The 2017 IUIS Phenotypic Classification for Primary Immunodeficiencies

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 re...

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Detalles Bibliográficos
Autores principales: Bousfiha, Aziz, Jeddane, Leïla, Picard, Capucine, Ailal, Fatima, Bobby Gaspar, H., Al-Herz, Waleed, Chatila, Talal, Crow, Yanick J., Cunningham-Rundles, Charlotte, Etzioni, Amos, Franco, Jose Luis, Holland, Steven M., Klein, Christoph, Morio, Tomohiro, Ochs, Hans D., Oksenhendler, Eric, Puck, Jennifer, Tang, Mimi L. K., Tangye, Stuart G., Torgerson, Troy R., Casanova, Jean-Laurent, Sullivan, Kathleen E.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer US 2017
Materias:
Original Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5742599/
https://www.ncbi.nlm.nih.gov/pubmed/29226301
http://dx.doi.org/10.1007/s10875-017-0465-8
Descripción
Sumario: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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