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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Main Author: Birgitta Strandvik
Format: Article
Language:English
Published: MDPI AG 2022-02-01
Series:Nutrients
Subjects:
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.
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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