Long-Term exercise intervention in patients with McArdle disease: clinical and aerobic fitness benefits
Introduction: The long-term effects of exercise in patients with McArdle disease—the paradigm of ‘exercise intolerance’—are unknown. This is an important question as the severity of the disease frequently increases with time. Purpose: To study the effects of a long-term exercise intervention on clin...
| Autores: | , , , , , , , , , , , , |
|---|---|
| Formato: | artículo |
| Fecha de publicación: | 2022 |
| País: | España |
| Recursos: | Universidad de Castilla-La Mancha |
| Repositorio: | RUIdeRA. Repositorio Institucional de la UCLM |
| OAI Identifier: | oai:ruidera.uclm.es:10578/29633 |
| Acesso em linha: | http://hdl.handle.net/10578/29633 |
| Access Level: | acceso abierto |
| Palavra-chave: | Myophosphorylase Exercise is medicine Glycogenosys type 5 Glycogen storage disease |
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Long-Term exercise intervention in patients with McArdle disease: clinical and aerobic fitness benefitsSantalla Hernández, AlfredoValenzuela, Pedro L.Rodríguez López, CarlosRodríguez Gómez, IreneNogales, GiselaPinós Figueras, TomásArenas, JoaquínMartín, Miguel ÁngelSantos Lozano, AlejandroMorán, MaríaFiuza Luces, CarmenAra Royo, IgnacioLucia, AlejandroMyophosphorylaseExercise is medicineGlycogenosys type 5Glycogen storage diseaseIntroduction: The long-term effects of exercise in patients with McArdle disease—the paradigm of ‘exercise intolerance’—are unknown. This is an important question as the severity of the disease frequently increases with time. Purpose: To study the effects of a long-term exercise intervention on clinical and fitness-related outcomes in McArdle patients. Methods: Seventeen patients (exercise group: N=10, 6 male, 38±18yrs; control: N=7, 4 male, 38±18yrs) participated in a twoyear unsupervised intervention including moderate-intensity aerobic (cycle-ergometer exercise for 1h) and resistance (high load-low repetition circuit) training on 5 and 2-3 days/week, respectively. Patients were assessed at baseline and postintervention. Besides safety, outcomes included clinical severity (e.g., exercise intolerance features) on a 0-3 scale (primary outcome), and aerobic fitness, gross muscle efficiency, and body composition (total/regional fat, muscle, and bone mass) (secondary outcomes). Results: The exercise program was safe and resulted in a reduction of one point (-1.0, 95% confidence interval -1.6—-0.5, p=0.025) in clinical severity vs. the control group, with 60% of participants in the exercise group becoming virtually asymptomatic and with no functional limitation in daily life activities. Compared with controls, the intervention induced significant and large benefits (all p<0.05) in the workload eliciting the ventilatory threshold (both in absolute (watts, +37%) and relative units (watts·kg-1 of total body mass or of lower-limb muscle mass, +44%)), peak oxygen uptake (ml·kg-1 ·min-1 , +28%) and peak workload (absolute (+27%) and relative units (+33%)). However, no significant changes were found for muscle efficiency nor for any measure of body composition. Conclusions: A two-year unsupervised intervention including aerobic and resistance exercise is safe and induces major benefits in the clinical course and aerobic fitness of patients with McArdle disease.The American College of Sports Medicine202220222022info:eu-repo/semantics/articleapplication/pdfapplication/pdfhttp://hdl.handle.net/10578/29633reponame:RUIdeRA. Repositorio Institucional de la UCLMinstname:Universidad de Castilla-La ManchaInglésinfo:eu-repo/semantics/openAccessoai:ruidera.uclm.es:10578/296332026-05-27T07:36:41Z |
| dc.title.none.fl_str_mv |
Long-Term exercise intervention in patients with McArdle disease: clinical and aerobic fitness benefits |
| title |
Long-Term exercise intervention in patients with McArdle disease: clinical and aerobic fitness benefits |
| spellingShingle |
Long-Term exercise intervention in patients with McArdle disease: clinical and aerobic fitness benefits Santalla Hernández, Alfredo Myophosphorylase Exercise is medicine Glycogenosys type 5 Glycogen storage disease |
| title_short |
Long-Term exercise intervention in patients with McArdle disease: clinical and aerobic fitness benefits |
| title_full |
Long-Term exercise intervention in patients with McArdle disease: clinical and aerobic fitness benefits |
| title_fullStr |
Long-Term exercise intervention in patients with McArdle disease: clinical and aerobic fitness benefits |
