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Clinical features and etiology of B udd– C hiari syndrome in C hinese patients: A single‐center study
Author(s) -
Cheng Delei,
Xu Hao,
Lu Zhaojun,
Hua Rong,
Qiu Huan,
Du Hongtao,
Xu Xinjian,
Zhang Jing
Publication year - 2013
Publication title -
journal of gastroenterology and hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.214
H-Index - 130
eISSN - 1440-1746
pISSN - 0815-9319
DOI - 10.1111/jgh.12140
Subject(s) - medicine , etiology , confidence interval , asymptomatic , gastroenterology , surgery
Abstract Background and Aim The clinical features and etiology of B udd– C hiari syndrome ( BCS ) vary from region to region, and there is lack of large sample studies about BCS in C hina. The aim of the present study was to study the clinical features and etiology of patients with incident BCS in C hina prospectively. Methods A consecutive case series of patients with incident BCS who were diagnosed in the A ffiliated H ospital of X uzhou M edical C ollege ( J iangsu, C hina) were enrolled from S eptember 2010 to D ecember 2011. All of the patients had continuous follow‐ups to record the symptoms, body features, laboratory and radiology findings, and treatment methods through M ay 2012. Results A total of 145 incident cases of BCS were identified. BCS was caused by hepatic venous obstruction in 31% of the patients, inferior vena cava obstruction in 6% of the patients, and 63% suffered from a combination of the two conditions. At least one etiological factor was present in 82% of the patients, with the most common being membranous obstruction (61%). Only 5% of the patients had myeloproliferative neoplasms with a JAK2 V617F mutation, and none of the patients had a factor V Leiden mutation. Eighteen months after a percutaneous transluminal angioplasty was performed, the survival rate and the asymptomatic survival rate were 99% (95% confidence interval, 95–100%) and 93% (95% confidence interval, 89–98%), respectively. Conclusion The most prevalent etiological factor for BCS in C hina is membranous obstruction. Moreover, most Chinese patients with chronic BCS are treated with percutaneous transluminal angioplasty and have an excellent clinical outcome.