Abstract
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.
Highlights
Two cohorts of clinically stable cystic fibrosis (CF) pancreatic-insufficient patients were compared before and after the implementation of the new recommendations on vitamin D supplementation: Cohort 1: years 2012–2013, collected retrospectively from a previous cross-sectional study [5]; Cohort 2: patients prospectively recruited in the period 2014–2016 for an experimental study
The implementation of new guidelines on Vitamin D supplementation has led to a progressive increase of the vitamin D dose that CF patients receive daily, improving the nutritional status of vitamin D in these patients
Our results suggested that initial dose is often inadequate for achieving target vitamin D serum levels
Summary
Between 40% and 90% of these patients have serum 25 OH vitamin D levels below 30 ng/mL [1], while approximately. CF patients often start with Vitamin D supplementation when they present exocrine pancreatic insufficiency, which can be developed over the years, but it normally appears at birth or during the first months of life. For this reason, supplementation usually starts from diagnosis, including those who were diagnosticated by neonatal screening (positive immunoreactive trypsin confirmed by abnormal sweat chloride concentration or identification of a CF disease-causing mutation in each copy of the cystic fibrosis transmembrane conductance regulator (CFTR) gene)
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