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The Association between the Respiratory System and Upper Limb Strength in Males with Duchenne Muscular Dystrophy: A New Field for Intervention?

Progressive, irreversible muscle weakness is the leading symptom of Duchenne muscular dystrophy (DMD), often resulting in death from respiratory muscle failure. Little is known about the relationship between the functioning of the respiratory system and the hand grip—a function which remains long pr...

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Autores principales: Sobierajska-Rek, Agnieszka, Wasilewska, Eliza, Śledzińska, Karolina, Jabłońska-Brudło, Joanna, Małgorzewicz, Sylwia, Wasilewski, Andrzej, Szalewska, Dominika
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9736927/
https://www.ncbi.nlm.nih.gov/pubmed/36497753
http://dx.doi.org/10.3390/ijerph192315675
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author Sobierajska-Rek, Agnieszka
Wasilewska, Eliza
Śledzińska, Karolina
Jabłońska-Brudło, Joanna
Małgorzewicz, Sylwia
Wasilewski, Andrzej
Szalewska, Dominika
author_facet Sobierajska-Rek, Agnieszka
Wasilewska, Eliza
Śledzińska, Karolina
Jabłońska-Brudło, Joanna
Małgorzewicz, Sylwia
Wasilewski, Andrzej
Szalewska, Dominika
author_sort Sobierajska-Rek, Agnieszka
collection PubMed
description Progressive, irreversible muscle weakness is the leading symptom of Duchenne muscular dystrophy (DMD), often resulting in death from respiratory muscle failure. Little is known about the relationship between the functioning of the respiratory system and the hand grip—a function which remains long preserved. This study aimed to investigate the interdependence between muscle strength and the function of both hand grip and the respiratory system in patients with DMD. Materials and Method: The study included cohort patients, aged 6–17, with DMD, recruited from the Rare Disease Centre, Gdansk, Poland. Clinical status (Vignos scale, Brook scale), pulmonary function (respiratory muscle strength—MIP, MEP); spirometry (FEV1; FVC), as well as upper limb function (performance of the upper limb—PUL 2.0) and hand grip strength (HGS) (hand-held dynamometer) were evaluated in all participants. Results: Finally, 53 boys (mean age 11.41 ± 3.70 years, 25 non-ambulant) were included. Each of the participants presented a lower %pv of MIP (48.11 ± 27), MEP (38.11 ± 22), PUL (75.64 ± 27), and HGS (33.28 ± 18). There were differences between the ambulatory and non-ambulatory groups in values of MIP, MEP, FVC, PUL, HGS (p < 0.001 for all), and FEV1 (p < 0.013). There were correlations between PUL, HGS, and MIP (R = 0.56; R = 0.61, p < 0.001 both), MEP (R = 0.59; R = 0.62, p < 0.001), FVC (R = 0.77; R = 0.77, p < 0.001), and FEV1 (R = 0.77; R = 0.79; p < 0.001). These correlations were found for all participants, but non-ambulatory patients presented stronger relationships. Conclusions: 1. The pulmonary and upper limb functions were within the normal range in ambulatory and low in non-ambulatory patients with DMD, but the muscle strength of both systems was low, regardless of the stage of the disease. 2. There seems to be an interdependence between the respiratory system and upper limb strength in terms of muscle strength and function in DMD patients, which is stronger in non-ambulatory patients. This may be the basis for the creation of a new personalized plan in rehabilitation—the simultaneous rehabilitation of the respiratory and upper limb muscles. Further studies on this theory should be conducted.
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spelling pubmed-97369272022-12-11 The Association between the Respiratory System and Upper Limb Strength in Males with Duchenne Muscular Dystrophy: A New Field for Intervention? Sobierajska-Rek, Agnieszka Wasilewska, Eliza Śledzińska, Karolina Jabłońska-Brudło, Joanna Małgorzewicz, Sylwia Wasilewski, Andrzej Szalewska, Dominika Int J Environ Res Public Health Article Progressive, irreversible muscle weakness is the leading symptom of Duchenne muscular dystrophy (DMD), often resulting in death from respiratory muscle failure. Little is known about the relationship between the functioning of the respiratory system and the hand grip—a function which remains long preserved. This study aimed to investigate the interdependence between muscle strength and the function of both hand grip and the respiratory system in patients with DMD. Materials and Method: The study included cohort patients, aged 6–17, with DMD, recruited from the Rare Disease Centre, Gdansk, Poland. Clinical status (Vignos scale, Brook scale), pulmonary function (respiratory muscle strength—MIP, MEP); spirometry (FEV1; FVC), as well as upper limb function (performance of the upper limb—PUL 2.0) and hand grip strength (HGS) (hand-held dynamometer) were evaluated in all participants. Results: Finally, 53 boys (mean age 11.41 ± 3.70 years, 25 non-ambulant) were included. Each of the participants presented a lower %pv of MIP (48.11 ± 27), MEP (38.11 ± 22), PUL (75.64 ± 27), and HGS (33.28 ± 18). There were differences between the ambulatory and non-ambulatory groups in values of MIP, MEP, FVC, PUL, HGS (p < 0.001 for all), and FEV1 (p < 0.013). There were correlations between PUL, HGS, and MIP (R = 0.56; R = 0.61, p < 0.001 both), MEP (R = 0.59; R = 0.62, p < 0.001), FVC (R = 0.77; R = 0.77, p < 0.001), and FEV1 (R = 0.77; R = 0.79; p < 0.001). These correlations were found for all participants, but non-ambulatory patients presented stronger relationships. Conclusions: 1. The pulmonary and upper limb functions were within the normal range in ambulatory and low in non-ambulatory patients with DMD, but the muscle strength of both systems was low, regardless of the stage of the disease. 2. There seems to be an interdependence between the respiratory system and upper limb strength in terms of muscle strength and function in DMD patients, which is stronger in non-ambulatory patients. This may be the basis for the creation of a new personalized plan in rehabilitation—the simultaneous rehabilitation of the respiratory and upper limb muscles. Further studies on this theory should be conducted. MDPI 2022-11-25 /pmc/articles/PMC9736927/ /pubmed/36497753 http://dx.doi.org/10.3390/ijerph192315675 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Sobierajska-Rek, Agnieszka
Wasilewska, Eliza
Śledzińska, Karolina
Jabłońska-Brudło, Joanna
Małgorzewicz, Sylwia
Wasilewski, Andrzej
Szalewska, Dominika
The Association between the Respiratory System and Upper Limb Strength in Males with Duchenne Muscular Dystrophy: A New Field for Intervention?
title The Association between the Respiratory System and Upper Limb Strength in Males with Duchenne Muscular Dystrophy: A New Field for Intervention?
title_full The Association between the Respiratory System and Upper Limb Strength in Males with Duchenne Muscular Dystrophy: A New Field for Intervention?
title_fullStr The Association between the Respiratory System and Upper Limb Strength in Males with Duchenne Muscular Dystrophy: A New Field for Intervention?
title_full_unstemmed The Association between the Respiratory System and Upper Limb Strength in Males with Duchenne Muscular Dystrophy: A New Field for Intervention?
title_short The Association between the Respiratory System and Upper Limb Strength in Males with Duchenne Muscular Dystrophy: A New Field for Intervention?
title_sort association between the respiratory system and upper limb strength in males with duchenne muscular dystrophy: a new field for intervention?
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9736927/
https://www.ncbi.nlm.nih.gov/pubmed/36497753
http://dx.doi.org/10.3390/ijerph192315675
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