肝内胆管稀少、单肾以及伴有糖尿病的萎缩性胰腺：非典型阿拉吉列综合征？
Paucity of intrahepatic bile ducts, solitary kidney and atrophic pancreas with diabetes mellitus: atypical Alagille syndrome?
作者信息
Devriendt K, Dooms L, Proesmans W, de Zegher F, Desmet V, Eggermont E
机构信息
Department of Paediatrics, University Hospital Leuven, Belgium.
出版信息
Eur J Pediatr. 1996 Feb;155(2):87-90. doi: 10.1007/BF02075756.
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).
未标记
报告了一名初步诊断为阿拉吉列综合征的儿童。额外的肾脏异常包括单侧肾缺如和一个伴有皮质下囊肿且功能减退的肾脏。5岁时，患儿患上胰岛素依赖型糖尿病，胰腺萎缩且胰岛细胞抗体呈阴性。
结论
该观察结果扩展了阿拉吉列综合征的表现，并提示其与肾-肝-胰发育异常（伊韦马克综合征）存在重叠。
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