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isolated congenital hepatic fibrosis - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

isolated congenital hepatic fibrosis

MONDO:0018840

Also known as:isolated CHFCongenital Hepatic Fibrosisnonsyndromic congenital hepatic fibrosis

Find Clinical TrialsTrials

Overview

A congenital disorder usually inherited in an autosomal recessive pattern. It affects the hepatobiliary system and the kidneys. It is characterized by liver fibrosis, portal hypertension, and renal cysts.

Clinical Trials3 registered · 2 recruiting

Diagnosis

Biomarker and diagnostic research for isolated congenital hepatic fibrosis has been reported in the published literature.

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

3 clinical trials registered, 2 recruiting. Interventions under study include other interventions. Research is primarily sponsored by academic and government institutions.

35 publications have been identified in PubMed for isolated congenital hepatic fibrosis. Research spans Case Report / Case Series (43%), Review / Meta-Analysis (17%), and Clinical Trial Publication (14%).

Research Type	Count	% of Total
Patient case studies	15	43%

Data assembled from 4 of 12 sources · Last updated Sep 19, 2026, 5:16 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:485426

GARD

Genetic and Rare Diseases Info Center

GARD:21995

Questions for Your Care Team

→Are any of the 3 active clinical trials a good fit for my situation?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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isolated congenital hepatic fibrosis

Overview

Diagnosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

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