Consensus Report on Nutrition for Pediatric Patients With Cystic Fibrosis

Laura K. Bachrach, M.D. Robert J. Beall, Ph.D. Preston W. Campbell, III, M.D. Susan C. Casey, B.S., R.D. Mitchell B. Cohen, M.D. Mary Corey, Ph.D. W. Hobart Davies, Ph.D. Judy A. Fulton, R.D. Richard J. Grand, M.D. John E. Grunow, M.D. Dana S. Hardin, M.D. Lesles Hendeles, Pharm.D. James E. Heubi, M.D. Van S. Hubbard, M.D. Hui-Chuan Kai, Ph.D. Sheila Innis, Ph.D. Elisabeth Luder, Ph.D., R.D. Karen MacGuiness, R.D. Richard K. Mathis, M.D. Annie McKenna, M.S., R.D., C.N.S. Antoinette Moran, M.D. Laurie Moyer-Mileur, Ph.D., R.D. Kimberly O. O’Brien, Ph.D. Hebe Quinton, M.S. Lynne M. Quittell, M.D. Ross W. Shepherd, M.D., FRACP Ronald J. Sokol, M.D. Lori J. Stark, Ph.D. John N. Udall, Jr., M.D., Ph.D. Babette Zemel, Ph.D. INTRODUCTION

[1]  G. Barnes,et al.  Guidelines for evaluation and treatment of gastroesophageal reflux in infants and children: recommendations of the North American Society for Pediatric Gastroenterology and Nutrition. , 2002, Journal of pediatric gastroenterology and nutrition.

[2]  A. Moran Diagnosis, screening, and management of cystic fibrosis–related diabetes , 2002, Current diabetes reports.

[3]  J. Rice,et al.  Growth hormone improves clinical status in prepubertal children with cystic fibrosis: results of a randomized controlled trial. , 2001, The Journal of pediatrics.

[4]  J. Rice,et al.  Growth hormone decreases protein catabolism in children with cystic fibrosis. , 2001, The Journal of clinical endocrinology and metabolism.

[5]  M. Corey,et al.  Treatment of vitamin K deficiency in cystic fibrosis: Effectiveness of a daily fat-soluble vitamin combination. , 2001, The Journal of pediatrics.

[6]  Y. Ghoos,et al.  13 C Mixed Triglyceride Breath Test: A Noninvasive Method to Assess Lipase Activity in Children , 2001, Journal of pediatric gastroenterology and nutrition.

[7]  E. Russek-Cohen,et al.  Stature as a prognostic factor in cystic fibrosis survival. , 2001, Journal of the American Dietetic Association.

[8]  L. Bachrach Acquisition of optimal bone mass in childhood and adolescence , 2001, Trends in Endocrinology & Metabolism.

[9]  R. Baker,et al.  Guidelines for Evaluation and Treatment of Gastroesophageal Reflux in Infants and Children: Recommendations of the North American Society for Pediatric Gastroenterology and Nutrition , 2001, Journal of pediatric gastroenterology and nutrition.

[10]  M. Kosorok,et al.  Early diagnosis of cystic fibrosis through neonatal screening prevents severe malnutrition and improves long-term growth. Wisconsin Cystic Fibrosis Neonatal Screening Study Group. , 2001, Pediatrics.

[11]  B. Keevil,et al.  Assessment of iron status in cystic fibrosis patients , 2000, Annals of clinical biochemistry.

[12]  S. Baker,et al.  Randomized, Double-Blind, Placebo-Controlled Pilot Trial of Megestrol Acetate in Malnourished Children With Cystic Fibrosis , 2000, Journal of pediatric gastroenterology and nutrition.

[13]  N. Krebs,et al.  Abnormalities in Zinc Homeostasis in Young Infants with Cystic Fibrosis , 2000, Pediatric Research.

[14]  G. Chan,et al.  Bone mineral status in prepubertal children with cystic fibrosis. , 2000, The Journal of pediatrics.

[15]  M R Kosorok,et al.  Risk of persistent growth impairment after alternate-day prednisone treatment in children with cystic fibrosis. , 2000, The New England journal of medicine.

[16]  D. Borowitz Evidence for the diagnosis of pancreatic insufficiency , 2000, Pediatric pulmonology.

[17]  S. Powers,et al.  Parent and child mealtime behavior in families of children with cystic fibrosis. , 2000, The Journal of pediatrics.

[18]  M. Kosorok,et al.  Nutritional Status of Patients With Cystic Fibrosis With Meconium Ileus: A Comparison With Patients Without Meconium Ileus and Diagnosed Early Through Neonatal Screening , 2000, Pediatrics.

