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1.
Clin Neurol Neurosurg ; 213: 107140, 2022 02.
Article in English | MEDLINE | ID: mdl-35091255

ABSTRACT

OBJECTIVE: Recent studies suggest that the clinical course and outcomes of patients with coronavirus disease 2019 (COVID-19) and myasthenia gravis (MG) are highly variable. We performed a systematic review of the relevant literature with a key aim to assess the outcomes of invasive ventilation, mortality, and hospital length of stay (HLoS) for patients presenting with MG and COVID-19. METHODS: We searched the PubMed, Scopus, Web of Science, and MedRxiv databases for original articles that reported patients with MG and COVID-19. We included all clinical studies that reported MG in patients with confirmed COVID-19 cases via RT-PCR tests. We collected data on patient background characteristics, symptoms, time between MG and COVID-19 diagnosis, MG and COVID-19 treatments, HLoS, and mortality at last available follow-up. We reported summary statistics as counts and percentages or mean±SD. When necessary, inverse variance weighting was used to aggregate patient-level data and summary statistics. RESULTS: Nineteen studies with 152 patients (mean age 54.4 ± 12.7 years; 79/152 [52.0%] female) were included. Hypertension (62/141, 44.0%) and diabetes (30/141, 21.3%) were the most common comorbidities. The mean time between the diagnosis of MG and COVID-19 was7.0 ± 6.3 years. Diagnosis of COVID-19 was confirmed in all patients via RT-PCR tests. Fever (40/59, 67.8%) and ptosis (9/55, 16.4%) were the most frequent COVID-19 and MG symptoms, respectively. Azithromycin and ceftriaxone were the most common COVID-19 treatments, while prednisone and intravenous immunoglobulin were the most common MG treatments. Invasive ventilation treatment was required for 25/59 (42.4%) of patients. The mean HLoS was 18.2 ± 9.9 days. The mortality rate was 18/152 (11.8%). CONCLUSION: This report provides an overview of the characteristics, treatment, and outcomes of MG in COVID-19 patients. Although COVID-19 may exaggerate the neurological symptoms and worsens the outcome in MG patients, we did not find enough evidence to support this notion. Further studies with larger numbers of patients with MG and COVID-19 are needed to better assess the clinical outcomes in these patients.


Subject(s)
COVID-19/complications , COVID-19/therapy , Myasthenia Gravis/complications , Myasthenia Gravis/therapy , Adolescent , Adult , COVID-19/mortality , Child , Female , Hospitalization , Humans , Male , Middle Aged , Myasthenia Gravis/mortality , Respiration, Artificial , Survival Rate , Young Adult
2.
J Korean Med Sci ; 36(39): e242, 2021 Oct 11.
Article in English | MEDLINE | ID: mdl-34636500

ABSTRACT

BACKGROUND: Myasthenia gravis (MG) is a rare classic autoimmune disease where immunosuppressant therapies have been successful to reduce MG attributable mortality fairly well. However, patients with refractory MG (rMG) among the actively treated MG (aMG) are nonresponsive to conventional therapy and display high disease severity, which calls for further research. We aimed to determine survival, prognosis, and clinical feature of patients with rMG compared to non-rMG. METHODS: Retrospective nationwide cohort study using Korea's healthcare database between 2002 and 2017 was conducted. Patients with rMG (n = 47) and non-rMG (n = 4,251) who were aged > 18 years, followed-up for ≥ 1 year, and prescribed immunosuppressants within 2 years after incident MG diagnosis were included. Patients with rMG were defined as administered plasma exchange or intravenous immunoglobulin at least 3 times per year after receiving ≥ 2 immunosuppressants. All-cause mortality, myasthenic crisis, hospitalization, pneumonia/sepsis, and emergency department (ED) visits were measured using Cox proportional hazard models and pharmacotherapy patterns for rMG were assessed. RESULTS: The rMG cohort included a preponderance of younger patients and women. The adjusted hazard ratio was 2.49 (95% confidence interval, 1.26-4.94) for mortality, 3.14 (2.25-4.38) for myasthenic crisis, 1.54 (1.15-2.06) for hospitalization, 2.69 (1.74-4.15) for pneumonia/sepsis, and 1.81 (1.28-2.56) for ED visits for rMG versus non-rMG. The immunosuppressant prescriptions were more prevalent in patients with rMG, while the difference was more remarkable before rMG onset rather than after rMG onset. CONCLUSION: Despite the severe prognosis of rMG, the strategies for pharmacotherapeutic regimens were similar in those two groups, suggesting that intensive monitoring and introduction of timely treatment options in the early phase of MG are required.


