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(23)Na MRI and myometry to compare eplerenone vs. glucocorticoid treatment in Duchenne dystrophy

In this pilot study we tested whether a low dose application of a mild diuretic substance such as eplerenone is beneficial in early stages of Duchenne muscular dystrophy using (23)Na und (1)H imaging, myometry, and clinical testing versus the glucocorticoid gold standard. Two 7-years old patients wi...

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Autores principales: A. Glemser, Philip, Jaeger, Heike, M. Nagel, Armin, E. Ziegler, Andreas, Simons, David, Schlemmer, Heinz-Peter, Lehmann-Horn, Frank, Jurkat-Rott, Karin, Weber, Marc-André
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Pacini Editore SRL 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5479105/
https://www.ncbi.nlm.nih.gov/pubmed/28690388
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author A. Glemser, Philip
Jaeger, Heike
M. Nagel, Armin
E. Ziegler, Andreas
Simons, David
Schlemmer, Heinz-Peter
Lehmann-Horn, Frank
Jurkat-Rott, Karin
Weber, Marc-André
author_facet A. Glemser, Philip
Jaeger, Heike
M. Nagel, Armin
E. Ziegler, Andreas
Simons, David
Schlemmer, Heinz-Peter
Lehmann-Horn, Frank
Jurkat-Rott, Karin
Weber, Marc-André
author_sort A. Glemser, Philip
collection PubMed
description In this pilot study we tested whether a low dose application of a mild diuretic substance such as eplerenone is beneficial in early stages of Duchenne muscular dystrophy using (23)Na und (1)H imaging, myometry, and clinical testing versus the glucocorticoid gold standard. Two 7-years old patients with DMD were examined on a 3T MRI system. (1)H MRI and (23)Na density-adapted 3-dimensional radial MRI sequences were performed both before and 1, 3 and 6 months after therapy with eplerenone respectively cortisone. We quantified fatty infiltration on T1-weighted images using subcutaneous fat as reference and fat fraction with a two-point DIXON sequence. Muscle oedema was quantified on STIR images and DIXON water maps with background noise as reference. We quantified Na(+) by a muscular tissue concentration sequence with a 51.3mM Na(+) with 5% agarose reference tube. A Na(+) IR-sequence was used for determination of mainly myoplasmic Na(+). Correspondingly myometry of muscles and tendons were assessed. Clinical tests (i.e. 4-steps-test) and blood counts (i.e. K(+)) were done by a pediatrician. Under eplerenone therapy we detected a reduction of muscular oedema, intracellular-weighted sodium IR signal and muscular sodium concentration. The oedema reduction in the DMD patient receiving eplerenone was more pronounced to the patient with cortisone. Myometric-measured tissue parameters such as muscle stiffness had a more pronounced effect in the child treated with eplerenone after a first increase in muscle stiffness both after eplerenone and cortisone treatment. Clinical abilities during both therapies were mostly constant. Eplerenone might be a possible new therapy option in DMD patients.
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spelling pubmed-54791052017-07-07 (23)Na MRI and myometry to compare eplerenone vs. glucocorticoid treatment in Duchenne dystrophy A. Glemser, Philip Jaeger, Heike M. Nagel, Armin E. Ziegler, Andreas Simons, David Schlemmer, Heinz-Peter Lehmann-Horn, Frank Jurkat-Rott, Karin Weber, Marc-André Acta Myol Original Articles In this pilot study we tested whether a low dose application of a mild diuretic substance such as eplerenone is beneficial in early stages of Duchenne muscular dystrophy using (23)Na und (1)H imaging, myometry, and clinical testing versus the glucocorticoid gold standard. Two 7-years old patients with DMD were examined on a 3T MRI system. (1)H MRI and (23)Na density-adapted 3-dimensional radial MRI sequences were performed both before and 1, 3 and 6 months after therapy with eplerenone respectively cortisone. We quantified fatty infiltration on T1-weighted images using subcutaneous fat as reference and fat fraction with a two-point DIXON sequence. Muscle oedema was quantified on STIR images and DIXON water maps with background noise as reference. We quantified Na(+) by a muscular tissue concentration sequence with a 51.3mM Na(+) with 5% agarose reference tube. A Na(+) IR-sequence was used for determination of mainly myoplasmic Na(+). Correspondingly myometry of muscles and tendons were assessed. Clinical tests (i.e. 4-steps-test) and blood counts (i.e. K(+)) were done by a pediatrician. Under eplerenone therapy we detected a reduction of muscular oedema, intracellular-weighted sodium IR signal and muscular sodium concentration. The oedema reduction in the DMD patient receiving eplerenone was more pronounced to the patient with cortisone. Myometric-measured tissue parameters such as muscle stiffness had a more pronounced effect in the child treated with eplerenone after a first increase in muscle stiffness both after eplerenone and cortisone treatment. Clinical abilities during both therapies were mostly constant. Eplerenone might be a possible new therapy option in DMD patients. Pacini Editore SRL 2017-03 /pmc/articles/PMC5479105/ /pubmed/28690388 Text en The journal and the individual contributions contained in it are protected by the copyright of Gaetano Conte Academy, Naples, Italy http://creativecommons.org/licenses/by-nc-nd/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivatives License, which permits for noncommercial use, distribution, and reproduction in any digital medium, provided the original work is properly cited and is not altered in any way. For details, please refer to http://creativecommons.org/licenses/by-nc-nd/3.0/
spellingShingle Original Articles
A. Glemser, Philip
Jaeger, Heike
M. Nagel, Armin
E. Ziegler, Andreas
Simons, David
Schlemmer, Heinz-Peter
Lehmann-Horn, Frank
Jurkat-Rott, Karin
Weber, Marc-André
(23)Na MRI and myometry to compare eplerenone vs. glucocorticoid treatment in Duchenne dystrophy
title (23)Na MRI and myometry to compare eplerenone vs. glucocorticoid treatment in Duchenne dystrophy
title_full (23)Na MRI and myometry to compare eplerenone vs. glucocorticoid treatment in Duchenne dystrophy
title_fullStr (23)Na MRI and myometry to compare eplerenone vs. glucocorticoid treatment in Duchenne dystrophy
title_full_unstemmed (23)Na MRI and myometry to compare eplerenone vs. glucocorticoid treatment in Duchenne dystrophy
title_short (23)Na MRI and myometry to compare eplerenone vs. glucocorticoid treatment in Duchenne dystrophy
title_sort (23)na mri and myometry to compare eplerenone vs. glucocorticoid treatment in duchenne dystrophy
topic Original Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5479105/
https://www.ncbi.nlm.nih.gov/pubmed/28690388
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