Chronic liver failure

Liver disease is rare in childhood, but new means of diagnosis and therapy, including transplantation, have altered its natural history and outcome. It is important that paediatricians are aware of these diseases and their management. Chronic liver failure may be caused by unresolved neonatal liver disease, either inherited biliary hypoplasia or extrahepatic biliary atresia, whereas in older children, autoimmune liver disease, cystic fibrosis and non-alcoholic steatohepatitis (fatty liver disease) are the most common causes. Treatment includes specific medication, nutritional support and liver transplantation, which now has a 90% survival with a good quality of life. Children with significant liver disease should be managed by shared care between a regional and a specialist centre.