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Nutrition in Cystic Fibrosis

A special issue of Nutrients (ISSN 2072-6643). This special issue belongs to the section "Nutrition and Public Health".

Deadline for manuscript submissions: closed (15 October 2021) | Viewed by 15460

Special Issue Editor

Department Pathophysiol & Transplantat, University of Milan, Cystic Fibrosis Reference Center, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Milan, 20122 Italy
Interests: cystic fibrosis; nutrition; liver disease

Special Issue Information

Dear Colleagues,

Cystic fibrosis (CF) is a multisystemic heterogeneous disease affecting the lungs, pancreas, liver, intestine, sweat glands, and reproductive tract. Lung disease is the main cause of morbidity and mortality. Several studies have indicated that maintaining a good nutritional status is critical to the long-term survival of CF patients. Getting the right nutrition remains the cornerstone of therapy for patients, since it is associated with better pulmonary function and survival. However, some patients still fail to achieve normal growth and nutritional status, and several aspects of the nutritional intervention remain poorly defined. It has become clear that CF patients require a personalized nutritional care and that accurate evaluation of energy balance and body composition should become a consistent part of the nutritional evaluation. The role of the dietitians within the CF multidisciplinary team has become increasingly important.There is an urgent need for evidence-based recommendations on the nutrient composition of the diet, in consideration of the increasing prevalence of fat-free mass depletion as well as overweight, diabetes, and the potential effects of different fatty acids on inflammation and immune response. In this issue of Nutrients dedicated to Nutrition in CF, the more controversial issues related to the “CF diet” will be examined, with particular regard to optimal macronutrient intake and essential fatty acid supplementation. A few clinical complications, such as sarcopenia and osteoporosis, are increasingly seen in CF patients and are at least in part related to malnutrition. The nutritional effects of CFTR modulators are the focus of increasing interest and will be reviewed with particular attention to physioetiological mechanisms.

Dr. Carla Colombo
Guest Editor

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Keywords

  • Cystic fibrosis
  • Nutritional status
  • Body composition
  • Sarcopenia
  • Essential fatty acid

Published Papers (5 papers)

