Liver and biliary disease in childhood

Acute liver disease or failure in children is most often due to viral hepatitis (A, B, E or sero-negative), paracetamol overdose or inherited metabolic liver disease. The clinical presentation includes jaundice, coagulopathy and encephalopathy. Uncomplicated acute hepatitis resolves spontaneously, but progressive acute liver failure is fatal in 70% of cases and requires referral to specialized units to prevent complications and for consideration for liver transplantation. Chronic liver disease may be due to unresolved neonatal liver disease, either inherited cholestasis or α1-antitrypsin. Chronic viral hepatitis B and C are rare but significant diseases, which require family support and long-term monitoring. Treatment for hepatitis B remains unsatisfactory, but combination therapy for hepatitis C is successful in over 70% of children. In older children, autoimmune liver disease or cystic fibrosis is the commonest causes, but non-alcoholic steatohepatitis is also common. Treatment includes specific medication, nutritional support and liver transplantation for end-stage disease. The long-term outcome of liver transplantation is excellent, more than 80% surviving with good quality of life.