Japanese periodical nationwide epidemiologic survey of aberrant portal hemodynamics
Japanese periodical nationwide epidemiologic survey of aberrant portal hemodynamics
复制标题
DOI：
10.1111/hepr.13343
复制
发表时间：
2019-08-01
影响因子：
4.2
通讯作者：
Nakamura, Yosikazu
中科院分区：
文献类型：
--
作者：
Ohfuji, Satoko;Furuichi, Yoshihiro;Nakamura, Yosikazu
目的特发性门静脉高压症（IPH）、肝外型门静脉阻塞（EHO）和Budd-Chiari综合征（BCS）均以不明原因的门静脉血流动力学异常为特征。本研究的目的是通过定期的全国性调查，探讨这些疾病的描述性流行病学的趋势。方法1999年、2005年和2015年使用相同的方案进行全国流行病学调查。调查对象是从日本全国的胃肠内科、外科、儿科、小儿外科等所有科室，按照床位数分层随机抽样选取的。我们要求每个部门完成一份关于上一年IPH、EHO和BCS患者年度人数的邮寄调查问卷。结果BCS患者的估计数量从1999年调查的280例（95%置信区间，200-360）增加到2015年调查的410例（300-530），而IPH和EHO患者的数量在15年间基本保持不变（IPH约为1000例; EHO约为770例）。在过去的15年中，IPH的平均发病年龄约为45岁，EHO为30岁，BCS为40岁。在这三项调查中，描述疾病从诊断时起加重的患者分别占IPH患者的10%、EHO患者的15%和BCS患者的20%。结论在日本，BCS的患病率正在增加，而IPH和EHO的患病率似乎稳定。在过去的15年里，包括腹泻在内的临床特征基本上保持不变。
Aim Idiopathic portal hypertension (IPH), extrahepatic portal obstruction (EHO), and Budd-Chiari syndrome (BCS) are characterized by aberrant portal hemodynamics of unknown etiology. The aim of this study was to explore trends in the descriptive epidemiology of these diseases through periodical nationwide surveys. Methods Nationwide epidemiologic surveys were undertaken in 1999, 2005, and 2015 using the same protocol. The survey targets were selected from all departments of gastrointestinal medicine, surgery, pediatrics, and pediatric surgery in Japan by stratified random sampling according to the number of beds. We asked each department to complete a mail-back questionnaire on the annual numbers of patients with IPH, EHO, and BCS during the preceding year. Results The estimated number of BCS patients increased from 280 (95% confidence interval, 200-360) in 1999 survey to 410 (300-530) in 2015 survey, whereas the number of IPH and EHO patients has remained largely unchanged during the 15 years (IPH was approximately 1000; EHO was approximately 770 in 2015 survey). The mean age at symptom onset was approximately 45 years for IPH, 30 years for EHO, and 40 years for BCS over the past 15 years. Those who described disease aggravation from the time of diagnosis accounted for approximately 10% of IPH, 15% of EHO, and 20% of BCS patients in each of the three surveys. Conclusions In Japan, the prevalence of BCS is increasing, while those of IPH and EHO appear to be stable. Clinical characteristics, including prognoses, have remained largely unchanged in the past 15 years.