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.

Renal artery stenosis

The presence of stenosis of the renal artery.


Total: 2

                       


(per page)
PMID (PMCID)
23641167
(3620816)
 OTHER
Hypertension and Biliary Ductopenia in a Patient with Duplication of Exon 6 of the JAG1 Gene.
Uberos J, Moreno L, Munoz-Hoyos A.
Clin Med Insights Pediatr. 2012;6:61-6.
The duplication of exon 6 of JAG1 has not previously been described as an alteration related to the Alagille syndrome with peripheral renal artery stenosis.
15772839
 FEMALE Child
Successful stenting for renal artery stenosis in a patient with Alagille syndrome.
Hirai H, Santo Y, Kogaki S, Kurotobi S, Etani Y, Mushiake S, Nakatsuchi Y, Nakajima S, Ozono K.
Pediatr Nephrol. 2005;20(6):831-3.
A 12-year-old girl with Alagille syndrome manifested severe hypertension caused by renal artery stenosis in a solitary functioning kidney.