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Clinical comparison between patients with selective immunoglobulin A deficiency and other primary immunodeficiencies.

Abstract
Primary immunodeficiencies (PID) are low-prevalence diseases. There are warning signs that may raise clinical suspicion. The objectives of this study were to describe the clinical characteristics and warning signs of patients with PID and to compare the clinical differences between selective immunoglobulin A (IgA) deficiency and other PIDs. Eighty-nine patients were studied; their median age at the time of diagnosis was 6 years old (4.08-11.67). Fifty-three (59.5%) patients were male. Fifty-four (60.7%) patients had selective IgA deficiency, and 35 (39.3%) had other PIDs. The main clinical manifestations were rhinopharyngitis in 65 (73.03%) patients and atopy in 39 (43.82%). Twenty- four (26.97%) patients showed warning signs, and none had selective IgA deficiency. Patients with other PIDs had a higher incidence of lower respiratory tract infection, sepsis, skin infections, mucocutaneous candidiasis, dental alterations, cardiovascular malformations, angioedema, hospitalizations and death. Ten (28.57%) patients received intravenous gammaglobulin, 15 (42.85%) antibiotic prophylaxis, and 2 (2.24%) antifungal prophylaxis.
AuthorsNatalia A Lozano, Alejandro Lozano, Laura V Sasia, Ricardo J Saranz, María Fernanda Agresta, María del Pilar Bovina Martijena, Luciano Ianiero, Andrés R Grenat
JournalArchivos argentinos de pediatria (Arch Argent Pediatr) Vol. 113 Issue 2 Pg. 141-5 (04 2015) ISSN: 1668-3501 [Electronic] Argentina
PMID25727826 (Publication Type: Comparative Study, Journal Article, Observational Study, Research Support, Non-U.S. Gov't)
Topics
  • Child
  • Child, Preschool
  • Diagnosis, Differential
  • Female
  • Humans
  • IgA Deficiency (diagnosis)
  • Immunologic Deficiency Syndromes (diagnosis)
  • Male
  • Retrospective Studies

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