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Paucity of intrahepatic bile ducts, solitary kidney and atrophic pancreas with diabetes mellitus: atypical Alagille syndrome?
- Source :
-
European journal of pediatrics [Eur J Pediatr] 1996 Feb; Vol. 155 (2), pp. 87-90. - Publication Year :
- 1996
-
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.<br />Conclusion: This observation extends the picture of Alagille syndrome and suggests an overlap with renal-hepatic-pancreatic dysplasia (Ivemark syndrome).
Details
- Language :
- English
- ISSN :
- 0340-6199
- Volume :
- 155
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- European journal of pediatrics
- Publication Type :
- Academic Journal
- Accession number :
- 8775219
- Full Text :
- https://doi.org/10.1007/BF02075756