title: Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review.
abstract: 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.
authors.affiliation: Division of Gastroenterology, Hepatology, and Nutrition, The Children's Hospital of Philadelphia, 3535 Market St, Philadelphia, PA 19104, USA. Stallingsv@email.chop.edu <Stallingsv@email.chop.edu>
authors.forename: Virginia A
authors.initials: VA
authors.lastname: Stallings
authors.forename: Lori J
authors.initials: LJ
authors.lastname: Stark
authors.forename: Karen A
authors.initials: KA
authors.lastname: Robinson
authors.forename: Andrew P
authors.initials: AP
authors.lastname: Feranchak
authors.forename: Hebe
authors.initials: H
authors.lastname: Quinton
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002664:Child Nutritional Physiological Phenomena; D003550:Cystic Fibrosis / Q000178:diet therapy* / Q000201:enzymology; D002149:Energy Intake; D057487:Enzyme Therapy; D004798:Enzymes / Q000008:administration & dosage; D019317:Evidence-Based Medicine; D010188:Exocrine Pancreatic Insufficiency / Q000178:diet therapy* / Q000201:enzymology; D005260:Female; D006128:Growth; D006801:Humans; D008297:Male; D009747:Nutritional Physiological Phenomena / Q000502:physiology*; D009752:Nutritional Status; D010179:Pancreas / Q000201:enzymology; D017410:Practice Guidelines as Topic; D015430:Weight Gain
chemical_list: D004798:Enzymes