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
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.
References (70)
- et al.
Cystic fibrosis adult care: Consensus conference report
Chest
(2004) - et al.
Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy
J Pediatr
(1995) - et al.
Nutritional status of an adult cystic fibrosis population
Nutrition
(2000) - et al.
Short-term nutritional supplementation during management of pulmonary exacerbations in cystic fibrosis: A controlled study, including effects of protein turnover
Am J Clin Nutr
(1988) - et al.
Relationships among nutritional status and skeletal and respiratory muscle function in cystic fibrosis: Does early dietary supplementation make a difference?
Am J Clin Nutr
(1993) - et al.
Contribution of behavior therapy to dietary treatment in cystic fibrosis: A randomized controlled study with 2-year follow-up
Behav Ther
(2003) - et al.
Stature as a prognostic factor in cystic fibrosis survival
J Am Diet Assoc
(2001) - et al.
Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis
J Pediatr
(2003) - et al.
Hidden depletion of fat-free mass and bone mineral density in adults with cystic fibrosis
Chest
(2003) - et al.
Nutrition in adults with cystic fibrosis
Clin Nutr
(1998)
Risk of death in cystic fibrosis patients with severely compromised lung function
Chest
Improvement of nutritional status and lung function after long-term nocturnal gastrostomy feedings in cystic fibrosis
J Pediatr
Impact of recent pulmonary exacerbations on quality of life in patients with cystic fibrosis
Chest
Longitudinal determinants of peak aerobic performance in children with cystic fibrosis
Chest
Improved outcomes of patients with cystic fibrosis admitted to the intensive care unit
J Cyst Fibros
Effectiveness of enteric coated pancreatic-enzymes given before meals in reducing steatorrhea in children with cystic-fibrosis
J Am Diet Assoc
Comparison of the use of body mass index percentiles and percentage of ideal body weight to screen for malnutrition in children with cystic fibrosis
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.
Cited by (531)
ESPEN-ESPGHAN-ECFS guideline on nutrition care for cystic fibrosis
2024, Clinical NutritionRemote endpoints for clinical trials in cystic fibrosis: Report from the U.S. CF foundation remote endpoints task force
2024, Journal of Cystic FibrosisStandards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues
2024, Journal of Cystic Fibrosis
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.
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.