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Classification of primary immunodeficiency disorders: One-fits-all does not help anymore

机译:原发性免疫缺陷疾病的分类:万能不再有用

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摘要

Primary immunodeficiency diseases (PIDs) include a heterogeneous group of conditions that affect development and/or function of the immune system. Most forms of PIDs are inherited as Mendelian traits. In the last years, advances in genetics and cell biology have permitted to define the molecular basis and the pathophysiology of several new forms of PIDs. These achievements are reflected in the latest classification of PIDs that has been produced by the International Union of Immunological Societies (IUIS) Expert Committee on Primary Immunodeficiency . By providing essential information on the clinical and immunological features of the PIDs, such classification is meant to facilitate the diagnostic approach to these disorders.
机译:原发性免疫缺陷疾病(PID)包括一组影响免疫系统发育和/或功能的异质性疾病。大多数形式的PID都作为孟德尔性状继承。近年来,遗传学和细胞生物学的进步已允许定义几种新形式的PID的分子基础和病理生理学。这些成就反映在国际免疫学会联合会(IUIS)原发性免疫缺陷专家委员会对PID的最新分类中。通过提供有关PID的临床和免疫学特征的基本信息,这种分类意在促进对这些疾病的诊断方法。

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