Department of Gastroenterology, Nanjing Drum Tower Hospital, Affiliated to Nanjing University Medical School, Nanjing, China.
Department of Gastroenterology, Peking University People's Hospital, Beijing, China.
J Gastroenterol Hepatol. 2019 Apr;34(4):634-642. doi: 10.1111/jgh.14612. Epub 2019 Feb 21.
Hepatic sinusoidal obstruction syndrome (HSOS) is a hepatic vascular disease presenting with abdominal distension, pain in the hepatic region, ascites, jaundice, and hepatomegaly. In China, this disease is often associated with the oral intake of plants that contain pyrrolidine alkaloids. The existing guidelines are limited to HSOS associated with hematopoietic stem cell transplantation in Western countries. The Hepatobiliary Diseases Committee of the Chinese Society of Gastroenterology convened an expert consensus conference on the diagnosis and treatment of PA-HSOS to evaluate current research in China and abroad. The "Nanjing criteria" developed by the committee to diagnose PA-HSOS include a confirmed history of PA-containing plant use and (i) abdominal distention and/or pain in the hepatic region, hepatomegaly, and ascites; (ii) elevation of serum total bilirubin or abnormal laboratory liver tests; (iii) evidence on enhanced computed tomography or magnetic resonance imaging; or (iv) pathological evidence that rules out other known causes of liver injury. Supportive symptomatic treatment, anticoagulant therapy, and placement of a transjugular intrahepatic portosystemic shunt for patients who do not respond to medical treatment are effective for the treatment of PA-HSOS. The benefits of glucocorticoids and prostaglandin E1 in PA-HSOS are not clear.
肝窦阻塞综合征(HSOS)是一种肝脏血管疾病,表现为腹胀、肝区疼痛、腹水、黄疸和肝肿大。在中国,这种疾病常与摄入含有吡咯里西啶生物碱的植物有关。现有的指南仅限于西方国家与造血干细胞移植相关的 HSOS。中华医学会消化病学分会肝胆疾病学组召开了吡咯里西啶生物碱相关性 HSOS 的诊断和治疗专家共识会议,对国内外的研究进行评估。该委员会制定的“南京标准”用于诊断 PA-HSOS,包括明确的含 PA 植物使用史和 (i) 腹胀和/或肝区疼痛、肝肿大和腹水;(ii) 血清总胆红素升高或实验室肝功能异常;(iii) 增强计算机断层扫描或磁共振成像的证据;或 (iv) 排除其他已知肝损伤原因的病理证据。对于不接受药物治疗的患者,支持对症治疗、抗凝治疗和经颈静脉肝内门体分流术放置是治疗 PA-HSOS 的有效方法。皮质激素和前列腺素 E1 在 PA-HSOS 中的益处尚不清楚。