Biopsy-proven liver cirrhosis in young children: A 10-year cohort study
Yi Dong1, Aiqin Li1, Shishu Zhu1
1The Fifth Medical Center (formerly Beijing 302 Hospital), Chinese PLA General Hospital, Beijing, China.
Insights
Hepatitis B virus (HBV) infection is a significant cause of pediatric cirrhosis in young children. Effective antiviral therapy can lead to hepatitis B surface antigen (HBsAg) loss in some patients, indicating potential for recovery.
Area of Science:
- Pediatric Gastroenterology and Hepatology
- Viral Hepatitis Research
- Childhood Liver Disease Studies
Background:
- Early childhood-onset cirrhosis presents a high mortality risk with limited research.
- Understanding the etiology and clinical course of cirrhosis in young children is crucial for improved outcomes.
- Biopsy-proven cirrhosis in infants, toddlers, and preschoolers requires dedicated investigation.
Purpose of the Study:
- To investigate the causes, clinical manifestations, and prognosis of biopsy-proven cirrhosis in early childhood.
- To identify key etiological factors contributing to pediatric cirrhosis.
- To evaluate the outcomes of antiviral therapy in children with hepatitis B virus (HBV)-related cirrhosis.
Main Methods:
- A 10-year prospective study (2010-2020) enrolled 139 children with biopsy-proven cirrhosis.
- Etiologies were determined through comprehensive clinical and laboratory assessments.
- Hepatitis B virus (HBV) infection was identified as a primary cause, with outcomes of antiviral therapy analyzed.
- Logistic regression was used to identify predictors of hepatitis B surface antigen (HBsAg) loss.
Main Results:
- Hepatitis B virus (HBV) infection was identified in 33.3% (31/93) of confirmed pediatric cirrhosis cases.
- Glycogen storage disease (16 cases) and Wilson disease (14 cases) were other significant etiologies.
- Antiviral therapy led to hepatitis B surface antigen (HBsAg) loss in 29.0% (9/31) of HBV-infected children.
- Baseline alanine aminotransferase levels independently predicted HBsAg loss (OR 1.008, p=0.028).
- One patient demonstrated histological improvement after a second biopsy.
Conclusions:
- Hepatitis B virus (HBV) infection is a major contributor to cirrhosis in young children.
- Antiviral therapy shows promise for managing HBV-related pediatric cirrhosis, with potential for viral marker clearance.
- Further research into the pathogenesis of HBV-related cirrhosis in early childhood is warranted.
Abstract:
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 cirrhosis in infants, toddlers and preschoolers. We enroled 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 aetiologies 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, nine 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, one patient who underwent second biopsies showed histological reverse. HBV infection is an important cause of paediatric cirrhosis in our study. The pathogenesis of HBV-related cirrhosis in early childhood deserves further studies.
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