Nutritional Status and Circulating Levels of Fat-Soluble Vitamins in Cystic Fibrosis Patients: A Cohort Study and Evaluation of the Effect of CFTR Modulators
Michela Francalanci,
Vito Terlizzi,
Cristina Fevola,
Giulia Di Rosa,
Valentina Pierattini,
Elena Roselli,
Paolo Bonomi,
Maria Chiara Cavicchi,
Valeria Galici,
Anna Silvia Neri,
Chiara Bianchimani,
Silvia Campana,
Daniela Dolce,
Novella Ravenni,
Erica Camera,
Tommaso Orioli,
Giovanni Taccetti
Affiliations
Michela Francalanci
Meyer Children’s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy
Vito Terlizzi
Meyer Children’s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy
Cristina Fevola
Meyer Children’s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy
Giulia Di Rosa
Meyer Children’s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy
Valentina Pierattini
Meyer Children’s Hospital IRCCS, Professional Dietetics, 50139 Florence, Italy
Elena Roselli
Meyer Children’s Hospital IRCCS, Professional Dietetics, 50139 Florence, Italy
Paolo Bonomi
Freelance Statistician, 20146 Milan, Italy
Maria Chiara Cavicchi
Meyer Children’s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy
Valeria Galici
Meyer Children’s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy
Anna Silvia Neri
Meyer Children’s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy
Chiara Bianchimani
Meyer Children’s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy
Silvia Campana
Meyer Children’s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy
Daniela Dolce
Meyer Children’s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy
Novella Ravenni
Meyer Children’s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy
Erica Camera
Meyer Children’s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy
Tommaso Orioli
Meyer Children’s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy
Giovanni Taccetti
Meyer Children’s Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy
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 20); 3 (2.7%) M and 5 (7.0%) F had a BMI 2 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.
