Paucity of intrahepatic bile ducts, solitary kidney and atrophic pancreas with diabetes mellitus: Atypical Alagille syndrome?
العنوان: | Paucity of intrahepatic bile ducts, solitary kidney and atrophic pancreas with diabetes mellitus: Atypical Alagille syndrome? |
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المؤلفون: | K. Devriendt, L. Dooms, W. Proesmans, F. de Zegher, E. Eggermont, V. Desmet |
المصدر: | European Journal of Pediatrics. 155:87-90 |
بيانات النشر: | Springer Science and Business Media LLC, 1996. |
سنة النشر: | 1996 |
مصطلحات موضوعية: | Male, Autoimmune disease, Kidney, Pathology, medicine.medical_specialty, medicine.diagnostic_test, business.industry, Biopsy, Intrahepatic bile ducts, medicine.disease, Alagille Syndrome, Diabetes Mellitus, Type 1, medicine.anatomical_structure, Liver, Dysplasia, Child, Preschool, Diabetes mellitus, Pediatrics, Perinatology and Child Health, Alagille syndrome, medicine, Humans, business, Pancreas |
الوصف: | 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). |
تدمد: | 1432-1076 0340-6199 |
URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b9ce67ca1b6f1b3070f5c5ee39ad760f https://doi.org/10.1007/bf02075756 |
حقوق: | CLOSED |
رقم الأكسشن: | edsair.doi.dedup.....b9ce67ca1b6f1b3070f5c5ee39ad760f |
قاعدة البيانات: | OpenAIRE |
تدمد: | 14321076 03406199 |
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