Isolated congenital hepatic fibrosis

Isolated congenital hepatic fibrosis

Definition

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.

Also known as Congenital Hepatic Fibrosis, nonsyndromic congenital hepatic fibrosis — per MONDO

Also identified as

Affected Organs

Organ Involvement Source
Alimentary part of gastrointestinal system Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Alimentary part of gastrointestinal system Disease Has Primary Anatomic Site NCIT · CC BY 4.0
Liver Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Liver Disease Has Primary Anatomic Site NCIT · CC BY 4.0