Excess weight in patients with cystic fibrosis: is it always beneficial?

Introduction: In recent years, guidelines for vitamin D supplementation have been updated and prophylactic recommended doses have been increased in patients with cystic fibrosis (CF). Objective: To evaluate safety and efficacy of these new recommendations. Results: Two cohorts of pancreatic insufficient CF patients were compared before (cohort 1: 179 patients) and after (cohort 2: 71 patients) American CF Foundation and European CF Society recommendations were published. Cohort 2 patients received higher Vitamin D doses: 1509 (1306–1711 95% CI) vs 1084 (983–1184 95% CI) IU/Day (p < 0.001), had higher 25 OH vitamin D levels: 30.6 (27.9–33.26 95% CI) vs. 27.4 (25.9–28.8 95% CI) ng/mL (p = 0.028), and had a lower prevalence of insufficient vitamin D levels (<30 ng/mL): 48% vs 65% (p = 0.011). Adjusted by confounding factors, patients in cohort 1 had a higher risk of vitamin D insufficiency: OR 2.23 (1.09–4.57 95% CI) (p = 0.028). Conclusion: After the implementation of new guidelines, CF patients received higher doses of vitamin D and a risk of vitamin D insufficiency decreased. Despite this, almost a third of CF patients still do not reach sufficient serum calcidiol levels.

This is a prospective, multicenter study of a cohort of 224 cystic fibrosis (CF) patients treated with CF transmembrane conductance regulator (CFTR) modulators (CFTRm). Our aim was to prospectively analyze the effect of CFTRm treatment on fat‐soluble vitamin serum levels. Demographic and clinical data were recorded, and fat‐soluble vitamin levels were analyzed at baseline, and at 6 and 12 months after starting treatment. Two groups were analyzed separately: patients receiving dual therapy lumacaftor/ivacaftor or tezacaftor/ivacaftor (Lum/Tez+Iva), and those on triple therapy with elexacaftor/tezacaftor/ivacaftor (ETI). We found that treatment with ETI produced a significant increase in vitamin D and A levels within the first 6 months, which was maintained at 12 months. However, with dual therapy, we observed an increase only in vitamin A levels within the first 6 months, which was not maintained at 12 months. No differences were found in vitamin E serum levels between the groups.