THE IMPACT OF MALNUTRITION ON LUNG FUNCTION IN CHILDREN WITH CYSTIC FIBROSIS

Alina Angelica Ciolpan; Iustina Violeta Stan; Valentina Daniela Comănici; Ioana Florentina Codreanu; Mihai Craiu; Ana Maria Alexandra Stănescu

Alina Angelica Ciolpan Carol Davila University of Medicine and Pharmacy, Bucharest, Romania & Alessandrescu-Rusescu National Institute for Mother and Child Health, Bucharest, Romania
Iustina Violeta Stan Carol Davila University of Medicine and Pharmacy, Bucharest, Romania & Alessandrescu-Rusescu National Institute for Mother and Child Health, Bucharest, Romania
Valentina Daniela Comănici Carol Davila University of Medicine and Pharmacy, Bucharest, Romania & Alessandrescu-Rusescu National Institute for Mother and Child Health, Bucharest, Romania
Ioana Florentina Codreanu Carol Davila University of Medicine and Pharmacy, Bucharest, Romania & Alessandrescu-Rusescu National Institute for Mother and Child Health, Bucharest, Romania
Mihai Craiu Carol Davila University of Medicine and Pharmacy, Bucharest, Romania & Alessandrescu-Rusescu National Institute for Mother and Child Health, Bucharest, Romania
Ana Maria Alexandra Stănescu Carol Davila University of Medicine and Pharmacy, Bucharest, Romania

Keywords: Cystic fibrosis, Nutritional status, Pulmonary function

Abstract

Cystic fibrosis (CF) is a genetic disorder that significantly impacts multiple organ systems, with malnutrition and respiratory dysfunction being prominent concerns. This study aims to evaluate the correlation between nutritional status and pulmonary function in pediatric patients with CF. This unicentric, retrospective, non-interventional study was conducted at the Pediatrics Department of the "Alessandrescu-Rusescu" National Institute for Mother and Child Health, Bucharest from January 1, 2018, to December 31, 2022. The study included patients under 18 years of age registered at the Regional Cystic Fibrosis Center "Alessandrescu-Rusescu" National Institute for Mother and Child Health, Bucharest. Anthropometric measurements and pulmonary function tests (FEV1) were analyzed, along with data on infections with Pseudomonas aeruginosa and methicillin-resistant Staphylococcus aureus (MRSA). The study cohort consisted of 51 patients with a mean age of 78 months. Nutritional deficiencies were observed in 72.5% of the patients, with mean weight and height values below the average for healthy populations. The mean FEV1 value among patients over 6 years old was 83.14%, indicating varying degrees of lung function impairment. Infections with Pseudomonas aeruginosa were detected in 35% of the patients, while MRSA infections were found in 11.76%. No statistical correlation was found between these infections and nutritional deficits. The findings underscore the critical interplay between nutritional status and pulmonary function in CF patients. A significant portion of the cohort exhibited nutritional deficiencies, which were associated with poorer pulmonary outcomes. The study highlights the importance of comprehensive nutritional and pulmonary management to improve health outcomes in pediatric CF patients. Further research is needed to explore additional factors influencing the relationship between nutrition and lung function in this population.

