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The prevalence of neuromyelitis optica in South East Wales

机译:威尔士东南部视神经脊髓炎的患病率

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Background and purpose: Neuromyeltis optica (NMO) is a neuroinflammatory disorder considered rare in Caucasian populations. However, accurate population-based epidemiological data for NMO and NMO spectrum disorder (NMO-SD) from Western populations employing validated diagnostic criteria remain limited. We sought therefore to estimate the prevalence and clinical features of NMO in a north European Caucasian population in South East Wales. Methods: Patients were identified by a comprehensive, multistage ascertainment strategy employing a regional neuroinflammatory disease register, hospital diagnostic databases personal physician referrals and regional requests for anti-aquaporin-4 antibodies (anti-AQP4). Results: Fourteen Caucasian patients (11 patients with NMO and three with NMO-SD) were identified in a population of 712572 (19.6/million; 95% CIs: 12.2-29.7). There was an excess of females (female:male 12:2), 11/14 were anti-AQP4 positive and 5/14 had disease onset under the age of 20years. Conclusion: This study suggests that NMO and related spectrum disorders are at least as frequent in Northern European populations as in non-Caucasian populations and that the demographic profile of prevalent patients differs from clinic-based cohorts.
机译:背景与目的:视神经脊髓炎(NMO)是一种在白种人中罕见的神经炎性疾病。但是,采用有效的诊断标准从西方人群获得的有关NMO和NMO谱系疾病(NMO-SD)的准确的基于人群的流行病学数据仍然有限。因此,我们试图评估NMO在东南威尔士的北欧高加索人群中的患病率和临床特征。方法:通过全面,多阶段的确定策略,使用区域神经炎性疾病登记簿,医院诊断数据库,个人医生推荐以及抗水通道蛋白4抗体(anti-AQP4)区域要求,对患者进行鉴定。结果:在712572的人群中确定了14例白人患者(11例NMO患者和3例NMO-SD患者)(百万分之19.6; 95%CI:12.2-29.7)。女性过多(女性:男性12:2),11/14的抗AQP4阳性和5/14的疾病发病年龄在20岁以下。结论:这项研究表明,北欧人群中的NMO和相关频谱异常至少与非高加索人群中的频率相同,而且普遍患者的人口统计学特征与基于临床的人群不同。

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