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Keywords:

  • Budd–Chiari syndrome;
  • clinical features;
  • etiology;
  • membranous obstruction;
  • percutaneous transluminal angioplasty

Abstract

Background and Aim

The clinical features and etiology of Budd–Chiari syndrome (BCS) vary from region to region, and there is lack of large sample studies about BCS in China. The aim of the present study was to study the clinical features and etiology of patients with incident BCS in China prospectively.

Methods

A consecutive case series of patients with incident BCS who were diagnosed in the Affiliated Hospital of Xuzhou Medical College (Jiangsu, China) were enrolled from September 2010 to December 2011. All of the patients had continuous follow-ups to record the symptoms, body features, laboratory and radiology findings, and treatment methods through May 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 China is membranous obstruction. Moreover, most Chinese patients with chronic BCS are treated with percutaneous transluminal angioplasty and have an excellent clinical outcome.