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In vitro contracture test results and anaesthetic management of a patient with emery-dreifuss muscular dystrophy for cardiac transplantation

Please always quote using this URN: urn:nbn:de:bvb:20-opus-123413
  • 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 laminEmery-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.show moreshow less

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Metadaten
Author: Frank Schuster, Carsten Wessig, Christoph Schimmer, Stephan Johannsen, Marc Lazarus, Ivan Aleksic, Rainer Leyh, Norbert Roewer
URN:urn:nbn:de:bvb:20-opus-123413
Document Type:Journal article
Faculties:Medizinische Fakultät / Klinik und Poliklinik für Anästhesiologie (ab 2004)
Language:English
Parent Title (English):Case Reports in Anesthesiology
Year of Completion:2012
Volume:2012
Issue:349046
Source:Case Reports in Anesthesiology. 2012:349046. doi:10.1155/2012/349046
DOI:https://doi.org/10.1155/2012/349046
Pubmed Id:http://www.ncbi.nlm.nih.gov/pubmed?term=PMC3438720
Dewey Decimal Classification:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 617 Chirurgie und verwandte medizinische Fachrichtungen
Tag:Emery-Dreifuss muscular dystrophy
Release Date:2015/12/21
Licence (German):License LogoCC BY: Creative-Commons-Lizenz: Namensnennung