Subject(s)
Myasthenia Gravis/diagnosis , Adolescent , Adult , Aged , Aged, 80 and over , Databases, Factual , Female , Hospitalization/statistics & numerical data , Humans , Immunosuppressive Agents/therapeutic use , Kaplan-Meier Estimate , Longitudinal Studies , Male , Middle Aged , Myasthenia Gravis/drug therapy , Myasthenia Gravis/mortality , Plasma Exchange , Prognosis , Proportional Hazards Models , Retrospective Studies , Young Adult
3.
Arthritis Rheumatol ; 73(5): 866-874, 2021 05.
Article in English | MEDLINE | ID: mdl-33258544

ABSTRACT

OBJECTIVE: To estimate the incidence of immune checkpoint inhibitor-related myositis (ICI-myositis) in cancer patients receiving ICIs, and to report associated clinical manifestations, patterns of care, and outcomes. METHODS: We identified a retrospective cohort of patients receiving ICIs between 2016 and 2019 seen at the University of Texas MD Anderson Cancer Center. Cases of ICI-myositis were identified using International Classification of Disease codes and confirmed by reviewing medical records and pathology, as available. RESULTS: A total of 9,088 patients received an ICI. Thirty-six patients (0.40%) were identified as having ICI-myositis: 17 patients (47%) with ICI-myositis alone and 19 (53%) with overlap manifestations (5 patients with myocarditis, 5 with myasthenia gravis, and 9 with both). The incidence of ICI-myositis was 0.31% in those receiving ICI monotherapy and 0.94% in those receiving combination ICI therapy (relative risk 3.1 [95% confidence interval 1.5-6.1]). Twenty-five patients (69%) received ≥1 treatment in addition to glucocorticoids: plasmapheresis in 17 patients (47%), intravenous immunoglobulin in 12 (33%), and biologics in 11 (31%). Patients with overlap conditions had worse outcomes than those with myositis alone, and 79% of them developed respiratory failure. Eight patients died as a result of ICI-myositis, and all had overlap syndrome with myasthenia gravis or myocarditis (P < 0.05); 75% of these patients had a concomitant infection. CONCLUSION: ICI-myositis is a rare but severe adverse event. More than half of the patients presented with overlap manifestations and had deleterious outcomes, including respiratory failure and death. None of the patients with ICI-myositis alone died as a result of adverse events. Optimal treatment strategies have yet to be determined.


Subject(s)
Immune Checkpoint Inhibitors/adverse effects , Myasthenia Gravis/chemically induced , Myocarditis/chemically induced , Myositis/chemically induced , Neoplasms/drug therapy , Adult , Aged , Aged, 80 and over , Cohort Studies , Female , Humans , Infections/epidemiology , Male , Middle Aged , Myasthenia Gravis/epidemiology , Myasthenia Gravis/mortality , Myocarditis/epidemiology , Myocarditis/mortality , Myositis/epidemiology , Myositis/mortality , Respiratory Insufficiency/epidemiology , Respiratory Insufficiency/etiology , Retrospective Studies
4.
Neuromuscul Disord ; 30(10): 815-824, 2020 10.
Article in English | MEDLINE | ID: mdl-32962871

