TY - JOUR
T1 - Nationwide survey of patients with primary immunodeficiency diseases in Japan
AU - Ishimura, Masataka
AU - Takada, Hidetoshi
AU - Doi, Takehiko
AU - Imai, Kousuke
AU - Sasahara, Yoji
AU - Kanegane, Hirokazu
AU - Nishikomori, Ryuta
AU - Morio, Tomohiro
AU - Heike, Toshio
AU - Kobayashi, Masao
AU - Ariga, Tadashi
AU - Tsuchiya, Shigeru
AU - Nonoyama, Shigeaki
AU - Miyawaki, Toshio
AU - Hara, Toshiro
N1 - Funding Information:
Acknowledgments The authors would like to thank the support of the Japanese Research Group on Primary Immunodeficiency Diseases, which is supported by Japan’s Ministry of Health, Labour and Welfare.
PY - 2011/12
Y1 - 2011/12
N2 - To determine the prevalence and clinical characteristics of patients with in Japan, we conducted a nationwide survey of primary immunodeficiency disease (PID) patients for the first time in 30 years.Questionnaireswere sent to 1,224 pediatric departments and 1,670 internal medicine departments of Japanese hospitals. A total of 1,240 patients were registered. The estimated number of patients with PID was 2,900 with a prevalence of 2.3 per 100,000 people and homogenous regional distribution in Japan. The male-tofemale ratio was 2.3:1 with a median age of 12.8 years. Adolescents or adults constituted 42.8% of the patients. A number of 25 (2.7%) and 78 (8.5%) patients developed malignant disorders and immune-related diseases, respectively, as complications of primary immunodeficiency disease. Close monitoring and appropriate management for these complications in addition to prevention of infectious diseases is important for improving the quality of life of PID patients.
AB - To determine the prevalence and clinical characteristics of patients with in Japan, we conducted a nationwide survey of primary immunodeficiency disease (PID) patients for the first time in 30 years.Questionnaireswere sent to 1,224 pediatric departments and 1,670 internal medicine departments of Japanese hospitals. A total of 1,240 patients were registered. The estimated number of patients with PID was 2,900 with a prevalence of 2.3 per 100,000 people and homogenous regional distribution in Japan. The male-tofemale ratio was 2.3:1 with a median age of 12.8 years. Adolescents or adults constituted 42.8% of the patients. A number of 25 (2.7%) and 78 (8.5%) patients developed malignant disorders and immune-related diseases, respectively, as complications of primary immunodeficiency disease. Close monitoring and appropriate management for these complications in addition to prevention of infectious diseases is important for improving the quality of life of PID patients.
KW - Epidemiology
KW - Japan
KW - Nationwide survey
KW - Primary immunodeficiency disease
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U2 - 10.1007/s10875-011-9594-7
DO - 10.1007/s10875-011-9594-7
M3 - Article
C2 - 21956496
AN - SCOPUS:84857050426
SN - 0271-9142
VL - 31
SP - 968
EP - 976
JO - Journal of Clinical Immunology
JF - Journal of Clinical Immunology
IS - 6
ER -
