蛋白C缺乏所致布加综合征：一种导致慢性肝病的罕见疾病。

Budd-Chiari Syndrome Due to Protein C Deficiency: A Rare Disorder to cause Chronic Liver Disease.

作者信息

Begum Rukshana, Mahtab Mamun Al, Mamun Ayub Al, Moben Ahmed Lutful, Hossain Sharker Mohammad Shahadat, Das Dulal Chandra, Malakar Debraj, Or Rashid Harun, Roy Partho Protim, Rahman Salimur

机构信息

Department of Hepatology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh.

出版信息

Euroasian J Hepatogastroenterol. 2016 Jul-Dec;6(2):194-197. doi: 10.5005/jp-journals-10018-1199. Epub 2016 Dec 1.

DOI:10.5005/jp-journals-10018-1199

PMID:29201759

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5578595/

Abstract

UNLABELLED

The Budd-Chiari syndrome (BCS) is a rare disorder due to chronic liver disease (CLD), which is caused by the obstruction of hepatic venous outflow that can be located at any place from the small hepatic venules up to the entrance of the inferior vena cava (IVC) into the right atrium. Among the causes of BCS, the rarer one is coagulation factor deficiencies. Here, we report a case of BCS associated with deficiency of protein C resulting in thrombus in IVC. The patient was a 50-year-old male, who had been suffering from recurrent abdominal and leg swelling for a long period of 7 years. He was evaluated thoroughly, and other causes of liver cirrhosis were excluded.

HOW TO CITE THIS ARTICLE

Begum R, Al Mahtab M, Al Mamun A, Moben AL, Hossain SMS, Das DC, Malakar D, Rashid HO, Roy PP, Rahman S. Budd-Chiari Syndrome Due to Protein C Deficiency: A Rare Disorder to cause Chronic Liver Disease. Euroasian J Hepato-Gastroenterol 2016;6(2):194-197.

摘要

未标注

布加综合征（BCS）是一种由慢性肝病（CLD）引起的罕见疾病，其病因是肝静脉流出道阻塞，阻塞部位可位于从小肝静脉到下腔静脉（IVC）进入右心房的入口处的任何位置。在BCS的病因中，较罕见的是凝血因子缺乏。在此，我们报告一例与蛋白C缺乏相关的BCS病例，该病例导致下腔静脉血栓形成。患者为一名50岁男性，长期反复出现腹部和腿部肿胀达7年之久。对其进行了全面评估，排除了肝硬化的其他病因。

如何引用本文

Begum R, Al Mahtab M, Al Mamun A, Moben AL, Hossain SMS, Das DC, Malakar D, Rashid HO, Roy PP, Rahman S. 蛋白C缺乏所致布加综合征：一种导致慢性肝病的罕见疾病。《欧亚肝脏胃肠病学杂志》2016年；6(2):194 - 197。