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Selective IgA deficiency: Clinical and immunological evaluation of 50 pediatric patients

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Abstract

Fifty children with IgA deficiency were followed for 1 to 4 years from 1975 to 1978. Thirty-five had complete deficiency of serum IgA (<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.

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Dedicated to Prof. Dr. H.-R. Wiedemann on the occasion of his 65th birthday

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Burgio, G.R., Duse, M., Monafo, V. et al. Selective IgA deficiency: Clinical and immunological evaluation of 50 pediatric patients. Eur J Pediatr 133, 101–106 (1980). https://doi.org/10.1007/BF00441577

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