Malnutrition is a common complication of chronic diseases in children and may lead to growth impairment (stunting). Malnutrition in cystic fibrosis (CF) results from increased energy expenditure, decreased energy intakes, malabsorption of ingested nutrients because of pancreatic insufficiency and chronic inflammation. Malnutrition and high levels of inflammatory cytokines affect IGF-1 production through interrelated mechanisms. Nutritional support was shown to improve both nutritional status and outcome in CF. However, some nutrients have a direct effect on the disease. n-3 fatty acids supplementation is able to correct lipid abnormalities resulting from a primary mechanism. Moreover, n-3 fatty acids have a direct effect on the inflammatory response, decreasing eicosanoid synthesis and modulating nuclear transcriptional factors nuclear factor ĸB and peroxisome proliferator-activated receptors γ. Nutritional support may be considered part of the care of the CF patient together with antibiotics, pancreatic enzymes and physiotherapy, influencing significantly the evolution of the disease.

1.
Kawchak DA, Zhao H, Scanlin TF, Tomezsko JL, Cnaan A, Stallings VA: Longitudinal, prospective analysis of dietary intake in children with cystic fibrosis. J Pediatr 1996;129:119–129.
2.
O’Rawe A, McIntosh I, Dodge JA, Brock DJ, Redmond AO, Ward R, et al: Increased energy expenditure in cystic fibrosis is associated with specific mutations. Clin Sci (Colch) 1992;82:71–76.
3.
Jelalian E, Stark LJ, Reynolds L, Seifer R: Nutrition intervention for weight gain in cystic fibrosis: A meta-analysis. J Pediatr 1998;132:486–492.
4.
Thomson MA, Quirk P, Swanson CE, Thomas BJ, Holt TL, Francis PJ, et al: Nutritional growth retardation is associated with defective lung growth in cystic fibrosis: A preventable determinant of progressive pulmonary dysfunction. Nutrition 1995;11:350–354.
5.
Navarro J, Rainisio M, Harms HK, Hodson ME, Koch C, Mastella G, et al: Factors associated with poor pulmonary function: Cross-sectional analysis of data from the ERCF. European Epidemiologic Registry of Cystic Fibrosis. Eur Respir J 2001;18:298–305.
6.
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;41:583–591.
7.
Lai HC, Corey M, FitzSimmons S, Kosorok MR, Farrell PM: Comparison of growth status of patients with cystic fibrosis between the United States and Canada. Am J Clin Nutr 1999;69:531–538.
8.
Farrell PM, Kosorok MR, Laxova A, Shen G, Koscik RE, Bruns WT, et al: Nutritional benefits of neonatal screening for cystic fibrosis. Wisconsin Cystic Fibrosis Neonatal Screening Study Group. N Engl J Med 1997;337:963–969.
9.
Laursen EM, Koch C, Petersen JH, Muller J: Secular changes in anthropometric data in cystic fibrosis patients. Acta Paediatr 1999;88:169–174.
10.
Stettler N, Kawchak DA, Boyle LL, Propert KJ, Scanlin TF, Stallings VA, et al: Prospective evaluation of growth, nutritional status and body composition in children with cystic fibrosis. Am J Clin Nutr 2000;72:407–413.
11.
Underwood LE, Thissen JP, Lemozy S, Ketelslegers JM, Clemmons DR: Hormonal and nutritional regulation of IGF-1 and its binding proteins. Horm Res 1994;42:145–151.
12.
Laursen EM, Lanng S, Rasmussen MH, Koch C, Skakkebaek NE, Muller J: Normal spontaneous and stimulated GH levels despite decreased IGF-1 concentrations in cystic fibrosis patients. Eur J Endocrinol 1999;140:315–321.
13.
De Benedetti F, Alonzi T, Moretta A, Lazzaro D, Costa P, Poli V, et al: Interleukin-6 causes growth impairment in transgenic mice through a decrease in insulin-like growth factor-1. A model for stunted growth in children with chronic inflammation. J Clin Invest 1997;99:643–650.
14.
Lopez-Calderon A, Ibanez de Caceres I, Soto L, Priego T, Martin AI, Villanua MA: The decrease in hepatic IGF-1 gene expression in arthritic rats is not associated with modifications in hepatic GH receptor mRNA. Eur J Endocrinol 2001;144:529–534.
15.
Huseman CA, Colombo JL, Brooks MA, Smay JR, Greger NG, Sammut PH, et al: Anabolic effect of biosynthetic growth hormone in cystic fibrosis patients. Pediatr Pulmonol 1996;22:90–95.
