The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies

被引：148

作者：

Bousfiha, Aziz ^{[1
]}

Jeddane, Leila ^{[1
]}

Al-Herz, Waleed ^{[2
,3
]}

Ailal, Fatima ^{[1
]}

Casanova, Jean-Laurent ^{[4
,5
,6
,7
,8
]}

Chatila, Talal ^{[9
]}

Conley, Mary Ellen ^{[4
]}

Cunningham-Rundles, Charlotte ^{[10
]}

Etzioni, Amos ^{[11
]}

Franco, Jose Luis ^{[12
]}

Gaspar, H. Bobby ^{[13
]}

Holland, Steven M. ^{[14
]}

Klein, Christoph ^{[15
]}

Nonoyama, Shigeaki ^{[16
]}

Ochs, Hans D. ^{[17
,18
]}

Oksenhendler, Eric ^{[19
,20
]}

Picard, Capucine ^{[5
,21
]}

Puck, Jennifer M. ^{[22
,23
]}

Sullivan, Kathleen E. ^{[24
]}

Tang, Mimi L. K. ^{[25
,26
,27
]}

机构：

[1] King Hassan II Univ, Clin Immunol Unit, A Harouchi Hosp, Ibn Roshd Med Sch, Casablanca, Morocco

[2] Kuwait Univ, Fac Med, Dept Pediat, Jabriya, Kuwait

[3] Al Sabah Hosp, Allergy & Clin Immunol Unit, Dept Pediat, Kuwait, Kuwait

[4] Rockefeller Univ, St Giles Lab Human Genet Infect Dis, Rockefeller Branch, New York, NY 10021 USA

[5] Howard Hughes Med Inst, New York, NY USA

[6] Necker Hosp Sick Children, Lab Human Genet Infect Dis, Necker Branch, INSERM UMR1163, Paris, France

[7] Univ Paris 05, Imagine Inst, Paris, France

[8] Necker Hosp Sick Children, Pediat Hematol & Immunol Unit, Paris, France

[9] Childrens Hosp, Div Immunol, Boston, MA 02115 USA

[10] Mt Sinai Sch Med, Dept Med & Pediat, New York, NY USA

[11] Technion Israel Inst Technol, Meyer Childrens Hospital, Haifa, Israel

[12] Univ Antioquia, Grp Primary Immunodeficiencies, Medellin, Colombia

[13] UCL Inst Child Hlth, London, England

[14] NIAID, Lab Clin Infect Dis, Bethesda, MD 20892 USA

[15] Univ Munich, Dr von Hauner Childrens Hosp, Munich, Germany

[16] Natl Def Med Coll, Dept Pediat, Saitama, Japan

[17] Univ Washington, Dept Pediat, Seattle, WA 98195 USA

[18] Seattle Childrens Res Inst, Seattle, WA USA

[19] Hop St Louis, AP HP, Dept Clin Immunol, Paris, France

[20] Univ Paris Diderot, Sorbonne Paris Cite, Paris, France

[21] Hop Necker Enfants Malad, AP HP, Ctr Etude Deficits Immunitaires, Paris, France

[22] Univ Calif San Francisco, Dept Pediat, San Francisco, CA USA

[23] UCSF Benioff Childrens Hosp, San Francisco, CA USA

[24] Childrens Hosp Philadelphia, Dept Pediat, Div Allergy Immunol, Philadelphia, PA 19104 USA

[25] Murdoch Childrens Res Inst, Melbourne, Vic, Australia

[26] Univ Melbourne, Dept Paediat, Melbourne, Vic, Australia

[27] Royal Childrens Hosp, Dept Allergy & Immunol, Melbourne, Vic, Australia

来源：

JOURNAL OF CLINICAL IMMUNOLOGY | 2015年 / 35卷 / 08期

关键词：

Primary immunodeficiencies; classification; IUIS PID expert committee; DIAGNOSIS; DEFICIENCY; WORLDWIDE; DISEASES;

D O I：

10.1007/s10875-015-0198-5

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

071005 [微生物学]; 100108 [医学免疫学];

摘要：

There are now nearly 300 single-gene inborn errors of immunity underlying phenotypes as diverse as infection, malignancy, allergy, auto-immunity, and auto-inflammation. For each of these five categories, a growing variety of phenotypes are ascribed to Primary Immunodeficiency Diseases (PID), making PIDs a rapidly expanding field of medicine. The International Union of Immunological Societies (IUIS) PID expert committee (EC) has published every other year a classification of these disorders into tables, defined by shared pathogenesis and/or clinical consequences. In 2013, the IUIS committee also proposed a more user-friendly, phenotypic classification, based on the selection of key phenotypes at the bedside. We herein propose the revised figures, based on the accompanying 2015 IUIS PID EC classification.

引用

页码：727 / 738

页数：12

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