Brazilian Guidelines for Nutrition in Cystic Fibrosis

Abstract

Objective: To develop a scientific consensus on nutrition in cystic fibrosis. Methods: Sixteen coordinators elaborated relevant questions on nutritional therapy in cystic fibrosis, which were divided into six sections: nutritional assessment, nutritional recommendations, nutritional intervention, dietary counseling, special situations and enzyme replacement, and gastrointestinal manifestations. Two to three specialists in the field were responsible for each section and obtaining answers formulated based on standardized bibliographic searches. The available literature was searched in the PubMed ® /MEDLINE database, after training and standardization of search strategies, to write the best level of evidence for the questions elaborated. Issues related to disagreement were discussed until a consensus was reached among specialists, based on the current scientific literature. Results: Forty-two questions were prepared and objectively answered, resulting in a consensus of nutritional therapy in cystic fibrosis. Conclusion: This work enabled establishing a scientific consensus for nutritional treatment of cystic fibrosis patients.

Effect of Consumption of Extravirgin Olive Oil on the Lipid Profile and on the Glycemia of Children with Cystic Fibrosis Seen at a Specialized Center in Belém-PA/ Brazil

“…The quality of fat consumed in the diet of people with CF may be important in terms of blood glucose, according to the findings of this research, in addition to the other studies cited that report the benefits of consumption of extra virgin olive oil in people without CF, but regarding glycemic changes It is also important to be careful when choosing carbohydrates, as foods with a low glycemic load can be beneficial for glycemic control also in HR 27,28 , and combined with extra virgin olive oil, it could further potentiate its effects on glucose control.…”

Nutritional counseling in childhood and adolescence: a systematic review

“…The efficacy of NC was measured by increasing BMI or weight of patients. Currently, NC has been already linked to an improvement in quality of life and wellbeing in pediatric patients with cystic fibrosis ( 49 – 52 ).…”

Evolution of nutritional management in children with cystic fibrosis – a narrative review

“…The supplementation with FSV became intrinsic to practice with recommendations provided by all international guidelines. [4][5][6][7][8] Although clinical deficiencies in vitamins A and E were rare with this approach, a new situation has emerged, with increasing reports of hypervitaminosis for those on CFTRm. [105][106][107][108] With a mounting focus on diet quality, challenges are emerging for nutrition professionals, including the reeducation of pwCF who were previously advised on the 'traditional legacy' diet.…”

“…It is well documented and established that optimising nutritional status improves lung function, 1 survival 2 and quality of life 3 . Consequently, international CF organisations across four continents developed nutritional guidelines, aiming to optimise patient nutritional status as part of their long‐term management goals 4–8 . Evidence now suggests that the implementation of these guidelines, as early as possible in patient care, has been instrumental in improving patient nutritional status over time (reflected as body mass index [BMI] changes), 9–11 and thus survival 12 .…”

“…The inherent fat malabsorption in PI pwCF contributed not just to poor weight and growth but deficiencies of fat‐soluble vitamins (FSV) which were historical hallmarks of CF. The supplementation with FSV became intrinsic to practice with recommendations provided by all international guidelines 4–8 . Although clinical deficiencies in vitamins A and E were rare with this approach, a new situation has emerged, with increasing reports of hypervitaminosis for those on CFTRm 105–108 …”