ABSTRACT

Myasthenia Gravis (MG) is an autoimmune disease with several comorbidities, however information on MG mortality remains sparse. We conducted a nationwide register-based study on mortality rates and causes of death among Swedish MG patients. Data was acquired from four Swedish National Board of Health and Welfare registers. A total of 4559 MG patients (2522 women, 55.3%) being alive entering the year 2006, were identified. 1121 patients (562 women, 50.1%) died during 2006-2016. Age- and gender-standardized mortality rate was 1.51 (95% CI 0.40) per 100 patients and did not differ from the Swedish general population. Time from MG diagnosis to death was 8.9 ±â€¯6.8 years. The most common ultimate cause of death was cancer (19.5%), followed by ischemic heart disease (13.3%) and MG (11.3%). When MG was stated as the ultimate cause of death, there was a strikingly higher likelihood of having influenza/pneumonia as a contributing cause of death (OR 2.5, p<0.0001). In conclusion, although we could not confirm a higher mortality rate in Swedish MG patients compared to the general population, and despite modern advancements in treatment, we observed that MG itself was stated as the third most common ultimate cause of death in Swedish MG patients.


Subject(s)
Cause of Death , Myasthenia Gravis/mortality , Myocardial Ischemia/mortality , Neoplasms/mortality , Registries/statistics & numerical data , Adult , Aged , Cohort Studies , Comorbidity , Female , Humans , Influenza, Human/mortality , Male , Middle Aged , Pneumonia/mortality , Sweden/epidemiology
5.
Int J Cardiol ; 299: 63-66, 2020 01 15.
Article in English | MEDLINE | ID: mdl-31611084

ABSTRACT

BACKGROUND: Patients with myasthenia gravis (MG) remain at a higher risk of developing takotsubo syndrome (TS), particularly during a myasthenic crisis (MC) event. The prevalence of MC-associated TS and its impact on subsequent in-hospital outcomes have not been explored previously. METHODS: We queried the National Inpatient Sample (NIS) databases (2007-2014) using weighted data and ICD-9 CM codes to evaluate the prevalence of MC-associated TS, demographics, comorbidities and inpatient outcomes of TS secondary to MC vs. other triggers. RESULTS: The nationwide prevalence of MC-associated TS was 0.3% (175/56,472). Of all 156,506 TS encounters, MC was present in 0.11% (n = 175) of cases. The groups were comparable in terms of demographics (median age 68-73 years, Caucasian >70%, females >80%). In comparison to non-MC TS, MC-associated TS demonstrated a higher frequency of coexisting diabetes and a lower frequency of smoking. The MC-TS cohort experienced significantly higher rates of all-cause mortality [8.6% vs. 4.7%, p = 0.014, unadjusted (OR1.91, p = 0.017) and adjusted (OR1.82, p = 0.038)] and complications including respiratory failure, the need of intubation/mechanical ventilation, and arrhythmia. The MC-TS cohort had fewer routine discharges and frequent transfers. The median stay was 6 days longer (10 vs. 4 days) and median hospital charges per admission were nearly $100,000 higher ($133,999 vs. $38,367) with MC-associated TS. CONCLUSIONS: This population-based analysis revealed a 15 times greater prevalence of secondary TS following MC as compared to the general inpatient population, a nearly 2 times higher odds of all-cause mortality, and significantly higher resource utilization in MC-associated TS as compared to TS triggered by other etiologies.


Subject(s)
Cost of Illness , Hospitalization/trends , Myasthenia Gravis/mortality , Takotsubo Cardiomyopathy/mortality , Aged , Aged, 80 and over , Cohort Studies , Databases, Factual/trends , Female , Humans , Male , Middle Aged , Myasthenia Gravis/diagnosis , Myasthenia Gravis/therapy , Respiration, Artificial/trends , Takotsubo Cardiomyopathy/diagnosis , Takotsubo Cardiomyopathy/therapy
6.
Neurology ; 94(3): e299-e313, 2020 01 21.
Article in English | MEDLINE | ID: mdl-31801833

