title: Hyperpolarized129Xe for investigation of mild cystic fibrosis lung disease in pediatric patients.
abstract: BACKGROUND
  Cystic fibrosis (CF) is a genetic disease which carries high morbidity and mortality from lung-function decline. Monitoring disease progression and treatment response in young patients is desirable, but serial imaging via CT is often considered prohibitive, and detailed functional information cannot be obtained using conventional imaging techniques. Hyperpolarized129Xe magnetic resonance imaging (MRI) can depict and quantify regional ventilation, but has not been investigated in pediatrics. We hypothesized that129Xe MRI is feasible and would demonstrate ventilation defects in mild CF lung disease with greater sensitivity than FEV1.
  
  
  METHODS
  11 healthy controls (age 6-16years) and 11 patients with mild CF (age 8-16years, Forced Expiratory Volume (FEV1) percent predicted >70%) were recruited for this study. Nine CF patients had an FEV1>85%. Each subject was imaged via hyperpolarized129Xe MRI, and the ventilation defect percentage (VDP) was measured. FEV1and VDP were compared between the groups.
  
  
  RESULTS
  FEV1for controls was 100.3%±8.5% (mean±sd) and for CF patients was 97.9%±16.0% (p=0.67). VDP was 6.4%±2.8% for controls and 18.3%±8.6% for CF (p<0.001). When considering the 9 CF patients with normal FEV1(>85%), the mean FEV1was 103.1%±12.3% (p=0.57 compared to controls) and VDP was 15.4%±6.3% (p=0.002).
  
  
  CONCLUSIONS
  Hyperpolarized129Xe MRI demonstrated ventilation defects in CF patients with normal FEV1and more effectively discriminated CF from controls than FEV1. Thus129Xe may be a useful outcome measure to detect mild CF lung disease, to investigate regional lung function in pediatric lung diseases, and to follow disease progression.
authors.affiliation: Center for Pulmonary Imaging Research, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States; Department of Physics, Washington University in St. Louis, St. Louis, MO, United States.
authors.forename: Robert P
authors.initials: RP
authors.lastname: Thomen
authors.affiliation: Center for Pulmonary Imaging Research, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.
authors.forename: Laura L
authors.initials: LL
authors.lastname: Walkup
authors.affiliation: Center for Pulmonary Imaging Research, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.
authors.forename: David J
authors.initials: DJ
authors.lastname: Roach
authors.affiliation: Center for Pulmonary Imaging Research, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States; Division of Pulmonary Medicine, Cincinnati Children's Hospital, Cincinnati, OH, United States.
authors.forename: Zackary I
authors.initials: ZI
authors.lastname: Cleveland
authors.affiliation: Division of Pulmonary Medicine, Cincinnati Children's Hospital, Cincinnati, OH, United States.
authors.forename: John P
authors.initials: JP
authors.lastname: Clancy
authors.affiliation: Center for Pulmonary Imaging Research, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States; Department of Physics, Washington University in St. Louis, St. Louis, MO, United States; Division of Pulmonary Medicine, Cincinnati Children's Hospital, Cincinnati, OH, United States. Electronic address: Jason.woods@cchmc.org.
authors.forename: Jason C
authors.initials: JC
authors.lastname: Woods
mesh_terms: D000293:Adolescent; D002648:Child; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000503:physiopathology; D018450:Disease Progression; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D011237:Predictive Value of Tests; D012123:Pulmonary Ventilation; D012129:Respiratory Function Tests / Q000379:methods; D012680:Sensitivity and Specificity; D014979:Xenon Isotopes / Q000494:pharmacology*
chemical_list: D014979:Xenon Isotopes; C000614971:Xenon-129
keywords: Cystic fibrosis; Hyperpolarized; Mri; Pediatric