[The Buckley syndrome: recurring, severe staphylococcal infections, eczema and hyperimmunoglobulinemia E. (author's transl)].
Fifteen patients aged between three and 27 years were examined clinically and immunologically. Common to all patients were severe recurring cutaneous and pulmonary staphyloccal infections, chronic eczema, eosinophilia and an extremely elevated serum IgE level. Eight of the patients had in addition facial dysplasia characterised by coarse features, prognathism and poorly formed external ears. Marked osteoporosis, particularly of the vertebral bodies, was observed in eight patients. A constant defect of granulocyte chemotaxis was found in only three patients; fluctuating or constantly normal chemotaxis occurred in six patients. Polycloncal hypergammaglobulinemia was detected in 14 patients, elevated IgD in two patients, a partial T-cell defect in two patients and a history of lack of antibody response in one patient. Therapeutic trails anti-H2 receptor-antihistamines did not produce lasting or satisfactory clinical or immunological results in the pathogenetically unidentified disease.
D000293:Adolescent; D000328:Adult; D000740:Anemia / Q000150:complications; D000917:Antibody Formation; D002633:Chemotaxis; D002648:Child; D002675:Child, Preschool; D004485:Eczema / Q000150:complications*; D004804:Eosinophils; D005260:Female; D006130:Growth Disorders; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications*; D007073:Immunoglobulin E; D007136:Immunoglobulins / Q000032:analysis; D007958:Leukocyte Count; D008297:Male; D009504:Neutrophils; D010024:Osteoporosis / Q000150:complications; D010587:Phagocytosis; D013203:Staphylococcal Infections / Q000150:complications*; D013577:Syndrome; D013601:T-Lymphocytes / Q000276:immunology
D007136:Immunoglobulins; D007073:Immunoglobulin E
Das Buckley-Syndrom: Rezidivierende, schwere Staphylokokken-infektionen, Ekzem und Hyperimmunglobulinmie E.