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P91 Delivery challenges of Bethlem myopathy, a rare congenital muscular dystrophy
Source: International Journal of Obstetric Anesthesia - May 1, 2023 Category: Anesthesiology Authors: E. Mann, S. Paterson, R. Burns Source Type: research

Atypical reaction to anesthesia in Duchenne/Becker muscular dystrophy
ConclusionOn preanesthetic assessment, the history of delayed motor development, as well as clinical and/or laboratory signs of myopathy, should encourage neurological evaluation, aiming at diagnosing subclinical myopathies and planning the necessary care to prevent anesthetic complications. Duchenne/Becker muscular dystrophy, although it does not increase susceptibility to MH, may lead to atypical fatal reactions in anesthesia.ResumoJustificativa/objetivosDistrofia muscular de Duchenne/Becker afeta a musculatura esquelética e leva a fraqueza muscular progressiva e risco de reações atípicas anestésicas após exposiç�...
Source: Brazilian Journal of Anesthesiology - July 10, 2018 Category: Anesthesiology Source Type: research

Influenza A Virus Infection Damages Zebrafish Skeletal Muscle and Exacerbates Disease in Zebrafish Modeling Duchenne Muscular Dystrophy
DISCUSSION Here, we investigated the effects of an infectious disease on skeletal muscle tissue alone and in combination with a genetic muscle disease. We found that human IAV can infect zebrafish muscle fibers and cause fiber damage via loss of sarcolemma integrity and/or loss of ECM adhesion external to the sarcolemma. Additionally, we showed that molecular and cellular markers of inflammation are present in muscle tissue in response to IAV infection. Finally, we showed that an infectious disease in combination with a genetic muscle disease greatly worsens the severity of muscle tissue degeneration. Taken together, our r...
Source: PLOS Currents Muscular Dystrophy - October 25, 2017 Category: Neurology Authors: Clarissa Henry Source Type: research

Atypical reaction to anesthesia in Duchenne/Becker muscular dystrophy
Conclusion On preanesthetic assessment, the history of delayed motor development, as well as clinical and/or laboratory signs of myopathy, should encourage neurological evaluation, aiming at diagnosing subclinical myopathies and planning the necessary care to prevent anesthetic complications. Duchenne/Becker muscular dystrophy, although it does not increase susceptibility to MH, may lead to atypical fatal reactions in anesthesia.
Source: Brazilian Journal of Anesthesiology - July 19, 2017 Category: Anesthesiology Source Type: research

Atypical reaction to anesthesia in Duchenne/Becker muscular dystrophy.
CONCLUSION: On preanesthetic assessment, the history of delayed motor development, as well as clinical and/or laboratory signs of myopathy, should encourage neurological evaluation, aiming at diagnosing subclinical myopathies and planning the necessary care to prevent anesthetic complications. Duchenne/Becker muscular dystrophy, although it does not increase susceptibility to MH, may lead to atypical fatal reactions in anesthesia. PMID: 28576279 [PubMed - as supplied by publisher]
Source: Revista Brasileira de Anestesiologia - June 5, 2017 Category: Anesthesiology Tags: Rev Bras Anestesiol Source Type: research

Anesthetic management of 877 pediatric patients undergoing muscle biopsy for neuromuscular disorders: a 20‐year review
ConclusionsIn this retrospective study, no patient exhibited signs or symptoms of hyperkalemia or MH probably because the incidence is very low and becomes even less likely due to the selection of the various anesthetic agents and strategies administered.
Source: Pediatric Anesthesia - April 24, 2016 Category: Anesthesiology Authors: Frederic Shapiro, Umeshkumar Athiraman, David J. Clendenin, Monica Hoagland, Navil F. Sethna Tags: Research Report Source Type: research

