title: Endocrine complications in primary immunodeficiency diseases in Japan.
abstract: BACKGROUND
  In spite of the accumulating evidence on the interaction between the immune and endocrine systems based on the recent progress in molecular genetics, there have been few epidemiological studies focused on the endocrine complications associated with primary immunodeficiency diseases (PID).
  
  
  OBJECTIVE
  To investigate the prevalence and clinical features of endocrine complications in patients with PID in a large-scale study.
  
  
  METHODS
  This survey was conducted on patients with PID who were alive on 1 December 2008 and those who were newly diagnosed and died between 1 December 2007 and 30 November 2008 in Japan. We investigated the prevalence and the clinical data of the endocrine complications in 923 patients with PID registered in the secondary survey.
  
  
  RESULTS
  Among 923 PID patients, 49 (5·3%) had endocrine disorders. The prevalence of the endocrine diseases was much higher in patients with PID than in the general population in the young age group, even after excluding patients with immune dysregulation.
  
  
  CONCLUSIONS
  Endocrine disorders are important complications of PID. Analysis of the endocrine manifestations in patients with PID in a large-scale study may provide further insights into the relationship between the immune and endocrine systems.
authors.affiliation: Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. t-nozaki@pediatr.med.kyushu-u.ac.jp
authors.forename: Takafumi
authors.initials: T
authors.lastname: Nozaki
authors.forename: Hidetoshi
authors.initials: H
authors.lastname: Takada
authors.forename: Masataka
authors.initials: M
authors.lastname: Ishimura
authors.forename: Kenji
authors.initials: K
authors.lastname: Ihara
authors.forename: Kohsuke
authors.initials: K
authors.lastname: Imai
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Morio
authors.forename: Masao
authors.initials: M
authors.lastname: Kobayashi
authors.forename: Shigeaki
authors.initials: S
authors.lastname: Nonoyama
authors.forename: Toshiro
authors.initials: T
authors.lastname: Hara
mesh_terms: D000293:Adolescent; D002648:Child; D004700:Endocrine System Diseases / Q000453:epidemiology / Q000209:etiology; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000453:epidemiology*; D007564:Japan / Q000453:epidemiology; D008297:Male; D015995:Prevalence