Advances in Clinical and Experimental Medicine
2014, vol. 23, nr 5, September-October, p. 775–783
Publication type: original article
Effect of Selected Factors Associated with the Clinical Course of the Disease on Nutritional Status in Children with Cystic Fibrosis
1 Department of Human Biology, University of Wroclaw, Poland
2 Anthropology Division, Institute of Mother and Child, Warszawa, Poland
3 Cystic Fibrosis Center, Institute of Mother and Child, Warszawa, Poland
Background. Malnutrition and delayed growth are commonly seen in children with cystic fibrosis and are indicators of poor prognosis. Understanding the factors that affect growth and nutritional status may improve care, treatment and longevity.
Objectives. To determine how nutritional status, as estimated using anthropometric measurements, in children with cystic fibrosis is affected by 1) the type of CFTR mutation, 2) colonization by Pseudomonas aeruginosa, and 3) age at diagnosis. The relationship between nutritional status and pulmonary function was also investigated.
Material and Methods. Anthropometric data on height, weight, circumferences and skinfold thicknesses were extracted from the medical histories of 41 boys and 48 girls diagnosed with cystic fibrosis who were treated at the Institute of Mother and Child in Warsaw, Poland. Muscle and fat tissue area were calculated from these measurements. The values for children with CF were compared to the Polish national growth reference charts. Multivariate linear regression tested the effect of three factors on nutritional status: colonization with P. aeruginosa, age at diagnosis, and the type of CFTR mutation.
Results. All values for children with CF were significantly lower than in the reference population. The results show that, in the children examined, nutritional status is more adversely affected than growth. Of the factors related to the clinical picture of the disease, only the presence of respiratory tract colonization by P. aeruginosa had an effect on physical development in the children examined. Neither the type of mutation present nor age at diagnosis had any significant effect.
Conclusion. Chronic colonization by P. aeruginosa interfered with growth and markedly worsened nutritional status, and was also associated with reductions in both total and lean body mass. Early nutritional intervention can improve nutritional state and pulmonary function in children with CF.
cystic fibrosis, anthropometry, malnutrition, muscularity, Pseudomonas aeruginosa.
- Sobczyńska-Tomaszewska A, Ołtarzewski M, Czerska K, Wertheim-Tysarowska, Sands D, Walkowiak J, Bal A, Mazurczak T, and the NBS CF working group: Newborn screening for cystic fibrosis: Polish 4 years’ experience with CFTR sequencing strategy. Eur J Hum Genet 2013, 21, 391–396.
- Pencharz P, Durie P: Pathogenesis of malnutrition in cystic fibrosis, and its treatment. Clin Nutr 2000, 19, 387–394.
- Sharma R, Florea V, Bolger A, Doehner W, Florea N, Coats A, Hodson M, Anker S, Henein M: Wasting as a independent predictor of mortality in patients with cystic fibrosis. Thorax 2001, 56, 746–750.
- Zemel BS, Jawad AF, FitzSimmons S, Stallings VA: Longitudinal relationship among growth, nutritional status, and pulmonary function in children with cystic fibrosis: Analysis of the Cystic Fibrosis Foundation National CF Patient Registry. J Pediatr 2000, 137, 374–380.
- Konstan M, Butler S, Wohl M, Stoddard M, Matousek R, Wagner J, Johnston CH, Morgan J: Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis. J Pediatr 2003, 142, 624–630.
- Hirche TO, Hirche H, Jongblut S, Stern M, Wagner TO, Wiedermann B. on behalf of the German CFQA Group: Statistical limitations of percent ideal body weight as measure for nutritional failure in patients with cystic fibrosis. J Cyst Fibros 2009, 8, 238–244.
- King SJ, Nyulasi IB, Strauss BJG, Kotsimbos T, Bailey M, Wilson J: Fat-free mass depletion in cystic fibrosis: Associated with lung disease severity but poorly detected by body mass index. Nutrition 2010, 26, 753–759.
- Haeusler G, Frisch H, Waldhor T, Gotz M: Perspectives of longitudinal growth in cystic fibrosis from birth to adult age. Eur J Pediatr 1994, 153, 158–163.
- Groeneweg M, Tan S, Boot AM, de Jongste JC, Bouquet J, Sinaasappel M: Assessment of nutritional status in children with cystic fibrosis: conventional anthropometry and bioelectrical impedance analysis. A cross-sectional study in Dutch patients. J Cyst Fibros 2002, 1, 276–280.
- Schechter MS, Shelton BJ, Margolis PA, Fitzsimmons SC: The association of socioeconomic status with outcomes in cystic fibrosis patients in the United States. Am J Respir Crit Care Med 2001, 163, 1331–1337.