[19]  S. Freedman,et al.  A membrane lipid imbalance plays a role in the phenotypic expression of cystic fibrosis in cftr(-/-) mice. , 1999, Proceedings of the National Academy of Sciences of the United States of America.

[20]  F. Accurso,et al.  Prospective, long-term study of fat-soluble vitamin status in children with cystic fibrosis identified by newborn screen. , 1999, The Journal of pediatrics.

[21]  H. McKay,et al.  A Six‐Year Longitudinal Study of the Relationship of Physical Activity to Bone Mineral Accrual in Growing Children: The University of Saskatchewan Bone Mineral Accrual Study , 1999, Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research.

[22]  M. Corey,et al.  Prevalence of vitamin K deficiency in cystic fibrosis. , 1999, The American journal of clinical nutrition.

[23]  T. Cheetham,et al.  The strengths and limitations of parental heights as a predictor of attained height , 1999, Archives of disease in childhood.

[24]  A. Quittner,et al.  Diagnosis, screening and management of cystic fibrosis related diabetes mellitus: a consensus conference report. , 1999, Diabetes research and clinical practice.

[25]  M. Kosorok,et al.  Comparison of growth status of patients with cystic fibrosis between the United States and Canada. , 1999, The American journal of clinical nutrition.

[26]  C. Madsen,et al.  Bone mineral content and body composition in children and young adults with cystic fibrosis , 1999, Pediatric pulmonology.

[27]  R. Sokol,et al.  Recommendations for management of liver and biliary tract disease in cystic fibrosis. Cystic Fibrosis Foundation Hepatobiliary Disease Consensus Group. , 1999, Journal of pediatric gastroenterology and nutrition.

[28]  R. Marcus,et al.  Bone acquisition and loss in children and adults with cystic fibrosis: a longitudinal study. , 1998, The Journal of pediatrics.

[29]  A. Moran,et al.  Abnormal glucose metabolism in cystic fibrosis. , 1998, The Journal of pediatrics.

[30]  LA Para,et al.  Proteolysis Associated With Insulin Resistance in Cystic Fibrosis , 1998, Pediatrics.

[31]  M. Kosorok,et al.  Growth status in children with cystic fibrosis based on the National Cystic Fibrosis Patient Registry data: evaluation of various criteria used to identify malnutrition. , 1998, The Journal of pediatrics.

[32]  N. Krebs,et al.  Effect of pancreatic enzymes on zinc absorption in cystic fibrosis. , 1998, Journal of pediatric gastroenterology and nutrition.

[33]  F. Accurso,et al.  Enteral nutrition for patients with cystic fibrosis: comparison of a semi-elemental and nonelemental formula. , 1998, The Journal of pediatrics.

[34]  R. Stern,et al.  Sodium Space and Intravascular Volume: Dietary Sodium Effects in Cystic Fibrosis and Healthy Adolescent Subjects , 1998, Pediatrics.

[35]  P. Frascarolo,et al.  Essential fatty acid deficiency in well nourished young cystic fibrosis patients , 1997, European Journal of Pediatrics.

[36]  L. Sokoll,et al.  Effect of vitamin K1 supplementation on vitamin K status in cystic fibrosis patients. , 1997, Journal of pediatric gastroenterology and nutrition.

[37]  J. Lloyd-Still,et al.  High-dose pancreatic-enzyme supplements and fibrosing colonopathy in children with cystic fibrosis. , 1997, The New England journal of medicine.

[38]  B. Strandvik,et al.  Vitamin A concentration in the liver decreases with age in patients with cystic fibrosis. , 1997, Journal of pediatric gastroenterology and nutrition.

[39]  C. Powers,et al.  Essential fatty acid deficiency and predisposition to lung disease in cystic fibrosis , 1996, Acta paediatrica.

[40]  N. Rifai,et al.  Vitamin A status in acute exacerbations of cystic fibrosis. , 1996, The American journal of clinical nutrition.

[41]  R. Sokol,et al.  Supplementation with carotenoids corrects increased lipid peroxidation in children with cystic fibrosis. , 1996, The American journal of clinical nutrition.

[42]  L. Molinari,et al.  Response to a single oral dose of all-rac-alpha-tocopheryl acetate in patients with cystic fibrosis and in healthy individuals. , 1996, The American journal of clinical nutrition.

