title: Incidence, clinical features, and risk factors of idiopathic pneumonia syndrome following hematopoietic stem cell transplantation in children.
abstract: BACKGROUND
  Idiopathic pneumonia syndrome (IPS) is a severe complication that can occur after hematopoietic stem cell transplantation (HSCT) and is often associated with a fatal outcome despite intensive supportive care.
  
  
  METHODS
  To assess the incidence and risk factors of IPS, we reviewed 251 consecutive patients (median age, 7.0 years) who received HSCT at the Department of Pediatrics, Nagoya University Hospital, between January 1990 and July 2009.
  
  
  RESULTS
  Twenty of 251 (cumulative incidence of IPS at 2 years after HSCT, 8.0%; 95% confidence interval (CI), 5.1-12.4%) patients developed IPS. The median duration from HSCT to diagnosis of IPS was 67 days (range, 12-486 days). Patients with IPS had significantly higher 5-year transplant-related mortality compared to patients without IPS (52% (95% CI, 19-77%) vs. 13% (95% CI, 5-25%), P < 0.001), and the probability of 5-year overall survival was significantly worse for patients with IPS (42% (95% CI, 25-64%) vs. 68% (95% CI, 59-76%), P = 0.01). By multivariate analysis, high risk in underlying disease (HR, 2.5; 95% CI, 1.0-6.7; P = 0.05) and a busulfan-containing regimen (HR, 3.5; 95% CI, 1.3-9.9; P < 0.01) were identified as the independent risk factors for developing IPS.
  
  
  CONCLUSIONS
  The prophylactic strategies for IPS in patients with these risk factors were warranted.
authors.affiliation: Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Hirotoshi
authors.initials: H
authors.lastname: Sakaguchi
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Takahashi
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Watanabe
authors.forename: Sayoko
authors.initials: S
authors.lastname: Doisaki
authors.forename: Hideki
authors.initials: H
authors.lastname: Muramatsu
authors.forename: Asahito
authors.initials: A
authors.lastname: Hama
authors.forename: Akira
authors.initials: A
authors.lastname: Shimada
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Yagasaki
authors.forename: Kazuko
authors.initials: K
authors.lastname: Kudo
authors.forename: Seiji
authors.initials: S
authors.lastname: Kojima
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006086:Graft vs Host Disease / Q000209:etiology; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D011014:Pneumonia / Q000453:epidemiology / Q000209:etiology* / Q000401:mortality; D012189:Retrospective Studies; D012307:Risk Factors