Nutrition and Cystic Fibrosis in Children
A special issue of Nutrients (ISSN 2072-6643). This special issue belongs to the section "Pediatric Nutrition".
Deadline for manuscript submissions: 5 November 2025 | Viewed by 2614
Special Issue Editor
Special Issue Information
Dear Colleagues,
Cystic Fibrosis (CF) is an autosomal dominant genetic disorder that impacts approximately 105,000 persons in 94 countries throughout the world. The primary F508 mutation in the cystic fibrosis transmembrane receptor (CFTR) gene was discovered in 1989 and is the dominant allele in 2/3 persons with CF. The classic presentation of CF remains that of malnutrition, lung disease, and pancreatic insufficiency. Malnutrition was one of the early targets of therapy and focused on a high-fat, high-calorie diet based on a pivotal study in the 1970s comparing the nutritional management practices of two centers in Toronto and Boston. Since then, many treatments have been developed to decrease morbidity and mortality in CF including CFTR modulators which aid in folding and stabilizing the CFTR protein. New highly effective modulator therapy (HEMT) has shown a significant improvement in weight gain due to an improvement in whole-body inflammation, decreased resting energy expenditure, and improvements in malabsorption. The impact of HEMT along with the recognition of more than 2000 mutations with variable clinical presentations is creating new challenges for clinicians. This Special Issue aims to collect manuscripts focusing on the change in the traditional approach to nutritional therapy, unveiling the importance for personalized medicine in CF.
Dr. Meghana Nitin Sathe
Guest Editor
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Keywords
- cystic fibrosis
- cystic fibrosis transmembrane regulator modulators
- elexacaftor/tezacaftor/ivacaftor
- ivacaftor
- nutritional therapy
- highly effective modulator therapy
- pancreatic insufficiency
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