Current spectrum of hepatobiliary disorders in northern India.

OBJECTIVE To evaluate the current spectrum of hepatobiliary disorders in children in Northern India. SETTING Tertiary level referral hospital. METHODS All children with hepatobiliary disorders presenting between January 1992 through July 1995 were evaluated by clinical assessment, liver function tests, viral and autoimmune markers, liver biopsy, copper studies and other relevant investigations. RESULTS Two hundred and thirty five children with hepatobiliary disorders were seen over three and a half years period (67 cases per year). Acute hepatitis (28%), chronic liver disease (36%) and neonatal cholestasis syndrome (NCS) (26%) were the most common patterns of liver diseases. Chronic liver diseases were constituted by ICC (2%), post-hepatitic etiology (13%), Wilson's disease (21%), autoimmune (4%), non-Wilsonian metabolic diseases (16%), hepatic venous outflow obstruction (2%) and non-cirrhotic portal fibrosis (1%). Cirrhosis was documented in 71% and chronic hepatitis in 12% of cases with chronic liver disease. Fulminant hepatic failure was the presentation in 4% of children with liver diseases. CONCLUSION Chronic liver diseases in Northern India are mainly constituted by post hepatitic, metabolic and cryptogenic etiology and ICC is rarely encountered. NCS is also one of the major subgroups of liver diseases in children.