Associations between peak oxygen uptake, lung function, and bronchiectasis in children with cystic fibrosis in the era of CFTR modulators.
cardiopulmonary exercise test
cystic fibrosis
peak oxygen uptake
pulmonary exacerbation
Journal
Pediatric pulmonology
ISSN: 1099-0496
Titre abrégé: Pediatr Pulmonol
Pays: United States
ID NLM: 8510590
Informations de publication
Date de publication:
06 2021
06 2021
Historique:
revised:
07
01
2021
received:
08
11
2020
accepted:
12
01
2021
pubmed:
19
1
2021
medline:
25
11
2021
entrez:
18
1
2021
Statut:
ppublish
Résumé
With the emergence of cystic fibrosis transmembrane conductance regulator (CFTR) modulators, forced expiratory volume in 1 s (FEV Spirometry and a maximal cardiopulmonary exercise test (CPET) were performed on the same day and compared to markers of disease severity. Markers of disease severity included a number of pulmonary exacerbations resulting in hospital admission within the preceding 12 months, body mass index, Pseudomonas aeruginosa (PsA) infection, and bronchiectasis. Fifty-two subjects (24 female) with CF participated in the study with a mean (SD) age of 13.8 (2.4) years, range 8-18 years. Forty-nine participants met satisfactory criteria for a maximal CPET. A significant correlation was found between relative VO In children with CF who have mild pulmonary disease, there is significant correlation between FEV
Sections du résumé
BACKGROUND
With the emergence of cystic fibrosis transmembrane conductance regulator (CFTR) modulators, forced expiratory volume in 1 s (FEV
METHODS
Spirometry and a maximal cardiopulmonary exercise test (CPET) were performed on the same day and compared to markers of disease severity. Markers of disease severity included a number of pulmonary exacerbations resulting in hospital admission within the preceding 12 months, body mass index, Pseudomonas aeruginosa (PsA) infection, and bronchiectasis.
RESULTS
Fifty-two subjects (24 female) with CF participated in the study with a mean (SD) age of 13.8 (2.4) years, range 8-18 years. Forty-nine participants met satisfactory criteria for a maximal CPET. A significant correlation was found between relative VO
CONCLUSIONS
In children with CF who have mild pulmonary disease, there is significant correlation between FEV
Substances chimiques
CFTR protein, human
0
Cystic Fibrosis Transmembrane Conductance Regulator
126880-72-6
Oxygen
S88TT14065
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1490-1495Informations de copyright
© 2021 Wiley Periodicals LLC.
Références
Nixon PA, Orenstein DM, Kelsey SF, Doershuk CF. The prognostic value of exercise testing in patients with cystic fibrosis. N Engl J Med. 1992;327(25):1785-1788.
Hebestreit H, Hulzebos EHJ, Schneiderman JE, et al. Cardiopulmonary exercise testing provides additional prognostic information in cystic fibrosis. Am J Respir Crit Care Med. 2019;199(8):987-995.
Pianosi P, Leblanc J, Almudevar A. Peak oxygen uptake and mortality in children with cystic fibrosis. Thorax. 2005;60(1):50-54.
Hulzebos EHJ, Bomhof-Roordink H, van de Weert-van Leeuwen PB, et al. Prediction of mortality in adolescents with cystic fibrosis. Med Sci Sports Exerc. 2014;46(11):2047-2052.
Vendrusculo FM, Heinzmann-Filho JP, da Silva JS, Perez Ruiz M, Donadio MVF. Peak oxygen uptake and mortality in cystic fibrosis: systematic review and meta-analysis. Respir Care. 2019;64(1):91-98.
Button BM, Wilson C, Dentice R, et al. Physiotherapy for cystic fibrosis in Australia and New Zealand: a clinical practice guideline. Respirology. 2016;21(4):656-667.
Hebestreit H, Arets HGM, Aurora P, et al. Statement on exercise testing in cystic fibrosis. Respiration. 2015;90(4):332-351.
Lang RL, Stockton K, Wilson C, Russell TG, Johnston LM. Exercise testing for children with cystic fibrosis: a systematic review. Pediatr Pulmonol. 2020;55(8):1996-2010.
Graham BL, Steenbruggen I, Miller MR, et al. Standardization of Spirometry 2019 Update. An official American Thoracic Society and European Respiratory Society Technical Statement. Am J Respir Crit Care Med. 2019;200(8):e70-e88.
Quanjer PH, Stanojevic S, Cole TJ, et al. Multi-ethnic reference values for spirometry for the 3-95-yr age range: the global lung function 2012 equations. Eur Respir J. 2012;40(6):1324-1343.
