Malnutrition in cystic fibrosis: A review

Nutrition in Clinical Practice

Volumn 28, Issue 6, 2013, Pages 676-683

  Culhane, Suzanne ^a   George, Carolyn ^a   Pearo, Brittany ^a   Spoede, Elizabeth ^a  

^a TEXAS CHILDREN S HOSPITAL   (United States)

Author keywords

cystic fibrosis; liver diseases; lung diseases; nutritional status; pancreas; pancreatic diseases

Indexed keywords

BICARBONATE; CORTICOSTEROID; IVACAFTOR; PANCREAS ENZYME;

ABDOMINAL PAIN; BACTERIAL OVERGROWTH; BODY WEIGHT; CALORIC INTAKE; CONSTIPATION; CYSTIC FIBROSIS; DEHYDRATION; DIABETES MELLITUS; DIARRHEA; DIET SUPPLEMENTATION; DIET THERAPY; DISEASE ASSOCIATION; DYSPHASIA; ENERGY BALANCE; ENERGY EXPENDITURE; ENERGY METABOLISM; ENZYME THERAPY; GASTROESOPHAGEAL REFLUX; GASTROINTESTINAL SYMPTOM; HEARTBURN; HUMAN; HYPERGLYCEMIA; LIPID ABSORPTION; LIVER DISEASE; LOWER ESOPHAGUS SPHINCTER; LUNG DISEASE; MALABSORPTION; MALNUTRITION; MUSCLE ATROPHY; NUTRITIONAL STATUS; PANCREAS EXOCRINE INSUFFICIENCY; PANCREAS INSUFFICIENCY; PATIENT COMPLIANCE; PH; REVIEW; STOMACH EMPTYING; VOMITING;

CYSTIC FIBROSIS; LIVER DISEASES; LUNG DISEASES; NUTRITIONAL STATUS; PANCREAS; PANCREATIC DISEASES;

CYSTIC FIBROSIS; ENERGY INTAKE; ENERGY METABOLISM; HUMANS; MALNUTRITION; NUTRITIONAL STATUS;

EID: 84888611730     PISSN: 08845336     EISSN: 19412452     Source Type: Journal    
DOI: 10.1177/0884533613507086     Document Type: Review

References (71)

