Nutrional status and dietary factors in cystic fibrosis patients with delta F508 mutation
Keywords:
Cystic fibrosis, Diet, Nutritional statusAbstract
Objective
To examine the association between nutritional status and dietary factors in children and adolescents with cystic fibrosis that are carriers of delta F508 mutation.
Methods
Cross-sectional study of cystic fibrosis children and adolescents. Nutritional status (body mass index percentile) and dietary intake (3-day diet records presented as a percentage of estimated energy requirement) were assessed.
Results
Thirty six patients (median of 8.6; interquartile range 6.8-12.5 years; 50% male). The Poisson regression analysis showed that the carriers for delta F508 mutation had 60% lower prevalence ratio of body mass index ≥25° (PR=0.4; 95%IC=0.2-0.8) and 90% lower prevalence ratio (PR=0.1; confidence interval 95%IC=0.02-0.3) of ≥150% of estimated energy requirement when compared with non-delta F508 mutation carriers. The model was adjusted for lung function, estimated energy requirement, and body mass index.
Conclusion
Carriers for delta F508 mutation showed lower body mass index percentile and lower daily caloric consumption when compared with patients without this mutation.
References
Mickle JE, Cutting GR. Genotype-phenotype relationships in cystic fibrosis. Med Clin North Am. 2000; 84(3):597-607.
Cystic Fibrosis Mutation Database. 2013 [2013 Jul 1]. Available from: http://www.genet.sickkids.on.ca/ Home.html
O’Sullivan BP, Freedman SD. Cystic fibrosis. The Lancet. 2009; 373(9678):1891-904. http://dx.doi. org/10.1016/S0140-6736(09)60327-5
Castellani C, Cuppens H, Macek Jr M, Cassiman JJ, Kerem E, Durie P, et al. Consensus on the use and interpretation of cystic fibrosis mutation analysis in clinical practice. J Cystic Fibrosis. 2008; 7(3):179-96. http://dx.doi.org/10.1016/j.jcf.2008.03.009
McKone EF, Emerson SS, Edwards KL, Aitken ML. Effect of genotype on phenotype and mortality in cystic fibrosis: A retrospective cohort study. The Lancet. 2003; 361(9370):1671-6. http://dx.doi.org/ 10.1016/S0140-6736(03)13368-5
Kerem E, Corey M, Kerem B, Rommens J, Markiewicz D, Levison H, et al. The relation between genotype and phenotype in cystic fibrosis: Analysis of the most common mutation (delta F508). New England J Med. 1990; 323(22):1517-22.
Keller BM, Casaulta Aebischer C, Kraemer R, Schöni MH. Growth in prepubertal children with cystic fibrosis, homozygous for the delta F508 mutation. J Cystic Fibrosis. 2003; 2(2):76-83. http://dx.doi.org/ 10.1016/S1569-1993(03)00023-7
Stephenson AL, Mannik LA, Walsh S, Brotherwood M, Robert R, Darling PB, 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(4):872-7. http://dx.doi.org/10.3945/ajcn.1 12.051409
Tomezsko JL, Stallings VA, Kawchak DA, Goin JE, Diamond G, Scanlin TF. Energy expenditure and genotype of children with cystic fibrosis. Pediatr Res. 1994; 35(4 Pt. 1):451-60. http://dx.doi.org/ 10.1203/00006450-199404000-00013
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(3):367-73.
