BACKGROUND: In @entity1577 ( @entity1577 ) the defective @entity1577 transmembrane conductance regulator protein may be responsible for the impaired transport of @entity2041 ( @entity2197 ), 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 @entity2197 in @entity1 with @entity1577 . METHODS: 54 adult and 51 pediatric @entity1 were randomized to receive inhaled @entity2197 or placebo twice daily for 12months. RESULTS: Twelve month treatment with inhaled @entity2197 did not achieve our predetermined primary outcome measure of 15% improvement in FEV1%. Only in @entity1 with moderate @entity358 , 3, 6 and 9months therapy with @entity2197 resulted in a statistically significant increase of FEV1 values from the baseline. Moreover @entity2197 therapy improved 6-minute walking test in pediatric population. @entity2197 was well tolerated by all @entity1 . CONCLUSIONS: Inhaled @entity2197 has slight positive effects in @entity1577 @entity1 with moderate @entity358 warranting further study. TRIAL REGISTRY: ClinicalTrials.gov; No.: NCT01450267; URL: www.clinicaltrialsgov.

Randomized, single blind, controlled trial of inhaled @entity2041 vs placebo in XXXX with @entity1577 .

@entity1 :: ('9606', 'Species') :: ['patients'] @entity2041 :: ('MESH:D005978', 'Chemical') :: ['glutathione'] @entity2197 :: ('CHEBI:16856', 'Chemical') :: ['GSH'] @entity358 :: ('MESH:D008171', 'Disease') :: ['lung disease'] @entity1577 :: ('MESH:D003550', 'Disease') :: ['CF', 'cystic fibrosis']
@entity1 :: (9606,Species) :: ['patients']
