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Prevalence of primary immunodeficiency in Korea

Title
Prevalence of primary immunodeficiency in Korea
Authors
Rhim J.W.Kim K.H.Kim D.S.Kim B.S.Kim J.S.Kim C.H.Kim H.M.Park H.J.Pai K.S.Son B.K.Shin K.S.Oh M.Y.Woo Y.J.Yoo Y.Lee K.S.Lee K.Y.Lee C.G.Lee J.S.Chung E.H.Choi E.H.Hahn Y.S.Park H.Y.Kim J.G.
Ewha Authors
김경효
SCOPUS Author ID
김경효scopus
Issue Date
2012
Journal Title
Journal of Korean Medical Science
ISSN
1011-8934JCR Link
Citation
vol. 27, no. 7, pp. 788 - 793
Indexed
SCI; SCIE; SCOPUS; KCI WOS scopus
Abstract
This study represents the first epidemiological study based on the national registry of primary immunodeficiencies (PID) in Korea. Patient data were collected from 23 major hospitals. A total of 152 patients with PID (under 19 yr of age), who were observed from 2001 to 2005, have been entered in this registry. The period prevalence of PID in Korea in 2005 is 11.25 per million children. The following frequencies were found: antibody deficiencies, 53.3% (n = 81), phagocytic disorders, 28.9% (n = 44); combined immunodeficiencies, 13.2% (n = 20); and T cell deficiencies, 4.6% (n = 7). Congenital agammaglobulinemia (n = 21) and selective IgA deficiency (n = 21) were the most frequently reported antibody deficiency. Other reported deficiencies were common variable immunodeficiencies (n = 16), X-linked agammaglobulinemia (n = 15), IgG subclass deficiency (n = 4). Phagocytic disorder was mostly chronic granulomatous disease. A small number of patients with Wiskott-Aldrich syndrome, hyper-IgE syndrome, and severe combined immunodeficiency were also registered. Overall, the most common first manifestation was pneumonia. This study provides data that permit a more accurate estimation PID patients in Korea. © 2012 The Korean Academy of Medical Sciences.
DOI
10.3346/jkms.2012.27.7.788
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의과대학 > 의학과 > Journal papers
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