Nationwide Survey of Patients with Primary Immunodeficiency Diseases in Japan
Nationwide Survey of Patients with Primary Immunodeficiency Diseases in Japan
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DOI：
10.1007/s10875-011-9594-7
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发表时间：
2011-12-01
影响因子：
9.1
通讯作者：
Hara, Toshiro
中科院分区：
文献类型：
--
作者：
Ishimura, Masataka;Takada, Hidetoshi;Hara, Toshiro
为了确定日本原发性免疫缺陷病（PID）患者的患病率和临床特征，我们30年来首次在全国范围内对PID患者进行了调查。问卷被发送到日本医院的1，224个儿科和1，670个内科。共有1 240名患者登记。PID患者的估计数量为2，900例，患病率为2.3/100，000人，在日本具有均匀的区域分布。男女比例为2.3：1，中位年龄为12.8岁。青少年或成人占42.8%。分别有25例（2.7%）和78例（8.5%）患者发生恶性疾病和免疫相关疾病，作为原发性免疫缺陷病的并发症。除了预防传染病外，密切监测和适当管理这些并发症对改善PID患者的生活质量非常重要。
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.