACE inhibitors are the drug of choice. Independent of their blood pressure lowering effect, they have been shown to decrease protein loss.

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[edit] Diet

Reduce sodium intake to 10002000 mg daily. Foods high in sodium include salt used in cooking and at the table, seasoning blends (garlic salt, Adobo, season salt, etc.) canned soups, canned vegetables containing salt, luncheon meats including turkey, ham, bologna, and salami, prepared foods, fast foods, soy sauce, ketchup, and salad dressings. On food labels, compare milligrams of sodium to calories per serving. Sodium should be less than or equal to calories per serving. Eat a moderate amount of high protein animal food: 3-5 oz per meal (preferably lean cuts of meat, fish, and poultry) Avoid saturated fats such as butter, cheese, fried foods, fatty cuts of red meat, egg yolks, and poultry skin. Increase unsaturated fat intake, including olive oil, canola oil, peanut butter, avocadoes, fish and nuts. Eat low-fat desserts. Increase intake of fruits and vegetables. No potassium or phosphorus restriction necessary. Monitor fluid intake, which includes all fluids and foods that are liquid at room temperature. Fluid management in nephrotic syndrome is tenuous, especially during an acute flare.

[edit] Complications
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Venous thrombosis: due to leak of anti-thrombin 3, which helps prevent thrombosis. This often occurs in the renal veins. Treatment is with oral anticoagulants (not heparin as heparin acts via anti-thrombin 3 which is lost in the proteinuria so it will be ineffective.) Hypercoagulopathy due to extravasation of fluid from the blood vessels (oedema) is also a risk for venous thrombosis. Infection: due to leakage of immunoglobulins, encapsulated bacteria such as Haemophilus influenzae and Streptococcus pneumoniae can cause infection. Acute renal failure is due to hypovolemia. Despite the excess of fluid in the tissues, there is less fluid in the vasculature. Decreased blood flow to the kidneys causes them to shutdown. Thus it is a tricky task to get rid of excess fluid in the body while maintaining circulatory euvolemia. Pulmonary edema: again due to fluid leak, sometimes it leaks into lungs causing hypoxia and dyspnoea. Growth retardation: does not occur in MCNS.It occurs in cases of relapses or resistance to therapy. Causes of growth retardation are protein deficiency from the loss of protein in urine, anorexia (reduced protein intake), and steroid therapy (catabolism). Vitamin D deficiency can occur. Vitamin D binding protein is lost. Hypothyroidism can occur. Thyroxine is reduced due to decreased thyroid binding globulin.

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Microcytic hypochromic anaemia is typical. It is iron-therapy resistant. Hypocalcemia can occur as a result of nephrotic syndrome. It may be significant enough to cause tetany. Hypocalcemia may be relative; calcium levels should be adjusted based on the albumin level and ionized calcium should be checked. Cushing's Syndrome

[edit] Prognosis
The prognosis depends on the cause of nephrotic syndrome. It is usually good in children, because minimal change disease responds very well to steroids and does not cause chronic renal failure. However other causes such as focal segmental glomerulosclerosis frequently lead to end stage renal disease. Factors associated with a poorer prognosis in these cases include level of proteinuria, blood pressure control and kidney function (GFR).

Nephrotic syndrome
URL of this page: http://www.nlm.nih.gov/medlineplus/ency/article/000490.htm

Nephrotic syndrome is a group of symptoms including protein in the urine (more than 3.5 grams per day), low blood protein levels, high cholesterol levels, high triglyceride levels, and swelling.

Causes
Nephrotic syndrome is caused by various disorders that damage the kidneys, particularly the basement membrane of the glomerulus. This immediately causes abnormal excretion of protein in the urine. The most common cause in children is minimal change disease, while membranous glomerulonephritis is the most common cause in adults. This condition can also occur as a result of infection (such as strep throat, hepatitis, or mononucleosis), use of certain drugs, cancer, genetic disorders, immune disorders, or diseases that affect multiple body systems including diabetes, systemic lupus erythematosus, multiple myeloma, and amyloidosis.