References

[1]V. Scotet, H. Gutierrez, and P. M. Farrell, “Neonatal Screening Review Newborn Screening for CF across the Globe-Where Is It Worthwhile?”, doi: 10.3390/ijns6010018.
[2]J. Guo, A. Garratt, and A. Hill, “Worldwide rates of diagnosis and effective treatment for cystic fibrosis,” J Cyst Fibros, vol. 21, no. 3, pp. 456–462, May 2022, doi: 10.1016/J.JCF.2022.01.009.
[3]W. B. Guggino and S. P. Banks-Schlegel, “Macromolecular interactions and ion transport in cystic fibrosis,” Am J Respir Crit Care Med, vol. 170, no. 7, pp. 815–820, Oct. 2004, doi: 10.1164/RCCM.200403-381WS.
[4]M. J. Goldman, Y. Yang, and J. M. Wilson, “Gene therapy in a xenograft model of cystic fibrosis lung corrects chloride transport more effectively than the sodium defect,” Nature Genetics 1995 9:2, vol. 9, no. 2, pp. 126–131, 1995, doi: 10.1038/ng0295-126.
[5]M. P. Boyle and K. De Boeck, “A new era in the treatment of cystic fibrosis: correction of the underlying CFTR defect,” Lancet Respir Med, vol. 1, no. 2, pp. 158–163, Apr. 2013, doi: 10.1016/S2213-2600(12)70057-7.
[6]J. E. Simpson, L. R. Gawenis, N. M. Walker, K. T. Boyle, and L. L. Clarke, “Chloride conductance of CFTR facilitates basal Cl-/HCO3- exchange in the villous epithelium of intact murine duodenum,” Am J Physiol Gastrointest Liver Physiol, vol. 288, no. 6, Jun. 2005, doi: 10.1152/AJPGI.00493.2004.
[7]E. M. Bradford, M. A. Sartor, L. R. Gawenis, L. L. Clarke, and G. E. Shull, “Reduced NHE3-mediated Na+ absorption increases survival and decreases the incidence of intestinal obstructions in cystic fibrosis mice,” Am J Physiol Gastrointest Liver Physiol, vol. 296, no. 4, Apr. 2009, doi: 10.1152/AJPGI.90520.2008.
[8]M. Rowland et al., “Outcome in cystic fibrosis liver disease,” Am J Gastroenterol, vol. 106, no. 1, pp. 104–109, Jan. 2011, doi: 10.1038/AJG.2010.316.
[9]K. Moyer and W. Balistreri, “Hepatobiliary disease in patients with cystic fibrosis,” Curr Opin Gastroenterol, vol. 25, no. 3, pp. 272–278, May 2009, doi: 10.1097/MOG.0B013E3283298865.
[10]P. J. Milla, “Cystic fibrosis: present and future,” Digestion, vol. 59, no. 5, pp. 579–588, Aug. 1998, doi: 10.1159/000007533.
[11]B. S. Zemel, A. F. Jawad, S. FitzSimmons, and V. A. Stallings, “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, vol. 137, no. 3, pp. 374–380, Sep. 2000, doi: 10.1067/MPD.2000.107891.
[12]E. H. Yen, H. Quinton, and D. Borowitz, “Better Nutritional Status in Early Childhood Is Associated with Improved Clinical Outcomes and Survival in Patients with Cystic Fibrosis,” J Pediatr, vol. 162, no. 3, pp. 530-535.e1, Mar. 2013, doi: 10.1016/J.JPEDS.2012.08.040.
[13]C. R. M. de M. Chaves, J. A. A. de Britto, C. Q. de Oliveira, M. M. Gomes, and A. L. P. da Cunha, “Association between nutritional status measurements and pulmonary function in children and adolescents with cystic fibrosis,” Jornal Brasileiro de Pneumologia, vol. 35, no. 5, pp. 409–414, 2009, doi: 10.1590/S1806-37132009000500004.
[14]J. A. Alvarez, T. R. Ziegler, E. C. Millson, and A. A. Stecenko, “Body composition and lung function in cystic fibrosis and their association with adiposity and normal-weight obesity,” Nutrition, vol. 32, no. 4, pp. 447–452, Apr. 2016, doi: 10.1016/J.NUT.2015.10.012.
[15]A. M. Salazar-Solarte, L. Torres-Canchala, F. Carrera-Gil, and D. M. Quimbayo-Wilches, “Pulmonary function and body composition parameters of children with cystic fibrosis from a hight complexity institution between 2015 and 2018,” Pediatr Neonatol, Apr. 2024, doi: 10.1016/J.PEDNEO.2023.11.006.
[16]A. A. Kilinc, O. F. Beser, E. P. Ugur, F. C. Cokugras, and H. Cokugras, “The effects of nutritional status and intervention on pulmonary functions in pediatric cystic fibrosis patients,” Pediatrics International, vol. 63, no. 3, pp. 316–322, Mar. 2021, Accessed: Jul. 04, 2024. [Online]. Available: https://www.academia.edu/117362395/The_effects_of_nutritional_status_and_intervention_on_pulmonary_functions_in_pediatric_cystic_fibrosis_patients
[17]D. B. Sanders et al., “Early Life Growth Trajectories in Cystic Fibrosis are Associated with Pulmonary Function at Age 6 Years,” J Pediatr, vol. 167, no. 5, p. 1081, Nov. 2015, doi: 10.1016/J.JPEDS.2015.07.044.
[18]A. A. Kilinc, O. F. Beser, E. P. Ugur, F. C. Cokugras, and H. Cokugras, “The effects of nutritional status and intervention on pulmonary functions in pediatric cystic fibrosis patients,” Pediatrics International, vol. 63, no. 3, pp. 316–322, Mar. 2021, doi: 10.1111/PED.14417.
[19]B. S. Zemel, A. F. Jawad, S. FitzSimmons, and V. A. Stallings, “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, vol. 137, no. 3, pp. 374–380, Sep. 2000, doi: 10.1067/MPD.2000.107891.
[20]K. L. Palmer, L. M. Aye, and M. Whiteley, “Nutritional cues control Pseudomonas aeruginosa multicellular behavior in cystic fibrosis sputum,” J Bacteriol, vol. 189, no. 22, pp. 8079–8087, Nov. 2007, doi: 10.1128/JB.01138-07/SUPPL_FILE/SUPPLEMENTAL_TABLE_6.ZIP.
[21]A. Oliver, R. Cantón, P. Campo, F. Baquero, and J. Blázquez, “High Frequency of Hypermutable Pseudomonas aeruginosa in Cystic Fibrosis Lung Infection,” Science (1979), vol. 288, no. 5469, pp. 1251–1253, May 2000, doi: 10.1126/SCIENCE.288.5469.1251.
[22] J. C. Davies, “Pseudomonas aeruginosa in cystic fibrosis: pathogenesis and persistence,” Paediatr Respir Rev, vol. 3, no. 2, pp. 128–134, Jun. 2002, doi: 10.1016/S1526-0550(02)00003-3.
[23] C. Rumpf, J. Lange, B. Schwartbeck, and B. C. Kahl, “Staphylococcus aureus and Cystic Fibrosis—A Close Relationship. What Can We Learn from Sequencing Studies?,” Pathogens, vol. 10, no. 9, Sep. 2021, doi: 10.3390/PATHOGENS10091177.