Paucity of intrahepatic bile ducts, solitary kidney and atrophic pancreas with diabetes mellitus: Atypical Alagille syndrome?
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- Devriendt, K., Dooms, L., Proesmans, W. et al. Eur J Pediatr (1996) 155: 87. doi:10.1007/BF02075756
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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.
This observation extends the picture of Alagille syndrome and suggests an overlap with renal-hepatic-pancreatic dysplasia (Iyemark syndrome).
Key wordsAlagille syndromeDiabetes mellitusIvemark syndromePancreas
glomerular filtration rate
insulin dependent diabetes mellitus
renal hepatic pancreatic dysplasia