부산대학교 도서관

Cystic fibrosis (CF) is a genetic disease caused by mutations in the gene encoding for the ion channel cystic fibrosis transmembrane conductance regulator (CFTR). The management of CF disease has evolved in recent decades from treating downstream disease manifestations affecting the airways, the lungs and the gastrointestinal system to addressing the CFTR gene defect. The advent of CFTR modulators, which correct the functionality of the defective CFTR, contributes to reshaping the landscape of CF demographics, prognosis and therapies, including nutritional management. A spectrum of clinical manifestations is emerging within the same patient population where undernutrition and nutritional deficiencies coexist with excessive weight gain and metabolic derangements. Such contrasting presentations challenge current practices, require adjustments to traditional approaches, and involve more individualised interventions. This narrative review examines the current state of knowledge on the nutritional management of people living with cystic fibrosis from early life to adulthood in the era of CFTR modulation. Highlights: Advances in research and care, including cystic fibrosis transmembrane conductance regulator (CFTR) modulators, have reshaped cystic fibrosis demographics, prognosis and management, including nutrition.A broad spectrum of nutritional manifestations, from underweight and nutritional deficiencies to overweight, metabolic derangements and disturbed eating behaviours, is now emerging.Because nutrition goals and concerns vary by age, genotype, medication use, access to resources, physical, mental and nutritional health, and individual preferences, interventions should be individualised and based on regular and proactive assessment. [ABSTRACT FROM AUTHOR]