[43]  M. V. van’t Hof,et al.  Long-term oral vitamin E supplementation in cystic fibrosis patients: RRR-alpha-tocopherol compared with all-rac-alpha-tocopheryl acetate preparations. , 1996, The American journal of clinical nutrition.

[44]  V. Carnielli,et al.  Medium-chain triglyceride absorption in patients with pancreatic insufficiency. , 1996, Scandinavian journal of gastroenterology.

[45]  R. Marcus,et al.  Correlates of osteopenia in patients with cystic fibrosis. , 1996, Pediatrics.

[46]  E. Frongillo,et al.  The relationship between body weight and mortality: a quantitative analysis of combined information from existing studies. , 1996, International journal of obesity and related metabolic disorders : journal of the International Association for the Study of Obesity.

[47]  C. Madsen,et al.  Bone density in children and adolescents with cystic fibrosis. , 1996, The Journal of pediatrics.

[48]  P. Durie,et al.  Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy. Consensus Committee. , 1995, The Journal of pediatrics.

[49]  M. V. van’t Hof,et al.  Response to oral β‐carotene supplementation in patients with cystic fibrosis: a 16‐month follow‐up study , 1995 .

[50]  P. Farrell,et al.  Oral supplementation with a high-fat, high-energy product improves nutritional status and alters serum lipids in patients with cystic fibrosis. , 1995, Journal of the American Dietetic Association.

[51]  M. V. van’t Hof,et al.  Response to oral beta-carotene supplementation in patients with cystic fibrosis: a 16-month follow-up study. , 1995, Acta paediatrica.

[52]  J. Nerup,et al.  Diabetes mellitus in cystic fibrosis: effect of insulin therapy on lung function and infections , 1994, Acta paediatrica.

[53]  Judith R. Mathews,et al.  Behavior at Mealtimes and the Young Child with Cystic Fibrosis , 1994 .

[54]  O. Bar-or,et al.  Relationships among nutritional status and skeletal and respiratory muscle function in cystic fibrosis: does early dietary supplementation make a difference? , 1993, The American journal of clinical nutrition.

[55]  R. Stern,et al.  Use of nonmedical treatment by cystic fibrosis patients. , 1992, The Journal of adolescent health : official publication of the Society for Adolescent Medicine.

[56]  G. Cleghorn,et al.  Nutritional Rehabilitation in Cystic Fibrosis: A 5 Year Follow‐up Study , 1992, Journal of pediatric gastroenterology and nutrition.

[57]  M. Corey,et al.  Decline of Exocrine Pancreatic Function in Cystic Fibrosis Patients with Pancreatic Sufficiency , 1992, Pediatric Research.

[58]  M. Corey,et al.  Genetic determination of exocrine pancreatic function in cystic fibrosis. , 1992, American journal of human genetics.

[59]  M. Corey,et al.  Prediction of mortality in patients with cystic fibrosis. , 1992, The New England journal of medicine.

[60]  V. Matkovic Calcium and peak bone mass , 1992, Journal of internal medicine.

[61]  P M Farrell,et al.  Nutritional assessment and management in cystic fibrosis: a consensus report. The Consensus Committee. , 1992, The American journal of clinical nutrition.

[62]  A. Malfroot,et al.  New insights on gastro-oesophageal reflux in cystic fibrosis by longitudinal follow up. , 1991, Archives of disease in childhood.

[63]  I. Kjellmer,et al.  Linear Growth in Children with Cystic Fibrosis: I. Birth to 8 Years of Age , 1991, Acta paediatrica Scandinavica.

[64]  C. Lamers,et al.  Omeprazole enhances the efficacy of pancreatin (pancrease) in cystic fibrosis. , 1991, Annals of internal medicine.

[65]  R. Levy,et al.  Treatment failure after substitution of generic pancrelipase capsules. , 1990, JAMA.

[66]  D Markiewicz,et al.  The relation between genotype and phenotype in cystic fibrosis--analysis of the most common mutation (delta F508). , 1990, The New England journal of medicine.

[67]  A. Stecenko,et al.  Treatment failure after substitution of generic pancrelipase capsules. Correlation with in vitro lipase activity. , 1990, JAMA.

[68]  R. Kane,et al.  Comparison of low, medium, and high carbohydrate formulas for nighttime enteral feedings in cystic fibrosis patients. , 1990, JPEN. Journal of parenteral and enteral nutrition.

[69]  R. Rayner,et al.  Night blindness and conjunctival xerosis caused by vitamin A deficiency in patients with cystic fibrosis. , 1989, Archives of disease in childhood.

[70]  Claire C. Gordon,et al.  Stature, recumbent length, and weight , 1988 .

