Nutritional management of people living with cystic fibrosis throughout life and disease continuum: Changing times, new challenges

被引:3
作者
Mailhot, Genevieve [1 ,2 ,5 ]
Denis, Marie-Helene [3 ]
Beauchamp-Parent, Caroline [1 ]
Jomphe, Valerie [4 ]
机构
[1] Fac Med, Dept Nutr, Montreal, PQ, Canada
[2] CHU Sainte Justine, Res Ctr, Montreal, PQ, Canada
[3] CHU Sainte Justine, Cyst Fibrosis Clin, Montreal, PQ, Canada
[4] Ctr Hosp Univ Montreal CHUM, Lung Transplant Program, Montreal, PQ, Canada
[5] Univ Montreal, Fac Med, Dept Nutr, Montreal, PQ H3T 1A8, Canada
基金
加拿大自然科学与工程研究理事会;
关键词
CFTR modulators; cystic fibrosis; life stages; nutrition; CLINICAL-PRACTICE GUIDELINES; ABNORMAL GLUCOSE-TOLERANCE; LONG-TERM SAFETY; EATING-DISORDERS; LUNG-FUNCTION; OPEN-LABEL; SODIUM DEFICIT; MECONIUM ILEUS; YOUNG-ADULTS; WEIGHT-GAIN;
D O I
10.1111/jhn.13214
中图分类号
R15 [营养卫生、食品卫生]; TS201 [基础科学];
学科分类号
100403 ;
摘要
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.
引用
收藏
页码:1675 / 1691
页数:17
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