title: A pilot study of the effect of inhaled buffered reduced glutathione on the clinical status of patients with cystic fibrosis.
abstract: OBJECTIVE
  To assess the impact of inhaled, buffered reduced glutathione (GSH) on clinical indicators of cystic fibrosis (CF) pathophysiology.
  
  
  METHODS
  A randomized, double-blind, placebo-controlled pilot study was conducted over an 8-week period. Nineteen subjects, age 6 to 19 years, with CF status documented by positive sweat chloride test results (> 60 mEq/L) were recruited for the trial. After matching on age and sex, 10 patients were randomly assigned to the treatment group and 9 patients to the placebo group. Primary outcomes were FEV1, FVC, forced expiratory flow at 25 to 75% of vital capacity, and peak flow; secondary outcomes were body mass index, 6-min walk distance, and self-reported cough frequency, mucus production/viscosity/color, wellness, improvement, and stamina. INTERVENTIONS AND ANALYSIS: Treatment was buffered GSH, and placebo was sodium chloride with a hint of quinine. The total daily dose of buffered GSH was approximately 66 mg/kg of body weight, and the total daily dose of placebo was approximately 15 mg/kg of body weight (quinine, 25 to 30 microg/kg). Doses were distributed across four inhalation sessions per day and spaced 3- to 4-h apart. General linear mixed models were used to analyze the data. The final sample size was nine subjects in the treatment group and seven subjects in the placebo group.
  
  
  RESULTS
  Mean change for peak flow was -6.5 L/min for the placebo group and +33.7 L/min for the GSH group (p = 0.04), and self-reported average improvement on a scale from 1 to 5 (1 being much worse and 5 being much better) was 2.8 for placebo and 4.7 for GSH (p = 0.004). Of the 13 primary and secondary outcomes examined, 11 outcomes favored the treatment group over the placebo group (p = 0.002), indicating a general tendency of improvement in the GSH group. No adverse events in the treatment group were noted.
  
  
  CONCLUSIONS
  This pilot study indicates the promise of nebulized buffered GSH to ameliorate CF disease, and longer, larger, and improved studies of inhaled GSH are warranted.
authors.affiliation: Utah Valley Regional Medical Center, Provo, UT, USA. UVCBISHO@ihc.com
authors.forename: Clark
authors.initials: C
authors.lastname: Bishop
authors.forename: Valerie M
authors.initials: VM
authors.lastname: Hudson
authors.forename: Sterling C
authors.initials: SC
authors.lastname: Hilton
authors.forename: Cathleen
authors.initials: C
authors.lastname: Wilde
mesh_terms: D000280:Administration, Inhalation; D000293:Adolescent; D000328:Adult; D002021:Buffers; D002648:Child; D003550:Cystic Fibrosis / Q000188:drug therapy* / Q000503:physiopathology; D004311:Double-Blind Method; D004848:Epithelium / Q000502:physiology; D005978:Glutathione / Q000008:administration & dosage / Q000627:therapeutic use*; D019803:Glutathione Disulfide / Q000378:metabolism; D006801:Humans; D008297:Male; D010865:Pilot Projects; D011446:Prospective Studies
chemical_list: D002021:Buffers; D005978:Glutathione; D019803:Glutathione Disulfide