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Erschienen in:

01.12.2011

Nationwide Survey of Patients with Primary Immunodeficiency Diseases in Japan

verfasst von: Masataka Ishimura, Hidetoshi Takada, Takehiko Doi, Kousuke Imai, Yoji Sasahara, Hirokazu Kanegane, Ryuta Nishikomori, Tomohiro Morio, Toshio Heike, Masao Kobayashi, Tadashi Ariga, Shigeru Tsuchiya, Shigeaki Nonoyama, Toshio Miyawaki, Toshiro Hara

Erschienen in: Journal of Clinical Immunology | Ausgabe 6/2011

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Abstract

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.

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Titel: Nationwide Survey of Patients with Primary Immunodeficiency Diseases in Japan
verfasst von: Masataka Ishimura
Hidetoshi Takada
Takehiko Doi
Kousuke Imai
Yoji Sasahara
Hirokazu Kanegane
Ryuta Nishikomori
Tomohiro Morio
Toshio Heike
Masao Kobayashi
Tadashi Ariga
Shigeru Tsuchiya
Shigeaki Nonoyama
Toshio Miyawaki
Toshiro Hara
Publikationsdatum: 01.12.2011
Verlag: Springer US
Erschienen in: Journal of Clinical Immunology / Ausgabe 6/2011
Print ISSN: 0271-9142
Elektronische ISSN: 1573-2592
DOI: https://doi.org/10.1007/s10875-011-9594-7

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