title: Increases in serum immunoglobulins to age-related normal levels in children with IgA and/or IgG subclass deficiency.
abstract: Immunoglobulins (Ig) A and G subclass deficiencies are common immune system disorders which cause morbidity especially between 2 and 6 yr of age. Prognosis of these defects and therapeutic approach is unclear. The aim of the present retrospective study was to review the clinical and laboratory records of 87 children with IgA and/or IgG subclass deficiency to determine whether these patients experience changes in serum Ig concentrations during follow-up and to give more clinic and laboratory information to the families about the course of these diseases. Among 87 patients studied, the most frequent defect was partial IgA deficiency combined with IgG3 subclass deficiency (41%). The other groups were as follows; partial IgA deficiency (32%), selective IgA deficiency (8%), partial IgA combined with IgG2-G4 subclass deficiency (6%), and IgG subclass deficiency (13%). The commonest clinical presentations were recurrent upper respiratory tract infections (76%), pneumonia (14%), acute gastroenteritis (3%), urinary tractus infection (3%), sinusitis (2%), and acute otitis media (2%). Atopy was widely represented in the patients studied (24%). The number of patients who were given prophylactic treatment with benzathine penicilline, prophylactic oral antibiotic, or oral bacterial extract to prevent infections was 68 (78%). Frequency of recurrent infections decreased from 7.9 +/- 4.9 per year to 2.5 +/- 2.3 in 68 patients receiving any prophylactic regimen; however, decrease in frequency of infections did not show any significant difference between different prophylactic groups. None of the patients in the selective IgA deficiency group had reached normal serum levels of IgA. At the age of 58.3 +/- 21.4 months, 52% of patients in partial IgA deficiency group and 51% of patients in partial IgA + IgG subclass deficiency group, serum IgA increased to normal ranges. Serum IgG subclass levels increased to normal range for age in 67% of patients in partial IgA + IgG subclass deficiency group and in 30% of patients in isolated IgG subclass deficiency group. The mean age for reaching age-related normal IgG subclass levels for these patients was 69.0 +/- 14.5 months. In conclusion, findings of this study suggest that IgA and/or IgG subclass deficiency may be either progressive or reversible disorders and emphasize the value of monitoring Ig levels in affected individuals.
authors.affiliation: Ege University, The Medical School, Department of Pediatrics, Izmir, Turkey. necil.kutukculer@ege.edu.tr
authors.forename: Necil
authors.initials: N
authors.lastname: Kutukculer
authors.forename: Neslihan Edeer
authors.initials: NE
authors.lastname: Karaca
authors.forename: Ozlem
authors.initials: O
authors.lastname: Demircioglu
authors.forename: Guzide
authors.initials: G
authors.lastname: Aksu
mesh_terms: D000367:Age Factors; D019072:Antibiotic Prophylaxis; D000907:Antibodies, Bacterial / Q000097:blood; D002648:Child; D002675:Child, Preschool; D005260:Female; D019741:Haemophilus influenzae type b / Q000276:immunology; D006801:Humans; D017098:IgA Deficiency / Q000276:immunology*; D017099:IgG Deficiency / Q000276:immunology*; D007070:Immunoglobulin A / Q000097:blood*; D007074:Immunoglobulin G / Q000097:blood* / Q000145:classification*; D007223:Infant; D008297:Male; D012189:Retrospective Studies
chemical_list: D000907:Antibodies, Bacterial; D007070:Immunoglobulin A; D007074:Immunoglobulin G