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Coburn-Miller C.,Children's Hospital of Buffalo | Casey S.,Seattle Childrens Hospital | Luong Q.,Therapeutics Development Network Coordinating Center | Cameron N.,Therapeutics Development Network Coordinating Center | And 8 more authors.
Clinical and Translational Science | Year: 2015

Malnutrition is one of the earliest clinical manifestations of cystic fibrosis (CF) and is associated with poorer pulmonary and cognitive outcomes and survival later in life. Infant growth can be a responsive measure for clinical research in this age group if obtained and characterized accurately. We report here the methods to standardize and implement research-quality anthropometric measurement of infants with cystic fibrosis in the Baby Observational Nutrition Study multicenter trial. © 2015 Wiley Periodicals, Inc.


PubMed | Seattle Childrens Hospital, Children's Hospital of Buffalo, Baylor College of Medicine, University of Rochester and 2 more.
Type: Journal Article | Journal: Clinical and translational science | Year: 2015

Malnutrition is one of the earliest clinical manifestations of cystic fibrosis (CF) and is associated with poorer pulmonary and cognitive outcomes and survival later in life. Infant growth can be a responsive measure for clinical research in this age group if obtained and characterized accurately. We report here the methods to standardize and implement research-quality anthropometric measurement of infants with cystic fibrosis in the Baby Observational Nutrition Study multicenter trial.


Conrad C.,Stanford University | Lymp J.,Therapeutics Development Network Coordinating Center | Thompson V.,Therapeutics Development Network Coordinating Center | Dunn C.,Stanford University | And 15 more authors.
Journal of Cystic Fibrosis | Year: 2015

Purpose: To evaluate the effects of oral N-acetylcysteine (NAC), which replenishes systemic glutathione, on decreasing inflammation and improving lung function in CF airways. Methods: A multicenter, randomized, double-blind proof of concept study in which 70 CF subjects received NAC or placebo orally thrice daily for 24weeks. Endpoints: primary, change in sputum human neutrophil elastase (HNE) activity; secondary, FEV1 and other clinical lung function measures; and safety, the safety and tolerability of NAC and the potential of NAC to promote pulmonary hypertension in subjects with CF. Results: Lung function (FEV1 and FEF25-75%) remained stable or increased slightly in the NAC group but decreased in the placebo group (p=0.02 and 0.02). Log10 HNE activity remained equal between cohorts (difference 0.21, 95% CI -0.07 to 0.48, p=0.14). Conclusions: NAC recipients maintained their lung function while placebo recipients declined (24week FEV1 treatment effect=150mL, p<0.02). However no effect on HNE activity and other selected biomarkers of neutrophilic inflammation were detected. Further studies on mechanism and clinical outcomes are warranted. © 2014.

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