Nutritional management of people living with cystic fibrosis throughout life and disease continuum: Changing times, new challenges.
J Hum Nutr Diet
; 36(5): 1675-1691, 2023 10.
Article
en En
| MEDLINE
| ID: mdl-37515397
ABSTRACT
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.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Fibrosis Quística
Tipo de estudio:
Prognostic_studies
Límite:
Humans
Idioma:
En
Revista:
J Hum Nutr Diet
Asunto de la revista:
CIENCIAS DA NUTRICAO
Año:
2023
Tipo del documento:
Article
País de afiliación:
Canadá