Document Detail


Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee.
MedLine Citation:
PMID:  17952897     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Primary immunodeficiency diseases (PIDs) are a genetically heterogeneous group of disorders that affect distinct components of the innate and adaptive immune system, such as neutrophils, macrophages, dendritic cells, complement proteins, natural killer cells, and T and B lymphocytes. The study of these diseases has provided essential insights into the functioning of the immune system. More than 120 distinct genes have been identified, whose abnormalities account for more than 150 different forms of PID. The complexity of the genetic,immunologic, and clinical features of PID has prompted the need for their classification, with the ultimate goal of facilitating diagnosis and treatment. To serve this goal, an international committee of experts has met every 2 years since 1970. In its last meeting in Jackson Hole, Wyo, after 3 days of intense scientific presentations and discussions, the committee has updated the classification of PID, as reported in this article.
Authors:
Raif S Geha; Luigi D Notarangelo; Jean-Laurent Casanova; Helen Chapel; Mary Ellen Conley; Alain Fischer; Lennart Hammarström; Shigeaki Nonoyama; Hans D Ochs; Jennifer M Puck; Chaim Roifman; Reinhard Seger; Josiah Wedgwood;
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The Journal of allergy and clinical immunology     Volume:  120     ISSN:  0091-6749     ISO Abbreviation:  J. Allergy Clin. Immunol.     Publication Date:  2007 Oct 
Date Detail:
Created Date:  2007-10-19     Completed Date:  2007-11-02     Revised Date:  2011-09-26    
Medline Journal Info:
Nlm Unique ID:  1275002     Medline TA:  J Allergy Clin Immunol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  776-94     Citation Subset:  AIM; IM    
Affiliation:
Division of Immunology, Children's Hospital, Boston, MA 02115, USA. faif.geha@childrens.harvard.edu
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MeSH Terms
Descriptor/Qualifier:
Humans
Immune System / physiology
Immunologic Deficiency Syndromes / classification*,  genetics,  immunology
Grant Support
ID/Acronym/Agency:
AI-35714/AI/NIAID NIH HHS; P01 AI035714-100005/AI/NIAID NIH HHS; R13 AI066891-01/AI/NIAID NIH HHS; R13 AI066891-02/AI/NIAID NIH HHS; R13 AI066891-03/AI/NIAID NIH HHS; R13 AI066891-04/AI/NIAID NIH HHS
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