The 2017 IUIS Phenotypic Classification for Primary Immunodeficiencies.

The 2017 IUIS Phenotypic Classification for Primary Immunodeficiencies.
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DOI:
10.1007/s10875-017-0465-8
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发表时间:
2018-01
影响因子:
9.1
通讯作者:
Sullivan KE
Sullivan KE
中科院分区:
医学2区
文献类型:
--
作者:
Bousfiha A;Jeddane L;Picard C;Ailal F;Bobby Gaspar H;Al-Herz W;Chatila T;Crow YJ;Cunningham-Rundles C;Etzioni A;Franco JL;Holland SM;Klein C;Morio T;Ochs HD;Oksenhendler E;Puck J;Tang MLK;Tangye SG;Torgerson TR;Casanova JL;Sullivan KE

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自20世纪90年代以来,国际免疫学会联合会(IUIS)PID专家委员会(EC),现在称为先天性免疫缺陷委员会,每隔一年发布一次先天性免疫缺陷的分类。这份完整的目录可作为全世界免疫学家和研究人员的参考。然而,它不适合临床医生在床边。对于这些人,IUIS PID EC自2013年以来一直在发布表型分类,事实证明该分类更加用户友好。现在有320种单基因先天性免疫缺陷,其表型多种多样,如感染、恶性肿瘤、过敏、自身免疫和自身炎症。我们在此提出了修订后的2017年表型分类,基于2017年IUIS先天免疫缺陷委员会分类。
Since the 1990s, the International Union of Immunological Societies (IUIS) PID expert committee (EC), now called Inborn Errors of Immunity Committee, has published every other year a classification of the inborn errors of immunity. This complete catalog serves as a reference for immunologists and researchers worldwide. However, it was unadapted for clinicians at the bedside. For those, the IUIS PID EC is now publishing a phenotypical classification since 2013, which proved to be more user-friendly. There are now 320 single-gene inborn errors of immunity underlying phenotypes as diverse as infection, malignancy, allergy, auto-immunity, and auto-inflammation. We herein propose the revised 2017 phenotypic classification, based on the accompanying 2017 IUIS Inborn Errors of Immunity Committee classification.
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