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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.

DOI:10.7860/JCDR/2016/18917.7924
PMID:27504332
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4963692/
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

1
Familial cholestasis: progressive familial intrahepatic cholestasis, benign recurrent intrahepatic cholestasis and intrahepatic cholestasis of pregnancy.家族性胆汁淤积症:进行性家族性肝内胆汁淤积症、良性复发性肝内胆汁淤积症和妊娠肝内胆汁淤积症。
Best Pract Res Clin Gastroenterol. 2010 Oct;24(5):541-53. doi: 10.1016/j.bpg.2010.07.010.
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Nasobiliary drainage induces long-lasting remission in benign recurrent intrahepatic cholestasis.鼻胆管引流可使良性复发性肝内胆汁淤积症获得持久缓解。
Hepatology. 2006 Jan;43(1):51-3. doi: 10.1002/hep.20998.
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Benign recurrent intrahepatic cholestasis type 2 is caused by mutations in ABCB11.2型良性复发性肝内胆汁淤积症由ABCB11基因突变引起。
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Benign recurrent intrahepatic cholestasis.
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A case of severe benign intrahepatic cholestasis treated with liver transplantation.一例采用肝移植治疗的严重良性肝内胆汁淤积症病例。
Am J Gastroenterol. 2002 Feb;97(2):475-7. doi: 10.1111/j.1572-0241.2002.05458.x.
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[Benign recurrent intrahepatic cholestasis: a seven-year follow-up report].[良性复发性肝内胆汁淤积症:七年随访报告]
Arq Gastroenterol. 2000 Oct-Dec;37(4):231-4. doi: 10.1590/s0004-28032000000400009. Epub 2001 Dec 5.
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Benign recurrent intrahepatic cholestasis.
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Ileal exclusion for Byler's disease: an alternative surgical approach with promising early results for pruritus.回肠旷置术治疗比勒氏病:一种对瘙痒症有良好早期效果的替代性手术方法。
J Pediatr Surg. 1998 Feb;33(2):220-4. doi: 10.1016/s0022-3468(98)90435-3.
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Morphological and biochemical studies of benign recurrent cholestasis.良性复发性胆汁淤积的形态学与生物化学研究
Gastroenterology. 1967 Mar;52(3):521-35.