It can accompany kidney disorders such as glomerulonephritis, focal and segmental glomerulosclerosis, and mesangiocapillary glomerulonephritis. Nephrotic syndrome can affect all age groups. In children, it is most common from age 2 to 6. This disorder occurs slightly more often in males than females.

Symptoms
Swelling (edema) is the most common symptom. It may occur:
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In the face and around the eyes (facial swelling) In the arms and legs, especially in the feet and ankles In the belly area (swollen abdomen)

Other symptoms include:

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Foamy appearance of the urine Weight gain (unintentional) from fluid retention Poor appetite High blood pressure

Exams and Tests

The doctor will perform a physical exam. Laboratory tests will be done to see how well the kidneys are working. They include:
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Creatine - blood test Blood urea nitrogen (BUN) Creatinine clearance Albumin blood test - may be low Urinalysis - reveals large amounts of urine protein

Fats are often also present in the urine. Blood cholesterol and triglyceride levels may increase. Kidney biopsy may be needed . Tests to rule out various causes may include the following:
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Glucose tolerance test Antinuclear antibody Rheumatoid factor Cryoglobulins Complement levels Hepatitis B and C antibodies VDRL serology

Serum protein electrophoresis

This disease may also alter the results of the following tests:
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Urinary casts Protein electrophoresis - urine Serum iron

Treatment
The goals of treatment are to relieve symptoms, prevent complications and delay progressive kidney damage. Treatment of the disorder that causes the condition is necessary to control nephrotic syndrome. Treatment may be needed for life. Controlling blood pressure is the most important measure to delay kidney damage. The goal is to keep blood pressure at or below 130/80 mmHg. Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) are the medicines most often used in this case. ACE inhibitors may also help decrease the amount of protein loss in the urine. Corticosteroids and other drugs that suppress or quiet the immune system may be used. High cholesterol and levels should be treated to reduce the risk of heart and blood vessel problems. However, a low-fat, low-cholesterol diet is usually not as helpful for people with nephrotic syndrome. Medications to reduce cholesterol and triglycerides may be needed, most commonly statins. A low salt diet may help with swelling in the hands and legs. Water pills (diuretics) may also help with this problem. Low protein diets may or may not be helpful. A moderate-protein diet (1 gram of protein per kilogram of body weight per day) may be suggested. Vitamin D may need to be replaced if nephrotic syndrome is chronic and unresponsive to therapy. Blood thinners may be required to treat or prevent clot formation.

Outlook (Prognosis)
The outcome varies; the syndrome may be acute and short-term or chronic and unresponsive to therapy. The cause and development of complications also affects the outcome.

Possible Complications
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Atherosclerosis and related heart diseases

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Renal vein thrombosis Acute kidney failure Chronic kidney disease Infections, including pneumococcal pneumonia Malnutrition Fluid overload, congestive heart failure, pulmonary edema

When to Contact a Medical Professional

Call your health care provider if symptoms which may indicate nephrotic syndrome occur. Call your health care provider if nephrotic syndrome persists or if new symptoms develop, including severe headache, fever, sores on the skin, cough, discomfort with urination, or decreased urine output. Go to the emergency room or call the local emergency number (such as 911) if convulsions occur.

Prevention
Appropriate treatment of conditions that can cause nephrotic syndrome may help prevent the syndrome.

What is nephrotic syndrome?

It is the name given to a condition when large amounts of protein leak out into the urine. Normal urine should contain almost no protein. In nephrotic syndrome the leak is large enough so that the levels of protein in the blood fall. This page has quite detailed information. If you want a more simple summary, go to our page with short information on nephrotic syndrome.

What trouble does it cause?

The most obvious symptom is usually swelling of the ankles and legs. Extra fluid may also accumulate in the abdomen and around the face, especially overnight. In children and young adults the ankles may be less affected and

the abdomen and face more affected. Most ankle swelling is caused by other diseases ; nephrotic syndrome is a rare cause of ankle swelling. Urine tests and blood samples are required to prove that nephrotic syndrome is the cause. The protein leak can sometimes make the urine frothy. Some people feel tired. Other problems can occur in nephrotic syndrome, probably as a result of some particular proteins that are missing because of the leak.

