Evidence-Based Practice Recommendations for Nutrition-Related Management of Children and Adults with Cystic Fibrosis and Pancreatic Insufficiency: Results of a Systematic Review

doi:10.1016/j.jada.2008.02.020

Journal of the American Dietetic Association

Volume 108, Issue 5, May 2008, Pages 832-839

https://doi.org/10.1016/j.jada.2008.02.020 Get rights and content

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.

Section snippets

Methods

Investigators at Johns Hopkins University conducted a systematic review in spring 2005 to assist the Subcommittee in making recommendations. English-language articles published from January 1988 to February 2005 reporting studies addressing the questions were identified for review. Searches were conducted in PubMed, EMBASE, the Cochrane Central Register of Controlled Trials, PASCAL, Allied and Complementary Medicine, and Agricultural On-line Access. Bibliographies of eligible articles and

New Analysis for Growth and Weight Monitoring in CF Care

The Working Group commissioned new analyses from the CF Foundation Patient Registry, reviewed the related literature, and prepared a report with recommendations for monitoring weight in adults and for monitoring growth in children. The 2005 CF Foundation Patient Registry included data from approximately 22,700 patients from 117 CF centers and 48 affiliated programs in the United States, and represents about 75% of the patient population. Each center and affiliate has institutional review board

Conclusions

These guidelines offer evidence-based recommendations for the increased energy intake required to support growth and development for children and weight maintenance in adults with CF and PI, and for improving weight and growth patterns when indicated. Finally, Registry data–based recommendations for the use of BMI for adults and BMI percentile for children for monitoring growth and weight status are provided. Desirable growth patterns in children and weight status in adults were defined based

V. A. Stallings is director, Nutrition Center, and director, Office of Faculty Affairs, Division of Gastroenterology, Hepatology, and Nutrition, The Children's Hospital of Philadelphia, Philadelphia, PA.

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EPI should be suspected in patients with high-risk clinical conditions, such as chronic pancreatitis, relapsing acute pancreatitis, pancreatic ductal adenocarcinoma, cystic fibrosis, and previous pancreatic surgery.
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L. J. Stark is professor of pediatrics and director, Division of Behavioral Medicine and Clinical Psychology, Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH.

K. A. Robinson is co-director, Johns Hopkins Evidence-based Practice Center, and research associate, Division of Internal Medicine, Johns Hopkins University School of Medicine, Baltimore, MD.

A. P. Feranchak is assistant professor pediatrics, Pediatric Gastroenterology, Hepatology, and Nutrition, University of Texas Southwestern Medical Center at Dallas.

H. Quinton is with the Dartmouth Hitchcock Medical Center, Lebanon, NH.

View full text

ResearchPerspectives in PracticeEvidence-Based Practice Recommendations for Nutrition-Related Management of Children and Adults with Cystic Fibrosis and Pancreatic Insufficiency: Results of a Systematic Review

Abstract

Section snippets

Methods

New Analysis for Growth and Weight Monitoring in CF Care

Conclusions

Chest

J Pediatr

Nutrition

Am J Clin Nutr

Am J Clin Nutr

Behav Ther

J Am Diet Assoc

J Pediatr

Chest

Clin Nutr

Chest

J Pediatr

Chest

Chest

J Cyst Fibros

J Am Diet Assoc

Am J Clin Nutr

Cystic Fibrosis Foundation Patient Registry: 2005 Annual Data Report to the Center Directors

Excess deaths associated with underweight, overweight, and obesity

JAMA

Exocrine pancreatic function

Cystic fibrosis

Consensus report on nutrition for pediatric patients with cystic fibrosis

J Pediatr Gastroenterol Nutr

The Guide to Clinical Preventive Services 2005

Energy supplements rich in linoleic acid improve body weight and essential fatty acid status of cystic fibrosis patients

J Pediatr Gastroenterol Nutr

Relation between dietary intake and nutritional status in cystic fibrosis

Arch Dis Child

A behavioral approach to increasing calorie consumption in children with cystic fibrosis

J Pediatr Psychol

Increasing calorie consumption in children with cystic-fibrosis—Replication with 2-year follow-up

J Appl Beh Analysis

Fat intake is low in cystic fibrosis despite unrestricted dietary practices

JPEN J Parenter Enteral Nutr

The management of enzymatic therapy in cystic fibrosis patients by an individualized approach

J Pediatr Gastroenterol Nutr

Efficacy of a nonrestricted fat diet in patients with cystic fibrosis

Am J Dis Child

Nutritional follow-up of cystic fibrosis patients: The role of nutrition education

J Pediatr Rio J

Dietary intakes of young children with cystic fibrosis: Is there a difference?

J Pediatr Gastroenterol Nutr

Supplemental nasogastric feeding in cystic fibrosis patients during treatment for acute exacerbation of chest disease

Aust Paediatr J

A randomized pilot study of behavioral treatment to increase calorie intake in toddlers with cystic fibrosis

Child Health Care

Oral nutritional supplements promote significant weight gain in cystic fibrosis patients

J Hum Nutr Diet.

Research
Perspectives in Practice
Evidence-Based Practice Recommendations for Nutrition-Related Management of Children and Adults with Cystic Fibrosis and Pancreatic Insufficiency: Results of a Systematic Review