Budd-Chiari综合征(BCS)是种因肝静脉流出道严重阻塞而引起的疾病。1990年日本对BCS进行了全国范围的流行病学、病因学及临床特征的调查。 病人及方法:流行病学初级调查中,向日本各大医院(200床位以上)的内、外、儿科分发了附有诊断标准的调查问卷,调查1989年就诊的BCS患者数。共收集到160例。二级调查中,进一步问询此160例BCS患者有关流行病学 Budd-Chiari syndrome (BCS) is a disease caused by a severe obstruction of the hepatic vein outflow tract. In 1990, Japan conducted a nationwide survey of epidemiology, etiology and clinical features of BCS. PATIENTS AND METHODS: In the primary epidemiological survey, questionnaires with diagnostic criteria were disseminated to pediatrics in major Japanese hospitals (over 200 beds) to investigate the number of BCS patients treated in 1989. A total of 160 cases were collected. In the second survey, further inquiries were made on the epidemiology of the 160 BCS patients