Spectrum of pediatric liver disease in a tertiary care center in western India

Abstract

Abstract Aim To study the spectrum of liver disease in children at a tertiary care liver specialized center in western India. Type of study Cross-sectional observational study. Setting Tertiary care liver specialized center. Subjects Children below 18 years age presenting with liver disease. Methods All successive children with primary liver disease seen from August 2006 to July 2009 were assessed as per set protocol. Clinical presentation and disease course, primary etiology of liver disease, and treatment offered were studied. As this was a cross-sectional study, long-term disease outcome was not primarily assessed. Results 113 children presented with liver disease. Acute liver disease was seen in 14% cases and comprised of systemic infections involving liver. Chronic liver disease (CLD) accounted for 86% of cases. Common etiology for CLD was metabolic disease (40/97; 41%) followed by Budd–Chiari syndrome (27/97; 28%) and extrahepatic biliary atresia (EHBA 10/97; 10%). Acute liver failure was seen in 12/113 cases (11%). A total of 26% cases with CLD presented with portal hypertension. Cirrhosis was detected on liver biopsy in 13/40 (32.5%) cases. Hepatopulmonary syndrome was seen in 2 cases. 80% cases of EHBA had progressive liver disease. Four cases underwent liver transplantation, indications being EHBA with biliary cirrhosis ( n =2), decompensated hepatic Wilson disease ( n =1), and hepatopulmonary syndrome ( n =1). Conclusions Metabolic liver disease, Budd–Chiari syndrome, and EHBA were the chief etiologies of CLD. Wilson disease and Gaucher disease were the commonest metabolic disorders seen. CLD was seen more common compared to acute liver disease.