PAUCITY OF INTRAHEPATIC BILE-DUCTS, SOLITARY KIDNEY AND ATROPHIC PANCREAS WITH DIABETES-MELLITUS - ATYPICAL ALAGILLE SYNDROME

Authors
DEVRIENDT K DOOMS L PROESMANS W DEZEGHER F DESMET V EGGERMONT E
Citation
K. Devriendt et al., PAUCITY OF INTRAHEPATIC BILE-DUCTS, SOLITARY KIDNEY AND ATROPHIC PANCREAS WITH DIABETES-MELLITUS - ATYPICAL ALAGILLE SYNDROME, European journal of pediatrics, 155(2), 1996, pp. 87-90
Citations number
15
Categorie Soggetti
Pediatrics
Journal title
European journal of pediatrics → ACNP
ISSN journal
03406199
Volume
155
Issue
2
Year of publication
1996
Pages
87 - 90
Database
ISI
SICI code
0340-6199(1996)155:2<87:POIBSK>2.0.ZU;2-C
Abstract
A child with the tentative diagnosis of Alagille syndrome is reported. Additional renal abnormalities are unilateral kidney agenesis and a k idney with subcortical cysts with decreased function. At the age of 5 years, insulin-dependent diabetes mellitus developed, with the pancrea s being atrophic and negative pancreatic islet cell antibodies. Conclu sion This observation extends the picture of Alagille syndrome suggest s an overlap with renal-hepatic-pancreatic dysplasia (Ivemark syndrome ).