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Updated: Mar 25, 2026

A Mouse Model of Chronic Liver Fibrosis for the Study of Biliary Atresia
Published on: February 3, 2023
Expression of transforming growth factor-β1 and connective tissue growth factor in congenital biliary atresia and
1Department of Gastroenterology, Children's Hospital of Zhejiang University, Hangzhou, China.
Insights
Transforming growth factor-β1 (TGF-β1) and connective tissue growth factor (CTGF) expression increases with liver fibrosis in infants. Early expression in biliary cells may contribute to congenital biliary atresia.
Area of Science:
- Pediatric Hepatology
- Gastroenterology
- Fibrosis Research
Background:
- Congenital biliary atresia and neonatal hepatitis are leading causes of cholestasis in infants.
- Liver fibrosis is a common complication in both conditions, impacting long-term outcomes.
- Understanding the molecular mechanisms driving fibrosis is crucial for developing targeted therapies.
Purpose of the Study:
- To investigate the expression of TGF-β1 and CTGF in infant liver tissues.
- To determine the relationship between TGF-β1 and CTGF expression and the degree of liver fibrosis.
- To explore the potential role of these growth factors in the pathogenesis of congenital biliary atresia.
Main Methods:
- Comparative analysis of liver tissue from 36 infants with cholestasis, divided into congenital biliary atresia and neonatal hepatitis groups.
- Histopathological assessment of liver fibrosis using H&E and Masson's trichrome staining.
- Immunohistochemical evaluation of TGF-β1 and CTGF expression levels and localization.
Main Results:
- Liver fibrosis was significantly more advanced in congenital biliary atresia compared to neonatal hepatitis (P < 0.01).
- TGF-β1 and CTGF expression was significantly higher in congenital biliary atresia patients, localized to hepatocytes and biliary epithelial cells, versus mainly hepatocytes in neonatal hepatitis (P < 0.01).
- Expression levels of both TGF-β1 and CTGF correlated positively and significantly with the severity of liver fibrosis (P < 0.01).
Conclusions:
- Liver fibrosis is a significant finding in both congenital biliary atresia and neonatal hepatitis.
- Increased TGF-β1 and CTGF expression is strongly associated with liver fibrosis progression in infants.
- Early expression of TGF-β1 and CTGF in biliary epithelial cells suggests a potential role in the pathogenesis of congenital biliary atresia.
Abstract:
We investigated the expression of transforming growth factor-β1 (TGF-β1) and connective tissue growth factor (CTGF) in the liver tissue of infants with congenital biliary atresia and neonatal hepatitis, as well as the relationship between the expression of the two factors and liver fibrosis. Thirty-six infants who met the cholestasis criteria were classified into congenital biliary atresia and neonatal hepatitis groups. All specimens were stained with hematoxylin and eosin and Masson's trichrome, and the degree of liver fibrosis was assessed. The scope and level of CTGF and TGF-β1 expression in the different specimens was evaluated by immunohistochemistry and observation. Liver fibrosis in the congenital biliary atresia group was more advanced than that in the neonatal hepatitis group, and the difference was significant (P < 0.01). In the neonatal hepatitis patients, CTGF and TGF-β1 were mainly expressed in the hepatocytes, while they were expressed in both hepatocytes and biliary epithelial cells in the congenital biliary atresia patients, and in these patients the expression was significantly stronger than in the neonatal hepatitis patients (P < 0.01). With the aggravation of hepatic fibrosis, CTGF and TGF-β1 expression levels in liver tissue gradually increased, and their expression levels were significantly correlated (P < 0.01). Liver fibrosis is present in both congenital biliary atresia and neonatal hepatitis patients. The gradual increase of CTGF and TGF-β1 expression levels in liver tissue is associated with liver fibrosis. Early expression of CTGF and TGF-β1 in biliary epithelial cells may be involved in the pathogenesis of congenital biliary atresia.
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