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  • Title: Paucity of intrahepatic bile ducts, solitary kidney and atrophic pancreas with diabetes mellitus: atypical Alagille syndrome?
    Author: Devriendt K, Dooms L, Proesmans W, de Zegher F, Desmet V, Eggermont E.
    Journal: Eur J Pediatr; 1996 Feb; 155(2):87-90. PubMed ID: 8775219.
    Abstract:
    UNLABELLED: A 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. CONCLUSION: This observation extends the picture of Alagille syndrome and suggests an overlap with renal-hepatic-pancreatic dysplasia (Ivemark syndrome).
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