非典型溶血性尿毒综合征(aHUS)是一种罕见但严重的疾病,通常具有遗传成分,并由替代补体途径的过度激活引起。随着研究转向改善aHUS的诊断和治疗,这将是重要的更好地了解其流行病学。我们的目的是进行系统的文献综述,以评估全球aHUS的发病率和患病率。在Embase和MEDLINE进行了全面的文献检索。此外,实践指南,国家/国际组织的数据库,和监管机构被搜查。从通过MEDLINE和Embase确定的2960种出版物中,105种出版物符合全文筛选条件,共有八篇全文符合入选标准。获得了欧洲和大洋洲的区域流行病学估计。法国有具体国家的数据,挪威,澳大利亚,和意大利。已确定的四项研究报告了aHUS的患病率,20岁或以下年龄组的患病率为每百万人口2.2至9.4,而唯一一项报告所有年龄段患病率的研究显示,患病率为每百万人口4.9。六项研究报告了aHUS的发病率,20岁以下年龄组的年发病率为每百万人口0.26至0.75,对于所有年龄段,年发病率为每百万人口0.23至1.9.据我们所知,这是为全面概述aHUS的全球发病率和患病率估计值而进行的首次系统评价.总的来说,所有研究的发病率估计都相似;然而,发现患病率数据的可变性更大.研究的局限性与研究之间aHUS定义的不一致以及缺乏评估欧洲以外aHUS发病率和患病率的流行病学研究有关。
Atypical hemolytic uremic syndrome (aHUS) is a rare but severe disorder that frequently has a genetic component and results from the overactivation of the alternative complement pathway. As research moves toward improved diagnosis and therapy of aHUS, it will be important to better understand its epidemiology. Our objective was to conduct a systematic literature
review to assess the incidence and prevalence estimates of aHUS globally. A comprehensive literature search was conducted in Embase and MEDLINE. Additionally, practice guidelines, databases of national/international organizations, and regulatory agencies were searched. From 2960 publications identified via MEDLINE and Embase, 105 publications were eligible for full-text screening, and a total of eight full-text articles met eligibility criteria for inclusion. Regional epidemiologic estimates were obtained for Europe and Oceania. Country-specific data were available for France, Norway, Australia, and Italy. Four of the identified studies reported on the prevalence of aHUS, prevalence in the age group of 20 years or younger was ranging from 2.2 to 9.4 per million population, while the only study that reported prevalence in all ages showed a prevalence of 4.9 per million population. Six studies reported on the incidence of aHUS, annual incidence in the age group of 20 years or younger was ranging from 0.26 to 0.75 per million population, and for all ages, annual incidence was ranging from 0.23 to 1.9 per million population. To our knowledge, this is the first systematic
review conducted to provide a comprehensive overview of global incidence and prevalence estimates of aHUS. In general, incidence estimates were similar across all the studies; however, prevalence data were found to be more variable. Study limitations were related to inconsistencies in the definitions of aHUS between studies and also a dearth of epidemiological studies assessing incidence and prevalence of aHUS outside of Europe.