REFERENCES
1. Meoli A, Fainardi V, Deolmi M,
Chiopris G, Marinelli F, Caminiti C, Esposito S, Pisi G. State of the
Art on Approved Cystic Fibrosis Transmembrane Conductance Regulator
(CFTR) Modulators and Triple-Combination Therapy. Pharmaceuticals
2021;14(9):928.
2. Wainwright CE, Elborn JS, Ramsey
BW, Marigowda G, Huang X, Cipolli M, Colombo C, Davies JC, De Boeck K,
Flume PA. Lumacaftor–ivacaftor in patients with cystic fibrosis
homozygous for Phe508del CFTR. New England Journal of Medicine
2015;373(3):220-231.
3. Keating D, Marigowda G, Burr L,
Daines C, Mall MA, McKone EF, Ramsey BW, Rowe SM, Sass LA, Tullis E.
VX-445–tezacaftor–ivacaftor in patients with cystic fibrosis and one
or two Phe508del alleles. New england journal of medicine
2018;379(17):1612-1620.
4. Hebestreit H, Schmid K, Kieser S,
Junge S, Ballmann M, Roth K, Hebestreit A, Schenk T, Schindler C,
Posselt H-G. Quality of life is associated with physical activity and
fitness in cystic fibrosis. BMC pulmonary medicine 2014;14(1):26.
5. Pérez M, Groeneveld IF,
Santana‐Sosa E, Fiuza‐Luces C, Gonzalez‐Saiz L, Villa‐Asensi JR,
López‐Mojares LM, Rubio M, Lucia A. Aerobic fitness is associated with
lower risk of hospitalization in children with cystic fibrosis.
Pediatric pulmonology 2014;49(7):641-649.
6. Hebestreit H, Hulzebos EH,
Schneiderman JE, Karila C, Boas SR, Kriemler S, Dwyer T, Sahlberg M,
Urquhart DS, Lands LC. Cardiopulmonary exercise testing provides
additional prognostic information in cystic fibrosis. American journal
of respiratory and critical care medicine 2018(ja).
7. Zemanick ET, Taylor-Cousar JL,
Davies J, Gibson RL, Mall MA, McKone EF, McNally P, Ramsey BW, Rayment
JH, Rowe SM. A phase 3 open-label study of
elexacaftor/tezacaftor/ivacaftor in children 6 through 11 years of age
with cystic fibrosis and at least one F508del allele. American Journal
of Respiratory and Critical Care Medicine 2021;203(12):1522-1532.
8. Causer AJ, Shute JK, Cummings MH,
Shepherd AI, Bright V, Connett G, Allenby MI, Carroll MP, Daniels T,
Saynor ZL. Cardiopulmonary exercise testing with supramaximal
verification produces a safe and valid assessment of VO2max in people
with cystic fibrosis: a retrospective analysis. Journal of Applied
Physiology 2018;125(4):1277-1283.
9. Saynor ZL, Barker AR, Oades PJ,
Williams CA. A protocol to determine valid V˙ O2max in young cystic
fibrosis patients. Journal of science and medicine in sport
2013;16(6):539-544.
10. Saynor ZL, Barker AR, Oades PJ,
Williams CA. Reproducibility of maximal cardiopulmonary exercise testing
for young cystic fibrosis patients. Journal of Cystic Fibrosis
2013;12(6):644-650.
11. Hildebrand M, VT VH, Hansen BH,
Ekelund U. Age group comparability of raw accelerometer output from
wrist-and hip-worn monitors. Medicine and science in sports and exercise
2014;46(9):1816-1824.
12. Savi D, Schiavetto S, Simmonds
NJ, Righelli D, Palange P. Effects of Lumacaftor/Ivacaftor on physical
activity and exercise tolerance in three adults with cystic fibrosis.
Journal of Cystic Fibrosis 2019;18(3):420-424.
13. Saynor ZL, Barker AR, Oades PJ,
Williams CA. The effect of ivacaftor in adolescents with cystic fibrosis
(G551D mutation): an exercise physiology perspective. Pediatric Physical
Therapy 2014;26(4):454-461.
14. Lamhonwah AM, Bear, C.E., Huan,
L.J., Chiaw, P.K., Ackerley, C.A. and Tein, I. Cystic fibrosis
transmembrane conductance regulator in human muscle: dysfunction causes
abnormal metabolic recovery in exercise. Annals of neurology
2010;67(6):802-808.
15. Valdivieso AG, Santa-Coloma TA.
CFTR activity and mitochondrial function. Redox biology
2013;1(1):190-202.
16. Edgeworth D, Keating D, Ellis M,
Button B, Williams E, Clark D, Tierney A, Heritier S, Kotsimbos T,
Wilson J. 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. Clinical Science
2017;131(15):2037-2045.
Table 1. Clinical and physical activity characteristics of the
cases before and after 6 weeks treatment with
Elexacaftor-Tezacaftor-Ivacaftor.
Figure 1. Measurements of
exercise capacity before and after 6 weeks treatment with
Elexacaftor-Tezacaftor-Ivacaftor. AT, anaerobic threshold;\(\dot{V}\)O2, pulmonary oxygen uptake.