The French national registry of primary immunodeficiency diseases
The French national registry of primary immunodeficiency diseases
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DOI：
10.1016/j.clim.2010.02.021
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发表时间：
2010-05-01
影响因子：
8.6
通讯作者：
Fischer, Alain
中科院分区：
文献类型：
--
作者：
Beaute, Julien;Mahlaoui, Nizar;Fischer, Alain
法国国家初级免疫缺陷参考中心(CEREDIH)成立于2005年，现已构成全国范围内的大学医疗中心儿科和成人医学科网络。该登记处共有3083名患者(主要是儿童)，总患病率为每10万名居民4.4例。主要是B细胞免疫缺陷是最常见的疾病(43%)。常见可变免疫缺陷的比例(CVIDs，14%)低于其他发达国家国家登记报告的比例。数据表明，尽管转诊到专家中心对儿童来说已经足够了，但在法国成年人还不是这样。原发免疫缺陷(PID)的分布在不同的地理区域之间差异很大，这表明患者护理的地区差异。作为世界上最大的国家IDs登记机构，CEREDIH为进一步研究和开展旨在提高医生(尤其是成人)对PIDs的认识的活动奠定了基础。(C)2010 Elsevier Inc.保留所有权利。
The French National Reference Center of Primary Immunodeficiencies (CEREDIH) was established in 2005 and now constitutes a nationwide network of pediatric and adult medicine departments in university medical centers. The registry comprises a total of 3,083 patients (mainly children), with an overall prevalence of 4.4 cases per 100,000 inhabitants. Predominantly B-cell immunodeficiencies are the most common diseases observed (43%). The proportion of common variable immunodeficiencies (CVIDs, 14%) is lower than reported by national registries in other developed countries. The data suggest that although referral to expert centers is fairly adequate for children, this is not yet the case in France for adults. The distribution of primary immunodeficiencies (PIDs) varied significantly across distinct geographical areas and this suggested regional differences in patient care. As the world's largest national registry of PIDs, CEREDIH provides a basis for both further studies and activities aimed at raising the physicians awareness of PIDs (notably in adults). (c) 2010 Elsevier Inc. All rights reserved.