16.
Hardin DS, Sy JP: Effects of growth hormone treatment in children with cystic fibrosis: the National Cooperative Growth Study experience. J Pediatr 1997;131:S65–S69.
17.
Hardin DS, Ellis KJ, Dyson M, Rice J, McConnell R, Seilheimer DK: Growth hormone decreases protein catabolism in children with cystic fibrosis. J Clin Endocrinol Metab 2001;86:4424–4428.
18.
Jeschke MG, Barrow RE, Herndon DN: Recombinant human growth hormone treatment in pediatric burn patients and its role during the hepatic acute phase response. Crit Care Med 2000;28:1578–1584.
19.
Vic P, Ategbo S, Gottrand F, Launay V, Loeuille GA, Elian JC, et al: Nutritional impact of antipseudomonas intravenous antibiotic courses in cystic fibrosis. Arch Dis Child 1997;76:437–440.
20.
Lai HC, FitzSimmons SC, Allen DB, Kosorok MR, Rosenstein BJ, Campbell PW, et al: Risk of persistent growth impairment after alternate-day prednisone treatment in children with cystic fibrosis. N Engl J Med 2000;342:851–859.
21.
Auerbach HS, Williams M, Kirkpatrick JA, Colten HR: Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis. Lancet 1985;ii:686–688.
22.
Konstan MW, Byard PJ, Hoppel CL, Davis PB: Effect of high-dose ibuprofen in patients with cystic fibrosis. N Engl J Med 1995;332:848–854.
23.
Rogiers V, Dab I, Crokaert R, Vis HL: Long chain non-esterified fatty acid pattern in plasma of cystic fibrosis patients and their parents. Pediatr Res 1980;14:1088–1091.
24.
Roulet M, Frascarolo P, Rappaz I, Pilet M: Essential fatty acid deficiency in well-nourished young cystic fibrosis patients. Eur J Pediatr 1997;156:952–956.
25.
Christophe AB, Warwick WJ, Holman RT: Serum fatty acid profiles in cystic fibrosis patients and their parents. Lipids 1994;29:569–575.
26.
Strandvik B, Bronnegard M, Gilljam H, Carlstedt-Duke J: Relation between defective regulation of arachidonic acid release and symptoms in cystic fibrosis. Scand J Gastroenterol Suppl 1988;143:1–4.
27.
Levistre R, Lemnaouar M, Rybkine T, Bereziat G, Masliah J: Increase of bradykinin-stimulated arachidonic acid release in a delta F508 cystic fibrosis epithelial cell line. Biochim Biophys Acta 1993;1181:233–239.
28.
Strandvik B, Berg U, Kallner A, Kusoffsky E: Effect on renal function of essential fatty acid supplementation in cystic fibrosis. J Pediatr 1989;115:242–250.
29.
Steinkamp G, Demmelmair H, Ruhl-Bagheri I, von der Hardt H, Koletzko B: 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.
30.
Van Egmond AW, Kosorok MR, Koscik R, Laxova A, Farrell PM: Effect of linoleic acid intake on growth of infants with cystic fibrosis. Am J Clin Nutr 1996;63:746–752.
31.
Belluzzi A, Brignola C, Campieri M, Pera A, Boschi S, Miglioli M: Effect of an enteric-coated fish-oil preparation on relapses in Crohn’s disease. N Engl J Med 1996;334:1557–1560.
32.
Kremer JM: n-3 Fatty acid supplements in rheumatoid arthritis. Am J Clin Nutr 2000;71(suppl):349–351.
33.
Kurlandsky LE, Bennink MR, Webb PM, Ulrich PJ, Baer LJ: The absorption and effect of dietary supplementation with ω-3 fatty acids on serum leukotriene B4 in patients with cystic fibrosis. Pediatr Pulmonol 1994;18:211–217.
34.
Lawrence R, Sorrell T: Eicosapentaenoic acid in cystic fibrosis: Evidence of a pathogenetic role for leukotriene B4. Lancet 1993;342:465–469.
35.
Heeckeren A, Walenga R, Konstan MW, Bonfield T, Davis PB, Ferkol T: Excessive inflammatory response of cystic fibrosis mice to bronchopulmonary infection with Pseudomonas aeruginosa. J Clin Invest 1997;100:2810–2815.
36.
Freedman SD, Katz MH, Parker EM, Laposata M, Urman MY, Alvarez JG: A membrane lipid imbalance plays a role in the phenotypic expression of cystic fibrosis in cftr(–/–) mice. Proc Natl Acad Sci USA 1999;96:13995–14000.
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