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.
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References
- Cystic Fibrosis Foundation Patient Registry: 2005 Annual Data Report to the Center Directors.Cystic Fibrosis Foundation, Bethesda, MD2006
- Excess deaths associated with underweight, overweight, and obesity.JAMA. 2005; 293: 1861-1867
- Exocrine pancreatic function.in: Walker W.A. Goulet O. Kleinman R.E. Sherman P.M. Schneider B.L. Sanderson I.R. Pediatric Gastrointestinal Disease. B.C. Decker, Hamilton, ON, Canada2004: 1607-1623
- Cystic fibrosis.in: Rudolph C.D. Rudolph A.M. Hostetter M.K. Lister G. Siegel N.J. Rudolph's Pediatrics. McGraw Hill, New York, NY2002: 1969-1980
- Consensus report on nutrition for pediatric patients with cystic fibrosis.J Pediatr Gastroenterol Nutr. 2002; 35: 246-259
- Cystic fibrosis adult care: Consensus conference report.Chest. 2004; 125: 1S-39S
- Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy.J Pediatr. 1995; 127: 681-684
- The Guide to Clinical Preventive Services 2005.US Department of Health and Human Services, Agency for Health Care Research and Quality, Rockville, MD2007 (Publication No. 05-0570, 1-192)
- Energy supplements rich in linoleic acid improve body weight and essential fatty acid status of cystic fibrosis patients.J Pediatr Gastroenterol Nutr. 2000; 31: 418-423
- Relation between dietary intake and nutritional status in cystic fibrosis.Arch Dis Child. 1998; 78: 443-447
- Nutritional status of an adult cystic fibrosis population.Nutrition. 2000; 16: 255-259
- A behavioral approach to increasing calorie consumption in children with cystic fibrosis.J Pediatr Psychol. 1990; 15: 309-326
- Increasing calorie consumption in children with cystic-fibrosis—Replication with 2-year follow-up.J Appl Beh Analysis. 1993; 26: 435-450
- Fat intake is low in cystic fibrosis despite unrestricted dietary practices.JPEN J Parenter Enteral Nutr. 1989; 13: 296-298
- The management of enzymatic therapy in cystic fibrosis patients by an individualized approach.J Pediatr Gastroenterol Nutr. 1988; 7: S36-S39
- Efficacy of a nonrestricted fat diet in patients with cystic fibrosis.Am J Dis Child. 1989; 143: 458-464
- 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; 48: 235-239
- 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; 57: 580-587
- Nutritional follow-up of cystic fibrosis patients: The role of nutrition education.J Pediatr Rio J. 2004; 80: 475-482
- Dietary intakes of young children with cystic fibrosis: Is there a difference?.J Pediatr Gastroenterol Nutr. 1996; 22: 254-258
- Supplemental nasogastric feeding in cystic fibrosis patients during treatment for acute exacerbation of chest disease.Aust Paediatr J. 1989; 25: 164-167
- A randomized pilot study of behavioral treatment to increase calorie intake in toddlers with cystic fibrosis.Child Health Care. 2003; 32: 297-311
- Oral nutritional supplements promote significant weight gain in cystic fibrosis patients.J Hum Nutr Diet. 1998; 11: 95-104
- Contribution of behavior therapy to dietary treatment in cystic fibrosis: A randomized controlled study with 2-year follow-up.Behav Ther. 2003; 34: 237-258
- Energy and protein intakes of patients with cystic fibrosis.J Hum Nutr Diet. 1992; 5: 333-342
- Nutritional rehabilitation increases resting energy expenditure without affecting protein turnover in patients with cystic fibrosis.J Pediatr Gastroenterol Nutr. 1991; 13: 383-390
- Percutaneous endoscopic gastrostomy in cystic fibrosis: Patient acceptance and effect of overnight tube feeding on nutritional status.Acta Gastroenterol Belg. 2004; 67: 241-244
- Five-year prospective analysis of dietary intake and clinical status in malnourished cystic fibrosis patients.J Hum Nutr Diet. 2003; 16: 225-231
- Predictive 5-year survivorship model of cystic fibrosis.Am J Epidemiol. 2001; 153: 345-352
- Stature as a prognostic factor in cystic fibrosis survival.J Am Diet Assoc. 2001; 101: 438-442
- Gender gap in cystic fibrosis mortality.Am J Epidemiol. 1997; 145: 794-803
- Longitudinal changes in growth parameters are correlated with changes in pulmonary function in children with cystic fibrosis.Pediatrics. 2003; 112: 588-592
- Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis.J Pediatr. 2003; 142: 624-630
- Normal growth in cystic fibrosis associated with a specialised centre.Arch Dis Child. 1999; 81: 241-246
- Relationship between nutritional status and lung function in cystic fibrosis: Cross sectional and longitudinal analyses from the German CF quality assurance (CFQA) project.Thorax. 2002; 57: 596-601
- Hidden depletion of fat-free mass and bone mineral density in adults with cystic fibrosis.Chest. 2003; 124: 2220-2228
- Nutritional growth-retardation is associated with defective lung growth in cystic-fibrosis: A preventable determinant of progressive pulmonary dysfunction.Nutrition. 1995; 11: 350-354
- Long-term survival and nutritional data in patients with cystic fibrosis treated in a Danish centre.Thorax. 1996; 51: 1023-1027
