Nutrition in Cystic Fibrosis—Some Notes on the Fat Recommendations
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 nutri...
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Format: | Article |
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MDPI AG
2022-02-01
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Series: | Nutrients |
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Online Access: | https://www.mdpi.com/2072-6643/14/4/853 |
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author | Birgitta Strandvik |
author_facet | Birgitta Strandvik |
author_sort | Birgitta Strandvik |
collection | DOAJ |
description | 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. |
first_indexed | 2024-03-09T21:18:15Z |
format | Article |
id | doaj.art-d0f598d32b1b4b6ebc9d46b35def21c1 |
institution | Directory Open Access Journal |
issn | 2072-6643 |
language | English |
last_indexed | 2024-03-09T21:18:15Z |
publishDate | 2022-02-01 |
publisher | MDPI AG |
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series | Nutrients |
spelling | doaj.art-d0f598d32b1b4b6ebc9d46b35def21c12023-11-23T21:29:22ZengMDPI AGNutrients2072-66432022-02-0114485310.3390/nu14040853Nutrition in Cystic Fibrosis—Some Notes on the Fat RecommendationsBirgitta Strandvik0Department of Biosciences and Nutrition, Karolinska Institutet, Huddinge, 14183 Stockholm, SwedenNutrition 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.https://www.mdpi.com/2072-6643/14/4/853energyarachidonic acidDHAlinoleic acidlipid mediatorCFTR modulators |
spellingShingle | Birgitta Strandvik Nutrition in Cystic Fibrosis—Some Notes on the Fat Recommendations Nutrients energy arachidonic acid DHA linoleic acid lipid mediator CFTR modulators |
title | Nutrition in Cystic Fibrosis—Some Notes on the Fat Recommendations |
title_full | Nutrition in Cystic Fibrosis—Some Notes on the Fat Recommendations |
title_fullStr | Nutrition in Cystic Fibrosis—Some Notes on the Fat Recommendations |
title_full_unstemmed | Nutrition in Cystic Fibrosis—Some Notes on the Fat Recommendations |
title_short | Nutrition in Cystic Fibrosis—Some Notes on the Fat Recommendations |
title_sort | nutrition in cystic fibrosis some notes on the fat recommendations |
topic | energy arachidonic acid DHA linoleic acid lipid mediator CFTR modulators |
url | https://www.mdpi.com/2072-6643/14/4/853 |
work_keys_str_mv | AT birgittastrandvik nutritionincysticfibrosissomenotesonthefatrecommendations |