- Umławska W, Susanne CH: Growth and nutritional status in children and adolescents with cystic fibrosis. Ann Hum Biol 2008, 35, 145–153.
- Geborek A, Hjelte L: Association between genotype and pulmonary phenotype in cystic fibrosis patients with severe mutation. J Cyst Fibr 2011, 10, 187–192.
- Pittman J, Calloway E, Kiser M, Yeatts J, Davis S, Drumm M, Schechter M, Leigh M, Emond M, Van Rie A, Knowles M: Age of Pseudomonas aeruginosa acquisition and subsequent severity of cystic fibrosis lung disease. Pediatr Pulmonol 2011, 46, 497–504.
- McKone E, Emerson ST, Edwards K, Aitken M: Effect of genotype on phenotype and mortality in cystic fibrosis: a retrospective cohort study. Lancet 2003, 361, 1671–1676.
- Kerem E, Conway S, Elborn S, Heijerman H: Standards of care for patients with cystic fibrosis: A European Consensus. J Cyst Fibros 2005, 4, 7–26.
- Martin R, Knusmann R: Anthropologie. Handbuch der vergleichenden. Biologie des Menschen. Vol. 1. Gustav Fischer, Stuttgart, New York 1988.
- Palczewska I, Niedźwiedzka Z: Wskaźniki rozwoju somatycznego dzieci i młodzieży warszawskiej. Med Wieku Rozwoj 2001, 5, 19–55.
- Lee T, Brownlee K, Conway S, Denton M, Littlewood J: Evaluation of a new definition for chronic Pseudomonas aeruginosa infection in cystic fibrosis patients. J Cyst Fibros 2003, 2, 29–34.
- Tomalak W, Radliński J, Pogorzelski A, Doniec Z: Reference values for forced inspiratory flows in children aged 7–15 years. Pediatr Pulmonol 2004, 38, 1–4.
- Willim G, Kurzawa R, Mazurek H, Hałuszka J, Jedrys-Kłucjasz U, Baran B, Radliński J: Wartości należne wskaźników oddechowych dzieci i młodzieży. IGiChP Oddział w Rabce, 1–62, 1998.
- Kosińska M, Szwed A, Cieślik J, Goździk J, Karbowy K: Assessment of the biological condition and nutritional status of adult patients with cystic fibrosis. Anthropol Rev 2005, 68, 53–64.
- Umławska W, Rams M: Physical development and pulmonary function in children and adolescents treated at two cystic fibrosis treatment centers in Poland. Arch Med Sci 2009, 5, 586–588.
- McNaughton SA, Stormont DA, Sheperd RW, Francis PWJ, Dean B: Growth failure in cystic fibrosis. J Pediatr Child Health 1999, 5, 86–92.
- Yen EH, Quinton H, Borowitz D: Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. J Pediatr 2013, 162, 530–535.
- Bradley GM, Blackman SM, Watson CP, Doshi VK, Cutting GR: Genetic modifiers of nutritional status in cystic fibrosis. Am J Clin Nutr 2012, 96, 299–308.
- Fitzsimmons SC: The changing epidemiology of cystic fibrosis. J Pediatr 1993, 122, 1–9.
- Pamukcu A, Bush A, Buchdahl R: Effects of Pseudomonas aeruginosa colonization on lung function and anthropometric variables in children with cystic fibrosis. Pediatr Pulmonol 1995, 19, 10–15.
- Kosorok MR, Zeng L, West SE, Rock MJ, Splaingard ML, Laxova A, Green CG, Collins J, Farrell PM: Acceleration of lung disease in children with cystic fibrosis after Pseudomonas aeruginosa acquisition. Pediatr Pulmonol 2001, 32, 277–287.
- Nixon GM, Armstrong DS, Corzino R, Olinsky A, Robertson CF, Grimwood K: Clinical outcome after early Pseudomonas aeruginosa infection in cystic fibrosis. J Pediatr 2001, 158, 699–704.
- Vinton NE, Padman R, Davis M, Harcke HT: Effects of Pseudomonas colonization on body composition and resting energy expenditure in children with cystic fibrosis. J Parenter Enteral Nutr 1999, 23, 233–236.
- Marin VB, Velandia S, Hunter B, Gattas V, Fielbaum O, Herrera O, Diaz E: Energy expenditure, nutritional status, and body composition in children with cystic fibrosis. Nutrition 2004, 20, 181–186.
- Bolton CE, Ionescu AA, Evans WD, Pettit RJ, Shale DJ: Altered tissue distribution in adults with cystic fibrosis. Thorax 2003, 5, 885–889.