| title_full_unstemmed |
Long-Term exercise intervention in patients with McArdle disease: clinical and aerobic fitness benefits |
| title_sort |
Long-Term exercise intervention in patients with McArdle disease: clinical and aerobic fitness benefits |
| dc.creator.none.fl_str_mv |
Santalla Hernández, Alfredo Valenzuela, Pedro L. Rodríguez López, Carlos Rodríguez Gómez, Irene Nogales, Gisela Pinós Figueras, Tomás Arenas, Joaquín Martín, Miguel Ángel Santos Lozano, Alejandro Morán, María Fiuza Luces, Carmen Ara Royo, Ignacio Lucia, Alejandro |
| author |
Santalla Hernández, Alfredo |
| author_facet |
Santalla Hernández, Alfredo Valenzuela, Pedro L. Rodríguez López, Carlos Rodríguez Gómez, Irene Nogales, Gisela Pinós Figueras, Tomás Arenas, Joaquín Martín, Miguel Ángel Santos Lozano, Alejandro Morán, María Fiuza Luces, Carmen Ara Royo, Ignacio Lucia, Alejandro |
| author_role |
author |
| author2 |
Valenzuela, Pedro L. Rodríguez López, Carlos Rodríguez Gómez, Irene Nogales, Gisela Pinós Figueras, Tomás Arenas, Joaquín Martín, Miguel Ángel Santos Lozano, Alejandro Morán, María Fiuza Luces, Carmen Ara Royo, Ignacio Lucia, Alejandro |
| author2_role |
author author author author author author author author author author author author |
| dc.subject.none.fl_str_mv |
Myophosphorylase Exercise is medicine Glycogenosys type 5 Glycogen storage disease |
| topic |
Myophosphorylase Exercise is medicine Glycogenosys type 5 Glycogen storage disease |
| description |
Introduction: The long-term effects of exercise in patients with McArdle disease—the paradigm of ‘exercise intolerance’—are unknown. This is an important question as the severity of the disease frequently increases with time. Purpose: To study the effects of a long-term exercise intervention on clinical and fitness-related outcomes in McArdle patients. Methods: Seventeen patients (exercise group: N=10, 6 male, 38±18yrs; control: N=7, 4 male, 38±18yrs) participated in a twoyear unsupervised intervention including moderate-intensity aerobic (cycle-ergometer exercise for 1h) and resistance (high load-low repetition circuit) training on 5 and 2-3 days/week, respectively. Patients were assessed at baseline and postintervention. Besides safety, outcomes included clinical severity (e.g., exercise intolerance features) on a 0-3 scale (primary outcome), and aerobic fitness, gross muscle efficiency, and body composition (total/regional fat, muscle, and bone mass) (secondary outcomes). Results: The exercise program was safe and resulted in a reduction of one point (-1.0, 95% confidence interval -1.6—-0.5, p=0.025) in clinical severity vs. the control group, with 60% of participants in the exercise group becoming virtually asymptomatic and with no functional limitation in daily life activities. Compared with controls, the intervention induced significant and large benefits (all p<0.05) in the workload eliciting the ventilatory threshold (both in absolute (watts, +37%) and relative units (watts·kg-1 of total body mass or of lower-limb muscle mass, +44%)), peak oxygen uptake (ml·kg-1 ·min-1 , +28%) and peak workload (absolute (+27%) and relative units (+33%)). However, no significant changes were found for muscle efficiency nor for any measure of body composition. Conclusions: A two-year unsupervised intervention including aerobic and resistance exercise is safe and induces major benefits in the clinical course and aerobic fitness of patients with McArdle disease. |
| publishDate |
2022 |
| dc.date.none.fl_str_mv |
2022 2022 2022 |
| dc.type.none.fl_str_mv |
info:eu-repo/semantics/article |
| format |
article |
| dc.identifier.none.fl_str_mv |
http://hdl.handle.net/10578/29633 |
| url |
http://hdl.handle.net/10578/29633 |
| dc.language.none.fl_str_mv |
Inglés |
| language_invalid_str_mv |
Inglés |
| dc.rights.none.fl_str_mv |
info:eu-repo/semantics/openAccess |
| eu_rights_str_mv |
openAccess |
| dc.format.none.fl_str_mv |
application/pdf application/pdf |
| dc.publisher.none.fl_str_mv |
The American College of Sports Medicine |
| publisher.none.fl_str_mv |
The American College of Sports Medicine |
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reponame:RUIdeRA. Repositorio Institucional de la UCLM instname:Universidad de Castilla-La Mancha |
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Universidad de Castilla-La Mancha |
| reponame_str |
RUIdeRA. Repositorio Institucional de la UCLM |
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RUIdeRA. Repositorio Institucional de la UCLM |
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