Infections

Patients are unusually susceptible to some infections.

In people who have nephrotic syndrome for a long time, Cholesterol cholesterol is often very high. This may increase the risk of narrowing of the arteries unless it is treated. Blood is more likely to clot in the veins, which may cause Blood thrombosis in the leg veins and occasionally elsewhere. clotting Some of these may require extra treatments to prevent them

Severe swelling of the ankles in nephrotic syndrome ... and after treatment. Cartoon by Beth Shortt, copyright www.edren.org. Contact us about re-use

What tests are necessary?

Lots of blood tests are useful. The most important test however is a renal biopsy. This test is designed to take a small piece of kidney to look at under the microscope. It is done with local anaesthetic and involves putting a needle into the kidney through the back; a scanner is used to find the kidney so the test is done in the X-ray department. More information on kidney biopsies

Can I do without a biopsy?

There are a few exceptions. In children the nephrotic syndrome is nearly always caused by a condition that responds easily to treatment, treatment is usually started first, and the biopsy only done if the protein leak is not cured. In diabetes, a biopsy may not be essential if there is strong evidence that it is likely to be related to diabetes. In adults, however many causes are possible, making a renal biopsy important - see next section). Up to top

What causes nephrotic syndrome?

In many cases, a definite cause is not found. Some have recognised underlying causes such as an allergic reactions to medicines, slow infections like Hepatitis B, and diabetes. It is divided into a number of types according to the appearances of the kidney under the microscope. These different types respond to treatment differently, and may mean different things for your future health. Disease Comments

Minimal change Most common in children usually disease responds quickly to steroids FSGS Membranous nephropathy Cause is unknown in most cases, some response to special treatment Cause is often unknown, but may occur with other diseases drugs and infections. Some improve with time but some deteriorate, these may

respond to treatment immunosuppression MCGN

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Rare cause of nephrotic syndrome, can be associated with other medical conditons Occurs as a long term complication in some patients. More likely if blood sugar has been poorly controlled. Excellent control of blood pressure and treatment with ACE inhibitors reduces the risk of renal failure More common in young women mainly affecting skin and joints, SLE affecting the kidney is rare and usually requires special treatment Usually a consequence of other chronic diseases like Rheumatoid Arthritis, or of chronic infections

Diabetes

SLE

Amyloid

What treatment is available?

The effects of fluid retention are managed by diuretics that force the kidney to put out more salt and water in the urine. This is helped by restricting the amount of salt in the diet and by avoiding excessive fluid intake. If a lot of fluid has been retained, it is important that diuretic therapy is carefully controlled by regular blood tests and weighing. Some patients may require to be admitted to hospital. Diet - dietary protein should not be increased above normal. Avoiding excessive salt is very important. Control of blood pressure (often high in people with kidney disease) is important in all patients. A type of blood pressure drug known as an ACE inhibitor has been proven to be particularly good at protecting kidney function and reducing the amount of protein in the urine. You are very likely to be prescribed one of these. More information on high blood pressure and kidney
diseases

Special treatment to prevent the complications mentioned above (infection, high cholesterol, thrombosis) is also important, especially if the nephrotic syndrome is likely to last for a long time. According to the type of kidney disease diagnosed by the biopsy, treatment to control the cause of nephrotic syndrome may be recommended: 1. Nothing: some cases of the nephrotic syndrome will improve with time and require no special treatment. Others are known to respond very poorly to any known treatment. 2. Steroid tablets (eg prednisolone): one form of the disease ('minimal change disease') is very sensitive to steroid treatment and short courses of these can be used, often with great success. Short-term use of steroids minimises their potential side-effects. 3. Immunosuppression: some of the more difficult cases are thought to be triggered by the body's own immune system, and treatment to control this can be used. These therapies come either as tablets or as drips which are given in hospital. These treatments are not commonly used because they can be toxic but they can also be very effective in some patients. Further information on immunosuppressive drugs used in kidney diseases is available here.