[71]  T. Lohman,et al.  Anthropometric Standardization Reference Manual , 1988 .

[72]  M. Corey,et al.  A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. , 1988, Journal of clinical epidemiology.

[73]  D Mukherjee,et al.  Head circumference reference data: birth to 18 years. , 1987, Pediatrics.

[74]  M. Miller,et al.  Iron deficiency in cystic fibrosis. , 1987, Archives of disease in childhood.

[75]  M. Goodchild Nutritional management of cystic fibrosis. , 1987, Digestion.

[76]  P. Zentler-Munro,et al.  Effect of cimetidine on enzyme inactivation, bile acid precipitation, and lipid solubilisation in pancreatic steatorrhoea due to cystic fibrosis. , 1985, Gut.

[77]  E. O'Loughlin,et al.  Gastroesophageal reflux in patients with cystic fibrosis. , 1985, The Journal of pediatrics.

[78]  D. Thissen,et al.  Parent-specific adjustments for evaluation of recumbent length and stature of children. , 1985, Pediatrics.

[79]  P. Durie,et al.  Colipase and lipase secretion in childhood-onset pancreatic insufficiency. Delineation of patients with steatorrhea secondary to relative colipase deficiency. , 1984, Gastroenterology.

[80]  J. Ater,et al.  Relative anemia and iron deficiency in cystic fibrosis. , 1983, Pediatrics.

[81]  P. Pencharz,et al.  Energy Needs and Growth in Children with Cystic Fibrosis , 1983, Journal of pediatric gastroenterology and nutrition.

[82]  A. R. Frisancho,et al.  New norms of upper limb fat and muscle areas for assessment of nutritional status. , 1981, The American journal of clinical nutrition.

[83]  H. Eigen,et al.  Changing feeding trends as a cause of electrolyte depletion in infants with cystic fibrosis. , 1981, Pediatrics.

[84]  R. Jacob,et al.  Some biochemical indices of nutrition in treated cystic fibrosis patients. , 1981, The American journal of clinical nutrition.

[85]  V. Rogiers,et al.  LONG CHAIN NON-ESTERIFIED FATTY ACID PATTERN IN PLASMA OF CYSTIC FIBROSIS PATIENTS AND THEIR PARENTS , 1980, Pediatric Research.

[86]  N. M. Morris,et al.  Validation of a self-administered instrument to assess stage of adolescent development , 1980, Journal of youth and adolescence.

[87]  P. Durie,et al.  Malabsorption of medium-chain triglycerides in infants with cystic fibrosis: correction with pancreatic enzyme supplement. , 1980, The Journal of pediatrics.

[88]  C. Denning,et al.  The effect of oral zinc supplementation on plasma levels of vitamin A and retinol-binding protein in cystic fibrosis. , 1979, The American journal of clinical nutrition.

[89]  T. Hahn,et al.  Reduced serum 25-hydroxyvitamin D concentration and disordered mineral metabolism in patients with cystic fibrosis. , 1979, The Journal of pediatrics.

[90]  R. Kraemer,et al.  RELATIVE UNDERWEIGHT IN CYSTIC FIBROSIS AND ITS PROGNOSTIC VALUE , 1978, Acta paediatrica Scandinavica.

[91]  D. McLaren,et al.  WEIGHT/LENGTH CLASSIFICATION OF NUTRITIONAL STATUS , 1975, The Lancet.

[92]  C. Howard,et al.  Letter: Hepatitis-B antigen and tobacco plants. , 1974, Lancet.

[93]  Intakes for Calcium , Phosphorus , Magnesium , Vitamin D , and Fluoride , 1974 .

[94]  C. Roy,et al.  Malabsorption of bile acids in children with cystic fibrosis. , 1973, The New England journal of medicine.

[95]  W. W. Read,et al.  Assessment of nutritional status of children. , 1973, Lancet.

[96]  K. Jeejeebhoy,et al.  Determination of fecal fats containing both medium and long chain triglycerides and fatty acids. , 1970, Clinical biochemistry.

[97]  S. Garn,et al.  Interaction of Nutrition and Genetics in the Timing of Growth and Development , 1966 .

[98]  P. A. Sant'agnese,et al.  Salt depletion in cold weather in infants with cystic fibrosis of the pancreas. , 1960 .

[99]  P. di Sant'Agnese,et al.  Salt depletion in cold weather in infants with cystic fibrosis of the pancreas. , 1960, Journal of the American Medical Association.

[100]  D. A. Sholl,et al.  Growth at Adolescence , 1962 .