1. Annual Data Report. Bethesda, MD: Cystic Fibrosis Foundation ; 2011 :
2. Corkins MR The A.S.P.E.N. Pediatric Nutrition Support Core Curriculum. Silver Spring, MD: American Society for Parenteral and Enteral Nutrition ; 2010 :
3. Corey M, McLaughlin FJ, Williams M, Levison H. A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol. 1988 ; 41 (6). 583-591
4. Stallings VA, Stark LJ, Robinson KA, Feranchak AP, Quinton H. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008 ; 108: 832-839
5. Borowitz D, Baker RD, Stallings V. Consensus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastr Nutr. 2002 ; 35: 246-259
6. Gottschlich MM The A.S.P.E.N. Nutrition Support Core Curriculum: A Case-Based Approach-The Adult Patient. Silver Spring, MD: American Society for Parenteral and Enteral Nutrition ; 2007 :
7. Davis PB. Cystic fibrosis since 1938. Am J Respir Crit Care Med. 2006 ; 173 (5). 475-482
8. Fieker A, Philpott J, Armand M. Enzyme replacement therapy for pancreatic insufficiency: present and future. Clin Exp Gastroenterol. 2011 ; 4: 55-73
9. Wouthyzen-Bakker M, Bodewes FA, Verkade HJ. Persistent fat malabsorption in cystic fibrosis: lessons for patient s and mice. J Cyst Fibros. 2011 ; 10: 150-158
10. Pencharz PB, Durie PR. Pathogenesis of malnutrition in CF. Clin Nutr. 2000 ; 19 (6). 387-394
11. Brady MS, Garson JL, Krug SK, et al. An enteric-coated high-buffered pancreas lipase reduces steatorrhea in patients with cystic fibrosis: a prospective, randomized study. J Am Diet Assoc. 2006 ; 106 (8). 1181-1196
12. Kalnins D, Ellis L, Corey M, et al. Enteric-coated pancreatic enzyme with bicarbonate is equal to standard enteric-coated enzyme in treating malabsorption in cystic fibrosis. J Pediatr Gastr Nutr. 2006 ; 42 (3). 256-261
13. Jones A. Drug therapies for reducing gastric acidity in people with cystic fibrosis. Cochran Collab. 2003 ; 2: 1-16
14. Borowitz D, Grand RJ, Durie PR. Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy. Consensus Committee. J Pediatr. 1995 ; 127 (5). 681-684
15. FitzSimmons SC, Burkhart GA, Borowitz D, et al. High-dose pancreatic-enzyme supplements and fibrosing colonopathy in children with cystic fibrosis. N Engl J Med. 1997 ; 336: 1283-1289
16. Smyth RL. Fibrosing colonopathy in cystic fibrosis. Arch Dis Child. 1996 ; 74: 464-468
17. Colombo C, Russo MC, Zazzeron L, Romano G. Liver disease in cystic fibrosis. J Pediatr Gastroenterol Nutr. 2006 ; 43 (1). S49-S55
18. Wilschanski M, Durie PR. Patterns of GI disease in adulthood associated with mutations in the CFTR gene. Gut. 2007 ; 56: 1153-1163
19. Peretti N, Marcil V, Drouin E, Levy E. Mechanism of lipid malabsorption in cystic fibrosis: the impact of essential fatty acid deficiency. Nutr Metab (Lond). 2005 ; 2: 11-29
20. Corbett K, Kelleher S, Rowland M, et al. Cystic fibrosis-associated liver disease: a population based study. J Pediatr. 2004 ; 145: 327-332
21. Kelly A, Moran A. Update on cystic fibrosis related diabetes. J Cyst Fibros. 2013 ; 12 (4). 318-331
22. Lang S, Hanse A, Thorsteinsson B, Nerup J, Koch C. Glucose tolerance in patients with cystic fibrosis: five year prospective study. BMJ. 1995 ; 311: 655-659
23. Austin A, Kalhan SC, Orenstein D, Nixon P, Arslanian S. Roles of insulin resistance and beta-cell dysfunction in the pathogenesis of glucose intolerance in cystic fibrosis. J Clin Endocrinol Metab. 1994 ; 79: 80-85
24. O'Riordan SM, Robinson PD, Donaghue KC, Moran A. Management of cystic fibrosis-related diabetes. Pediatr Diabetes. 2008 ; 9: 338-344
25. Hameed S, Morton JR, Jaffe A, et al. Early glucose abnormalities in cystic fibrosis are preceded by poor weight gain. Diabetes Care. 2010 ; 33: 221-226
26. Moran A, Pekow P, Grover P, et al. Insulin therapy to improve BMI in cystic fibrosis-related diabetes therapy trial. Diabetes Care. 2009 ; 32: 1783-1788
