Glutamine supplementation in cystic fibrosis: A randomized placebo-controlled trial.

RATIONALE: Pulmonary infection and malnutrition in cystic fibrosis are associated with decreased survival. Glutamine has a possible anti-microbial effect, with a specific impact against Pseudomonas aeruginosa. We aimed to test the hypothesis that oral glutamine supplementation (21 g/day) for 8 weeks in adults with cystic fibrosis would decrease pulmonary inflammation and improve clinical status. METHODS: The study design was a randomized double-blind placebo-controlled study design with an iso-nitrogenous placebo. The primary analysis was intention to treat, and the primary outcome was change in induced sputum neutrophils. RESULTS: Thirty-nine individuals were recruited and thirty-six completed the study. Glutamine supplementation had no impact on any of the outcome measures in the intention-to-treat analysis. In the per protocol analysis, glutamine supplementation was associated with an increase in induced sputum neutrophils (P = 0.046), total cells (P = 0.03), and in Pseudomonas isolation agar colony forming units (P = 0.04) compared to placebo. CONCLUSIONS: There was no effect of glutamine supplementation on markers of pulmonary inflammation in the intention-to-treat analysis.

Are measures of body habitus associated with mortality in cystic fibrosis?

BACKGROUND: Nutrition is an important component of clinical care for patients with cystic fibrosis. We aimed to test the hypothesis that increased BMI, height, and level of creatinine as a biomarker for lean muscle mass are associated with lower mortality and whether differences in these measures may contribute toward sex differences in survival in cystic fibrosis. METHODS: Using a cohort study design, we analyzed data from the UK Cystic Fibrosis Registry for patients who attended an annual assessment visit in 2007 and were followed-up until July 2009. RESULTS: Of 1,517 individuals, 62 died during the follow-up period. The odds of death were higher among patients in the lowest quintile of serum creatinine compared with the rest of the study population (OR, 3.28; 95% CI, 1.79-5.98). Increased height and higher BMI were also associated with lower risk of death. The higher mortality in female patients (OR, 1.48; 95% CI, 0.93-2.34) was reversed by adjustment using the absolute values for height, BMI, and serum creatinine level (adjusted OR, 0.44; 95% CI, 0.21-0.90) but not by the use of sex-specific values for these exposure variables. CONCLUSIONS: Lower muscle mass, shorter stature, and a low BMI are associated with increased mortality in cystic fibrosis. These measures of body habitus may contribute to the sex-specific survival differences in individuals with cystic fibrosis.