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Research

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12 pages, 877 KiB  
Article
Change in Nutrient and Dietary Intake in European Children with Cystic Fibrosis after a 6-Month Intervention with a Self-Management mHealth Tool
by Joaquim Calvo-Lerma, Mieke Boon, Jessie Hulst, Carla Colombo, Inês Asseiceira, María Garriga, Etna Masip, Ine Claes, Anna Bulfamante, Hettie M. Janssens, Maria Roca, Saioa Vicente, Victoria Fornés, Laura Zazzeron, Bo van Schijndel, Sandra Woodcock, Luisa Pereira, Kris de Boeck and Carmen Ribes-Koninckx
Nutrients 2021, 13(6), 1801; https://doi.org/10.3390/nu13061801 - 26 May 2021
Cited by 7 | Viewed by 3598
Abstract
Cystic Fibrosis (CF) is a life-long genetic disease, causing increased energy needs and a healthy diet with a specific nutrient distribution. Nutritional status is an indicator of disease prognosis and survival. This study aimed at assessing the effectiveness of a self-management mobile app [...] Read more.
Cystic Fibrosis (CF) is a life-long genetic disease, causing increased energy needs and a healthy diet with a specific nutrient distribution. Nutritional status is an indicator of disease prognosis and survival. This study aimed at assessing the effectiveness of a self-management mobile app in supporting patients with CF to achieve the dietary goals set by the CF nutrition guidelines. A clinical trial was conducted in pancreatic insufficient children with CF, followed in six European CF centres, where the self-management app developed within the MyCyFAPP project was used for six months. To assess secondary outcomes, three-day food records were compiled in the app at baseline and after 3 and 6 months of use. Eighty-four subjects (mean 7.8 years old) were enrolled. Compared to baseline, macronutrient distribution better approximated the guidelines, with protein and lipid increasing by 1.0 and 2.1% of the total energy intake, respectively, by the end of the study. Consequently, carbohydrate intake of the total energy intake decreased significantly (−2.9%), along with simple carbohydrate intake (−2.4%). Regarding food groups, a decrease in ultra-processed foods was documented, with a concomitant increase in meat and dairy. The use of a self-management mobile app to self-monitor dietary intake could become a useful tool to achieve adherence to guideline recommendations, if validated during a longer period of time or against a control group. Full article
(This article belongs to the Special Issue Nutrition in Cystic Fibrosis)
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13 pages, 441 KiB  
Article
DeltaF508 CFTR Hetero- and Homozygous Paediatric Patients with Cystic Fibrosis Do Not Differ with Regard to Nutritional Status
by Aleksandra Mędza, Katarzyna Kaźmierska, Bartosz Wielgomas, Lucyna Konieczna, Ilona Olędzka, Agnieszka Szlagatys-Sidorkiewicz and Katarzyna Sznurkowska
Nutrients 2021, 13(5), 1402; https://doi.org/10.3390/nu13051402 - 21 Apr 2021
Cited by 3 | Viewed by 1867
Abstract
The purpose of this study was to compare the nutritional status between deltaF508 CFTR hetero- and homozygous paediatric patients with cystic fibrosis. We assessed the percentage profiles of fatty acids measured in erythrocyte membranes and the serum levels of vitamins A, D3, E [...] Read more.
The purpose of this study was to compare the nutritional status between deltaF508 CFTR hetero- and homozygous paediatric patients with cystic fibrosis. We assessed the percentage profiles of fatty acids measured in erythrocyte membranes and the serum levels of vitamins A, D3, E and K1 in the studied groups. We also measured the weights and heights and calculated the body mass indexes (BMIs). The studied groups consisted of 34 heterozygous and 30 homozygous patients. No statistically significant differences were found in the serum vitamins or erythrocyte membrane fatty acid profiles between the hetero- and homozygous patient groups, except for heptadecanoic acid (p = 0.038). The mean percentiles of height, weight and BMI did not differ significantly between the two groups. The homozygous and heterozygous paediatric patients with cystic fibrosis were similar in terms of their nutritional statuses. Full article
(This article belongs to the Special Issue Nutrition in Cystic Fibrosis)
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Review

