Primary immunodeficiency diseases: an update
Primary immunodeficiency diseases: an update
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DOI:
10.1046/j.1365-2249.2003.02110.x
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发表时间:
2003-04-01
影响因子:
4.6
通讯作者:
Rosen, F
中科院分区:
文献类型:
--
作者:
Chapel, H;Geha, R;Rosen, F
The last full report of the IUIS Scientific Committee for Primary Immunodeficiencies [PIDs] was published in Clinical and Experimental Immunology over 3 years ago [1]. This covered the relevant basic immunological principles, cellular, genetic, humoral (including cytokine) and induction aspects of immune responses to those microbial antigens involved in human infections. All primary immunodeficiencies (and the investigations required for diagnosis) are discussed in turn, ranging from combined deficiencies of T and B cells, predominantly humoral defects, T cell defects (including those of cytokine and cytokine receptor production), complement and phagocyte deficiencies and those immunodeficiencies associated with genetic defects in related systems. A section on therapies and brief descriptions of causes of secondary immunodeficiencies completed the knowledge of ID known at that time.The report was timely and has been much quoted in the literature as a reference point for papers on mechanisms of both PID diseases and their treatments. The citation index must be substantial. The most recent meeting of this committee took place in July 2001. During the intervening 2 years there were few new types of PID; most of the newly described diseases were extensions of current phenotypes such as: