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

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

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  • 1Division of Gastroenterology, Hepatology, and Nutrition, The Children's Hospital of Philadelphia, 3535 Market St, Philadelphia, PA 19104, USA. <>


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.

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