소아 IgA 결핍증의 임상양상과 면역학적 특징
The Clinical Manifestations and Immunological Characteristics ofSelective IgA Deficiency in Children
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Purpose:Selective immunoglobulin A deficiency (IgAD) is the most common primary immunodeficiency disease. The aim of the present study is to analyze clinical manifestations and immunological characteristics of selective IgAD in children. Methods:Twenty-one children aged 1-14 years with the serum IgA level of 2 standard deviation (2 SD) below normal for age who had recurrent infection were enrolled in this study at Uijeongbu St. Mary’s Hospital of the Catholic University of Korea, between January 2006 and August 2007. Recurrent infection was defined as the presence of at least 6 febrile infection episodes per year before this study. Among IgA deficient patients, we excluded panhypogammaglobulinemic patients. Results:The average frequency of admission was 2.2±2.0 per year. The most common causative disease at admission was lower respiratory infection (53.6%). Immunoglobulin G (IgG) subclass deficiency was corcurrent in 9 patients (42.9%): deficiency of IgG2 in 1, IgG3 in 5, IgG4 in 1 and IgG3＋IgG4 in 2. Five patients (23.8%) had a decrease in CD3＋ cells and 8 patients (38%) had a decrease in CD4＋ cells. One patient progressed to common variable immunodeficiency (CVID). Serum IgA levels of 76.5% of the patients increased to 2 SD above normal for age after 15.9±4.5 months. Conclusion:The patients with IgAD in this study showed frequent respiratory and gastrointestinal infections and combined with other immunodeficiencies including IgG subclass deficiency. Our data suggest that it is necessary to examine the immunological status including IgA level in patients with frequent infections and that those with IgAD should be observed for the potential progression to CVID.
Abstract
Purpose:Selective immunoglobulin A deficiency (IgAD) is the most common primary immunodeficiency disease. The aim of the present study is to analyze clinical manifestations and immunological characteristics of selective IgAD in children. Methods:Twenty-one children aged 1-14 years with the serum IgA level of 2 standard deviation (2 SD) below normal for age who had recurrent infection were enrolled in this study at Uijeongbu St. Mary’s Hospital of the Catholic University of Korea, between January 2006 and August 2007. Recurrent infection was defined as the presence of at least 6 febrile infection episodes per year before this study. Among IgA deficient patients, we excluded panhypogammaglobulinemic patients. Results:The average frequency of admission was 2.2±2.0 per year. The most common causative disease at admission was lower respiratory infection (53.6%). Immunoglobulin G (IgG) subclass deficiency was corcurrent in 9 patients (42.9%): deficiency of IgG2 in 1, IgG3 in 5, IgG4 in 1 and IgG3＋IgG4 in 2. Five patients (23.8%) had a decrease in CD3＋ cells and 8 patients (38%) had a decrease in CD4＋ cells. One patient progressed to common variable immunodeficiency (CVID). Serum IgA levels of 76.5% of the patients increased to 2 SD above normal for age after 15.9±4.5 months. Conclusion:The patients with IgAD in this study showed frequent respiratory and gastrointestinal infections and combined with other immunodeficiencies including IgG subclass deficiency. Our data suggest that it is necessary to examine the immunological status including IgA level in patients with frequent infections and that those with IgAD should be observed for the potential progression to CVID.
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