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.

Intrahepatic cholestasis

Impairment of bile flow due to obstruction in the small bile ducts within the liver.


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PMID (PMCID)
7725553
 MALE Infant, Newborn
[Arteriohepatic dysplasia (Alagille syndrome)].
Esberg BH.
Ugeskr Laeger. 1995;157(11):1531-2.
Alagille syndrome (AS) is the second most frequent cause of intrahepatic cholestasis in children under one year of age.
1482647
 MALE Child
IgA nephritis in a patient with Alagille syndrome and a transplanted liver.
Gilboa N, Hopp L, Agostini RM.
Pediatr Nephrol. 1992;6(6):559-61.
Alagille syndrome (arteriohepatic dysplasia) is a major cause of intrahepatic cholestasis in infancy.
1482647
 MALE Child
IgA nephritis in a patient with Alagille syndrome and a transplanted liver.
Gilboa N, Hopp L, Agostini RM.
Pediatr Nephrol. 1992;6(6):559-61.
Alagille syndrome (arteriohepatic dysplasia) is a major cause of intrahepatic cholestasis in infancy.
1779119
 MALE Adult
[Ocular anomalies in Alagille's syndrome].
Ricci B, Lepore D, Iossa M, Santo A, Chiaretti A.
J Fr Ophtalmol. 1991;14(8-9):481-5.
The authors examine a family, in which two brothers were affected by a severe expression of arteriohepatic dysplasia (ADH, Alagille syndrome), an autosomal dominant disorder associated with intrahepatic cholestasis, characteristic facial appearance, congenital embryotoxon.
1779119
 MALE Adult
[Ocular anomalies in Alagille's syndrome].
Ricci B, Lepore D, Iossa M, Santo A, Chiaretti A.
J Fr Ophtalmol. 1991;14(8-9):481-5.
The authors examine a family, in which two brothers were affected by a severe expression of arteriohepatic dysplasia (ADH, Alagille syndrome), an autosomal dominant disorder associated with intrahepatic cholestasis, characteristic facial appearance, congenital embryotoxon.
3687146
 FEMALE
[Alagille syndrome, a rare differential diagnosis of intrahepatic cholestasis].
Kurtz W, Strohm WD, Lambrecht E, Leuschner U.
Z Gastroenterol. 1987;25(10):668-72.
[Alagille syndrome, a rare differential diagnosis of intrahepatic cholestasis].
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.
484950
 MIXED_SAMPLE Infant, Newborn
Arteriohepatic dysplasia: a benign syndrome of intrahepatic cholestasis with multiple organ involvement.
Riely CA, Cotlier E, Jensen PS, Klatskin G.
Ann Intern Med. 1979;91(4):520-7.
Arteriohepatic dysplasia: a benign syndrome of intrahepatic cholestasis with multiple organ involvement.