J Korean Med Sci.  2012 Jul;27(7):788-793. 10.3346/jkms.2012.27.7.788.

Prevalence of Primary Immunodeficiency in Korea

• Rhim JW
• Kim KH
• Kim DS
• Kim BS
• Kim JS
• Kim CH
• Kim HM
• Park HJ
• Pai KS
• Son BK
• Shin KS
• Oh MY
• Woo YJ
• Yoo Y
• Lee KS
• Lee KY
• Lee CG
• Lee JS
• Chung EH
• Choi EH
• Hahn YS
• Park HY
• Kim JG

• KMID: 2157925
• DOI: http://doi.org/10.3346/jkms.2012.27.7.788

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.

Keyword

Primary Immunodeficiencies; Registry; Korea

MeSH Terms

Adolescent
Agammaglobulinemia/congenital/epidemiology
Age Distribution
Child
Child, Preschool
Common Variable Immunodeficiency/epidemiology
Female
Genetic Diseases, X-Linked/epidemiology
Humans
IgA Deficiency/epidemiology
IgG Deficiency/epidemiology
Immunologic Deficiency Syndromes/*epidemiology
Infant
Infant, Newborn
Job's Syndrome/epidemiology
Male
Prevalence
Questionnaires
Registries
Republic of Korea/epidemiology
Severe Combined Immunodeficiency/epidemiology
Sex Distribution
Wiskott-Aldrich Syndrome/epidemiology
Young Adult

Figure

• 

  Fig. 1 Map of Korea showing the provinces 1-7 and the prevalence of primary immunodeficiencies according to the regions.

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