• Eur. J. Pediatr. · Feb 1996

    Case Reports

    Paucity of intrahepatic bile ducts, solitary kidney and atrophic pancreas with diabetes mellitus: atypical Alagille syndrome?

    • K Devriendt, L Dooms, W Proesmans, F de Zegher, V Desmet, and E Eggermont.
    • Department of Paediatrics, University Hospital Leuven, Belgium.
    • Eur. J. Pediatr. 1996 Feb 1; 155 (2): 87-90.

    UnlabelledA child with the tentative diagnosis of Alagille syndrome is reported. Additional renal abnormalities are unilateral kidney agenesis and a kidney with subcortical cysts with decreased function. At the age of 5 years, insulin-dependent diabetes mellitus developed, with the pancreas being atrophic and negative pancreatic islet cell antibodies.ConclusionThis observation extends the picture of Alagille syndrome and suggests an overlap with renal-hepatic-pancreatic dysplasia (Ivemark syndrome).

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