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.

Acholic stools

Clay colored stools lacking bile pigment.


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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.
In addition, they had the characteristic facial appearance of Alagille syndrome with cardiac, vertebral, and eye anomalies, and all had passed acholic stools from the neonatal period.