A1 Refereed original research article in a scientific journal
C4A deficiency in children and adolescents with recurrent respiratory infections
Authors: Kainulainen L, Peltola V, Seppanen M, Viander M, He QS, Lokki ML, Ruuskanen O
Publisher: ELSEVIER SCIENCE INC
Publication year: 2012
Journal: Human Immunology
Journal name in source: HUMAN IMMUNOLOGY
Journal acronym: HUM IMMUNOL
Number in series: 5
Volume: 73
Issue: 5
First page : 498
Last page: 501
Number of pages: 4
ISSN: 0198-8859
DOI: https://doi.org/10.1016/j.humimm.2012.02.015
Abstract
Increased susceptibility to recurrent viral and bacterial respiratory infections in children and young adults is not well understood. To evaluate the role of complement factor C4 in the defense against respiratory infections, we studied complement factor C4 allotypes C4A and C4B and copy numbers of C4A and C4B genes in 84 children and young adults with recurrent acute otitis media, sinusitis, or pneumonia and in 74 healthy controls. The occurrence of C4A gene deficiency was significantly higher in patients compared with controls (26% vs 14%, p = 0.048). Girls predominated in the group of patients with C4A deficiency (73% girls and 27% boys, p = 0.004). The lectin pathway of complement was more often functionally impaired in patients with C4A deficiency than in patients with no C4A deficiency (41% vs 13%, p = 0.033). Classical and alternative pathways were normal in individuals with C4 null alleles. C4A deficiency is 1 of the minor defects of the innate immunity that may predispose children and young adults to recurrent respiratory infections. C4 gene testing should be added to the list of investigations when the cause for recurrent acute otitis media, maxillary sinusitis, or pneumonia in children and young adults is sought. (C) 2012 American Society for Histocompatibility and lmmunogenetics. Published by Elsevier Inc. All rights reserved.
Increased susceptibility to recurrent viral and bacterial respiratory infections in children and young adults is not well understood. To evaluate the role of complement factor C4 in the defense against respiratory infections, we studied complement factor C4 allotypes C4A and C4B and copy numbers of C4A and C4B genes in 84 children and young adults with recurrent acute otitis media, sinusitis, or pneumonia and in 74 healthy controls. The occurrence of C4A gene deficiency was significantly higher in patients compared with controls (26% vs 14%, p = 0.048). Girls predominated in the group of patients with C4A deficiency (73% girls and 27% boys, p = 0.004). The lectin pathway of complement was more often functionally impaired in patients with C4A deficiency than in patients with no C4A deficiency (41% vs 13%, p = 0.033). Classical and alternative pathways were normal in individuals with C4 null alleles. C4A deficiency is 1 of the minor defects of the innate immunity that may predispose children and young adults to recurrent respiratory infections. C4 gene testing should be added to the list of investigations when the cause for recurrent acute otitis media, maxillary sinusitis, or pneumonia in children and young adults is sought. (C) 2012 American Society for Histocompatibility and lmmunogenetics. Published by Elsevier Inc. All rights reserved.
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