Wasserman K, Hensen JE, Sue DY, et al. Principles of Exercise Testing and Interpretation. Philadelphia, PA: Lippincott Williams & Wilkins; 2005:585.
Wasserman K, Whipp BJ, Koyl SN, Beaver WL. Anaerobic threshold and respiratory gas exchange during exercise. J Appl Physiol. 1973;35(2):236-243.
Shimizu M, Myers J, Buchanan N, et al. The ventilatory threshold: method, protocol, and evaluator agreement. Am Heart J. 1991;122(2):509-516.
Radtke T, Crook S, Kaltsakas G, et al. ERS statement on standardisation of cardiopulmonary exercise testing in chronic lung diseases. Eur Respir Rev. 2019;28:154.
Urquhart DS, Vendrusculo FM. Clinical interpretation of cardiopulmonary exercise testing in cystic fibrosis and implications for exercise counselling. Paediatr Respir Rev. 2017;24:72-78.
Hebestreit H, Kieser S, Junge S, et al. Long-term effects of a partially supervised conditioning programme in cystic fibrosis. Eur Respir J. 2010;35(3):578-583.
Radtke T, Nevitt SJ, Hebestreit H, Kriemler S. Physical exercise training for cystic fibrosis. Cochrane Database Syst Rev. 2017;11:CD002768.
Paranjape SM, Barnes LA, Carson KA, von Berg K, Loosen H, Mogayzel PJ. Exercise improves lung function and habitual activity in children with cystic fibrosis. J Cyst Fibros. 2012;11(1):18-23.
Weir E, Burns PD, Devenny A, Young D, Paton JY. Cardiopulmonary exercise testing in children with cystic fibrosis: one centre's experience. Arch Dis Child. 2017;102(5):440-444.
Edgeworth D, Keating D, Ellis M, et al. Improvement in exercise duration, lung function and well-being in G551D-cystic fibrosis patients: a double-blind, placebo-controlled, randomized, cross-over study with ivacaftor treatment. Clin Sci (Lond). 2017;131(15):2037-2045.
Middleton PG, Mall MA, Dřevínek P, et al. Elexacaftor-tezacaftor-ivacaftor for cystic fibrosis with a single Phe508del allele. N Engl J Med. 2019;381(19):1809-1819.
Pérez M, Groeneveld IF, Santana-Sosa E, et al. Aerobic fitness is associated with lower risk of hospitalization in children with cystic fibrosis. Pediatr Pulmonol. 2014;49(7):641-649.
Wilkes DL, Schneiderman JE, Nguyen T, et al. Exercise and physical activity in children with cystic fibrosis. Paediatr Respir Rev. 2009;10(3):105-109.
Bhatt JM. Treatment of pulmonary exacerbations in cystic fibrosis. Eur Respir Rev. 2013;22(129):205-216.
Welsh L, Robertson CF, Ranganathan SC. Increased rate of lung function decline in Australian adolescents with cystic fibrosis. Pediatr Pulmonol. 2014;49(9):873-877.
Cropp GJ, et al. Exercise tolerance and cardiorespiratory adjustments at peak work capacity in cystic fibrosis. Am Rev Respir Dis. 1982;126(2):211-216.
Pastré J, Prévotat A, Tardif C, Langlois C, Duhamel A, Wallaert B. Determinants of exercise capacity in cystic fibrosis patients with mild-to-moderate lung disease. BMC Pulm Med. 2014;14:74.
Almajed A, Lands LC. The evolution of exercise capacity and its limiting factors in cystic fibrosis. Paediatr Respir Rev. 2012;13(4):195-199.
Vandenbranden SL, McMullen A, Schechter MS, et al. Lung function decline from adolescence to young adulthood in cystic fibrosis. Pediatr Pulmonol. 2012;47(2):135-143.
Radtke T, Hebestreit H, Gallati S, et al. CFTR Genotype and maximal exercise capacity in cystic fibrosis: a cross-sectional study. Ann Am Thorac Soc. 2018;15(2):209-216.
Oikonomou A, Manavis J, Karagianni P, et al. Loss of FEV1 in cystic fibrosis: correlation with HRCT features. Eur Radiol. 2002;12(9):2229-2235.
Bar-Yoseph R, Ilivitzki A, Cooper DM, et al. Exercise capacity in patients with cystic fibrosis vs. non-cystic fibrosis bronchiectasis. PLOS One. 2019;14(6):e0217491.
Marcotte JE, Canny GJ, Grisdale R, et al. Effects of nutritional status on exercise performance in advanced cystic fibrosis. Chest. 1986;90(3):375-379.