Spectrum of significant liver diseases at autopsy in children in tertiary care institute

Background To study the etiology of hepatic diseases in children at autopsy in North India. Material and methods One hundred and eighty-one pediatric autopsy cases, (age range 0-14 years) who presented with signs and symptoms of hepatic diseases were analyzed. Results Of these 66.9% were males. Metabolic disorders (38.7%) were the most common followed by hepatitis (viral, autoimmune and others) (19.9%), infiltration by leukaemia/lymphoma (11.0%), infections (6.1%), histiocytic disorders (5.5%), vascular disorders (2.2%), extra hepatic biliary atresia (EHBA, 2.2%), congenital hepatic fibrosis (1.1%), progressive familial intrahepatic cholestasis (0.6%), benign recurrent intrahepatic cholestasis (0.6%), tumors (0.6%), and other causes (12.2%). Conclusion Liver diseases are common in children with a male dominance. Amongst the metabolic disorders, cystic fibrosis (CF), Reye syndrome, Indian childhood cirrhosis (ICC), galactosemia and Wilson disease were the most common. In CF, cholestasis was seen in 6 and invasive fungal infection in 5 cases. Both classical and atypical types of ICC were noted. Predominant macrovesicular steatosis was present in 2 cases of Reye syndrome. In galactosemia, steatosis was absent in 3 cases wherein frank micronodular cirrhosis was present. Acute hepatitis was more common than chronic. It being an autopsy study, incidence of EHBA was low.