Analysis of motor and respiratory function in Duchenne muscular dystrophy patients
•Duchenne muscular dystrophy patients presented motor and respiratory deterioration.•Non-ambulatory condition was associated with worse motor function measure and spirometry.•Deterioration of motor and respiratory function showed correlation between each other.•The assessments should be performed on...
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| Veröffentlicht in: | Respiratory physiology & neurobiology 2019-04, Vol.262, p.1-11 |
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| container_title | Respiratory physiology & neurobiology |
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| creator | Luiz, Lívia Cocato Marson, Fernando Augusto Lima Bresciani Almeida, Celize Cruz Toro, Adyléia Aparecida Dalbo Contrera Nucci, Anamarli Ribeiro, José Dirceu |
| description | •Duchenne muscular dystrophy patients presented motor and respiratory deterioration.•Non-ambulatory condition was associated with worse motor function measure and spirometry.•Deterioration of motor and respiratory function showed correlation between each other.•The assessments should be performed on a routine basis.
Duchenne muscular dystrophy(DMD) shows motor and respiratory impairment.
19 DMD patients (DMDG) (nine ambulatory and 10 non-ambulatory) were evaluated through motor function measure (MFM), 6-minute walk test (6MWT), respiratory muscle strength, cough peak flow, spirometry and volumetric capnography (VCap) tools. Control group that performed spirometry and VCap (CG1-n = 17) were different from those that performed the 6MWT (CG2-n = 8).
The follow tools were assessed (p |
| doi_str_mv | 10.1016/j.resp.2019.01.009 |
| format | Article |
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Duchenne muscular dystrophy(DMD) shows motor and respiratory impairment.
19 DMD patients (DMDG) (nine ambulatory and 10 non-ambulatory) were evaluated through motor function measure (MFM), 6-minute walk test (6MWT), respiratory muscle strength, cough peak flow, spirometry and volumetric capnography (VCap) tools. Control group that performed spirometry and VCap (CG1-n = 17) were different from those that performed the 6MWT (CG2-n = 8).
The follow tools were assessed (p < 0.05): (i) MFM: Ambulatory patients showed higher values than non-ambulatory patients; (ii) 6MWT: DMDG walked a shorter distance and showed higher respiratory rate at rest and heart rate (HR) at rest than CG2; (iii) Spirometry: DMDG and non-ambulatory patients had minor values achieved in spirometry when compared with CG1 and ambulatory patients, respectively; (iv) VCap: DMDG when compared with CG1 showed: (<11 years-old) lower values in VCap parameters; (>11 years-old): higher HR and lower slope 2. There was correlation between spirometry, mainly for zFEV1/FVC, and MFM.
DMDG showed motor (MFM/6MWT) and respiratory (spirometry/VCap) deterioration when compared with CG. Non-ambulatory condition was associated with worse MFM and spirometry.</description><identifier>ISSN: 1569-9048</identifier><identifier>EISSN: 1878-1519</identifier><identifier>DOI: 10.1016/j.resp.2019.01.009</identifier><identifier>PMID: 30660861</identifier><language>eng</language><publisher>Netherlands: Elsevier B.V</publisher><subject>Adolescent ; Child ; Cross-Sectional Studies ; Disease Progression ; Duchenne ; Genetic disease ; Heart Rate ; Humans ; Lung function ; Male ; Motor Activity ; Motor Disorders - etiology ; Motor Disorders - physiopathology ; motor skills ; Muscle Strength ; Muscular dystrophy ; Muscular Dystrophy, Duchenne - physiopathology ; Respiration ; Respiration Disorders - etiology ; Respiration Disorders - physiopathology ; Respiratory Muscles - physiopathology ; Spirometry ; Walking - physiology</subject><ispartof>Respiratory physiology & neurobiology, 2019-04, Vol.262, p.1-11</ispartof><rights>2019 Elsevier B.V.</rights><rights>Copyright © 2019 Elsevier B.V. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c356t-bed6fb7a8a071a1dc070d91b190f5ae39b2efbdcb5f9e90d5838733a8150123f3</citedby><cites>FETCH-LOGICAL-c356t-bed6fb7a8a071a1dc070d91b190f5ae39b2efbdcb5f9e90d5838733a8150123f3</cites><orcidid>0000-0003-4955-4234</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/S1569904818301204$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,776,780,3537,27901,27902,65306</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/30660861$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Luiz, Lívia Cocato</creatorcontrib><creatorcontrib>Marson, Fernando Augusto Lima</creatorcontrib><creatorcontrib>Bresciani Almeida, Celize Cruz</creatorcontrib><creatorcontrib>Toro, Adyléia Aparecida Dalbo Contrera</creatorcontrib><creatorcontrib>Nucci, Anamarli</creatorcontrib><creatorcontrib>Ribeiro, José Dirceu</creatorcontrib><title>Analysis of motor and respiratory function in Duchenne muscular dystrophy patients</title><title>Respiratory physiology & neurobiology</title><addtitle>Respir Physiol Neurobiol</addtitle><description>•Duchenne muscular dystrophy patients presented motor and respiratory deterioration.•Non-ambulatory condition was associated with worse motor function measure and spirometry.•Deterioration of motor and respiratory function showed correlation between each other.•The assessments should be performed on a routine basis.
