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一名年轻成年人的良性复发性肝内胆汁淤积症

Benign Recurrent Intrahepatic Cholestasis in a Young Adult.

作者信息

Kumar Prabhat, Charaniya Riyaz, Ahuja Arvind, Mittal Sakshi, Sahoo Ratnakar

机构信息

Senior Resident, Department of Medicine, PGIMER & Dr RML Hospital , New Delhi, India .

Postgraduate Resident, Department of Medicine, PGIMER & Dr RML Hospital , New Delhi, India .

出版信息

J Clin Diagn Res. 2016 Jun;10(6):OD01-2. doi: 10.7860/JCDR/2016/18917.7924. Epub 2016 Jun 1.

Abstract

Benign Recurrent Intrahepatic Cholestasis (BRIC) is a rare genetic disorder characterized by recurrent episodes of cholestatic jaundice. The initial episode of jaundice generally occurs before second decade of life and can persist for several weeks to months before resolving spontaneously. It is a benign disease and even after repeated episodes of jaundice, fibrosis of liver cell does not occur. We had a young adult patient who was having recurrent episodes of cholestatic jaundice with intervening symptom free period for last 20 years. He had first episode of jaundice at the age of eight and since then had several similar episodes. Diagnosis was made by classical clinical presentation and histopathological findings. We intend to report this case due to rarity of this disease in India.

摘要

良性复发性肝内胆汁淤积症(BRIC)是一种罕见的遗传性疾病,其特征为胆汁淤积性黄疸反复发作。黄疸的首发通常发生在20岁之前,可持续数周或数月后自行消退。这是一种良性疾病,即使黄疸反复发作,肝细胞也不会发生纤维化。我们有一位年轻成年患者,在过去20年中反复出现胆汁淤积性黄疸,期间有症状缓解期。他8岁时首次出现黄疸,此后又有几次类似发作。通过典型的临床表现和组织病理学检查结果做出了诊断。由于这种疾病在印度罕见,我们打算报告此病例。

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本文引用的文献

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Benign recurrent intrahepatic cholestasis.
Clin Liver Dis. 1999 Aug;3(3):509-28, viii. doi: 10.1016/s1089-3261(05)70083-0.

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