Are there any long term complications?

In some cases there are. Some people with severe disease do not respond to treatment. After years of heavy protein leakage, the kidneys can fail, and some people will progress onto kidney failure with the need for dialysis or a transplant to keep them well. The renal biopsy and other tests help to predict the likelihood of this. [[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[[

Nephrotic Syndrome Overview, Signs & Symptoms, Causes

Overview of Nephrotic Syndrome
Nephrotic syndrome (NS) is a condition that is often caused by any of a group of diseases that damage the kidneys' filtering system, the glomeruli. The structure of the glomeruli prevents most

protein from getting filtered through into the urine. Normally, a person loses less than 150 mg of protein in the urine in a 24-hour period. Nephrotic-range proteinuria, the urination of more than 3.5 grams of protein during a 24-hour period, or 25 times the normal amount, is the primary indicator of NS.

Incidence and Prevalence of Nephrotic Syndrome

About two in every 10,000 people experience nephrotic syndrome. Nephrotic syndrome prevalence is difficult to establish in adults because the condition is usually a result of an underlying disease. In children, it is diagnosed in more boys than girls, usually between 2 and 3 years of age. Article Continues Below Ads by Google Improve Kidney Health Probiotics to promote removal ofnitrogenous waste for kidney health www.kibowbiotech.com Diagnosis Parkinson's? Learn more about PD and readabout other people's experiences www.parkinsons-voices.eu Tingling Hands? Numbness in your Arms, Hands or Fingers?Here is the solution. www.tingling-hands.net Test-it urine strips Rapid & accurate urine screeningCall now - distributors wanted www.lifeassay.com

Nephrotic Syndrome Signs and Symptoms

In addition to proteinuria, there are three main symptoms of nephrotic syndrome associated with protein leaking into the urine:
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Hypoalbuminemia (low level of albumin in the blood) Edema (swelling) Hypercholesterolemia (high level of cholesterol in the blood)

Hypoalbuminemia is a low level of albumin (a protein) in the blood due to proteinuria. Low albumin in the blood causes fluid to move from the blood into the tissue, causing swelling. The

kidney perceives the decrease of fluid in the blood and aggressively retains as much fluid and salt as it can. This contributes to the body's fluid-overload state. Nephrotic-related swelling makes tissue puffy, soft, and impressionable to the touch. Edema is most common in the legs and feet, especially after standing all day. It can cause feelings of tightness in the extremities and may affect mobility. In later stages, swelling may occur in the abdomen (ascites), hands, and around the eyes in the morning (called periorbital edema). In later stages, the whole body may swell (anasarca). Some people gain weight after fluid builds up in their bodies for a long time. Hypercholestrolemia, high blood cholesterol, is common in nephrotic syndrome). In addition to albumin, other important enzymes involved in cholesterol metabolism slip through the glomeruli, which contribute to high blood cholesterol.

Nephrotic Syndrome Complications

Nephrotic syndrome is associated with renal failure. The disease that causes NS can damage the glomeruli and can interfere with their ability to clean the blood. The edema that is present in the legs may also be occurring in the kidney tissue itself and can interfere with the kidneys' ability to clean the blood. Renal failure can either be gradual (CRF) or acute (ARF). A hypercoaguable state, in which the blood abnormally overclots, is also seen in some patients with NS. This means that they are at risk for developing a blood clot in the legs or in the renal veins that transport blood from the kidney. Some patients take blood thinners to prevent this complication.

