-
Je něco špatně v tomto záznamu ?
The long-term effect of elexacaftor/tezacaftor/ivacaftor on cardiorespiratory fitness in adolescent patients with cystic fibrosis: a pilot observational study
N. Stastna, L. Hrabovska, P. Homolka, L. Homola, M. Svoboda, K. Brat, L. Fila
Jazyk angličtina Země Anglie, Velká Británie
Typ dokumentu časopisecké články, pozorovací studie
NLK
BioMedCentral
od 2001-12-01
BioMedCentral Open Access
od 2001
Directory of Open Access Journals
od 2001
Free Medical Journals
od 2001
PubMed Central
od 2001
Europe PubMed Central
od 2001
ProQuest Central
od 2009-01-01
Open Access Digital Library
od 2001-09-01
Open Access Digital Library
od 2001-01-01
Open Access Digital Library
od 2001-01-01
Medline Complete (EBSCOhost)
od 2001-01-01
Health & Medicine (ProQuest)
od 2009-01-01
ROAD: Directory of Open Access Scholarly Resources
od 2001
Springer Nature OA/Free Journals
od 2001-12-01
- MeSH
- aminofenoly * terapeutické užití MeSH
- benzodioxoly * terapeutické užití MeSH
- chinolony * terapeutické užití MeSH
- cystická fibróza * farmakoterapie patofyziologie MeSH
- dítě MeSH
- fixní kombinace léků * MeSH
- indoly * terapeutické užití MeSH
- kardiorespirační zdatnost MeSH
- lidé MeSH
- mladiství MeSH
- pilotní projekty MeSH
- prospektivní studie MeSH
- pyrazoly * terapeutické užití MeSH
- pyridiny * terapeutické užití MeSH
- pyrrolidiny MeSH
- pyrroly terapeutické užití MeSH
- spotřeba kyslíku MeSH
- tolerance zátěže účinky léků MeSH
- zátěžový test MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- pozorovací studie MeSH
BACKGROUND: Physical activity is a crucial demand on cystic fibrosis treatment management. The highest value of oxygen uptake (VO2peak) is an appropriate tool to evaluate the physical activity in these patients. However, there are several other valuable CPET parameters describing exercise tolerance (Wpeak, VO2VT1, VO2VT2, VO2/HRpeak, etc.), and helping to better understand the effect of specific treatment (VE, VT, VD/VT etc.). Limited data showed ambiguous results of this improvement after CFTR modulator treatment. Elexacaftor/tezacaftor/ivacaftor medication improves pulmonary function and quality of life, whereas its effect on CPET has yet to be sufficiently demonstrated. METHODS: We performed a single group prospective observational study of 10 adolescent patients with cystic fibrosis who completed two CPET measurements between January 2019 and February 2023. During this period, elexacaftor/tezacaftor/ivacaftor treatment was initiated in all of them. The first CPET at the baseline was followed by controlled CPET at least one year after medication commencement. We focused on interpreting the data on their influence by the novel therapy. We hypothesized improvements in cardiorespiratory fitness following treatment. We applied the Wilcoxon signed-rank test. The data were adjusted for age at the time of CPET to eliminate bias of aging in adolescent patients. RESULTS: We observed significant improvement in peak workload, VO2 peak, VO2VT1, VO2VT2, VE/VCO2 slope, VE, VT, RQ, VO2/HR peak and RR peak. The mean change in VO2 peak was 5.7 mL/kg/min, or 15.9% of the reference value (SD ± 16.6; p= 0.014). VO2VT1 improved by 15% of the reference value (SD ± 0.1; p= 0.014), VO2VT2 improved by 0.5 (SD ± 0.4; p= 0.01). There were no differences in other parameters. CONCLUSION: Exercise tolerance improved after elexacaftor/tezacaftor/ivacaftor treatment initiation. We suggest that the CFTR modulator alone is not enough for recovering physical decondition, but should be supplemented with physical activity and respiratory physiotherapy. Further studies are needed to examine the effect of CFTR modulators and physical therapy on cardiopulmonary exercise tolerance.
