@entity358 of primary XXXX .

@entity12089 @entity1071 @entity17454 @entity61363 @entity10190 @entity72653 @entity465 @entity1200 @entity358 @entity74 @entity1117
In the fifty years since Ogden @entity12089 , more than 100 additional @entity465 have been described. These disorders may involve one or more components of the immune system, including T, B, and NK lymphocytes; phagocytic cells; and complement proteins. Most are recessive traits, some of which are caused by mutations in genes on the X chromosome, others in genes on autosomal chromosomes. Until the past decade, there was little insight into the fundamental problems underlying a majority of these conditions. Many of the primary @entity1200 have now been mapped to specific chromosomal locations, and the fundamental biologic errors have been identified in more than 3 dozen. Within the past decade the molecular bases of 7 X-linked @entity1200 have been reported: X-linked immunodeficiency with Hyper IgM, @entity1071 , @entity12089 , X-linked severe @entity465 , the @entity17454 , nuclear factor kappaB essential modulator ( @entity10190 or @entity10190 ), and the @entity72653 . The abnormal genes in @entity1117 ( @entity1117 ) and @entity61363 had been identified several years earlier. In addition, there are now many @entity74 for which the molecular bases have been discovered. These new advances will be reviewed, with particular emphasis on the @entity358 of some of these diseases. In some cases there are unique features of @entity358 in specific defects. Infections obviously account for most of these complications, but the host reaction to infection often leads to characteristic findings that can be helpful diagnostically. Finally, advances in treatment of the underlying diseases as well as their infectious complications will be covered.
