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Decreased quality of life in Duchenne muscular disease patients related to functional neurological and cardiac impairment

L. Juříková, L. Masárová, R. Panovský, M. Pešl, KŽ. Revendová, O. Volný, V. Feitová, T. Holeček, V. Kincl, P. Danhofer, S. Voháňka, J. Haberlová, K. Podolská

. 2024 ; 15 (-) : 1360385. [pub] 20240208

Status not-indexed Language English Country Switzerland

Document type Journal Article

Persistent link   https://www.medvik.cz/link/bmc24005827

In this prospective study involving 37 Duchenne muscular dystrophy (DMD) patients aged 8-18 years and older, we examined the impact of neurological and cardiac factors on quality of life (QoL). Our findings revealed a negative correlation between upper limb movement and overall mobility, self-service, and usual activities. Ambulatory and non-ambulatory DMD patients showed significant differences in mobility-related parameters. Cardiac evaluations demonstrated associations between mitral annular plane systolic excursion (MAPSE) and mobility-related aspects. The PEDSQL 3.0 neuromuscular model questionnaire further highlighted age-related and movement-related correlations with QoL. The loss of ambulatory status and reduced upper limb movement were negatively associated with QoL, while upper limb movement positively correlated with septal MAPSE. However, no significant associations were found between MAPSE and anxiety/depression. These findings underscore the multifaceted impact of DMD on QoL and emphasize the importance of considering both neurological and cardiac factors in comprehensive patient care.

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$a In this prospective study involving 37 Duchenne muscular dystrophy (DMD) patients aged 8-18 years and older, we examined the impact of neurological and cardiac factors on quality of life (QoL). Our findings revealed a negative correlation between upper limb movement and overall mobility, self-service, and usual activities. Ambulatory and non-ambulatory DMD patients showed significant differences in mobility-related parameters. Cardiac evaluations demonstrated associations between mitral annular plane systolic excursion (MAPSE) and mobility-related aspects. The PEDSQL 3.0 neuromuscular model questionnaire further highlighted age-related and movement-related correlations with QoL. The loss of ambulatory status and reduced upper limb movement were negatively associated with QoL, while upper limb movement positively correlated with septal MAPSE. However, no significant associations were found between MAPSE and anxiety/depression. These findings underscore the multifaceted impact of DMD on QoL and emphasize the importance of considering both neurological and cardiac factors in comprehensive patient care.
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$a Panovský, Roman $u International Clinical Research Center, St. Anne's University Hospital, Faculty of Medicine, Masaryk University, Brno, Czechia $u 1st Department of Internal Medicine-Cardio-angiology, Faculty of Medicine, St. Anne's University Hospital, Brno, Czechia
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$a Pešl, Martin $u International Clinical Research Center, St. Anne's University Hospital, Faculty of Medicine, Masaryk University, Brno, Czechia $u 1st Department of Internal Medicine-Cardio-angiology, Faculty of Medicine, St. Anne's University Hospital, Brno, Czechia $u Department of Biology, Faculty of Medicine, Masaryk University, Brno, Czechia
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$a Revendová, Kamila Žondra $u Department of Neurology, University Hospital Ostrava, Brno, Czechia $u Centre for Clinical Neurosciences, Faculty of Medicine, University Ostrava, Ostrava, Czechia
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$a Volný, Ondřej $u International Clinical Research Center, St. Anne's University Hospital, Faculty of Medicine, Masaryk University, Brno, Czechia $u Department of Neurology, University Hospital Ostrava, Brno, Czechia $u Centre for Clinical Neurosciences, Faculty of Medicine, University Ostrava, Ostrava, Czechia
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$a Feitová, Věra $u International Clinical Research Center, St. Anne's University Hospital, Faculty of Medicine, Masaryk University, Brno, Czechia $u Department of Medical Imaging, St. Anne's University Hospital, Brno, Czechia
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$a Holeček, Tomaš $u International Clinical Research Center, St. Anne's University Hospital, Faculty of Medicine, Masaryk University, Brno, Czechia $u Department of Medical Imaging, St. Anne's University Hospital, Brno, Czechia $u Department of Biomedical Engineering, University of Technology, Brno, Czechia
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$a Kincl, Vladimír $u International Clinical Research Center, St. Anne's University Hospital, Faculty of Medicine, Masaryk University, Brno, Czechia $u 1st Department of Internal Medicine-Cardio-angiology, Faculty of Medicine, St. Anne's University Hospital, Brno, Czechia
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$a Danhofer, Pavlína $u Department of Paediatric Neurology, Faculty of Medicine of Masaryk University, University Hospital Brno, Brno, Czechia
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$a Haberlová, Jana $u Department of Paediatric Neurology, 2nd Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czechia
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$a Podolská, Karolína $u Department of Paediatric Neurology, 2nd Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czechia
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