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Mapping Hepatic Stellate Cell Morphology in Mouse Models of Liver Fibrosis
Published on: February 13, 2026
[Congenital hepatic fibrosis. Report of five cases]
Paul Harris1, Daniel Fodor, Felipe Cavagnaro
1Departamento de Pediatría, Secciones de Gastroenterología Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago. pharris@med.puc.cl
Insights
Congenital hepatic fibrosis (CHF) in children can be linked to polycystic kidney disease. Early diagnosis and management are crucial for severe liver damage and portal hypertension.
Area of Science:
- Pediatric Hepatology
- Nephrology
- Genetics
Background:
- Congenital hepatic fibrosis (CHF) is an autosomal dominant disorder.
- CHF is frequently associated with polycystic kidney disease (PKD).
Observation:
- This study reviewed 5 pediatric cases of CHF (ages 2-14).
- Medical management and associated renal disease were evaluated.
Findings:
- Three patients had autosomal recessive PKD diagnosed before liver manifestations, experiencing severe liver damage and portal hypertension requiring shunts (TIPS or surgical).
- Two patients with asymptomatic hepatomegaly and normal renal function had a more benign clinical course.
Implications:
- CHF diagnosis should be considered in children with PKD.
- Persistent, hard hepatomegaly, especially left lobe predominance, warrants suspicion for CHF even without known renal disease.
Background:
Congenital hepatic fibrosis (CHF) is an autosomic dominant disease that has been associated with polycystic kidney disease.
Aim:
To describe the medical management of 5 children with CHF and to evaluate the presence and extension of the associated renal disease.
Patients And Methods:
Retrospective review of the medical charts of 5 children with CHF, aged 2 to 14 years.
Results:
Three children presented autosomic recessive polycystic kidney disease, which was diagnosed before the appearance of liver disease manifestations. They presented a more severe liver damage, with a more aggressive clinical course requiring use of transjugular intrahepatic porto-systemic shunts (TIPS) or surgical porto-systemic shunts to control portal hypertension. The other two children, in whom the diagnosed was based on asymptomatic hepatomegaly, had normal renal function and structure with a more benign clinical course.
Conclusions:
The diagnosis of CHF should be suspected not only in children with polycystic kidney disease but in those children with persistent, hard consistency, left lobe predominance hepatomegaly.
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