In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation
Emery-Dreifuss muscular dystrophy (EDMD) is a hereditary neuromuscular disorder characterized by slowly progressive muscle weakness, early contractures, and dilated cardiomyopathy. We reported an uneventful general anaesthesia using total intravenous anaesthesia (TIVA) for cardiac transplantation in...
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| Format: | Article |
| Language: | English |
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Wiley
2012-01-01
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| Series: | Case Reports in Anesthesiology |
| Online Access: | http://dx.doi.org/10.1155/2012/349046 |
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| author | Frank Schuster Carsten Wessig Christoph Schimmer Stephan Johannsen Marc Lazarus Ivan Aleksic Rainer Leyh Norbert Roewer |
| author_facet | Frank Schuster Carsten Wessig Christoph Schimmer Stephan Johannsen Marc Lazarus Ivan Aleksic Rainer Leyh Norbert Roewer |
| author_sort | Frank Schuster |
| collection | DOAJ |
| description | Emery-Dreifuss muscular dystrophy (EDMD) is a hereditary neuromuscular disorder characterized by slowly progressive muscle weakness, early contractures, and dilated cardiomyopathy. We reported an uneventful general anaesthesia using total intravenous anaesthesia (TIVA) for cardiac transplantation in a 19-year-old woman suffering from EDMD. In vitro contracture test results of two pectoralis major muscle bundles of the patient suggest that exposition to triggering agents does not induce a pathological sarcoplasmic calcium release in the lamin A/C phenotype. However, due to the lack of evidence in the literature, we would recommend TIVA for patients with EDMD if general anaesthesia is required. |
| format | Article |
| id | doaj-art-84a86f91d0e44a3fbd170521897dfe18 |
| institution | Kabale University |
| issn | 2090-6382 2090-6390 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Anesthesiology |
| spelling | doaj-art-84a86f91d0e44a3fbd170521897dfe182025-02-03T01:12:10ZengWileyCase Reports in Anesthesiology2090-63822090-63902012-01-01201210.1155/2012/349046349046In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac TransplantationFrank Schuster0Carsten Wessig1Christoph Schimmer2Stephan Johannsen3Marc Lazarus4Ivan Aleksic5Rainer Leyh6Norbert Roewer7Department of Anaesthesia and Critical Care, University of Wuerzburg, 97080 Wuerzburg, GermanyDepartment of Neurology, University of Wuerzburg, 97080 Wuerzburg, GermanyDepartment of Cardiothoracic and Thoracic Vascular Surgery, University of Wuerzburg, 97080 Wuerzburg, GermanyDepartment of Anaesthesia and Critical Care, University of Wuerzburg, 97080 Wuerzburg, GermanyDepartment of Anaesthesia and Critical Care, University of Wuerzburg, 97080 Wuerzburg, GermanyDepartment of Cardiothoracic and Thoracic Vascular Surgery, University of Wuerzburg, 97080 Wuerzburg, GermanyDepartment of Cardiothoracic and Thoracic Vascular Surgery, University of Wuerzburg, 97080 Wuerzburg, GermanyDepartment of Anaesthesia and Critical Care, University of Wuerzburg, 97080 Wuerzburg, GermanyEmery-Dreifuss muscular dystrophy (EDMD) is a hereditary neuromuscular disorder characterized by slowly progressive muscle weakness, early contractures, and dilated cardiomyopathy. We reported an uneventful general anaesthesia using total intravenous anaesthesia (TIVA) for cardiac transplantation in a 19-year-old woman suffering from EDMD. In vitro contracture test results of two pectoralis major muscle bundles of the patient suggest that exposition to triggering agents does not induce a pathological sarcoplasmic calcium release in the lamin A/C phenotype. However, due to the lack of evidence in the literature, we would recommend TIVA for patients with EDMD if general anaesthesia is required.http://dx.doi.org/10.1155/2012/349046 |
| spellingShingle | Frank Schuster Carsten Wessig Christoph Schimmer Stephan Johannsen Marc Lazarus Ivan Aleksic Rainer Leyh Norbert Roewer In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation Case Reports in Anesthesiology |
| title | In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation |
| title_full | In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation |
| title_fullStr | In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation |
| title_full_unstemmed | In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation |
| title_short | In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation |
| title_sort | in vitro contracture test results and anaesthetic management of a patient with emery dreifuss muscular dystrophy for cardiac transplantation |
| url | http://dx.doi.org/10.1155/2012/349046 |
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