Primary immunodeficiency diseases: An update
Primary immunodeficiency diseases: An update
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DOI：
10.1016/j.jaci.2004.06.044
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发表时间：
2004-09-01
影响因子：
14.2
通讯作者：
Geha, R
中科院分区：
文献类型：
--
作者：
Notarangelo, L;Casanova, JL;Geha, R
虽然相对罕见，原发性免疫缺陷疾病（pid）提供了一个很好的窗口进入免疫系统的功能。在20世纪60年代后期，对这些疾病及其相关感染和遗传学的观察，将免疫系统分为不道德免疫和细胞介导免疫。这些疾病在诊断和治疗方面也构成挑战。从1970年开始，由世界卫生组织召集的一个委员会为当时已知的原发性免疫缺陷疾病制定了统一的命名法。从那时起，以及后来在国际免疫学会联合会的支持下，国际专家委员会每2至3年召开一次会议，更新pid的分类。在过去的15年中，超过100个pip的分子基础已经被阐明。这一最新情况是该委员会2003年6月在葡萄牙辛特拉举行的最近一次会议的结果，该会议经过了2(1)/ 2天的科学讨论。
Although relatively rare, primary immune deficiency diseases (PIDs) provide an excellent window into the functioning of the immune system. In the late 1960s, observations on these diseases, with their associated infections and genetics, bisected the immune system into Immoral immunity and cell-mediated immunity. These diseases also represent a challenge in their diagnosis and treatment. Beginning in 1970, a unified nomenclature for the then-known primary immunodeficiency diseases was created by a committee convened by the World Health Organization. Since then, and later under the aegis of the International Union of Immunological Societies, an international committee of experts has met every 2 to 3 years to update the classification of PIDs. During the past 15 years, the molecular basis of more than 100 PIDs has been elucidated. This update results from the latest meeting of this committee in Sintra, Portugal, June 2003, which followed 2(1)/(2) days of scientific discussions.