title: X-linked hyper IgM syndrome.
abstract: X-linked hyper IgM syndrome is largely caused by defects in the CD40L (CD154). The patients have low levels of IgG and have difficulty with respiratory infections as well as opportunistic infections. The morbidity of this syndrome is high; however, early diagnosis and therapy should reduce morbidity and mortality.
authors: Division of Immunology, Children's Hospital, Boston, MA 02115, USA.
authors: L C
authors: LC
authors: Schneider
mesh_terms: D000293:Adolescent; D019013:CD40 Antigens / Q000378:metabolism; D023201:CD40 Ligand / Q000378:metabolism; D002648:Child; D005260:Female; D006580:Genetic Carrier Screening; D006801:Humans; D006942:Hypergammaglobulinemia / Q000209:etiology / Q000235:genetics / Q000276:immunology; D007075:Immunoglobulin M; D008297:Male; D014960:X Chromosome
chemical_list: D019013:CD40 Antigens; D007075:Immunoglobulin M; D023201:CD40 Ligand