Water T2 could predict functional decline in patients with dysferlinopathy
Background: Water T2 (T2) mapping is increasingly being used in muscular dystrophies to assess active muscle damage. It has been suggested as a surrogate outcome measure for clinical trials. Here, we investigated the prognostic utility of T2 to identify changes in muscle function over time in limb g...
| Autores: | , , , , , , , , , , , , , , , , , , , , , , , , , |
|---|---|
| Tipo de recurso: | artículo |
| Fecha de publicación: | 2022 |
| País: | España |
| Institución: | Universitat Autònoma de Barcelona |
| Repositorio: | Dipòsit Digital de Documents de la UAB |
| Idioma: | inglés |
| OAI Identifier: | oai:ddd.uab.cat:284169 |
| Acceso en línea: | https://ddd.uab.cat/record/284169 https://dx.doi.org/urn:doi:10.1002/jcsm.13063 |
| Access Level: | acceso abierto |
| Palabra clave: | Magnetic resonance imaging Water T2 Limb girdle muscular dystrophy Limb girdle muscular dystrophy R2 Limb girdle muscular dystrophy 2B |
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Water T2 could predict functional decline in patients with dysferlinopathyMoore, Ursula|||0000-0002-0643-5596Caldas de A. Araújo, Ericky|||0000-0002-1352-0879Reyngoudt, Harmen|||0000-0002-0777-2711Gordish-Dressman, Heather|||0000-0002-2330-5427Smith, Fiona E.Wilson, IanJames, Meredith K.|||0000-0001-8078-7744Mayhew, Anna G.|||0000-0001-7085-9011Rufibach, Laura E.|||0000-0001-8208-0574Day, John W.|||0000-0002-0086-9529Jones, Kristi J.Bharucha-Goebel, Diana|||0000-0002-7437-0982Salort-Campana, Emmanuelle|||0000-0002-1846-3017Pestronk, Alan|||0000-0002-8991-5770Walter, Maggie C.|||0000-0002-7611-4827Paradas, Carmen|||0000-0002-6917-2236Stojkovic, Tanya|||0000-0002-4054-2838Mori-Yoshimura, Madoka|||0000-0003-4312-9114Bravver, ElenaPegoraro, Elena|||0000-0002-7740-4156Mendell, Jerry R.|||0000-0002-2691-0200Bushby, Kate|||0000-0001-8818-4008Blamire, Andrew|||0000-0002-8749-1257Straub, Volker|||0000-0001-9046-3540Carlier, Pierre G.|||0000-0002-2315-6927Diaz-Manera, Jordi|||0000-0003-2941-7988Magnetic resonance imagingWater T2Limb girdle muscular dystrophyLimb girdle muscular dystrophy R2Limb girdle muscular dystrophy 2BBackground: Water T2 (T2) mapping is increasingly being used in muscular dystrophies to assess active muscle damage. It has been suggested as a surrogate outcome measure for clinical trials. Here, we investigated the prognostic utility of T2 to identify changes in muscle function over time in limb girdle muscular dystrophies. Methods: Patients with genetically confirmed dysferlinopathy were assessed as part of the Jain Foundation Clinical Outcomes Study in dysferlinopathy. The cohort included 18 patients from two sites, both equipped with 3-tesla magnetic resonance imaging (MRI) systems from the same vendor. T2 value was defined as higher or lower than the median in each muscle bilaterally. The degree of deterioration on four functional tests over 3 years was assessed in a linear model against covariates of high or low T2 at baseline, age, disease duration, and baseline function. Results: A higher T2 at baseline significantly correlated with a greater decline on functional tests in 21 out of 35 muscles and was never associated with slower decline. Higher baseline T2 in adductor magnus, vastus intermedius, vastus lateralis, and vastus medialis were the most sensitive, being associated bilaterally with greater decline in multiple timed tests. Patients with a higher than median baseline T2 (. 22022-01-0120222022-01-01Articlehttp://purl.org/coar/resource_type/c_6501VoRhttp://purl.org/coar/version/c_970fb48d4fbd8a85info:eu-repo/semantics/articleapplication/pdfhttps://ddd.uab.cat/record/284169https://dx.doi.org/urn:doi:10.1002/jcsm.13063reponame:Dipòsit Digital de Documents de la UABinstname:Universitat Autònoma de BarcelonaInglésengopen accesshttp://purl.org/coar/access_right/c_abf2Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.https://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:ddd.uab.cat:2841692026-06-06T12:50:31Z |
