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Essential Fatty Acid Deficiency in Cystic Fibrosis Disease Progression: Role of Genotype and Sex

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Summary: Nutrition plays a crucial role in cystic fibrosis (CF) due to its association with increased energy consumption, specific nutritional deficiencies, and malabsorption related to pancreatic insufficiency. The deterioration of lung function in CF patients has been linked to nutrition. However, despite the general recommendation of high energy intake, clinical deterioration remains difficult to control, suggesting the need for identifying and specifying special nutritional needs. Lipid abnormalities, particularly in essential or conditionally essential fatty acids, are commonly observed in CF patients. This article focuses on the qualitative aspects of fat metabolism, which has been neglected in dietary fat recommendations that only emphasize fat quantity. The deficiency of linoleic acid, an essential fatty acid important for membrane structure and function, has been recognized for over 60 years in CF patients. The ratio between arachidonic acid and docosahexaenoic acid, conditionally essential fatty acids in the n-6 and n-3 series respectively, is often increased. Recent discoveries regarding the connection between CFTR modulators and lipid metabolism have generated new interest in this field, and advancements in technology provide opportunities for more personalized therapy.

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