title: Pneumothorax in patients with severe combined immunodeficiency.
abstract: BACKGROUND
  Most infants with pneumothorax have underlying conditions. Pneumocystis jirovecii pneumonia (PCP) frequently occurs in patients with severe combined immunodeficiency (SCID). The aim of this study was to determine clinical features of PCP-associated pneumothorax in SCID patients.
  
  
  METHODS
  The medical records of four SCID patients with pneumothorax were retrospectively reviewed.
  
  
  RESULTS
  All four patients were diagnosed as having SCID at the time of contracting PCP. All patients received mechanical ventilation because of severe respiratory failure. Only one patient was successfully extubated and was alive following hematopoietic stem cell transplantation (HSCT); of the remaining patients, however, two died of respiratory failure, and one patient died of early HSCT-related complications.
  
  
  CONCLUSIONS
  Pneumothorax associated with PCP can occur in SCID patients, and they may have a poor prognosis. If pneumothorax occurs in infants, both respiratory management and prompt investigation of the underlying conditions are needed, considering the possibility of PCP associated with SCID.
authors.affiliation: Department of Pediatrics, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Toyama, Japan.
authors.forename: Akihiro
authors.initials: A
authors.lastname: Hoshino
authors.forename: Kohsuke
authors.initials: K
authors.lastname: Imai
authors.forename: Yusei
authors.initials: Y
authors.lastname: Ohshima
authors.forename: Motoko
authors.initials: M
authors.lastname: Yasutomi
authors.forename: Masashi
authors.initials: M
authors.lastname: Kasai
authors.forename: Masaru
authors.initials: M
authors.lastname: Terai
authors.forename: Keiko
authors.initials: K
authors.lastname: Ishigaki
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Morio
authors.forename: Toshio
authors.initials: T
authors.lastname: Miyawaki
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Kanegane
mesh_terms: D006801:Humans; D007223:Infant; D008297:Male; D045363:Pneumocystis carinii; D011020:Pneumonia, Pneumocystis / Q000150:complications*; D011030:Pneumothorax / Q000150:complications*; D012189:Retrospective Studies; D016511:Severe Combined Immunodeficiency / Q000150:complications*
keywords: Pneumocystis jirovecii; pneumothorax; severe combined immunodeficiency