Incidence
Age
Sex
Geography	- in the West, alcohol is the most common cause - worldwide, viral infection is the commonest cause
Aetiology	- nerosis of liver cells followed by fibrosis and nodule formation -> deranged liver function - liver architecture becomes diffusely abnormal -> disrupted blood flow -> portal hypertension Common causes - alcohol - hepatitis B±D - hepatitis C - ? other viruses Uncommon causes - 1º biliary cirrhosis - 2º biliary cirrhosis (prolonged (months) large duct biliary obstruction due to e.g. strictures, stones, sclerosing cholangitis) - autoimmune hepatitis - hereditary haemochromatosis - Budd-Chiari - Wilson's - Drugs e.g. methotrexate - a1-antitrypsin deficiency - cystic fibrosis Pathogenesis - chronic injury -> inflammation -> necrosis -> activation of stellate cells -> fibrosis - in the space of Disse, normal matrix is replaced by collagens and fibronectin - subendothelial fibrosis -> loss of endothelial fenestrations -> impaired liver function
Presentation
Investigations	LFTs - may be normal - there is normally some elevation of AST/ALT and ALP - if the patient is decompensated, all biochemistry is deranged - serum albumin - outlook is best if this > 25 g/L - PT is prolonged in relation to severity of disease U+Es - low Na suggests severe disease - dilution secondary to defect in free water clearance - may be due to diuretic therapy
Macro
Micro	Micronodular cirrhosis - regenerating nodules > 3 mm in size - liver involved uniformly - commonly due to alcohol damage or biliary tract disease Macronodular cirrhosis - variable sized nodules - larger nodules can contain normal tissue - often seen following HBV infection Determining cause - viral markers - serum autoantibodies - serum immunoglobulins - miscellaneous measures - in young patients, serum copper and serum a1-antitrypsin - serum TIBC and ferritin (screen for hereditary haemochromatosis) USS liver - size and shape - fatty change and fibrosis -> diffuse increased echogenicity - vascular architecture and surface nodularity - patency of portal and hepatic veins - presence of HCC CT - hepatosplenomegaly - dilated collateral vessels - arterial-phase contrast scans are useful for detection of HCC Barium swallow/OGD - detection and treatment of varices Scintiscanning Liver biopsy - type and severity of disease
Staging
Serum markers	- a-fetoprotein - screen for HCC
Management	- manage complications - 6-monthly USS and measurement of AFP - progression may be halted by treating underlying cause - alcohol should be avoided Indications for transplantation - all patients with Child's grade C cirrhosis should be considered - Primary biliary cirrhosis - Chronic hepatitis B; recurrence occurs in 60-70%, with cirrhosis; HBV immunoglobulin prophylaxis reduces recurrence to 30% - Chronic HCV infection; prognosis is good, although HCV recurs - Autoimmune hepatitis (patients not responding to medical management) - Alcoholic liver disease (if "well-motivated") - Primary metabolic disorders (e.g. Wilson's disease, a1-antitrypsin deficiency - scleosing cholangitis
Prognosis	- variable, depending on aetiology Poor prognostic indicators - albumin < 25 g/L - Na < 120 mmol/L - prolonged PTT/INR - persistent jaundice - failure of response to therapy - ascites - haemorrhage from varices - neuropsychiatric complications - small liver - persistent hypotension - failure to treat cause Child's grading - grades A, B, C based on presence of jaundice, encephalopathy, ascites and serum albumin level - grade A has best prognosis; this includes prognosis in surgical procedures
Complications	- portal hypertension and GI haemorrhage - ascites - portosystemic encephalopathy - renal failure (hepatorenal syndrome) - HCC

Hepatobiliary medicine

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