Prevalence of Primary Immunodeficiency in Korea.
10.3346/jkms.2012.27.7.788
- Author:
Jung Woo RHIM
1
;
Kyung Hyo KIM
;
Dong Soo KIM
;
Bong Seong KIM
;
Jung Soo KIM
;
Chang Hwi KIM
;
Hwang Min KIM
;
Hee Ju PARK
;
Ki Soo PAI
;
Byong Kwan SON
;
Kyung Sue SHIN
;
Moo Young OH
;
Young Jong WOO
;
Young YOO
;
Kun Soo LEE
;
Kyung Yil LEE
;
Chong Guk LEE
;
Joon Sung LEE
;
Eun Hee CHUNG
;
Eun Hwa CHOI
;
Youn Soo HAHN
;
Hyun Young PARK
;
Joong Gon KIM
Author Information
1. Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea.
- Publication Type:Original Article ; Research Support, Non-U.S. Gov't
- Keywords:
Primary Immunodeficiencies;
Registry;
Korea
- MeSH:
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
- From:Journal of Korean Medical Science
2012;27(7):788-793
- CountryRepublic of Korea
- Language:English
-
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.
