Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review

J Am Diet Assoc. 2008 May;108(5):832-9. doi: 10.1016/j.jada.2008.02.020.


The Cystic Fibrosis Foundation established a process of systematic review of evidence to inform the development of clinical care guidelines and encourage evidence-based practice. The Subcommittee on Growth and Nutrition reviewed the evidence in two areas: energy intake and dosing for pancreatic enzyme replacement therapy. Evidence-based recommendations are presented here. Also, an ad hoc working group conducted a review of the literature and performed new analyses using the Cystic Fibrosis Foundation Patient Registry to update the recommendations for growth and weight-status monitoring. These Registry data-based recommendations are presented.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review
  • Systematic Review

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child Nutritional Physiological Phenomena
  • Cystic Fibrosis / diet therapy*
  • Cystic Fibrosis / enzymology
  • Energy Intake
  • Enzyme Therapy*
  • Enzymes / administration & dosage
  • Evidence-Based Medicine
  • Exocrine Pancreatic Insufficiency / diet therapy*
  • Exocrine Pancreatic Insufficiency / enzymology
  • Female
  • Growth
  • Humans
  • Male
  • Nutritional Physiological Phenomena / physiology*
  • Nutritional Status*
  • Pancreas / enzymology
  • Practice Guidelines as Topic
  • Weight Gain


  • Enzymes