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The nutritional status in CF: Being certain about the uncertainties
Dimitri Declercq1, Svenya Van Meerhaeghe1, Sophie Marchand2
1Center for Cystic Fibrosis, Department of Pediatrics, Ghent University Hospital, C. Heymanslaan 10, Belgium.
Clinical Nutrition ESPEN
|January 22, 2019
Summary
Optimizing nutritional therapy for cystic fibrosis (CF) is crucial for lung function and longevity. This review emphasizes adapting nutrition, including pancreatic enzyme replacement therapy (PERT), based on individual patient needs and recent evidence.
Area of Science:
- Medical Nutrition Therapy
- Cystic Fibrosis Research
- Pulmonary Medicine
Background:
- Nutritional therapy is fundamental in managing cystic fibrosis (CF).
- Maintaining optimal nutritional status is strongly linked to pulmonary function and patient longevity.
- Continuous adaptation of nutritional strategies is essential for preserving or improving health outcomes in CF patients.
Purpose of the Study:
- To review and update nutritional therapy recommendations for CF.
- To incorporate the latest evidence published since the ESPEN-ESPGHAN-ECFS guidelines.
- To address key areas including growth monitoring, body composition, protein intake, and pancreatic enzyme therapy.
Main Methods:
- Conducted a literature search in PubMed, Scopus, and Web of Science (June 2014 - June 2017).
- Focused on research concerning growth charts, body composition, protein intake, and pancreatic enzyme replacement therapy (PERT) in CF.
- Included 17 studies after initial screening of 1810 hits.
Main Results:
- Growth charts were discussed in 3 studies, body composition in 6.
- Protein intake and digestion were addressed in 4 studies, and PERT in 4 studies.
- Identified a need for updated evidence on these nutritional aspects.
Conclusions:
- Nutritional therapy in CF requires redefinition based on age, pancreatic function, and disease stage.
- Macronutrient balance is critical and varies throughout life, necessitating precise PERT.
- Improved nutritional assessment requires proper use of growth charts and consensus on body composition metrics.
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