| dc.title.none.fl_str_mv |
Water T2 could predict functional decline in patients with dysferlinopathy |
| title |
Water T2 could predict functional decline in patients with dysferlinopathy |
| spellingShingle |
Water T2 could predict functional decline in patients with dysferlinopathy Moore, Ursula|||0000-0002-0643-5596 Magnetic resonance imaging Water T2 Limb girdle muscular dystrophy Limb girdle muscular dystrophy R2 Limb girdle muscular dystrophy 2B |
| title_short |
Water T2 could predict functional decline in patients with dysferlinopathy |
| title_full |
Water T2 could predict functional decline in patients with dysferlinopathy |
| title_fullStr |
Water T2 could predict functional decline in patients with dysferlinopathy |
| title_full_unstemmed |
Water T2 could predict functional decline in patients with dysferlinopathy |
| title_sort |
Water T2 could predict functional decline in patients with dysferlinopathy |
| dc.creator.none.fl_str_mv |
Moore, Ursula|||0000-0002-0643-5596 Caldas de A. Araújo, Ericky|||0000-0002-1352-0879 Reyngoudt, Harmen|||0000-0002-0777-2711 Gordish-Dressman, Heather|||0000-0002-2330-5427 Smith, Fiona E. Wilson, Ian James, Meredith K.|||0000-0001-8078-7744 Mayhew, Anna G.|||0000-0001-7085-9011 Rufibach, Laura E.|||0000-0001-8208-0574 Day, John W.|||0000-0002-0086-9529 Jones, Kristi J. Bharucha-Goebel, Diana|||0000-0002-7437-0982 Salort-Campana, Emmanuelle|||0000-0002-1846-3017 Pestronk, Alan|||0000-0002-8991-5770 Walter, Maggie C.|||0000-0002-7611-4827 Paradas, Carmen|||0000-0002-6917-2236 Stojkovic, Tanya|||0000-0002-4054-2838 Mori-Yoshimura, Madoka|||0000-0003-4312-9114 Bravver, Elena Pegoraro, Elena|||0000-0002-7740-4156 Mendell, Jerry R.|||0000-0002-2691-0200 Bushby, Kate|||0000-0001-8818-4008 Blamire, Andrew|||0000-0002-8749-1257 Straub, Volker|||0000-0001-9046-3540 Carlier, Pierre G.|||0000-0002-2315-6927 Diaz-Manera, Jordi|||0000-0003-2941-7988 |
| author |
Moore, Ursula|||0000-0002-0643-5596 |
| author_facet |
Moore, Ursula|||0000-0002-0643-5596 Caldas de A. Araújo, Ericky|||0000-0002-1352-0879 Reyngoudt, Harmen|||0000-0002-0777-2711 Gordish-Dressman, Heather|||0000-0002-2330-5427 Smith, Fiona E. Wilson, Ian James, Meredith K.|||0000-0001-8078-7744 Mayhew, Anna G.|||0000-0001-7085-9011 Rufibach, Laura E.|||0000-0001-8208-0574 Day, John W.|||0000-0002-0086-9529 Jones, Kristi J. Bharucha-Goebel, Diana|||0000-0002-7437-0982 Salort-Campana, Emmanuelle|||0000-0002-1846-3017 Pestronk, Alan|||0000-0002-8991-5770 Walter, Maggie C.|||0000-0002-7611-4827 Paradas, Carmen|||0000-0002-6917-2236 Stojkovic, Tanya|||0000-0002-4054-2838 Mori-Yoshimura, Madoka|||0000-0003-4312-9114 Bravver, Elena Pegoraro, Elena|||0000-0002-7740-4156 Mendell, Jerry R.|||0000-0002-2691-0200 Bushby, Kate|||0000-0001-8818-4008 Blamire, Andrew|||0000-0002-8749-1257 Straub, Volker|||0000-0001-9046-3540 Carlier, Pierre G.|||0000-0002-2315-6927 Diaz-Manera, Jordi|||0000-0003-2941-7988 |
| author_role |
author |
| author2 |
Caldas de A. Araújo, Ericky|||0000-0002-1352-0879 Reyngoudt, Harmen|||0000-0002-0777-2711 Gordish-Dressman, Heather|||0000-0002-2330-5427 Smith, Fiona E. Wilson, Ian James, Meredith K.|||0000-0001-8078-7744 Mayhew, Anna G.|||0000-0001-7085-9011 Rufibach, Laura E.|||0000-0001-8208-0574 Day, John W.|||0000-0002-0086-9529 Jones, Kristi J. Bharucha-Goebel, Diana|||0000-0002-7437-0982 Salort-Campana, Emmanuelle|||0000-0002-1846-3017 Pestronk, Alan|||0000-0002-8991-5770 Walter, Maggie C.|||0000-0002-7611-4827 Paradas, Carmen|||0000-0002-6917-2236 Stojkovic, Tanya|||0000-0002-4054-2838 Mori-Yoshimura, Madoka|||0000-0003-4312-9114 Bravver, Elena Pegoraro, Elena|||0000-0002-7740-4156 Mendell, Jerry R.|||0000-0002-2691-0200 Bushby, Kate|||0000-0001-8818-4008 Blamire, Andrew|||0000-0002-8749-1257 Straub, Volker|||0000-0001-9046-3540 Carlier, Pierre G.|||0000-0002-2315-6927 Diaz-Manera, Jordi|||0000-0003-2941-7988 |