27. Hameed S, Morton JR, Field PI, et al. Once daily insulin detemir in cystic fibrosis with insulin deficiency. Arch Dis Child. 2012 ; 97: 464-467
28. Bizzarri C, Lucidi V, Ciampalini P, Bella S, Russo B, Cappa M. Clinical effects of early treatment with insulin glargine in patients with cystic fibrosis and impaired glucose tolerance. J Endocrinol Invest. 2006 ; 29: RC1-4
29. Mozzillo E, Franzese A, Valerio G, et al. One-year glargine treatment can improve the course of lung disease in children and adolescents with cystic fibrosis and early glucose derangements. Pediatr Diabetes. 2009 ; 10: 162-167
30. Shepherd SL, Hovel MF, Harwood IR, Granger LD, Hofstetter CR, Molgaard C. A comparative study of psychosocial assets of adults with cystic fibrosis and their healthy peers. Chest. 1990 ; 97: 1310-1016
31. Czyzewski DI, Koocher G. Medical compliance and coping with cystic fibrosis. J Child Psychol Psych. 1987 ; 28: 311-319
32. Hammons AK, Fiese B. Mealtime interactions in families of a child with cystic fibrosis: A meta-analysis. J Cyst Fibros. 2010 ; 9: 377-384
33. Stark LJ, Opipari LC, Spieth LE, et al. Contribution of behavior therapy in dietary treatment of cystic fibrosis: a randomized controlled study with 2-year follow up. Behav Ther. 2003 ; 34: 237-258
34. Quitnner AL, Tolbert VE, Regoli MJ, Orenstein D, Hollingsworth JL, Eigen H. Development of the role- play inventory of situations and coping strategies for parents of children with cystic fibrosis. J Pediatr Psychol. 1996 ; 21: 209-235
35. Stark LJ, Jelalian E, Powers SW, et al. Parent and child mealtime behaviors in families of children with cystic fibrosis. J Pediatr. 2000 ; 136: 195-200
36. Goodwin B. Nutrition issues in cystic fibrosis. Pract Gastroenterol. 2005 ; 27: 76-94
37. Matel JL. Nutritional management of cystic fibrosis. J Parenter Enteral Nutr. 2012 ; 36: 60s-67s
38. Fieker A, Philpott J, Armand M. Enzyme replacement therapy for pancreatic insufficiency: present and future. Clin Exp Gas. 2011 ; 4: 55-73
39. O'Brien CE, Harden H, Com G. A survey of nutrition practices for patients with cystic fibrosis. Nutr Clin Pract. 2013 ; 28: 237-241
40. Erskine JM, Lingard C, Sontag M. Update on enteral nutrition support for cystic fibrosis. Nutr Clin Pract. 2007 ; 22: 223-232
41. Michel SH, Maqbool A, Hanna MD, Mascarenhas M. Nutrition management of pediatric patients who have cystic fibrosis. Pediatr Clin N Am. 2009 ; 56: 1123-1141
42. Bradley GM, Carson KA, Leonard AR, Mogayzel PJ, Oliva-Hemker M. Nutritional outcomes following gastrostomy in children with cystic fibrosis. Pediatr Pulm. 2012 ; 47 (8). 473-478
43. Trabulsi J, Ittenbach RF, Schall JI, et al. Evaluation of formulas for calculating total energy requirements of preadolescent children with cystic fibrosis. Am J Clin Nutr. 2007 ; 85: 144-151
44. Gelfond D, Borowitz D. Gastrointestinal complications of cystic fibrosis. Clin Gastroenterol Hepatol. 2013 ; 11: 333-343
45. Borowitz D, Durie PR, Clarke LL, et al. Gastrointestional outcomes and confounders in cystic fibrosis. J Pediatr Gastr Nutr. 2005 ; 41: 273-285
46. Fridge JL, Conrad C, Gerson L, Castillo RO, Cox K. Risk factors for small bowel bacterial overgrowth in Cystic Fibrosis. JPGN. 2007 ; 44: 212-218
47. Houwen RH, van der Doef HP, Sermet I, et al. Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristic and treatment of DIOS. J Pediatr Gastr Nutr. 2009 ; 49: 54-58
48. Goralski JL, Lercher DM, Davis SD, Dellon ES. Eosinophilic esophagitis in cystic fibrosis: a case series and review of literature. J Cystic Fibrosis. 2012 ; 12: 9-14
49. Nasr SZ, Drury D. Appetite stimulants use in cystic fibrosis. Pediatr Pulmonol. 2008 ; 43: 209-219
