title: Randomized, single blind, controlled trial of inhaled glutathione vs placebo in patients with cystic fibrosis.
abstract: BACKGROUND
  In cystic fibrosis (CF) the defective CF transmembrane conductance regulator protein may be responsible for the impaired transport of glutathione (GSH), the first line defense of the lung against oxidative stress. The aim of this single-blind, randomized, placebo-controlled trial was to evaluate the effect of inhaled GSH in patients with CF.
  
  
  METHODS
  54 adult and 51 pediatric patients were randomized to receive inhaled GSH or placebo twice daily for 12 months.
  
  
  RESULTS
  Twelve month treatment with inhaled GSH did not achieve our predetermined primary outcome measure of 15% improvement in FEV1%. Only in patients with moderate lung disease, 3, 6 and 9 months therapy with GSH resulted in a statistically significant increase of FEV1 values from the baseline. Moreover GSH therapy improved 6-minute walking test in pediatric population. GSH was well tolerated by all patients.
  
  
  CONCLUSIONS
  Inhaled GSH has slight positive effects in CF patients with moderate lung disease warranting further study.
  
  
  BACKGROUND
  ClinicalTrials.gov; No.: NCT01450267; URL: www.clinicaltrialsgov.
authors.affiliation: Department of Cardiothoracic and Respiratory Sciences, Second University of Naples, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Calabrese
authors.affiliation: Department of Translational Medical Sciences, Cystic Fibrosis Center, University "Federico II" of Naples, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Tosco
authors.affiliation: Department of Translational Medical Sciences, Cystic Fibrosis Center, University "Federico II" of Naples, Italy.
authors.forename: P
authors.initials: P
authors.lastname: Abete
authors.affiliation: Department of Translational Medical Sciences, Cystic Fibrosis Center, University "Federico II" of Naples, Italy.
authors.forename: V
authors.initials: V
authors.lastname: Carnovale
authors.affiliation: Department of Translational Medical Sciences, Cystic Fibrosis Center, University "Federico II" of Naples, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Basile
authors.affiliation: Department of Translational Medical Sciences, Cystic Fibrosis Center, University "Federico II" of Naples, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Magliocca
authors.affiliation: Department of Pediatrics, Cystic Fibrosis Center, Sapienza University of Rome, Italy.
authors.forename: S
authors.initials: S
authors.lastname: Quattrucci
authors.affiliation: Department of Pediatrics, Cystic Fibrosis Center, Sapienza University of Rome, Italy.
authors.forename: S
authors.initials: S
authors.lastname: De Sanctis
authors.affiliation: Department of Pediatrics, Cystic Fibrosis Center, Sapienza University of Rome, Italy.
authors.forename: F
authors.initials: F
authors.lastname: Alatri
authors.affiliation: Department of Cardiothoracic and Respiratory Sciences, Second University of Naples, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Mazzarella
authors.affiliation: Department of Cardiothoracic and Respiratory Sciences, Second University of Naples, Italy.
authors.forename: L
authors.initials: L
authors.lastname: De Pietro
authors.affiliation: Department of Cardiothoracic and Respiratory Sciences, Second University of Naples, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Turino
authors.affiliation: Pediatric Pneumology, Santobono-Pausilipon-Annunziata Children's Hospital, Naples, Italy.
authors.forename: E
authors.initials: E
authors.lastname: Melillo
authors.affiliation: Department of Translational Medical Sciences, Cystic Fibrosis Center, University "Federico II" of Naples, Italy.
authors.forename: P
authors.initials: P
authors.lastname: Buonpensiero
authors.affiliation: Department of Translational Medical Sciences, Cystic Fibrosis Center, University "Federico II" of Naples, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Di Pasqua
authors.affiliation: Department of Translational Medical Sciences, Cystic Fibrosis Center, University "Federico II" of Naples, Italy. Electronic address: raia@unina.it.
authors.forename: V
authors.initials: V
authors.lastname: Raia
mesh_terms: D000280:Administration, Inhalation; D000293:Adolescent; D000328:Adult; D000975:Antioxidants / Q000008:administration & dosage / Q000493:pharmacokinetics; D001692:Biological Transport / Q000187:drug effects*; D002648:Child; D003550:Cystic Fibrosis / Q000188:drug therapy / Q000378:metabolism; D016903:Drug Monitoring / Q000379:methods; D005080:Exercise Test / Q000187:drug effects; D005260:Female; D005541:Forced Expiratory Volume / Q000187:drug effects; D005978:Glutathione / Q000008:administration & dosage / Q000493:pharmacokinetics; D006801:Humans; D008168:Lung / Q000187:drug effects / Q000378:metabolism; D008297:Male; D018384:Oxidative Stress / Q000187:drug effects; D012720:Severity of Illness Index; D016037:Single-Blind Method; D016896:Treatment Outcome
chemical_list: D000975:Antioxidants; D005978:Glutathione
keywords: Cystic fibrosis; Glutathione; Therapy