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- Title
Nutritional Status and Circulating Levels of Fat-Soluble Vitamins in Cystic Fibrosis Patients: A Cohort Study and Evaluation of the Effect of CFTR Modulators.
- Authors
Francalanci, Michela; Terlizzi, Vito; Fevola, Cristina; Di Rosa, Giulia; Pierattini, Valentina; Roselli, Elena; Bonomi, Paolo; Cavicchi, Maria Chiara; Galici, Valeria; Neri, Anna Silvia; Bianchimani, Chiara; Campana, Silvia; Dolce, Daniela; Ravenni, Novella; Camera, Erica; Orioli, Tommaso; Taccetti, Giovanni
- Abstract
Background: Improved therapy in CF has led to an overall improvement in nutritional status. The objectives of our study are: to cross-sectionally assess nutritional status and serum levels of fat-soluble vitamins; to retrospectively evaluate the efficacy of modulators on nutritional status and fat-soluble vitamin levels. Methods: In patients younger than 2 years of age, we evaluated growth, in patients aged 2–18 years, we assessed BMI z-scores, and in adults, we assessed absolute BMI values. Levels of 25(OH)D, vitamins A, and E were measured. Results: A cross-sectional analysis was conducted on 318 patients, 109 (34.3%) with pancreatic sufficiency. Only three patients were under 2 years old. In 135 patients aged 2–18 years, the median BMI z-score was 0.11, and 5 (3.7%) patients had malnutrition (z-score ≤ 2SD). In 180 adults, the median BMI was 21.8 kg/m2. Overall, 15 (13.7%) males (M) and 18 (25.3%) females (F) were underweight (18 < BMI > 20); 3 (2.7%) M and 5 (7.0%) F had a BMI < 18. Suboptimal 25(OH)D levels were found in patients with pancreatic insufficiency. The prevalence of deficiency of vitamins A and E is low. After one year of treatment with modulators, the increase in BMI was more consistent (M: 1.58 ± 1.25 kg/m2 F: 1.77 ± 1.21 kg/m2) in elexacaftor/tezacaftor/ivacaftor (ETI)-treated patients compared with other modulators, with a significant increase in levels of all fat-soluble vitamins. Conclusions: Malnutrition is present in a limited number of subjects. The prevalence of subjects with suboptimal 25(OH)D levels is high. ETI showed a beneficial effect on nutritional status and circulating levels of fat-soluble vitamins.
- Subjects
DRUG efficacy; NUTRITIONAL assessment; COMBINATION drug therapy; VITAMIN E; ANTI-inflammatory agents; CROSS-sectional method; FIBROSIS; RETROSPECTIVE studies; VITAMIN D; CYSTIC fibrosis; LEANNESS; VITAMIN A; DESCRIPTIVE statistics; MALNUTRITION; BODY mass index; MEMBRANE proteins; NUTRITIONAL status; LONGITUDINAL method; PHARMACODYNAMICS; THERAPEUTICS; EVALUATION
- Publication
Children, 2023, Vol 10, Issue 2, p252
- ISSN
2227-9067
- Publication type
Article
- DOI
10.3390/children10020252