Kugelmas M
Division of Digestive Diseases and Nutrition, University of Kentucky, Lexington 40536-0084, USA.
Hepatogastroenterology. 1998 Sep-Oct;45(23):1381-6.
The Budd-Chiari syndrome encompasses a group of conditions that cause partial or complete obstruction of the hepatic venous outflow tract. This leads to hepatocyte necrosis which can manifest as a devastating fulminant illness, or a more indolent condition that eventually presents with features of portal hypertension. Doppler ultrasonography of the hepatic veins and inferior vena cava has become the initial diagnostic test of choice, but most patients require venography and liver biopsy prior to definitive therapy. Multiple therapeutic modalities have been used for the Budd-Chiari syndrome, including medical, radiological, and surgical approaches. The role, indications, and outcome of liver transplantation for individuals with the Budd-Chiari syndrome are discussed.
布加综合征包括一组导致肝静脉流出道部分或完全梗阻的病症。这会导致肝细胞坏死，可表现为严重的暴发性疾病，或表现为门静脉高压特征的更为隐匿的病症。肝静脉和下腔静脉的多普勒超声检查已成为首选的初步诊断测试，但大多数患者在进行确定性治疗之前需要进行静脉造影和肝活检。布加综合征已采用多种治疗方式，包括药物、放射和手术方法。本文讨论了布加综合征患者肝移植的作用、适应症和结果。
