Nutritional Status in the First 2 Years of Life in Cystic Fibrosis Diagnosed by Newborn Screening.

OBJECTIVE: To evaluate nutritional status and associated factors in a cystic fibrosis (CF) cohort diagnosed by newborn screening and followed up to month 24. METHODS: A prospective longitudinal multicenter study assessing nutritional status according to pancreatic status, feeding modalities, prescriptions, pulmonary outcome, and biological nutritional parameters. RESULTS: One hundred and five infants were recruited and 99 completed the study. Nutritional care management prevented undernutrition and stunting in those with exocrine pancreatic sufficiency (EPS), but affected (13/87) 15% and (21/86) 24%, respectively, of infants with exocrine pancreatic insufficiency (EPI). The logistic regression model found a positive association between both weight and length z scores "at risk" at month 24, and initial pulmonary symptoms (odds ratio [OR] 0.06, P < 0.01 and OR 0.08, P < 0.01, respectively); these symptoms were less frequent when age at first visit was earlier than 1.2 months (33% vs 67%, P = 0.02); stunting was also associated with high-calorie density intake and Staphylococcus aureus (OR 0.05, P = 0.01 and OR 0.17, P < 0.01). Pulmonary outcome did not differ according to pancreatic status; breast-feeding for at least 3 months delayed first acquisition of Pseudomonas aeruginosa. Despite sodium and fat-soluble vitamin supplementation, half of both cohorts had low urinary sodium output and half of the EPI cohort had low vitamin D levels. CONCLUSIONS: Our data shed light on the fact that stunting was more frequent than undernutrition, while both parameters involved only patients with pancreatic insufficiency. Modalities of feeding were not associated with nutritional status; breast-feeding may provide some protection against acquisition of P aeruginosa.

Moderate intake of docosahexaenoic acid raises plasma and platelet vitamin E levels in cystic fibrosis patients.

Patients with cystic fibrosis have increased oxidative stress and impaired antioxidant systems. Moderate intake of docosahexaenoic acid (DHA) may favor the lowering of oxidative stress. In this randomized, double-blind, cross-over study, DHA or placebo capsules, were given daily to 10 patients, 5mg/kg for 2 weeks then 10mg/kg DHA for the next 2 weeks (or placebo). After 9 weeks of wash-out, patients took placebo or DHA capsules. Biomarkers of lipid peroxidation and vitamin E were measured at baseline, and after 2 and 4 weeks of treatment in each phase. The proportions of DHA increased both in plasma and platelet lipids after DHA supplementations. The lipid peroxidation markers did not significantly decrease, in spite of a trend, after the first and/or the second dose of DHA but plasma and platelet vitamin E amounts increased significantly after DHA supplementation. Our findings reinforce the antioxidant potential of moderate DHA intake in subjects displaying increased oxidative stress.

Exercise with incorporated expiratory manoeuvres was as effective as breathing techniques for airway clearance in children with cystic fibrosis: a randomised crossover trial.

QUESTION: : Can a session of exercise with incorporated expiratory manoeuvres substitute for a session of breathing techniques for airway clearance in children with cystic fibrosis? Are children with cystic fibrosis as co-operative and satisfied with the exercise regimen as with the breathing techniques?. DESIGN: Randomised, cross-over trial with concealed allocation and intention-to-treat analysis. PARTICIPANTS: 34 children with cystic fibrosis in a stable clinical state. INTERVENTIONS: Participants underwent two 20-min airway clearance interventions on two scheduled clinic days: one involving three bouts of various whole-body exercise modalities each followed by independent expiratory manoeuvres, and the other involving breathing control, thoracic expansions with manual expiratory compressions, and the forced expiratory technique. OUTCOME MEASURES: Wet weight of expectorated sputum, change in lung function, co-operation with treatment, perceived treatment quality, and satisfaction with treatment were all assessed after each intervention. RESULTS: The wet weight of sputum after exercise was 0.6g higher after the exercise intervention, which was not statistically or clinically significant (95% CI -0.2 to 1.4). However, lung function and participant satisfaction with the treatment were both significantly better after the exercise intervention. Co-operation with treatment and perceived treatment quality were equally high for each intervention. CONCLUSION: A session of various whole-body exercises interspersed with independent expiratory manoeuvres could be an acceptable substitute for a session of breathing control, thoracic expansions with manual expiratory compressions, and the forced expiratory technique in children with mild cystic fibrosis lung disease.