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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.