The precipitating cause is believed to involve accumulation of inspissated secretions within cholangioles. The inspissated material may appear as amorphous material, plugs or concretions. The material is PAS positive and diastase resistant. Accumulation of this material can distend bile ducts which may rupture, resulting in a cholangiolitis followed by fibrosis.
Prevalence: Hepatic fibrosis tends to be seen more often in patients who survive into adulthood, but it can be found in infants.
Clinical and laboratory features:
(1) elevation of serum liver function tests, including alkaline phosphatase
(2) hepatomegaly, which may be irregular
(3) development of portal hypertension with esophageal varices and splenomegaly
Pathologic features:
(1) yellow-to-green-colored nodularity of the liver in affected areas, which may range from focal to diffuse with multiple, fused nodules. It is believed that diffuse areas arise when focal areas coalesce over time.
(2) fibrous septae that delineate hepatocellular nodules (the diagnosis of cirrhosis requires that nodules be regenerative)
(3) variable portal inflammation with fibrosis and bile duct proliferation
The diagnosis requires exclusion of other causes of hepatic fibrosis.