Citace poskytuje Crossref.org
- 000
- 00000naa a2200000 a 4500
- 001
- bmc24013847
- 003
- CZ-PrNML
- 005
- 20240905133922.0
- 007
- ta
- 008
- 240725s2024 enk f 000 0|eng||
- 009
- AR
- 024 7_
- $a 10.1186/s12890-024-03069-8 $2 doi
- 035 __
- $a (PubMed)38807122
- 040 __
- $a ABA008 $b cze $d ABA008 $e AACR2
- 041 0_
- $a eng
- 044 __
- $a enk
- 100 1_
- $a Stastna, Nela $u Department of Pulmonology, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic. nela.stastna@fnmotol.cz $u Faculty of Medicine, Masaryk University, Brno, Czech Republic. nela.stastna@fnmotol.cz
- 245 14
- $a The long-term effect of elexacaftor/tezacaftor/ivacaftor on cardiorespiratory fitness in adolescent patients with cystic fibrosis: a pilot observational study / $c N. Stastna, L. Hrabovska, P. Homolka, L. Homola, M. Svoboda, K. Brat, L. Fila
- 520 9_
- $a BACKGROUND: Physical activity is a crucial demand on cystic fibrosis treatment management. The highest value of oxygen uptake (VO2peak) is an appropriate tool to evaluate the physical activity in these patients. However, there are several other valuable CPET parameters describing exercise tolerance (Wpeak, VO2VT1, VO2VT2, VO2/HRpeak, etc.), and helping to better understand the effect of specific treatment (VE, VT, VD/VT etc.). Limited data showed ambiguous results of this improvement after CFTR modulator treatment. Elexacaftor/tezacaftor/ivacaftor medication improves pulmonary function and quality of life, whereas its effect on CPET has yet to be sufficiently demonstrated. METHODS: We performed a single group prospective observational study of 10 adolescent patients with cystic fibrosis who completed two CPET measurements between January 2019 and February 2023. During this period, elexacaftor/tezacaftor/ivacaftor treatment was initiated in all of them. The first CPET at the baseline was followed by controlled CPET at least one year after medication commencement. We focused on interpreting the data on their influence by the novel therapy. We hypothesized improvements in cardiorespiratory fitness following treatment. We applied the Wilcoxon signed-rank test. The data were adjusted for age at the time of CPET to eliminate bias of aging in adolescent patients. RESULTS: We observed significant improvement in peak workload, VO2 peak, VO2VT1, VO2VT2, VE/VCO2 slope, VE, VT, RQ, VO2/HR peak and RR peak. The mean change in VO2 peak was 5.7 mL/kg/min, or 15.9% of the reference value (SD ± 16.6; p= 0.014). VO2VT1 improved by 15% of the reference value (SD ± 0.1; p= 0.014), VO2VT2 improved by 0.5 (SD ± 0.4; p= 0.01). There were no differences in other parameters. CONCLUSION: Exercise tolerance improved after elexacaftor/tezacaftor/ivacaftor treatment initiation. We suggest that the CFTR modulator alone is not enough for recovering physical decondition, but should be supplemented with physical activity and respiratory physiotherapy. Further studies are needed to examine the effect of CFTR modulators and physical therapy on cardiopulmonary exercise tolerance.
- 650 _2
- $a lidé $7 D006801
- 650 12
- $a cystická fibróza $x farmakoterapie $x patofyziologie $7 D003550
- 650 _2
- $a mladiství $7 D000293
- 650 _2
- $a mužské pohlaví $7 D008297
- 650 _2
- $a ženské pohlaví $7 D005260
- 650 _2
- $a prospektivní studie $7 D011446
- 650 _2
- $a pilotní projekty $7 D010865
- 650 12
- $a indoly $x terapeutické užití $7 D007211
- 650 12
- $a benzodioxoly $x terapeutické užití $7 D052117
- 650 12
- $a chinolony $x terapeutické užití $7 D015363
- 650 12
- $a aminofenoly $x terapeutické užití $7 D000627
- 650 12
- $a pyrazoly $x terapeutické užití $7 D011720
- 650 12
- $a pyridiny $x terapeutické užití $7 D011725
- 650 12
- $a fixní kombinace léků $7 D004338
- 650 _2
- $a kardiorespirační zdatnost $7 D000072599
- 650 _2
- $a zátěžový test $7 D005080
- 650 _2
- $a pyrroly $x terapeutické užití $7 D011758
- 650 _2
- $a tolerance zátěže $x účinky léků $7 D017079
- 650 _2
- $a spotřeba kyslíku $7 D010101
- 650 _2
- $a dítě $7 D002648
- 650 _2
- $a pyrrolidiny $7 D011759
- 655 _2
- $a časopisecké články $7 D016428
- 655 _2
- $a pozorovací studie $7 D064888
- 700 1_
- $a Hrabovska, Lenka $u Department of Sports Medicine and Rehabilitation, St. Anne's University Hospital and Faculty of Medicine, Masaryk University, Brno, Czech Republic
- 700 1_
- $a Homolka, Pavel $u Department of Sports Medicine and Rehabilitation, St. Anne's University Hospital and Faculty of Medicine, Masaryk University, Brno, Czech Republic
- 700 1_
- $a Homola, Lukas $u Department of Paediatric Infectious Diseases, University Hospital Brno and Faculty of Medicine, Masaryk University, Brno, Czech Republic
- 700 1_
- $a Svoboda, Michal $u Institute of Biostatistics and Analyses Ltd. and Faculty of Medicine, Masaryk University, Brno, Czech Republic
- 700 1_
- $a Brat, Kristian $u Department of Pulmonology, University Hospital Brno and Faculty of Medicine, Masaryk University, Brno, Czech Republic
- 700 1_
- $a Fila, Libor $u Department of Pulmonology, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic
- 773 0_
- $w MED00008206 $t BMC pulmonary medicine $x 1471-2466 $g Roč. 24, č. 1 (2024), s. 260
- 856 41
- $u https://pubmed.ncbi.nlm.nih.gov/38807122 $y Pubmed
- 910 __
- $a ABA008 $b sig $c sign $y - $z 0
- 990 __
- $a 20240725 $b ABA008
- 991 __
- $a 20240905133916 $b ABA008
- 999 __
- $a ok $b bmc $g 2143576 $s 1225713
- BAS __
- $a 3
- BAS __
- $a PreBMC-MEDLINE
- BMC __
- $a 2024 $b 24 $c 1 $d 260 $e 20240528 $i 1471-2466 $m BMC pulmonary medicine $n BMC Pulm Med $x MED00008206
- LZP __
- $a Pubmed-20240725
