Congenital Hepatic Fibrosis
DEFINITION:
Fibrous enlargement of the portal tracts with abnormally shaped bile ducts and no cirrhosis
EPIDEMIOLOGY:
Autosomal recessive; Most commonly presents in childhood, but can be diagnosed at any age
Incidence 1 in 10,000 to 1 in 20,000 births
Often associated with Caroli’s Syndrome; See also Liver- Hepatobiliary Cystic Disease (Chapter 4.16)
Often associated with autosomal recessive polycystic kidney disease in children
ETIOLOGIES:
Recent association with a mutation in polycystic kidney and hepatic disease 1 gene (PKHD1) gene
Exact pathogenesis is unclear
PATHOPHYSIOLOGY:
Histopathologic features:Related posts:
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