Causes of Nephrotic Syndrome

There are a number of different disorders that can cause NS. Diabetes and, to a lesser extent, hypertension can cause diffuse damage to the glomeruli and can ultimately lead to NS. The following diseases can cause specific damage to the glomeruli and often result in the development of heavy proteinuria and in many instances NS:
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Amyloidosis (the stiffening and subsequent malfunction of the kidney due to fibrous protein deposit in the tissue) Congential nephrosis Focal segmental glomerular sclerosis (FSGS) (creates scar tissue in the glomerulus, damaging its protein-repellant membrane) Glomerulonephritis (GN) o Diffuse mesangial proliferative GN (affecting the messangium) o Membranous (damages the protein-repellant membrane) o Postinfectious (occurs after an infection) IgA nephropathy (Berger's disease) (deposit of specific immunoglobulin A causing an inflammatory reaction and leading to glomerulonephritis)

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Minimal change disease (Nil's disease) Pre-eclampsia (rarely associated with NS, more often associated with heavy proteinuria)

Many of these diseases tend to occur more often in certain age groups:
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Less than 1 year old o Congenital nephrosis Less than 15 years old o Min change o FSGS o Other Age 15 to 40 years o Min change o FSGS o Other Over age 40 o Membranous GN o Diabetic nephropathy Over 60 o Amyloidosis may account for up to 20% of cases

Kidneys - nephrotic syndrome

Summary
Nephrotic syndrome is a condition of the kidneys. It is usually caused by one of the diseases that damage the kidneys' filtering system, allowing protein to be filtered out into the urine (proteinuria). Symptoms include foamy and frothy urine, unexplained weight loss, oedema (fluid retention or swelling), muscle wasting, stomach pain and dizziness. Treatment includes medications and dietary changes. Share this article
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Nephrotic syndrome is a condition of the kidneys. It is usually caused by one of the diseases that damage the kidneys filtering system. This allows protein to be filtered out into the urine (proteinuria).

When the protein level in the blood drops, liquid seeps out of the smallest blood vessels (capillaries) all over the body and settles into the surrounding tissue, causing fluid swelling (oedema). Treatment includes medications and dietary changes. Minimal change disease (lipoid nephrosis) is the most common form of nephrotic syndrome in children. How your body is affected Blood is cleaned in the kidneys as it passes through tiny filters called nephrons. Each kidney contains about one million nephrons. The kidneys remove waste products from the blood (such as products of food metabolism), while maintaining a balance of nutrients, salts and water. Normally, protein is not removed when the kidneys filter waste from the blood. However, when the kidneys are damaged, protein leaks through the damaged filters and is removed from the body in the urine, along with the waste products. The two proteins that are most likely to be present in the urine when this happens are albumin (controls blood volume) and globulin (largely made up of antibody proteins). Normally a person loses less than 150mg of protein in the urine in a 24-hour period. A person with nephrotic syndrome can lose more than 3.5g of protein in the urine during a 24-hour period or 25 times the normal amount. Signs and symptoms The symptoms of nephrotic syndrome include:
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Foamy and frothy urine Unexplained weight loss General malaise (feeling unwell) Oedema (fluid retention or swelling), particularly around the abdomen (belly area), legs and eyes Muscle wasting Stomach pain Dizziness when standing up from a lying or sitting position (orthostatic hypotension).

Causes of nephrotic syndrome Some of the causes of nephrotic syndrome include:

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Changes to the immune system (minimal change or lipoid nephrosis) this type is most common in children. It is called minimal change because the kidney filters appear normal under a microscope. The cause is thought to be changes in certain cells of the immune system. The function of the kidneys is normal and the outlook for recovery is usually excellent. Inflammation local inflammation or swelling damages and scars the kidney filters. Examples of this are focal glomerulosclerosis and membranous nephropathy. Treatment

may not resolve the condition and the kidneys may gradually lose their ability to filter wastes and excess water from the blood. Secondary nephrotic syndrome can be caused by certain conditions including diabetes, drugs, cancer and systemic lupus erythematosus (SLE).

Complications Complications of nephrotic syndrome can include:

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Dehydration low protein levels may lead to a reduction in blood volume. In severe cases, intravenous fluids may be given to boost the bodys water content. Blood clots in the leg veins and occasionally in the kidney veins. Blood clots can also go into the lungs and cause chest pain, breathlessness or coughing up of blood. Infection infection and inflammation (peritonitis) of the peritoneal cavity. This is the thin elastic lining that contains the pancreas, stomach, intestines, liver, gallbladder and other organs. A fever may indicate infection. Kidney failure without treatment, the kidneys may fail in extreme cases.

