Nutrition in Cystic Fibrosis-Some Notes on the Fat Recommendations

Birgitta Strandvik, Birgitta Strandvik

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 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.

Keywords: CFTR modulators; DHA; arachidonic acid; energy; linoleic acid; lipid mediator.

Conflict of interest statement

The author declares no conflict of interest.

Figures

Figure 1
Figure 1
Major pathways of polyunsaturated fatty acid synthesis. Simplified major transformation steps to long-chain polyunsaturated fatty acids from the essential fatty acids, linoleic (LA) and α-linolenic (ALA) acids and the endogenously synthesized oleic acid (OA), which all compete for the same desaturases (FADS1 and FADS2) and elongases. The major metabolites are indicated in boxes, proinflammatory and ellipses (anti-inflammatory) lipid mediators. ARA, arachidonic acid; EPA, eicosapentaenoic acid; DHA, docosahexaenoic acid; LT, leukotrienes; TX, tromboxanes.

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