Objective To define the clinical spectrum of chronic liver disease among Egyptian children.

Methods 148 children (aged 2 months to 14 years) with suspected chronic liver disease were studied. Liver function tests and abdominal ultrasound, liver biopsy, upper gastrointestinal endoscopy and other special investigations were done whenever indicated.

Results The most common chronic liver diseases in order of frequency were: (1) Chronic hepatitis in 54 children (36.5%) (16 had idiopathic, 15 autoimmune disease, nine cytomegalovirus infection, eight hepatitis C, two hepatitis B, two pan-hypopituitarism with neonatal hepatitis, one Epstein–Barr virus infection and one drug induced). (2) Metabolic liver disease in 50 children (33.8%) (19 had glycogen storage disease (GSD), 18 Niemann–Pick disease, four galactosaemia, three Gaucher disease, two Wilson disease, two hereditary tyrosinaemia, one α1 antitrypsin deficiency and one hereditary fractose intolerance). (3) Biliary diseases in 19 children (12.8%) (nine had biliary atresia, six Byler disease, two alagille syndrome, one choledochalcyst and one sclerosing cholangitis). (4) Cirrhosis in 11 children (7.4%) (five had biliary, two post-hepatic, three GSD and one cryptogenic cirrhosis). The less common entities were autosomal dominant polycystic kidney disease in five children, congenital hepatic fibrosis in three children, portal vein thrombosis in three children, bilharzial hepatic fibrosis in two children and one child had hepatic carcinoma.

Conclusions The study has provided background information on the occurrence of specific liver diseases in Egyptian children. The majority of the children studied had congenital, autoimmune, or cryptogenic disorders and 14% had viral causes.