期刊
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
卷 124, 期 6, 页码 1161-1178出版社
MOSBY-ELSEVIER
DOI: 10.1016/j.jaci.2009.10.013
关键词
Primary immunodeficiencies; T cells; B cells; severe combined immunodeficiency; predominantly antibody deficiencies; DNA repair defects; phagocytes; complement; immune dysregulation syndromes; innate immunity; autoinflammatory disorders
资金
- Jeffrey Modell Foundation
- National Institute of Allergy and Infectious Diseases [R13-AI-066891]
- National Institutes of Health [Al-35714]
- Baxter Healthcare
- Talecris
- Biotest
- Baxter Corp.
- March of Dimes
- European Community
- Swedish Research Council
More than 50 years after Ogdeon Bruton's discovery of congenital agammaglobulinemia, human primary immunodeficiencies (PIDs) continue to unravel novel molecular and cellular mechanisms that govern development and function of the human immune system. This report provides the updated classification of PIDs that has been compiled by the International Union of Immunological Societies Expert Committee on Primary Immunodeficiencies after its biannual meeting in Dublin, Ireland, in June 2009. Since the appearance of the last classification in 2007, novel forms of PID have been discovered, and additional pathophysiology mechanisms that account for PID in human beings have been unraveled. Careful analysis and prompt recognition of these disorders is essential to prompt effective forms of treatment and thus to improve survival and quality of life in patients affected with PIDs. (J Allergy Clin Immunol 2009;124:1161-78.)
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