Dystrophinopathies
Semin Neurol 2015; 35: 369-384DOI: 10.1055/s-0035-1558982The dystrophinopathies fall along a spectrum of muscular dystrophy phenotypes, with variable involvement of skeletal and cardiac muscle. The diagnosis of dystrophinopathy should be suspected in any patient with a highly elevated creatine kinase level beyond the context of rhabdomyolysis secondary to toxic or metabolic myopathy. Genetic testing for dystrophinopathy is highly sensitive and specific, and identifying a proband will often lead to implications for several relatives at risk for cardiomyopathy, weakness, or anesthetic reactions. Management of the dystrophino...
Source: Seminars in Neurology - October 6, 2015 Category: Neurology Authors: Brandsema, John F.Darras, Basil T. Source Type: research

The FVB Background Does Not Dramatically Alter the Dystrophic Phenotype of Mdx Mice
Discussion To meet the practical needs of our transgenic studies, we crossed the BL10-background mdx mice with FVB/NJ mice. Recent studies suggest that the so-called “wild type” inbred mice may actually carry various changes in their genome. For example, the commonly used A/J mice were recently show to display progressive muscular dystrophy due to a mutation in the dysferlin gene 24. The FVB strain was also found to carry mutations in several genes of the visual system 25. It is thus important to determine whether the FVB background alters the dystrophic phenotype of the original mdx mice. After seven generations of ba...
Source: PLOS Currents Muscular Dystrophy - February 10, 2015 Category: Neurology Authors: Nalinda B. Wasala Source Type: research

The HDAC Inhibitor TSA Ameliorates a Zebrafish Model of Duchenne Muscular Dystrophy
Discussion Our results identify a dmd morpholino cocktail that induces a high penetrance of muscle lesions and strongly resembles the zebrafish dmd mutant phenotype. We also show that the HDAC inhibitor TSA rescues both dmd-MO and dmd mutant muscle lesions. By comparing different approaches to scoring muscle lesions, our study confirms a previous report 13 that simple assessment of the muscle birefringence pattern in whole larvae, using a stereomicroscope, is a reliable approach to scoring the dmd-MO or dmd mutant phenotype following chemical treatment. Thus, our work identifies optimal morpholino and phenotypic scoring ap...
Source: PLOS Currents Muscular Dystrophy - September 17, 2013 Category: Neurology Authors: njohnso8 Source Type: research

Analysis of histomorphology in malignant hyperthermia-susceptible patients.
CONCLUSION: Despite the inability of the histomorphological examination to identify consistent features in MHS patients, histology may serve as a potential adjunct to CHCT and aid in the identification of other myopathies. Nevertheless, the specifics of its utility ought to be assessed in other studies and by way of formal cost-effectiveness analysis. PMID: 23888335 [PubMed - as supplied by publisher]
Source: Canadian Journal of Anaesthesia - July 26, 2013 Category: Anesthesiology Authors: Orlov D, Keith J, Rosen D, Croul S, Kraeva N, Riazi S Tags: Can J Anaesth Source Type: research

A Novel Method for Targeting Intramuscular Diaphragm Injections Using Ultrasound and Electromyography (P02.220)
CONCLUSIONS: This study demonstrates the feasibility and practicality of using ultrasound to guide injections and needle EMG in animal models in an effort to reduce complications associated with conventional blind technique. Further, this new method demonstrates the feasibility of targeted diaphragm injections with gene replacement therapy in muscle diseases.Supported by: MDA (Muscular Dystrophy Association); AFM (Association Francais Contre les Myopathy); Joshua Frase Foundation.Disclosure: Dr. Sarwal has nothing to disclose. Dr. Serfas has nothing to disclose. Dr. Mitchell has nothing to disclose. Dr. Cartwright has noth...
Source: Neurology - February 14, 2013 Category: Neurology Authors: Sarwal, A., Serfas, J. D., Mitchell, E., Cartwright, M., Beggs, A., Buj-Bello, A., Childers, M. Tags: P02 Clinical Neurophysiology: EMG Source Type: research