ABSTRACT

OBJECTIVE: To determine demographic characteristics, clinical features, treatment regimens, and outcome of myasthenic crisis (MC) requiring mechanical ventilation (MV). METHODS: Analysis of patients who presented with MC between 2006 and 2015 in a German multicenter retrospective study. RESULTS: We identified 250 cases in 12 participating centers. Median age at crisis was 72 years. Median duration of MV was 12 days. Prolonged ventilation (>15 days) depended on age (p = 0.0001), late-onset myasthenia gravis (MG), a high Myasthenia Gravis Foundation of America Class before crisis (p = 0.0001 for IVb, odds ratio [OR] = infinite), number of comorbidities (>3 comorbidities: p = 0.002, OR 2.99), pneumonia (p = 0.0001, OR 3.13), and resuscitation (p = 0.0008, OR 9.15). MV at discharge from hospital was necessary in 20.5% of survivors. Patients with early-onset MG (p = 0.0001, OR 0.21), thymus hyperplasia (p = 0.002, OR 0), and successful noninvasive ventilation trial were more likely to be ventilated for less than 15 days. Noninvasive ventilation in 92 cases was sufficient in 38%, which was accompanied by a significantly shorter duration of ventilation (p = 0.001) and intensive care unit (ICU) stay (p = 0.01). IV immunoglobulins, plasma exchange, and immunoadsorption were more likely to be combined sequentially if the duration of MV and the stay in an ICU extended (p = 0.0503, OR 2.05). Patients who received plasma exchange or immunoadsorption as first-line therapy needed invasive ventilation significantly less often (p = 0.003). In-hospital mortality was 12%, which was significantly associated with the number of comorbidities (>3) and complications such as acute respiratory distress syndrome and resuscitation. Main cause of death was multiorgan failure, mostly due to sepsis. CONCLUSION: Mortality and duration of MC remained comparable to previous reports despite higher age and a high disease burden in our study. Prevention and treatment of complications and specialized neurointensive care are the cornerstones in order to improve outcome.


Subject(s)
Myasthenia Gravis/therapy , Respiration, Artificial/mortality , Adolescent , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Myasthenia Gravis/mortality , Young Adult
7.
Intern Med J ; 49(12): 1537-1540, 2019 12.
Article in English | MEDLINE | ID: mdl-31808263

ABSTRACT

Myasthenia gravis (MG) is a disorder affecting neuromuscular transmission with heterogeneous manifestations and treatments. This study describes clinical features and management of MG patients at a metropolitan hospital in Australia. Overall findings were consistent with previously published data. However, frequency of intravenous immuno-globulin use was higher, reasons for which are explored. Management is best conducted through specialist clinics with necessary expertise and standardised treatment protocols.


Subject(s)
Immunoglobulins, Intravenous/therapeutic use , Myasthenia Gravis/epidemiology , Myasthenia Gravis/therapy , Thymectomy/statistics & numerical data , Australia , Female , Hospitals, Urban , Humans , Male , Middle Aged , Myasthenia Gravis/mortality , Retrospective Studies , Thymectomy/adverse effects
8.
Eur Neurol ; 81(5-6): 287-293, 2019.
Article in English | MEDLINE | ID: mdl-31661691

ABSTRACT

INTRODUCTION: Myasthenic crisis (MC) is a life-threatening condition usually occurred in patients with myasthenia gravis (MG). OBJECTIVE: On the basis of a retrospective case series review, we try to develop a scoring system to evaluate the probability for in-hospital death in MC patients. METHODS: We retrospectively reviewed 78 MC patients who were hospitalized from January 2014 to December 2018. Clinical and laboratory data including 17 variables were analyzed univariately. The main clinical outcome was defined as the in-hospital death. Then eligible variables were evaluated by a stepwise multivariate regression and a scoring system was then generated. Calibration and discrimination methods were used to evaluate and validate the model performance. RESULTS: The overall in-hospital mortality was 11.5% (9/78) in the MC cohort. Five clinical variables including Myasthenia Gravis Foundation of America (MGFA) classification at onset, septic shock, thymoma classification, cardiac arrest, and the lowest serum albumin were found to be associated with in-hospital mortality. Further 3 variables entered the final regression, and internal validation showed that the area under the curve was 0.919 (95% CI 0.788-1.000). A scoring system with a full credit of 7 points was generated to predict the in-hospital mortality (MGFA at onset, 2 points; septic shock, 4 points; and cardiac arrest, 3 points). CONCLUSIONS: A 7-point scoring model was established on the basis of a retrospective review of MC patients to predict the in-hospital mortality.