Diagnosis Diagnosing nephrotic syndrome involves a number of tests, including:

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Urine tests excessive protein makes the urine appear frothy and foamy. A dipstick urine test can also detect protein levels (proteinuria). A 24-hour urine collection or a spot urine protein/creatinine ratio may be done if urine protein is found on the dipstick. These tests measure the amount of protein more precisely and identify whether kidney damage is mild, moderate or heavy. Blood tests to check the blood protein and creatinine (waste made by muscle activity) levels. Biopsy a small sample of kidney tissue is taken and examined in a laboratory.

Further tests Sometimes further tests may be required. These may include:
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Ultrasound an examination of the kidneys using sound waves to outline the structure of organs. Computed tomography (CT) scan or magnetic resonance imaging (MRI) using radio-frequency wavelengths and a strong magnetic field to provide clear and detailed pictures of internal organs and tissues.

Treatment Minimal change nephrotic syndrome fixes itself in around 40 per cent of cases. Other causes of nephrotic syndrome are also often treatable. It is essential to consult a kidney specialist (nephrologist) who can develop a management plan for your condition. Treatment depends on the severity of the condition, but may include:

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Specific medications treatment for some of the causes (for example, steroids for minimal change, immunosuppression for membranous nephropathy or focal sclerosis) this may lead to complete or partial remissions of the nephrotic syndrome Diuretics to control the swelling tendency Medication to control high blood pressure.

All patients with persisting nephrotic syndrome should be treated with:

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Angiotensin active agents (ACE inhibitors or angiotensin blockers) to reduce the amount of proteinuria A low salt diet to help swelling and to assist in the reduction of proteinuria Statins to help with cholesterol control and to reduce proteinuria.

Where to get help

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Your doctor Kidney Health Information Service Tel. 1800 4 KIDNEY (543 639), or TTY users phone 1800 555 677 then ask for 1800 454 363

Things to remember
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Nephrotic syndrome is the failure of the kidneys to maintain enough protein in the blood. Minimal change disease (lipoid nephrosis) is the most common form of nephrotic syndrome in children. Treatment includes medications and dietary changes. Summary Bee stings have previously been implicated in the development of nephrotic syndrome, though the reported cases in the literature are poorly documented. A three-years-old girl developed generalized edema eight days after a bee sting. The physical examination and laboratory findings were relevant with nephrotic syndrome. Her serum IgE level was to be increased. Prompt remission with resolution of edema and normalization of the laboratory findings was obtained after given the corticosteroid treatment. Due to existence of association between development of nephrotic syndrome and hypersensitivity, cases of bee sting must be closely followed-up for multiple immunemediated complications. 2006, F rat niversitesi, T p Fakltesi y Top y Summary y Introduction y Case Presentation y Disscussion y References Introduction The most common type of nephrotic syndrome in childhood is the idiopathic one, which histologically presents as a minimal lesion disease with excellent response to corticosteroid treatment 1. The etiology and pathogenesis of the idiopathic nephrotic syndrome remains obscure 2. Numerous examples of abnormal immune responsiveness have been described in minimal change nephrotic syndrome. The association of minimal