Duchenne muscular dystrophy(DMD) shows motor and respiratory impairment.
19 DMD patients (DMDG) (nine ambulatory and 10 non-ambulatory) were evaluated through motor function measure (MFM), 6-minute walk test (6MWT), respiratory muscle strength, cough peak flow, spirometry and volumetric capnography (VCap) tools. Control group that performed spirometry and VCap (CG1-n = 17) were different from those that performed the 6MWT (CG2-n = 8).
The follow tools were assessed (p < 0.05): (i) MFM: Ambulatory patients showed higher values than non-ambulatory patients; (ii) 6MWT: DMDG walked a shorter distance and showed higher respiratory rate at rest and heart rate (HR) at rest than CG2; (iii) Spirometry: DMDG and non-ambulatory patients had minor values achieved in spirometry when compared with CG1 and ambulatory patients, respectively; (iv) VCap: DMDG when compared with CG1 showed: (<11 years-old) lower values in VCap parameters; (>11 years-old): higher HR and lower slope 2. There was correlation between spirometry, mainly for zFEV1/FVC, and MFM.
DMDG showed motor (MFM/6MWT) and respiratory (spirometry/VCap) deterioration when compared with CG. Non-ambulatory condition was associated with worse MFM and spirometry.</description><subject>Adolescent</subject><subject>Child</subject><subject>Cross-Sectional Studies</subject><subject>Disease Progression</subject><subject>Duchenne</subject><subject>Genetic disease</subject><subject>Heart Rate</subject><subject>Humans</subject><subject>Lung function</subject><subject>Male</subject><subject>Motor Activity</subject><subject>Motor Disorders - etiology</subject><subject>Motor Disorders - physiopathology</subject><subject>motor skills</subject><subject>Muscle Strength</subject><subject>Muscular dystrophy</subject><subject>Muscular Dystrophy, Duchenne - physiopathology</subject><subject>Respiration</subject><subject>Respiration Disorders - etiology</subject><subject>Respiration Disorders - physiopathology</subject><subject>Respiratory Muscles - physiopathology</subject><subject>Spirometry</subject><subject>Walking - physiology</subject><issn>1569-9048</issn><issn>1878-1519</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2019</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kM1q3TAQRkVpaH7aF-iiaNmN3RmrtiXoJiRtUwgEQroWsjQiutiSK9kFv319uWmXXc0MnO-DOYy9R6gRsPt0qDOVuW4AVQ1YA6hX7AJlLytsUb3e97ZTlYLP8pxdlnIAwB578YadC-g6kB1esMfraMathMKT51NaUuYmOn4sDtns58b9Gu0SUuQh8tvVPlOMxKe12HU0mbutLDnNzxufzRIoLuUtO_NmLPTuZV6xn9--Pt3cVfcP33_cXN9XVrTdUg3kOj_0Rhro0aCz0INTOKAC3xoSamjID84OrVekwLVSyF4II7EFbIQXV-zjqXfO6ddKZdFTKJbG0URKa9EN9krItldyR5sTanMqJZPXcw6TyZtG0EeX-qCPL-ujSw2od5d76MNL_zpM5P5F_srbgS8ngPYvfwfKutjdgCUXMtlFuxT-1_8H0fuHQQ</recordid><startdate>201904</startdate><enddate>201904</enddate><creator>Luiz, Lívia Cocato</creator><creator>Marson, Fernando Augusto Lima</creator><creator>Bresciani Almeida, Celize Cruz</creator><creator>Toro, Adyléia Aparecida Dalbo Contrera</creator><creator>Nucci, Anamarli</creator><creator>Ribeiro, José Dirceu</creator><general>Elsevier B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0003-4955-4234</orcidid></search><sort><creationdate>201904</creationdate><title>Analysis of motor and respiratory function in Duchenne muscular dystrophy patients</title><author>Luiz, Lívia Cocato ; Marson, Fernando Augusto Lima ; Bresciani Almeida, Celize Cruz ; Toro, Adyléia Aparecida Dalbo Contrera ; Nucci, Anamarli ; Ribeiro, José Dirceu</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c356t-bed6fb7a8a071a1dc070d91b190f5ae39b2efbdcb5f9e90d5838733a8150123f3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2019</creationdate><topic>Adolescent</topic><topic>Child</topic><topic>Cross-Sectional Studies</topic><topic>Disease Progression</topic><topic>Duchenne</topic><topic>Genetic disease</topic><topic>Heart Rate</topic><topic>Humans</topic><topic>Lung function</topic><topic>Male</topic><topic>Motor Activity</topic><topic>Motor Disorders - etiology</topic><topic>Motor Disorders - physiopathology</topic><topic>motor skills</topic><topic>Muscle Strength</topic><topic>Muscular dystrophy</topic><topic>Muscular Dystrophy, Duchenne - physiopathology</topic><topic>Respiration</topic><topic>Respiration Disorders - etiology</topic><topic>Respiration Disorders - physiopathology</topic><topic>Respiratory Muscles - physiopathology</topic><topic>Spirometry</topic><topic>Walking - physiology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Luiz, Lívia Cocato</creatorcontrib><creatorcontrib>Marson, Fernando Augusto Lima</creatorcontrib><creatorcontrib>Bresciani Almeida, Celize Cruz</creatorcontrib><creatorcontrib>Toro, Adyléia Aparecida Dalbo Contrera</creatorcontrib><creatorcontrib>Nucci, Anamarli</creatorcontrib><creatorcontrib>Ribeiro, José Dirceu</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Respiratory physiology & neurobiology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Luiz, Lívia Cocato</au><au>Marson, Fernando Augusto Lima</au><au>Bresciani Almeida, Celize Cruz</au><au>Toro, Adyléia Aparecida Dalbo Contrera</au><au>Nucci, Anamarli</au><au>Ribeiro, José Dirceu</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Analysis of motor and respiratory function in Duchenne muscular dystrophy patients</atitle><jtitle>Respiratory physiology & neurobiology</jtitle><addtitle>Respir Physiol Neurobiol</addtitle><date>2019-04</date><risdate>2019</risdate><volume>262</volume><spage>1</spage><epage>11</epage><pages>1-11</pages><issn>1569-9048</issn><eissn>1878-1519</eissn><abstract>•Duchenne muscular dystrophy patients presented motor and respiratory deterioration.•Non-ambulatory condition was associated with worse motor function measure and spirometry.•Deterioration of motor and respiratory function showed correlation between each other.•The assessments should be performed on a routine basis.
Duchenne muscular dystrophy(DMD) shows motor and respiratory impairment.
19 DMD patients (DMDG) (nine ambulatory and 10 non-ambulatory) were evaluated through motor function measure (MFM), 6-minute walk test (6MWT), respiratory muscle strength, cough peak flow, spirometry and volumetric capnography (VCap) tools. Control group that performed spirometry and VCap (CG1-n = 17) were different from those that performed the 6MWT (CG2-n = 8).
The follow tools were assessed (p < 0.05): (i) MFM: Ambulatory patients showed higher values than non-ambulatory patients; (ii) 6MWT: DMDG walked a shorter distance and showed higher respiratory rate at rest and heart rate (HR) at rest than CG2; (iii) Spirometry: DMDG and non-ambulatory patients had minor values achieved in spirometry when compared with CG1 and ambulatory patients, respectively; (iv) VCap: DMDG when compared with CG1 showed: (<11 years-old) lower values in VCap parameters; (>11 years-old): higher HR and lower slope 2. There was correlation between spirometry, mainly for zFEV1/FVC, and MFM.
DMDG showed motor (MFM/6MWT) and respiratory (spirometry/VCap) deterioration when compared with CG. Non-ambulatory condition was associated with worse MFM and spirometry.</abstract><cop>Netherlands</cop><pub>Elsevier B.V</pub><pmid>30660861</pmid><doi>10.1016/j.resp.2019.01.009</doi><tpages>11</tpages><orcidid>https://orcid.org/0000-0003-4955-4234</orcidid></addata></record> |
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| source | MEDLINE; Elsevier ScienceDirect Journals |
| subjects | Adolescent Child Cross-Sectional Studies Disease Progression Duchenne Genetic disease Heart Rate Humans Lung function Male Motor Activity Motor Disorders - etiology Motor Disorders - physiopathology motor skills Muscle Strength Muscular dystrophy Muscular Dystrophy, Duchenne - physiopathology Respiration Respiration Disorders - etiology Respiration Disorders - physiopathology Respiratory Muscles - physiopathology Spirometry Walking - physiology |
| title | Analysis of motor and respiratory function in Duchenne muscular dystrophy patients |
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