Subject(s)
Hospital Mortality , Myasthenia Gravis/mortality , Adult , Asian People , Cohort Studies , Female , Humans , Male , Middle Aged , Myasthenia Gravis/complications , Retrospective Studies , Risk Factors
9.
F1000Res ; 72018.
Article in English | MEDLINE | ID: mdl-30443340

ABSTRACT

Autoimmune myasthenia gravis (MG) is a neuromuscular junction disorder marked clinically by fatigable muscle weakness and serologically by the presence of autoantibodies against acetylcholine receptors (AChRs), muscle-specific kinase (MuSK), or lipoprotein-related protein 4 (LPR4). Over the past few decades, the mortality of patients with MG has seen a dramatic decline secondary to evolving interventions in critical care and medical management. In the past 2 to 3 years, there have been several changes in standard of care for the treatment of MG. These changes include confirmation of the benefit of thymectomy versus medical management alone in AChR patients and a new US Food and Drug Administration-approved medication for refractory MG. There are also several exciting new prospective drugs in the pipeline, which are in different stages of clinical trial testing.


Subject(s)
Combined Modality Therapy/methods , Myasthenia Gravis/therapy , Standard of Care/trends , Animals , Autoantibodies/immunology , Combined Modality Therapy/standards , Disease Management , Humans , Myasthenia Gravis/mortality , Salvage Therapy/methods
10.
Muscle Nerve ; 58(5): 708-712, 2018 11.
Article in English | MEDLINE | ID: mdl-29572981

ABSTRACT

INTRODUCTION: The objective of this study was to estimate mortality and survival in a large cohort of myasthenia gravis (MG) patients from Belgrade, Serbia, during the period 1979-2008. METHODS: Data for all patients with MG were collected from hospital records and the Belgrade MG Registry. RESULTS: Within the 30-year study period, death occurred in 107 (20%) of 562 patients with MG, with MG-related fatality below 2%. The average MG mortality rate was 1.76 per 1,000,000 population (1.26/1,000,000 women, 2.45/1,000,000 men). A statistically significant increase was recorded for the average standardized mortality rate for all patients (P < 0.01). The mean survival from disease onset was 34.3 ± 2.0 years. Significantly shorter survival was observed in men compared with women and in patients older than 50 years compared with younger ones (P < 0.01). DISCUSSION: We observed long survival and low frequency of MG-related fatalities but increasing average standardized mortality rate, most notably in older men with MG. Muscle Nerve 58: 708-712, 2018.


Subject(s)
Myasthenia Gravis/epidemiology , Myasthenia Gravis/mortality , Adult , Age Distribution , Aged , Antibodies/blood , Cohort Studies , Electromyography , Female , Humans , Male , Middle Aged , Receptors, Nicotinic/immunology , Serbia/epidemiology , Survival Analysis
11.
Neuroepidemiology ; 50(3-4): 153-159, 2018.
Article in English | MEDLINE | ID: mdl-29558746

ABSTRACT

PURPOSE: The objective of the study was to evaluate changes of epidemiological parameters in patients with myasthenia gravis (MG) in Slovakia during the period 1977-2015. METHODS: Data from medical records of MG patients registered in Slovakia were analyzed. Epidemiological rates (incidence, prevalence, mortality) were assessed for several periods to identify changes and drifts over the period of study. RESULTS: Out of 2,074 patients, 892 were males (43.0%) and 1,193 were females (57.0%). The thymoma associated MG (TAMG) was diagnosed in 123 patients (5.9%). The mean age at onset shifted from 35.8 years in 1977-1989 to 60.0 years in -2010-2015. The crude incidence increased from 0.36/100,000 in 1977-1989 to 1.74/100,000 in 2010-2015. The average annual incidence of TAMG was 0.05/100,000. Maximum increase of MG incidence was registered among the elderly (≥50 years), particularly in the group of patients in the age range 70-79 years (0.34/100,000 in 1977-1989→7.10/100,000 in 2010-2015) and 80-89 years (0.00/100,000 in 1977-1989→5.31/100,000 in 2010-2015). The crude MG prevalence on December 31, 2015 was 24.75/100,000. The average MG mortality was 0.27/100,000. CONCLUSION: The age at onset and incidence increased significantly over the study period due to marked increase of MG incidence in elderly, particularly over 70 years. Possible reasons for this trend are discussed.


