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.

Hepatic failure



Total: 1

                      


(per page)
PMID (PMCID)
11283905
MALE
Successful heterotopic segmental liver transplantation from a live donor to a patient with Alagille syndrome.
Haberal M, Arda IS, Karakayali H, Emiroglu R, Bilgin N, Arslan G, Coskun M, Boyacioglu S.
J Pediatr Surg. 2001;36(4):667-71.
It is unusual to see the clinical picture of hepatic failure resulting in cirrhosis and requiring transplantation, but liver transplantation is indicated in Alagille syndrome patients who have chronic cholestasis.