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Clinical Characteristics and Outcomes of Adults with Budd-Chiari Syndrome: A Single-Center Experience in Colombia

医学 布加综合征 单中心 中心(范畴论) 儿科 内科学 下腔静脉 化学 结晶学
作者
Ximena Morales,Carolina Salinas,Geovanny Hernández-Cely,Cristina Torres,Martín Garzón,Rocío López,Juan Manuel Sánchez Pérez,Felipe Durán-Torres,Adriana Varón,Oscar Beltrán
出处
期刊:Digestive Diseases [S. Karger AG]
卷期号:: 1-7
标识
DOI:10.1159/000547006
摘要

Budd-Chiari syndrome is defined as the obstruction of hepatic venous flow. Limited evidence exists in Colombia regarding the characterization of these patients. This study aimed to describe the clinical characteristics, management, and survival of patients diagnosed with Budd-Chiari syndrome in a Colombian hepatology referral center from 2010 to 2022. This is a descriptive retrospective longitudinal study of a cohort of patients with Budd-Chiari syndrome in adult patients diagnosed with Budd-Chiari syndrome in a Colombian hepatology center from January 2010 to January 2022. 31 patients diagnosed with Budd-Chiari syndrome were included. Of the patients, 61.3% (n = 19) were female. The median age was 28 years [interquartile range (IQR) 23-38]. Acquired thrombophilias were the main risk factor (58.1%, n = 18), mainly secondary to antiphospholipid syndrome (55.5%, n = 10). The primary clinical manifestation presented was ascites 87.1% (n = 27). At diagnosis, 74.2% (n = 23) were in a chronic course, and 5 patients had cirrhosis documented by biopsy. The Rotterdam score was class II (intermediate prognosis) in 48.3% (n = 14). Of the patients, 86% (n = 25) had anticoagulation therapy. Five patients (16.1%) underwent transjugular intrahepatic portosystemic shunt. Eleven patients died (35.5%), with a median survival time from diagnosis to death of 337.1 days (IQR 46.5-647.5). Budd-Chiari syndrome is a poorly characterized disease in the Colombian population. This study shows that acquired thrombophilias are the leading risk factor associated with this medical condition in this population, with clinical features and mortality rates similar to those reported in the literature.

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