Prevalence of primary immunodeficiency in Korea.
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.
Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea.
Jung Woo
JW
Rhim
Kyung Hyo
KH
Kim
Dong Soo
DS
Kim
Bong Seong
BS
Kim
Jung Soo
JS
Kim
Chang Hwi
CH
Kim
Hwang Min
HM
Kim
Hee Ju
HJ
Park
Ki Soo
KS
Pai
Byong Kwan
BK
Son
Kyung Sue
KS
Shin
Moo Young
MY
Oh
Young Jong
YJ
Woo
Young
Y
Yoo
Kun Soo
KS
Lee
Kyung Yil
KY
Lee
Chong Guk
CG
Lee
Joon Sung
JS
Lee
Eun Hee
EH
Chung
Eun Hwa
EH
Choi
Youn Soo
YS
Hahn
Hyun Young
HY
Park
Joong Gon
JG
Kim
D000293:Adolescent; D000361:Agammaglobulinemia / Q000151:congenital / Q000453:epidemiology; D017677:Age Distribution; D002648:Child; D002675:Child, Preschool; D017074:Common Variable Immunodeficiency / Q000453:epidemiology; D005260:Female; D040181:Genetic Diseases, X-Linked / Q000453:epidemiology; D006801:Humans; D017098:IgA Deficiency / Q000453:epidemiology; D017099:IgG Deficiency / Q000453:epidemiology; D007153:Immunologic Deficiency Syndromes / Q000453:epidemiology*; D007223:Infant; D007231:Infant, Newborn; D007589:Job Syndrome / Q000453:epidemiology; D008297:Male; D015995:Prevalence; D012042:Registries; D056910:Republic of Korea / Q000453:epidemiology; D016511:Severe Combined Immunodeficiency / Q000453:epidemiology; D017678:Sex Distribution; D011795:Surveys and Questionnaires; D014923:Wiskott-Aldrich Syndrome / Q000453:epidemiology; D055815:Young Adult