title: Clinical manifestations in selective IgA deficiency in childhood. A follow-up report.
abstract: Clinical manifestations in 40 children with selective IgA deficiency were studied during a follow-up period of 2-10 years. The patients were divided into two groups: group I consisted of 25 children with "sporadic" IgA deficiency and group II of 15 children with "familial" IgA deficiency. Respiratory tract infections including otitis media were frequent in both groups. Concomitant IgG2-IgG4 deficiency was found in two patients in group I. Longitudinal serum IgG levels were elevated significantly in both groups. Atopic complaints were observed in 10 children of the "sporadic" group, but only in two of the "familial" group. However, elevated serum IgE levels were more often found in group II. Two children of group I were mentally retarded and chromosomal examination showed abnormalities in both. Anti-IgA antibodies were detected in one child in group I and three children in group II. These three patients had an IgA deficient mother with class-specific anti-IgA antibodies. Concomitant IgG4-IgE deficiency was found in all four.
authors.affiliation: Department of Paediatrics, University Hospital Nijmegen, The Netherlands.
authors.forename: P C
authors.initials: PC
authors.lastname: De Laat
authors.forename: C M
authors.initials: CM
authors.lastname: Weemaes
authors.forename: R
authors.initials: R
authors.lastname: Gonera
authors.forename: P J
authors.initials: PJ
authors.lastname: Van Munster
authors.forename: J A
authors.initials: JA
authors.lastname: Bakkeren
authors.forename: G B
authors.initials: GB
authors.lastname: Stoelinga
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002869:Chromosome Aberrations; D025063:Chromosome Disorders; D002887:Chromosomes, Human, Pair 18; D002892:Chromosomes, Human, Pair 22; D004406:Dysgammaglobulinemia / Q000150:complications* / Q000235:genetics / Q000276:immunology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006969:Hypersensitivity, Immediate / Q000209:etiology / Q000276:immunology; D017098:IgA Deficiency; D007070:Immunoglobulin A / Q000097:blood; D007073:Immunoglobulin E / Q000097:blood; D007074:Immunoglobulin G / Q000097:blood; D007075:Immunoglobulin M / Q000097:blood; D007223:Infant; D008297:Male; D010033:Otitis Media / Q000209:etiology; D012008:Recurrence; D012141:Respiratory Tract Infections / Q000209:etiology
chemical_list: D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D007073:Immunoglobulin E