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.

Nephropathy

A nonspecific term referring to disease or damage of the kidneys.


Total: 3

                       


(per page)
PMID (PMCID)
20042397
 MIXED_SAMPLE Child
An unusual cause of hypertension and renal failure: a case series of a family with Alagille syndrome.
Shrivastava R, Williams A, Mikhail A, Roberts D, Richards M, Aithal V.
Nephrol Dial Transplant. 2010;25(5):1501-6.
Alagille syndrome should be considered in the differential diagnosis of adults with renovascular disease and children with agenesis/dysgenesis of kidney and reflux nephropathy even in the absence of hepatic disease.
3252966
 FEMALE
Nephropathy in arteriohepatic dysplasia (Alagille's syndrome).
Wolfish NM, Shanon A.
Child Nephrol Urol. 1988;9(3):169-72.
Nephropathy in arteriohepatic dysplasia (Alagille's syndrome).
6862162
 OTHER Infant, Newborn
Tubulointerstitial nephropathy associated with arteriohepatic dysplasia.
Hyams JS, Berman MM, Davis BH.
Gastroenterology. 1983;85(2):430-4.
An infant with a variant of arteriohepatic dysplasia (characteristic facies, valvular and peripheral pulmonic stenosis, hypercholesterolemia, intrahepatic cholestasis) and a severe tubulointerstitial nephropathy is described.