[Susceptibility to infections and hyper IgE: 19 new case reports ].
Nineteen patients exhibiting a susceptibility to infections due to staphylococcus and fungi, allergy and hyper IgE are reported (syndrome described by R. Buckley). Prognathism, coarse features and marked osteoporosis were observed in more than half the patients. Immune disorders were characterized by a defective chemotaxis of granulocytes and depressed in vivo and in vitro immune responses to antigens. These cellular abnormalities could be secondary to a dysregulation of IgE synthesis resulting in an excess of secretion of histamine, heparin and other substances from mast cells. According to this possible mechanism, we suggest a therapy associating anti-histaminic substances which block H1 and H2 receptors and an agent which inhibits mast cell degranulation.
D000293:Adolescent; D000328:Adult; D000917:Antibody Formation; D002634:Chemotaxis, Leukocyte; D002648:Child; D002675:Child, Preschool; D004485:Eczema / Q000276:immunology; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000209:etiology*; D006967:Hypersensitivity / Q000276:immunology; D007111:Immunity, Cellular; D007073:Immunoglobulin E / Q000302:isolation & purification*; D007239:Infections / Q000276:immunology*; D008297:Male; D010024:Osteoporosis / Q000276:immunology; D011378:Prognathism / Q000276:immunology; D011379:Prognosis; D013577:Syndrome
D007073:Immunoglobulin E
Syndrome de susceptibilité aux infections avec hyper IgE. A propos de 19 nouvelles observations.