The nutritional status in CF: Being certain about the uncertainties

Abstract

Background: Nutritional therapy is one of the cornerstones in cystic fibrosis (CF) therapy. There is a strong association between nutritional status and pulmonary function and thus longevity. Therefore nutritional therapy should be continuously adapted to preserve or improve the nutritional status. This narrative review was written to reconsider nutritional therapy in CF based on the latest evidence available since the publication of the ESPEN - ESPGHAN - ECFS guidelines on nutrition care for infants, children and adults with CF.

Methods: A literature search in Pubmed, Scopus and Web of Science was conducted to identify new research focusing on the use of growth charts, body composition, protein intake and pancreatic enzyme therapy (PERT) in CF between June 2014 and June 2017.

Results: The search strategy resulted in a total of 1810 hits across the databases. After reviewing title and abstract only 17 studies were included of which 2 animal studies. The use of growth charts was discussed in 3 studies, body composition in 6, protein intake and digestion in 4 and PERT in 4.

Conclusion: According to the current guidelines and the available evidence of the discussed topics, it is important that the nutritional therapy in CF is redefined according to age, pancreatic function and disease stage. Macronutrients balances are of importance and change over lifetime. As a consequence an accurate PERT intake is required and thus further research on timing and dosage is necessary. To improve the nutritional assessment a proper use of the growth charts and a consensus on body composition measurements, references and thresholds is advised.

Keywords: BMI; Body composition; Cystic fibrosis; Nutritional status.