Preumont V, Hermans MP, Lebecque P, Buysschaert M. Glucose homeostasis and genotype-phenotype interplay in cystic fibrosis patients with CFTR gene delta F508 mutation. Diabetes Care. 2007; 30(5):1187-92. http://dx.doi.org/10.2337/dc06-19 15
Strandvik B, Gronowitz E, Enlund F, Martinsson T, Wahlstrom J. Essential fatty acid deficiency in relation to genotype in patients with cystic fibrosis. J Pediatr. 2001; 139(5):650-5. http://dx.doi.org/ 10.1067/mpd.2001.118890
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 Dietetic Assoc. 2008; 108(5):832-9. http://dx.doi. org/10.1016/j.jada.2008.02.020
Associação Brasileira de Empresas de Pesquisa. Critério de classificação econômica Brasil. São Paulo: Abep; 2013 [acesso 2012 jun 12]. Disponível em: http://www.abep.org/novo/Content.aspx? ContentID=301
Hammerschlag MR, Harding L, Macone A, Smith AL, Goldmann D. Bacteriology of sputum in cystic fibrosis: Evaluation of dithiothreitol as a mucolytic agent. J Clin Microbiol. 1980; 11(6):552-7.
Gilligan P, Kiska D, Appleman M. Cystic fibrosis microbiology. Cumitech 43. Washington (DC): ASM Press; 2006.
Pellegrino R, Viegi G, Brusasco V, Crapo R, Burgos F, Casaburi R, et al. Interpretative strategies for lung function tests. Euro Respir J. 2005; 26(5):948-68. http://dx.doi.org/10.1183/09031936.05.00 035205
Borowitz D, Baker RD, Stallings V. Consensus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2002; 35(3):246-59. http://dx.doi.org/10.1097/01.MPG.0 000025580.85615.14
Frisancho AR. New norms of upper limb fat and muscle areas for assessment of nutritional status. Am J Clin Nutr. 1981; 34(11):2540-5.
World Health Organization. Physical status: The use and interpretation of anthropometry. Report of a WHO Expert Committee. Geneva: WHO; 1995.
Wold Health Organization, Multicentre Growth Reference Study Group. WHO Child Growth Standards: Length/height-for-age, weight-for-age, weight-for-length, weight-for-height and body mass index-for-age: Methods and development. Geneva: WHO; 2006.
Universidade Estadual de Campinas. Tabela Brasi leira de Composição de Alimentos - TACO. 4ª ed. rev. ampl. Campinas: Unicamp; 2011.
Slater B, Marchioni DL, Fisberg RM. Estimando a prevalência da ingestão inadequada de nutrientes. Rev Saúde Pública. 2004; 38:599-605. http://dx. doi.org/10.1590/S0034-89102004000400019
Willett WC, Howe GR, Kushi LH. Adjustment for total energy intakein epidemiologic studies. Am J Clin Nutr. 1997; 65(Suppl.):1220S-8S.
Instituto de Medicina. Dietary reference intakes for energy, carbohydrate, fiber, fat, fatty acids, cholesterol, protein, and amino acids. Washington (DC): Institute of Medicine; 2002.
Sinaasappel M, Stern M, Littlewood J, Wolfe S, Steinkamp G, Heijerman HGM, et al. Nutrition in patients with cystic fibrosis: A European Consensus. J Cystic Fibrosis. 2002; 1(2):51-75.
Kastner-Cole D, Palmer CNA, Ogston SA, Mehta A, Mukhopadhyay S. Overweight and obesity in delta F508 homozygous cystic fibrosis. J Pediatr. 2005; 147(3):402-4. http://dx.doi.org/10.1016/j. jpeds.2005.06.003
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(1):119-29.
White H, Wolfe SP, Foy J, Morton A, Conway SP, Brownlee KB. Nutritional Intake and status in children with cystic fibrosis: Does age matter? J Pediatric Gastroenterol Nutr. 2007; 44(1):116-23. http://dx.doi.org/10.1097/01.mpg.000023792 9.45846.78
Bines JE, Truby HD, Armstrong DS, Phelan PD, Grimwood K. Energy metabolism in infants with cystic fibrosis. J Pediatr. 2002; 140(5):527-33. http:/ /dx.doi.org/10.1067/mpd.2002.123284
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2023 Carina de Sousa SANTOS, Thais STEEMBURGO
This work is licensed under a Creative Commons Attribution 4.0 International License.
