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Published on: February 10, 2015
Early type I collagen deposition is associated with prognosis in biliary atresia
Luis Ricardo Longo-Santos1, Walcy Rosolia Teodoro2, Evandro Sobroza de Mello3
1Department of Pediatric Surgery, University of São Paulo Medical School (FMUSP), São Paulo, Brazil.
Insights
Type I collagen in initial liver biopsies predicts disease progression in biliary atresia (BA) patients, aiding prognosis for pediatric liver transplantation (LTx). This helps identify children needing timely intervention for this serious liver condition.
Area of Science:
- Pediatric Hepatology
- Gastroenterology
- Translational Research
Background:
- Biliary atresia (BA) is a pediatric cholestatic liver disease leading to fibrosis and liver transplantation (LTx).
- Predictive histopathological markers are needed to forecast BA progression to end-stage liver disease.
Purpose of the Study:
- To identify histopathological or immunohistochemical markers in BA liver biopsies.
- To correlate these markers with patient prognosis and disease progression.
Main Methods:
- Histological and morphometric analysis of liver fibrosis in 36 BA patients.
- Indirect immunofluorescence (IF) for collagens I, III, IV, and V in initial and follow-up biopsies.
- Correlation of collagen deposition with time to Kasai hepatoportoenterostomy (KPE) and LTx.
Main Results:
- Perisinusoidal type III and V collagen deposition was prominent initially.
- Type I and IV collagen deposition indicated disease progression (p<0.01).
- Higher initial type I collagen levels correlated with faster progression to LTx (p=0.04).
Conclusions:
- Morphometric assessment of perisinusoidal type I collagen via IF in initial biopsies predicts progression time to LTx in post-surgical BA.
- Type I collagen serves as a potential prognostic biomarker for biliary atresia.
Background:
Biliary atresia (BA) is a cholestatic liver disease of children that progresses to hepatic fibrosis. BA is the main indication of pediatric liver transplantation (LTx). Histopathological markers in liver biopsies could be useful for predicting progression to end-stage disease.
Objective:
To establish histopathological or immunohistochemical markers in liver biopsies of BA patients and correlate those markers with prognosis.
Method:
Histological analysis of biliary alterations and morphometric assessment of liver fibrosis were performed, in addition to indirect immunofluorescence assays (IF) for type I, III, IV and V collagens in initial and final liver biopsies of 36 patients with BA who underwent Kasai hepatoportoenterostomy (KPE) and LTx in the last 20years at a single center.
Results:
Histopathological markers had no correlation with evolutive time until LTx. The perisinusoidal deposition of type III and V collagens was more prominent in the initial biopsies (p<0.01), whereas deposition of type I and IV collagens indicated progression (p<0.01). Patients with large amounts of perisinusoidal type I collagen in the initial biopsies had worse progression time curves until LTx (p=0.04).
Conclusion:
Morphometric assessment of perisinusoidal deposition of type I collagen by IF in the initial biopsy can correlate with progression time to LTx in post-surgical BA.
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