title: Selective IgA deficiency: clinical and immunological evaluation of 50 pediatric patients.
abstract: Fifty children with IgA deficiency were folllowed for 1 to 4 years from 1975 to 1978. Thirty-five had complete deficiency of serum IgA (less than 2.5 IU/ml) and 15 partial deficiency (serum IgA below the 10th centile for age). Patients with another associated immunodeficiency, such as ataxia-telangiectasia, were not included. Most children with complete deficiency of IgA had recurrent respiratory and/or gastrointestinal infections, about half with onset in the first year of life, while partial deficiency of IgA has probably little if any importance for anti-infectious immunity but is important in the pathogenesis of atopy. Atopic diseases were frequent in both groups. Chromosomal abnormalities were found in 2 patients: trisomy 21 in one and in the other a ring chromosome 18. No important defects in cellular immunity were detected but some isolated, borderline abnormalities were often present.
mesh_terms: D002648:Child; D002675:Child, Preschool; D002869:Chromosome Aberrations / Q000276:immunology; D025063:Chromosome Disorders; D002902:Chromosomes, Human, 16-18; D004314:Down Syndrome / Q000276:immunology; D004406:Dysgammaglobulinemia / Q000276:immunology*; D005260:Female; D005759:Gastroenteritis / Q000276:immunology; D006801:Humans; D017098:IgA Deficiency; D007111:Immunity, Cellular; D007070:Immunoglobulin A / Q000032:analysis; D007223:Infant; D008297:Male; D012008:Recurrence; D012141:Respiratory Tract Infections / Q000276:immunology
chemical_list: D007070:Immunoglobulin A