Alagille syndrome

A rare syndrome variably characterized by chronic cholestasis due to paucity of intrahepatic bile ducts, peripheral pulmonary artery stenosis, vertebrae segmentation anomalies, characteristic facies, posterior embryotoxon/anterior segment abnormalities, pigmentary retinopathy, and dysplastic kidneys.

Bile duct proliferation

Proliferative changes of the bile ducts.


Total: 1

                       


(per page)
PMID (PMCID)
15807312
 MALE Infant
Hepatocellular carcinoma occurring in alagille syndrome.
Kim B, Park SH, Yang HR, Seo JK, Kim WS, Chi JG.
Pathol Res Pract. 2005;201(1):55-60.
Liver biopsies were diagnosed as bile duct paucity, compatible with Alagille syndrome in two cases, but the third case showed marked bile duct proliferation at the initial liver biopsy when 7 months old, which made diagnosis difficult.