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change nephrotic syndrome with allergy and with certain genetic markers of immune responsiveness, as well as the excellent response of proteinuria to immunomodulatory agents, has suggested a causal relationship between the immunological and renal abnormalities. Studies of humoral and cellular immune functions support the hypothesis that immune regulation is abnormal in this clinical entity 3. The association between nephrotic syndrome and allergic phenomena was originally recognized about 40 years ago, when case reports appeared which described nephrotic syndrome triggered by bee sting, poison ivy and pollen hypersensitivity 4,5. A rare case of nephrotic syndrome following a bee sting in a female child was reported. y Top y Summary y Introduction y Case Presentation y Disscussion y References Case Presentation A three-years-old girl was presented with appearance of generalized edema. Her history revealed that, eight days before the admission she had a bee sting on her neck, which produced redness and edema in the same area. Eight days after from this event the child had marked edema to be onset from her eyelids and face; afterwards the edema extended to legs and eventually the whole body. She had no fever. There was no history of atopy. Generalized edema and ascites were determined on her physical examination, and arterial blood pressure measurements were within normal limits. The laboratory investigations revealed an erythrocyte sedimentation rate of 70 mm/h and normal complete blood count. Serum total protein was 4.08 g/dl, albumin 1.51 g/dl, total cholesterol 420 mg/dl, triglycerides 280 mg/dl, serum urea 42 mg/dl, serum creatinine 0.4 mg/dl, creatinine clearance 65 ml/min. Complement C3 was 1.04 g/L (0.90-1.80 g/L), C4 0.25 g/L (0.100.40 g/L), IgA 2.08 g/L (0.90-3.95 g/L), IgM 1.20 g/L (0.40-2.30 g/L), IgG 8.82 g/L (716 g/L) and IgE 490 u/ml (0.00-190 u/ml). HBs antigen was negative. Examination of her urine revealed marked proteinuria with the presence of one to two leukocytes per high-power field; this prompted referral for hospital treatment. The total proteinuria was 3.5 g/day. Human albumin was administered once because of her marked edema and ascite; then prednisolon treatment introduced at the dose of 60 mg/m2/day. On the fourth day of treatment the child s urine was protein free, and there was a significant increase in her diuresis (3.2 ml/kg/h) and resolution of the edema. The steroid regimen was changed to every other day. Following next months at the steroid regimen was tapered gradually and stopped end of sixth month. Three months after end of the treatment, recurrence was not observed. y Top y Summary y Introduction y Case Presentation y Disscussion y References Discussion Numerous studies have confirmed that there is an increased incidence of atopy (infantile eczema, allergic rhinitis and asthma) both in patients with steroid-sensitive nephrotic

syndrome and their first degree relatives, although the occurrence of overt atopic disease at the time of nephrosis is uncommon 6,7. Several authors have reported a role for allergy in the development and maintenance of this disease, especially during childhood 2. There appears to be a pathogenic relationship between respiratory and food allergy and proteinuria in some cases of nephrotic syndrome 8. Atopic individuals presenting with nephrotic syndrome, especially if the nephrosis is frequently relapsing, may have increased levels of serum IgE and interleukin-4 (IL-4). Hypersensitivity type I responses with increased IgE and IL-4 have long been known to be involved in atopic diseases 9,10. In the literature there are reports on the occurrence of nephrotic syndrome after insect sting, particularly bee stings 1-11, but these are very rare case. Shishkin 8 presented a series of 52 patients with various morphological forms of glomerulonephritis and history of allergy; 71% of his patients had elevated serum concentrations of IgE. Most of these patients had minimal change nephrotic syndrome and in one case the nephrotic syndrome ensued after insect sting. Also, in this study it was reported that normal levels of IgE were more frequently associated with steroid-resistant nephrotic syndrome characterized by a progressive decline of renal functions and severe damage to the glomeruli. In a study by Elming and Solling 12, it was suggested that there may be a nonspecifically increased urinary excretion of albumin following insect sting, but none of their patients had developed nephrotic syndrome during follow up period. Cuoghi et al. 13, in a series of 180 children with nephrotic syndrome found that three children had relapses triggered by the insect sting and all of them went to remission with steroid treatment. Clinical severity of nephrotic syndrome following bee sting and response to steroid / cytotoxic treatment is variably different 14,15. Nephrotic syndrome in the case presented here was caused by a bee sting and also her serum IgE level was found elevated. Thus, existence of association between development to be of nephrotic syndrome and hypersensitivity can be considered, because it is reported that minimal change nephrotic syndrome patients with atopy history have increased levels of serum IgE 2,9,10. Also in agreement with the relevant literatures, our case had a very favorable clinical course with prompt response to corticosteroid treatment. All this conditions reveal that minimal change nephrotic syndrome associated with allergy has a very favorable response to corticosteroid treatment. Cases of insect sting, particularly bee stings, must be closely followed up because of the multiple problems, especially for immune-mediated complications such as nephrotic syndrome.