- Nutrition in adults with cystic fibrosis.Clin Nutr. 1998; 17: 211-215
- Pulmonary function correlates in the prediction of long-term weight gain in cystic fibrosis patients with gastrostomy tube feedings.J Pediatr Gastroenterol Nutr. 1998; 27: 53-56
- Behavioral intervention to improve calorie intake of children with cystic fibrosis: Treatment vs wait list control.J Pediatr Gastroenterol Nutr. 1996; 22: 240-253
- Wasting as an independent predictor of mortality in patients with cystic fibrosis.Thorax. 2001; 56: 746-750
- Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis.Pediatr Pulmonol. 2002; 34: 91-100
- Factors associated with poor pulmonary function: Cross-sectional analysis of data from the ERCF.Eur Respir J. 2001; 18: 298-305
- Risk of death in cystic fibrosis patients with severely compromised lung function.Chest. 1998; 113: 1230-1234
- Prognostic factors in cystic fibrosis in a single center in Brazil: A survival analysis.Pediatr Pulmonol. 2002; 34: 3-10
- Factors affecting clinical outcome in gastrostomy-fed children with cystic fibrosis.Pediatr Pulmonol. 2004; 37: 324-329
- Factors influencing the course of cystic fibrosis in Czech patients: Experience of the Prague Clinic.Paediatr Paedol. 1995; 30: 19-24
- A nocturnal nasogastric feeding programme in cystic fibrosis adults.J Hum Nutr Diet. 1994; 7: 257-262
- Percutaneous endoscopic gastrostomy feeding in patients with cystic fibrosis.Gut. 1999; 44: 87-90
- Improvement of nutritional status and lung function after long-term nocturnal gastrostomy feedings in cystic fibrosis.J Pediatr. 1994; 124: 244-249
- Morbidity and mortality data of cystic fibrosis patients.Saudi Med J. 2003; 24: 730-735
- Impact of recent pulmonary exacerbations on quality of life in patients with cystic fibrosis.Chest. 2002; 121: 64-72
- Longitudinal determinants of peak aerobic performance in children with cystic fibrosis.Chest. 2003; 124: 2215-2219
- Improved outcomes of patients with cystic fibrosis admitted to the intensive care unit.J Cyst Fibros. 2004; 3: 8-14
- Comparison of weight-based dosages of enteric-coated microtablet enzyme preparations in patients with cystic-fibrosis.J Pediatr Gastroenterol Nutr. 1994; 19: 191-197
- Effectiveness of enteric coated pancreatic-enzymes given before meals in reducing steatorrhea in children with cystic-fibrosis.J Am Diet Assoc. 1992; 92: 813-817
- Comparison of a high lipase pancreatic-enzyme extract with a regular pancreatin preparation in adult cystic-fibrosis patients.Aliment Pharmacol Ther. 1994; 8: 603-607
- Comparison of two pancreatic enzyme supplements in patients with cystic fibrosis.Ann Allergy Asthma Immunol. 1990; 7: 109-118
- High dose pancreatic enzymes in cystic fibrosis.Arch Dis Child. 1990; 65: 311-312
- Comparative in vitro and in vivo studies of enteric-coated pancrelipase preparations for pancreatic insufficiency.J Pediatr Gastroenterol Nutr. 1993; 17: 407-413
- Two enteric coated microspheres in cystic fibrosis.Arch Dis Child. 1990; 65: 594-597
- Reducing pancreatic enzyme dose does not compromise growth in cystic fibrosis.J Hum Nutr Diet. 1998; 11: 487-492
- Maintenance of growth in cystic fibrosis despite reduction in pancreatic enzyme supplementation.Arch Dis Child. 1998; 78: 377-378
- Pancreatic enzyme supplementation in cystic fibrosis patients before and after fibrosing colonopathy.J Pediatr Gastroenterol Nutr. 1998; 26: 80-84
- CDC growth charts: United States.Adv Data. 2000; 314: 1-27
- Clinical guidelines on the identification, evaluation, and treatment of overweight and obesity in adults: Executive summary.Am J Clin Nutr. 1998; 68: 899-917
- Pulmonary function growth velocity in children 6 to 18 years of age.Am Rev Respir Dis. 1993; 148: 1502-1508
- Spirometric reference values from a sample of the general US population.Am J Respir Crit Care Med. 1999; 159: 179-187
- 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. 2004; 80: 982-991
Biography
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.
Biography
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.
Biography
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.
Biography
A. P. Feranchak is assistant professor pediatrics, Pediatric Gastroenterology, Hepatology, and Nutrition, University of Texas Southwestern Medical Center at Dallas.
Biography
H. Quinton is with the Dartmouth Hitchcock Medical Center, Lebanon, NH.
Article info
Publication history
Accepted:
September 20,
2007
Identification
Copyright
© 2008 American Dietetic Association. Published by Elsevier Inc. All rights reserved.
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- Dietetic Management of People with Cystic FibrosisJournal of the American Dietetic AssociationVol. 108Issue 12
- PreviewWe read with interest the report by Stallings and colleagues on the recommendations of the US Cystic Fibrosis Foundation's Subcommittee on Growth and Nutrition (1). This guidance will assist dietetic management of people with cystic fibrosis, particularly with regard to identifying those at nutritional risk.
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