50. Powers SW, Patton SR, Henry R, Heidemann M, Stark LJ. A tool to individualize nutrition care for children with cystic fibrosis: reliability, validity, and utility of the CF individualized nutritional assessment of kids eating. Child Health Care. 2005 ; 32: 113-131
51. Shepherd RW, Holt TL, Vasques-Velasquez L, Coward WA, Prentice A, Lucas A. Increased energy expenditure in young children with cystic fibrosis. Lancet. 1988 ; 1: 1300-1303
52. Fried MD, Durie PR, Tsui LC, Corey M, Levison H, Pencharz PB. The cystic fibrosis gene and resting energy expenditure. J Pediatr. 1991 ; 199: 913-916
53. Girardet JP, Tounian P, Sardet A, et al. Resting energy expenditure in infants with cystic fibrosis. J Pediatr Gastroenterol Nutr. 1994 ; 18: 214-219
54. Tomezko JL, Stallings VA, Kawchak DA, Goin JE, Diamond G, Scanlin TF. Energy expenditure and genotype in children with cystic fibrosis. Pediatr Res. 1994 ; 35: 451-460
55. Zemel BS, Kawchak DA, Cnann A, Zhao H, Scanlin TF, Stallings VA. Prospective evaluation of resting energy expenditure, nutritional status, pulmonary function and genotype in children with cystic fibrosis. Pediatr Res. 1996 ; 40: 578-586
56. Allen JR, McCauley JC, Selby AM, et al. Differences in resting energy expenditure between male and female children with cystic fibrosis. J Pediatr. 2003 ; 142: 15-19
57. Moudiou T, Galli-Tsinopoulou A, Nousia-Arvanitakis S. Effect of exocrine pancreatic function on resting energy expenditure in cystic fibrosis. Acta Paediatrica. 2007 ; 96: 1521-1525
58. Barclay A, Allen JR, Blyler E, et al. Resting energy expenditure in females with cystic fibrosis-is it affected by puberty?. Eur J Clin Nutr. 2007 ; 61: 1207-1212
59. Grungow J, Azcue M, Berall G, Pencharz P. Energy expenditure in cystic fibrosis during activities of daily living. J Pediatr. 1992 ; 122: 243-246
60. Barry S, Gallagher C. Corticosteroids and skeletal muscle function in cystic fibrosis. J Appl Physiol. 2003 ; 95: 1379-1384
61. Thomson MA, Wilmott RW, Wainwright C, Masters B, Francis PJ, Shepherd RW. Resting energy expenditure, pulmonary inflammation, and genotype in the early course of cystic fibrosis. J Pediatr. 1996 ; 129: 367-373
62. Divangahi M, Balghi H, Danialou G, et al. Lack of CFTR in skeletal muscle predisposes to muscle wasting and diaphragm muscle pump failure in cystic fibrosis mice. PLoS Genet. 2009 ; 5: e1000586
63. Stephenson AL, Mannik LA, Walsh S, et al. Longitudinal trends in nutritional status and the relation between lung function and BMI in cystic fibrosis: a population-based cohort study. Am J Clin Nutr. 2013 ; 97: 872-877
64. Stallings VA, Fung EB, Hofley PM, Scanlin TF. Acute pulmonary exacerbation is not associated with increased energy expenditure in children with cystic fibrosis. J Pediatr. 1998 ; 132: 493-499
65. Naon H, Hack S, Shelton MT, Gotthoffer RC, Gozal D. Resting energy expenditure. Evolution during antibiotic treatment for pulmonary exacerbations in cystic fibrosis. Chest. 1993 ; 103: 1819-1825
66. Shepherd RW, Holt TL, Cleghorn G, Ward LC, Isles A, Francis P. 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
67. RamseyB. Baby Observational and Nutritional Study (BONUS). Bethesda, MD: National Library of Medicine; 2000-2013. Available from: http://clinicaltrials. gov/ct2/show/NCT01424696.
68. Rhodes B, Nash EF, Tullis E, et al. Prevalence of dyslipidemia in adults with cystic fibrosis. J Cyst Fibros. 2010 ; 9: 24-28
69. Davis PB, Yasothan U, Kirkpatrick P. Ivacaftor. Nature Rev Drug Disc. 2012 ; 11 (5). 349-350
70. Davies JC, Wainright CE, Canny GJ, et al. Efficacy and safety of Ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation. Am J Respir Crit Care Med. 2013 ; 187 (11). 1219-1225
71. Cystic Fibrosis Foundation. Kalydeco. Available at: http://www.cff.org/ treatments/therapies/kalydeco/. Accessed June 27, 2013.