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      Predictors of extubation outcomes following myasthenic crisis

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          Abstract

          Objective

          Myasthenic crisis (MC) is considered the most severe adverse event in patients with myasthenia gravis. The present retrospective study was performed to evaluate the predictors of clinical outcomes in patients with MC.

          Methods

          The medical charts of 33 patients (19 women, 14 men) with 76 MC attacks from 2002 to 2014 were retrospectively reviewed. Early extubation (≤7 days) and prolonged ventilation (>15 days) during the MC were used to assess patient outcomes.

          Results

          Among the 33 patients, 24 (72.7%) had positive acetylcholine receptor antibody test results and 20 (60.6%) experienced recurrent MC attacks (≥2 episodes) during follow-up (median 83.6 months, range 1.5–177 months). Plasma exchange during an MC was significantly associated with early extubation. Male sex, older age (>50 years), atelectasis, and ventilator-associated pneumonia significantly contributed to prolonged ventilation. In 22 patients who underwent thymectomy, both the duration between MC attacks and the mean number of MC attacks were significantly reduced after surgery.

          Conclusions

          Plasma exchange during MC attacks was found to be important for early extubation; older patients and those with atelectasis or ventilator-associated pneumonia were more vulnerable to prolonged ventilation. Thymectomy may be useful to prevent recurrence of MC.

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          Most cited references28

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          Myasthenia gravis.

          D Drachman (1994)
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            Myasthenic crisis: clinical features, mortality, complications, and risk factors for prolonged intubation.

            We retrospectively reviewed the hospital records of 53 patients admitted for 73 episodes of myasthenic crisis at Columbia-Presbyterian Medical Center over a period of 12 years, from 1983 to 1994. Median age at the onset of first crisis was 55 (range, 20 to 82), the ratio of women to men was 2:1, and the median interval from onset of symptoms to first crisis was 8 months. Infection (usually pneumonia or upper respiratory infection) was the most common precipitating factor (38%), followed by no obvious cause (30%) and aspiration (10%). Twenty-five percent of patients were extubated at 7 days, 50% at 13 days, and 75% at 31 days; the longest crisis exceeded 5 months. Using survival analysis and backward stepwise Cox regression, we identified three independent predictors of prolonged intubation: (1) pre-intubation serum bicarbonate > or = 30 mg/dl (p = 0.0004, relative hazard 4.5), (2) peak vital capacity day 1 to 6 post-intubation 50 (p = 0.01, relative hazard 2.4). The proportion of patients intubated longer than 2 weeks was 0% among those with no risk factors, 21% with one risk factor, 46% with two risk factors, and 88% with three risk factors (p = 0.0004). Complications independently associated with prolonged intubation included atelectasis (p = 0.002), anemia treated with transfusion (p = 0.03), Clostridium difficile infection (p = 0.01), and congestive heart failure (p = 0.03). Three episodes of crisis were fatal, for a mortality rate of 4% (3/73); four additional patients died after extubation. All seven deaths were due to overwhelming medical comorbidity. Over half of those who survived were functionally dependent (home or institutionalized) at discharge. In addition to prospective controlled studies of immunotherapies, the prevention and treatment of medical complications offers the best opportunity for further improving the outcome of myasthenic crisis.
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              Myasthenia gravis: management of myasthenic crisis and perioperative care.

              Vern Juel (2004)
              Myasthenic crisis may be defined as respiratory failure or delayed postoperative extubation for more than 24 hours resulting from myasthenic weakness. Myasthenic crisis results from weakness of upper airway muscles leading to obstruction and aspiration, weakness of respiratory muscles leading to reduced tidal volumes, or from weakness of both muscle groups. About one-fifth of patients with myasthenia gravis experience crisis, usually within the first year of illness. Over the last four decades, prognosis from myasthenic crisis has dramatically improved from a mortality rate of 75% to the current rate of less than 5%. Common precipitating factors for myasthenic crisis include respiratory infections, aspiration, sepsis, surgical procedures, rapid tapering of immune modulation, beginning treatment with corticosteroids, exposure to drugs that may increase myasthenic weakness, and pregnancy. Myasthenic crisis should not be fatal, as long as patients receive timely respiratory support and appropriate immunotherapy to reduce myasthenic weakness of the upper airway and respiratory muscles. Myasthenic patients with oropharyngeal or respiratory muscle weakness should receive preoperative plasma exchange or intravenous immunoglobulin therapy to a minimal level of weakness to prevent postoperative complications.
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                Author and article information

                Journal
                J Int Med Res
                J. Int. Med. Res
                IMR
                spimr
                The Journal of International Medical Research
                SAGE Publications (Sage UK: London, England )
                0300-0605
                1473-2300
                17 November 2016
                December 2016
                : 44
                : 6
                : 1524-1533
                Affiliations
                [1 ]Department of Thoracic Surgery, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, People’s Republic of China
                [2 ]Clinical Trials Unit, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, People’s Republic of China
                [3 ]Department of Neurology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, People’s Republic of China
                [4 ]Department of Surgery, Davis Heart and Lung Research Institute, The Ohio State University Wexner Medical Center, Columbus, OH, USA
                Author notes
                [*]Chao Cheng, Department of Thoracic Surgery, The First Affiliated Hospital of Sun Yat-sen University, 58 Zhongshan 2 nd Road, Guangzhou 510080, P.R. China. Email: drchengchao@ 123456163.com .
                Article
                10.1177_0300060516669893
                10.1177/0300060516669893
                5536745
                27856933
                337af608-17a7-4de7-8d76-3ed26cce6651
                © The Author(s) 2016

                This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 3.0 License ( http://www.creativecommons.org/licenses/by-nc/3.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access page( https://us.sagepub.com/en-us/nam/open-access-at-sage).

                History
                : 6 March 2016
                : 26 August 2016
                Categories
                Clinical Reports

                myasthenia gravis,myasthenic crisis,early extubation,thymectomy,plasma exchange,prolonged ventilation,surgery

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