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.…
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: | https://pubmed.ncbi.nlm.nih.gov/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): | CC BY: Creative-Commons-Lizenz: Namensnennung |