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
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
).