| author2_role |
author author author author author author author author author author author author author author author author author author author author author author author author author |
| dc.subject.none.fl_str_mv |
Magnetic resonance imaging Water T2 Limb girdle muscular dystrophy Limb girdle muscular dystrophy R2 Limb girdle muscular dystrophy 2B |
| topic |
Magnetic resonance imaging Water T2 Limb girdle muscular dystrophy Limb girdle muscular dystrophy R2 Limb girdle muscular dystrophy 2B |
| description |
Background: Water T2 (T2) mapping is increasingly being used in muscular dystrophies to assess active muscle damage. It has been suggested as a surrogate outcome measure for clinical trials. Here, we investigated the prognostic utility of T2 to identify changes in muscle function over time in limb girdle muscular dystrophies. Methods: Patients with genetically confirmed dysferlinopathy were assessed as part of the Jain Foundation Clinical Outcomes Study in dysferlinopathy. The cohort included 18 patients from two sites, both equipped with 3-tesla magnetic resonance imaging (MRI) systems from the same vendor. T2 value was defined as higher or lower than the median in each muscle bilaterally. The degree of deterioration on four functional tests over 3 years was assessed in a linear model against covariates of high or low T2 at baseline, age, disease duration, and baseline function. Results: A higher T2 at baseline significantly correlated with a greater decline on functional tests in 21 out of 35 muscles and was never associated with slower decline. Higher baseline T2 in adductor magnus, vastus intermedius, vastus lateralis, and vastus medialis were the most sensitive, being associated bilaterally with greater decline in multiple timed tests. Patients with a higher than median baseline T2 (. |
| publishDate |
2022 |
| dc.date.none.fl_str_mv |
2 2022-01-01 2022 2022-01-01 |
| dc.type.none.fl_str_mv |
Article http://purl.org/coar/resource_type/c_6501 VoR http://purl.org/coar/version/c_970fb48d4fbd8a85 |
| dc.type.openaire.fl_str_mv |
info:eu-repo/semantics/article |
| format |
article |
| dc.identifier.none.fl_str_mv |
https://ddd.uab.cat/record/284169 https://dx.doi.org/urn:doi:10.1002/jcsm.13063 |
| url |
https://ddd.uab.cat/record/284169 https://dx.doi.org/urn:doi:10.1002/jcsm.13063 |
| dc.language.none.fl_str_mv |
Inglés eng |
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Inglés |
| language |
eng |
| dc.rights.none.fl_str_mv |
open access http://purl.org/coar/access_right/c_abf2 https://creativecommons.org/licenses/by/4.0/ |
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info:eu-repo/semantics/openAccess |
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open access http://purl.org/coar/access_right/c_abf2 https://creativecommons.org/licenses/by/4.0/ |
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openAccess |
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application/pdf |
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reponame:Dipòsit Digital de Documents de la UAB instname:Universitat Autònoma de Barcelona |
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Universitat Autònoma de Barcelona |
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Dipòsit Digital de Documents de la UAB |
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Dipòsit Digital de Documents de la UAB |
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