Document Detail


Nationwide Survey of Patients with Primary Immunodeficiency Diseases in Japan.
MedLine Citation:
PMID:  21956496     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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.
Authors:
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
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-9-29
Journal Detail:
Title:  Journal of clinical immunology     Volume:  -     ISSN:  1573-2592     ISO Abbreviation:  -     Publication Date:  2011 Sep 
Date Detail:
Created Date:  2011-9-29     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8102137     Medline TA:  J Clin Immunol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan, ischii@pediatr.med.kyushu-u.ac.jp.
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