[Hyperimmunoglobulinemia E syndrome with recurrent infections (Buckley's syndrome) associated with membranoproliferative glomerulonephritis. A case in an adult].
A 35-year old male patient who, since the age of 17, had membranoproliferative glomerulonephritis presented with recurrent atopic eczema and sinusitis. Outbursts of eczema, sinusitis and proteinuria occurred simultaneously. Signs of immune deficiency, with permanently extreme IgE levels (greater then 10.000 I.U/ml), defective neutrophil chemotaxis and monocyte phagocytotic function (Buckley's syndrome) were present. The role of these abnormalities in the pathogenesis of glomerulonephritis (chronic antigen production associated with focal infection and resulting in the formation of immune complexes, defective elimination of these circulating immune complexes) is discussed.
D000293:Adolescent; D000328:Adult; D000936:Antigen-Antibody Complex / Q000032:analysis; D002634:Chemotaxis, Leukocyte; D003876:Dermatitis, Atopic / Q000276:immunology; D005921:Glomerulonephritis / Q000276:immunology*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications*; D007073:Immunoglobulin E / Q000032:analysis*; D007239:Infections / Q000276:immunology*; D008297:Male; D009504:Neutrophils / Q000502:physiology; D012008:Recurrence; D012852:Sinusitis / Q000276:immunology; D013577:Syndrome
D000936:Antigen-Antibody Complex; D007073:Immunoglobulin E
Syndrome d'hyperimmunoglobulinémie E avec infections récurrentes (syndrome de Buckley) associé à une glomérulonéphrite membrano-proliférative. Une observation chez un adulte.