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10 pages, 639 KiB  
Review
Breastfeeding in Cystic Fibrosis: A Systematic Review on Prevalence and Potential Benefits
by Carla Colombo, Gianfranco Alicandro, Valeria Daccò, Alessandra Consales, Fabio Mosca, Carlo Agostoni and Maria Lorella Giannì
Nutrients 2021, 13(9), 3263; https://doi.org/10.3390/nu13093263 - 18 Sep 2021
Cited by 2 | Viewed by 2218
Abstract
Breastfeeding (BF) is considered the normative standard of feeding for all infants. However, the impact of BF in patients with cystic fibrosis (CF) is not completely defined. Therefore, we conducted a systematic review to evaluate BF prevalence in the CF population and its [...] Read more.
Breastfeeding (BF) is considered the normative standard of feeding for all infants. However, the impact of BF in patients with cystic fibrosis (CF) is not completely defined. Therefore, we conducted a systematic review to evaluate BF prevalence in the CF population and its impact on anthropometric and pulmonary outcomes. We searched MEDLINE, Embase and the Cochrane Library for original articles published in English up to 4 December 2020 that report the prevalence of BF and/or any measure of association between BF and anthropometric or pulmonary outcomes. Nine observational studies were identified (six retrospective cohort studies, one prospective cohort study, one survey and one case–control study within a retrospective cohort). The BF rate in CF patients is lower than that of the healthy population (approximately 50–60% of infants were breastfed at any time). The benefits in anthropometric outcomes of BF for >2 months in this at-risk population are unclear. A few relatively small studies suggest a potential benefit of BF in reducing lung infections, although data are inconsistent. The currently available data are insufficient to draw definite conclusions on the benefits of exclusive BF in anthropometric and pulmonary outcomes in CF. Clinical trials evaluating well-defined BF promotion interventions are needed. Full article
(This article belongs to the Special Issue Nutrition in Cystic Fibrosis)
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11 pages, 276 KiB  
Review
The Impact of Highly Effective CFTR Modulators on Growth and Nutrition Status
by Rosara Bass, Jefferson N. Brownell and Virginia A. Stallings
Nutrients 2021, 13(9), 2907; https://doi.org/10.3390/nu13092907 - 24 Aug 2021
Cited by 27 | Viewed by 3628
Abstract
Patients with cystic fibrosis (CF) are at increased risk of malnutrition and growth failure due to multiple factors as a result of suboptimal or absent function of the CFTR chloride channel protein. Dysfunctional CFTR contributes to increased energy expenditure, exocrine pancreatic insufficiency causing [...] Read more.
Patients with cystic fibrosis (CF) are at increased risk of malnutrition and growth failure due to multiple factors as a result of suboptimal or absent function of the CFTR chloride channel protein. Dysfunctional CFTR contributes to increased energy expenditure, exocrine pancreatic insufficiency causing impaired dietary macronutrient digestion and absorption, intestinal dysbiosis, and impaired bile acid homeostasis. Poor nutritional status as a result of these mechanisms is associated with decreased lung function, worse clinical outcomes, and ultimately, increased mortality. Nutritional interventions addressing these mechanisms, such as pancreatic enzyme-replacement therapy and enteral caloric supplementation, have improved nutritional status and, by association, clinical outcomes. In the last decade, the advent of medications targeting defective CFTR proteins has revolutionized the care of patients with CF by reducing the overall impact of CFTR dysfunction. Below, we summarize the effects of highly effective CFTR modulators on nutritional status overall as well as specific factors including bile acid metabolism, pancreatic function, energy expenditure, and intestinal dysbiosis. The future of CF nutrition care will require a paradigm shift away from focusing on methods addressing CFTR dysfunction such as excess calorie provision and toward an individualized, holistic approach in the context of specific mutations and CFTR-directed therapy. Full article
(This article belongs to the Special Issue Nutrition in Cystic Fibrosis)

Other

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11 pages, 636 KiB  
Commentary
Nutrition in Cystic Fibrosis—Some Notes on the Fat Recommendations
by Birgitta Strandvik
Nutrients 2022, 14(4), 853; https://doi.org/10.3390/nu14040853 - 18 Feb 2022
Cited by 8 | Viewed by 3381
Abstract
Nutrition is important in cystic fibrosis (CF) because the disease is associated with a higher energy consumption, special nutritional deficiencies, and malabsorption mainly related to pancreatic insufficiency. The clinical course with deterioration of lung function has been shown to relate to nutrition. Despite [...] Read more.
Nutrition is important in cystic fibrosis (CF) because the disease is associated with a higher energy consumption, special nutritional deficiencies, and malabsorption mainly related to pancreatic insufficiency. The clinical course with deterioration of lung function has been shown to relate to nutrition. Despite general recommendation of high energy intake, the clinical deterioration is difficult to restrain suggesting that special needs have not been identified and specified. It is well-known that the CF phenotype is associated with lipid abnormalities, especially in the essential or conditionally essential fatty acids. This review will concentrate on the qualitative aspects of fat metabolism, which has mainly been neglected in dietary fat recommendations focusing on fat quantity. For more than 60 years it has been known and confirmed that the patients have a deficiency of linoleic acid, an n-6 essential fatty acid of importance for membrane structure and function. The ratio between arachidonic acid and docosahexaenoic acid, conditionally essential fatty acids of the n-6 and n-3 series, respectively, is often increased. The recently discovered relations between the CFTR modulators and lipid metabolism raise new interests in this field and together with new technology provide possibilities to specify further specify personalized therapy. Full article
(This article belongs to the Special Issue Nutrition in Cystic Fibrosis)
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