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胆管稀少:新生儿胆汁淤积的一种罕见病因。

Paucity of biliary ducts: A rare etiology of neonatal cholestasis.

作者信息

Figiel Steven Cornell, Franco Arie, Pucar Darko, Lewis Kristopher Neal, Lee Jeffrey Roberts

机构信息

Department of Radiology, Georgia Health Sciences University, Augusta, GA, USA.

出版信息

J Radiol Case Rep. 2012 Feb;6(2):29-38. doi: 10.3941/jrcr.v6i2.892. Epub 2012 Feb 1.

Abstract

We report a case of a newborn with cholestasis that was diagnosed as nonsyndromic Alagille syndrome. The main feature of the disease is a paucity of biliary ducts. There are two known types of the disease: the syndromic type which is associated with other congenital defects and the nonsyndromic type without other anomalies detected at birth. We describe the case and discuss its clinical and radiologic findings. We also discuss the various etiologies of cholestasis that are included in the differential diagnosis.

摘要

我们报告一例新生儿胆汁淤积症,该患儿被诊断为非综合征性阿拉吉耶综合征。该病的主要特征是胆管稀少。已知该病有两种类型:综合征型与其他先天性缺陷相关,非综合征型在出生时未检测到其他异常。我们描述了该病例并讨论了其临床和影像学表现。我们还讨论了鉴别诊断中所包含的胆汁淤积症的各种病因。

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Paucity of biliary ducts: A rare etiology of neonatal cholestasis.胆管稀少:新生儿胆汁淤积的一种罕见病因。
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Prenat Diagn. 2011 Sep;31(9):881-6. doi: 10.1002/pd.2795. Epub 2011 Jun 27.
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Pediatr Pulmonol. 2007 Dec;42(12):1207-14. doi: 10.1002/ppul.20725.
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