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KMID : 0191120120270070788
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Journal of Korean Medical Science
2012 Volume.27 No. 7 p.788 ~ p.793
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Prevalence of Primary Immunodeficiency in Korea
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Rhim Jung-Woo
Kim Kyung-Hyo
Kim Dong-Soo
Kim Bong-Seong
Kim Jung-Soo
Kim Chang-Hwi
Kim Hwang-Min
Park Hee-Ju
Pai Ki-Soo
Son Byong-Kwan
Shin Kyung-Sue
Oh Moo-Young
Woo Young-Jong
Yoo Young
Lee Kun-Soo
Lee Kyung-Yil
Lee Chong-Guk
Lee Joon-Sung
Chung Eun-Hee
Choi Eun-Hwa
Hahn Youn-Soo
Park Hyun-Young
Kim Joong-Gon
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Abstract
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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.
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KEYWORD
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Primary Immunodeficiencies, Registry, Korea
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