Cirrhosis

causative PATH PT Dx* Management/

Diseases
Chronic Inflammation Abdominal pain/dark urine/pale stools/ weight HepcAb Hep B:
loss/ Pruritis/ fatigue/Jaundice/ Hepatomegaly. HepbAb Immune- enhancing therap
+/- Splenomegaly. Targeted antiviral therapy:
Nucleos(t)ide analogues: la
Viral B&C IVDrugs = Hep C adefovir, tenofovir, enteca
Sex= Hep B Last 2 are the most effectiv
Hep C:
IFN, PEG, RBV, PI: Protease

- Copper metabolism with accumulation of CU to toxic levels in - Present with acute fulminant liver failure. 1st: Slit lamp (eye checkup; examine the eye). • CU chelation
many organs, mainly in eyes, liver and brain. - Cu deposition in: - Ceruloplasmin (CU carrier protein) - Penicillamine
- Due to genetic mutations in CU ion transporter gene (ATP7B) • Basal ganglia => Chorea. - Urinary CU - Zinc, Trientene
resulting principally in impaired excretion of CU. - Often associated with wide variety of Best: biopsy  CU • Liver transplantation
Wilson's
neurological & psychiatric manifestations. • Family Screening
• Liver => cirrhosis.
• Eyes => Kaischer Fleischer rings.
- Typical triad of cirrhosis.
- Primary hemochromatosis - Mutations in HFE gene on Asymptomatic in early stage. Iron overload: • Best: Phlebotomy/Venes
chromosome 6. - DM/Bronze diabetes (rare) • 1st: Ferritin (250 mcg/L in Women, 300 mcg/L Men). treatment option.
- Skin bronzing (hyperpigmentation). Ferritin >1000 mcg/L => suggest liver damage + fibrosis or - Removal of a unit of bloo
Dysregulation of iron intestinal absorption resulting in excess Iron - Testicular atrophy. cirrhosis. - Initially weekly
accumulation in organs (total body iron up to 50g) with >30% - Arthritis. • Transferrin Saturation >45% - Ferritin of < 50 and TF% <
Hemochro accumulating in liver. - Cardiomyopathy. HFE gene mutation (C282Y) – required for diagnosis: • Fe chelators (e.g. Desferr
matosis Increased iron storage due to inappropriately increase iron • Homozygotes for C282Y mutation >98% of primary - Secondary hemosiderosis
absorption resulting in multi-organ iron overload and damage hemochromatosis - Intolerant of venesection
• Minor mutation - Homozygote for H63D mutation - less Fe
overload
Liver biopsy:
• Hepatic iron overload (Hepatic iron index)
Best is biopsy:  Fe.
- Metabolic inherited disease due to impaired function of alpha-1 - Shortness of breath. • Low serum alpha-1 Antitrypsin level. - Liver (+/- lung transplanta
antitrypsin. - Mild hepatitis • A1-AT Phenotyping/Genotyping – PiZZ variant causes most Advanced cases
{Anti-trypsin inhibit Elastase in the lung. In this condition due to - Mild abnormal LFTs severe disease. - No smoking as accelerate
A1 Anti-
anti-trypsin deficiency Elastase is not inhibited (regulated), - Emphysema on CXR. • Liver biopsy – intracellular A1-AT – PAS + granules & fibrosis/
Trypsin
therefore COPD take place). cirrhosis, + PAS macrophages.

- Liver disease characterized by accumulation of cytoplasmic

inclusions (PAS granules)

* In Dx for:
Wilson's => never pick serum CU for Dx.
Hemochromatosis => never pick serum Fe for Dx.
SIRS = Systemic Inflammatory Response Syndrome
 1stHit: 1) Insulin resistance. 2) Lipid deposition in liver parenchyma. - Steatosis and Non-alcoholic Steatohepatitis • No excess alcohol (<30g for men, <20g for women). - Liver transplantation
2ndHit: 1) oxidative stress. 2) Adipokinase. (NASH) - Majority asymptomatic. • Negative investigations for other causes of chronic hepatitis - Treat Diabetes, Hypertensi
Cirrhosis  1) progressive fibrosis. 2) Liver failure, HCC. - Diabetes, obesity. (e.g. Hepatitis B, C) Hypercholesterolemia.
Spectrum: - Complications of cirrhosis with jaundice, • Bright echogenic liver on ultrasound - Lifestyle modifications (e.g. W
Steatosis (Fat vesicles in hepatocytes)  NASH (steatosis + inflammation + fibrosis) encephalopathy and ascites. • Liver biopsy. and Exercise, Mediterranean low calor
Cirrhosis • lipids Aim for weight loss of 10% BW
NAFLD Bariatric surgery
• Features of the metabolic syndrome:
- Waist >94cm men, >80cm for women
- Fasting glucose>5.6 or on Rx
- Raised triglycerides>1.7mmol/l
- Low HDL<1.0mmol/l
- BP>135/85mmHg

Alcohol consumption. - SIRS features – fever, leukocytosis 1) Long Hx of alcohol consumption. • Alcohol abstinence:
Spectrum: - Acute hepatitis with jaundice and tender 2) Abnormal LFTs: - Benzodiazepines for severe
Normal liver Alcoholic steatosis  Alcoholic Steatohepatitis hepatomegaly - Moderate rise in AST/ALT <300 (if >300 consider other chlordiazepoxide, Diazepam,
(ASH)+ fibrosis  Alcohol related cirrhosis. - Liver dysfunction – ascites, coagulopathy, diagnosis) • Treat infection if present.
encephalopathy - AST > ALT (high AST:ALT ratio) • Nutrition
Acute - Alcoholic steatosis, Alcoholic Steatohepatitis (ASH)+ fibrosis & 3) Liver dysfunction: - High dose I.V. Vitamin B1 (Th
Alcoholic Alcohol related cirrhosis can turn into  Acute Alcoholic Hepatitis. - Raised INR, Wernicke's encephalopathy is high)
- Raised bilirubin - Dietitian support
Hepatitis - Low threshold for nasoente
4) Enlarged liver on USS, ascites.
5) Liver biopsy: - Treat complications of liver
- Neutrophil infiltration dysfunction/failure
- Ballooning of hepatocytes - Ascites - Diuretics
- Mallory bodies - Encephalopathy – Lactulose
• Corticosteroids in severe ca