Nationwide Survey of Patients with Primary Immunodeficiency Diseases in Japan

被引：67

作者：

Ishimura, Masataka ^{[1
]}

Takada, Hidetoshi ^{[1
]}

Doi, Takehiko ^{[1
]}

Imai, Kousuke ^{[2
]}

Sasahara, Yoji ^{[3
]}

Kanegane, Hirokazu ^{[4
]}

Nishikomori, Ryuta ^{[5
]}

Morio, Tomohiro ^{[6
]}

Heike, Toshio ^{[5
]}

Kobayashi, Masao ^{[7
]}

Ariga, Tadashi ^{[8
]}

Tsuchiya, Shigeru ^{[3
]}

Nonoyama, Shigeaki ^{[2
]}

Miyawaki, Toshio ^{[4
]}

Hara, Toshiro ^{[1
]}

机构：

[1] Kyushu Univ, Grad Sch Med Sci, Dept Pediat, Higashi Ku, Fukuoka 8128582, Japan

[2] Natl Def Med Coll, Dept Pediat, Tokorozawa, Saitama 359, Japan

[3] Tohoku Univ, Sch Med, Dept Pediat, Sendai, Miyagi 980, Japan

[4] Toyama Univ, Grad Sch Med & Pharmaceut Sci, Dept Pediat, Toyama 930, Japan

[5] Kyoto Univ, Grad Sch Med, Dept Pediat, Kyoto, Japan

[6] Tokyo Med & Dent Univ, Grad Sch, Dept Pediat, Bunkyo Ku, Tokyo, Japan

[7] Hiroshima Univ, Grad Sch Biomed Sci, Dept Pediat, Hiroshima, Japan

[8] Hokkaido Univ, Grad Sch Med, Dept Pediat, Sapporo, Hokkaido, Japan

来源：

JOURNAL OF CLINICAL IMMUNOLOGY | 2011年 / 31卷 / 06期

关键词：

Primary immunodeficiency disease epidemiology; nationwide survey; Japan; CHRONIC GRANULOMATOUS-DISEASE; INTRAVENOUS IMMUNOGLOBULIN; AGAMMAGLOBULINEMIA; DEFICIENCY; DISORDERS; TRANSPLANTATION; EXPERIENCE; DATABASE; REGISTRY; UPDATE;

D O I：

10.1007/s10875-011-9594-7

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

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. Questionnaires were 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-to-female 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.

引用

页码：968 / 976

页数：9

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