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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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Detalles Bibliográficos
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
Descripción
Sumario: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.