Young children with liver cirrhosis have a significantly high risk of mortality. However, there are few studies regarding early childhood-onset cirrhosis. This study aims to explore the causes, clinical findings and prognosis of biopsy-proven LC in infants, toddlers and preschoolers. We enrolled young children with biopsy-proven cirrhosis from January 2010. Till January 2020, the study has been going on for 10 years. A total of 139 cirrhotic children were enrolled, including 87 boys and 52 girls. The median age at initially histological diagnosis of cirrhosis was 2 years old (range: 1 month-6 years). Sixty-two patients reported yellowish discoloration of sclera and/or skin as an initial symptom. Ninety-three patients had definite etiologies while 46 had indeterminate causes. Among the confirmed cases, 31 had hepatitis B virus (HBV) infection, accounting for 33.3%. Subsequently, glycogen storage disease was diagnosed in 16 cases and Wilson disease in 14 cases. In these patients with HBV infection, 9 finally achieved hepatitis B surface antigen (HBsAg) loss (29.0%) after effective antiviral therapy during the follow-up. Logistic regression revealed that baseline alanine aminotransferase (odds ratio 1.008, P=0.028) was the independent predictor of HBsAg loss. Furthermore, 1 patient who underwent second biopsies showed histological reverse. HBV infection is an important cause of paediatric cirrhosis in our study. The Ppathogenesis of HBV-related cirrhosis in early childhood deserves further studies.
Biopsy-proven liver cirrhosis in young children: A 10-year cohort study.