Article ; Online: Nutritional management of people living with cystic fibrosis throughout life and disease continuum: Changing times, new challenges.
Journal of human nutrition and dietetics : the official journal of the British Dietetic Association
2023 Volume 36, Issue 5, Page(s) 1675–1691
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 ...
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. |
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MeSH term(s) | Humans ; Cystic Fibrosis/complications ; Cystic Fibrosis/genetics ; Cystic Fibrosis/therapy ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; Cystic Fibrosis Transmembrane Conductance Regulator/therapeutic use ; Prognosis ; Lung ; Precision Medicine |
Chemical Substances | Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6) |
Language | English |
Publishing date | 2023-07-28 |
Publishing country | England |
Document type | Journal Article ; Review |
ZDB-ID | 645183-4 |
ISSN | 1365-277X ; 0952-3871 ; 1465-8178 |
ISSN (online) | 1365-277X |
ISSN | 0952-3871 ; 1465-8178 |
DOI | 10.1111/jhn.13214 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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