Subject(s)
Myasthenia Gravis/epidemiology , Age of Onset , Female , Humans , Incidence , Male , Myasthenia Gravis/mortality , Prevalence , Slovakia/epidemiology , Survival Rate
12.
J Clin Pathol ; 71(7): 637-641, 2018 Jul.
Article in English | MEDLINE | ID: mdl-29439008

ABSTRACT

AIMS: We explored the relationships between programmed death-1 (PD-1) and programmed death-ligand 1 (PD-L1) expression and the pathological and clinical features of thymic epithelial tumours and thymic hyperplasia. METHODS: We evaluated PD-1 and PDL-1 expressions within epithelial and microenvironmental components in thymic epithelial tumours (n=44) and thymic hyperplasias (n=8), immunohistochemically. We compared the results with demographic, clinical and histopathological features of the cases. RESULTS: We found 48% epithelial expression and 82.7% microenvironment expression for PD-1 and 11.5% epithelial expression and 34.6% microenvironment expression for PD-L1. There was no PD-1 expression, in either the epithelial or microenvironment, in the thymic hyperplasia group. PD-1 and PD-L1 positivity was more significant in thymic epithelial tumours than thymic hyperplasia. Patients with PD-1-positive microenvironments exhibited significantly shorter mean estimated survival time than their negative counterparts. CONCLUSION: These findings suggest that anti-PD-1 and anti-PD-L1 therapies may benefit patients due to high release of PD-1 and PD-L1 in thymic epithelial tumours.


Subject(s)
B7-H1 Antigen/analysis , Biomarkers, Tumor/analysis , Myasthenia Gravis/metabolism , Neoplasms, Glandular and Epithelial/chemistry , Programmed Cell Death 1 Receptor/analysis , Thymus Gland/chemistry , Thymus Hyperplasia/metabolism , Thymus Neoplasms/chemistry , Adolescent , Adult , Aged , Female , Humans , Immunohistochemistry , Kaplan-Meier Estimate , Male , Middle Aged , Myasthenia Gravis/mortality , Myasthenia Gravis/pathology , Neoplasms, Glandular and Epithelial/mortality , Neoplasms, Glandular and Epithelial/pathology , Retrospective Studies , Thymus Gland/pathology , Thymus Hyperplasia/mortality , Thymus Hyperplasia/pathology , Thymus Neoplasms/mortality , Thymus Neoplasms/pathology , Tumor Microenvironment , Young Adult
13.
J Crit Care ; 45: 90-94, 2018 06.
Article in English | MEDLINE | ID: mdl-29413729

ABSTRACT

BACKGROUND: There are limited data on the characteristics, incidence, and mortality of patients with myasthenia gravis (MG) admitted to the ICU. AIMS: To study the epidemiology, characteristics and outcome of patients with MG in Australian and New Zealand (ANZ) ICUs over a decade. METHODS: We performed a retrospective observational, cross sectional study of data from the Australian and New Zealand Intensive Care Society (ANZICS) Adult Patient Database (APD). We collected data on all adult patients admitted with a primary diagnosis of MG to 159 Australian and 19 New Zealand ICUs between January 1, 2005 and December 31, 2015.We extracted detailed relevant data and performed statistical assessment. RESULTS: We identified 245 patients admitted to ICU with the primary diagnosis of MG, with an incidence increasing from 1 to 2.5 per thousand ICU admissions (P<0.0001) and from 1 to 2.2 per million people (P=0.02). Mean age was 60years with more patients being female (53.7% vs 47.3%) and 91 (37.1%) patients received mechanical ventilation. Hospital mortality occurred in 13 (5.3%) patients with a mortality rate lower than in other ICU patients. CONCLUSIONS: In ANZ, the ICU and population incidence of MG has increased over the last decade. However, its mortality rate was low.


Subject(s)
Critical Care , Hospital Mortality , Intensive Care Units , Myasthenia Gravis/physiopathology , Adult , Aged , Cross-Sectional Studies , Female , Humans , Incidence , Male , Middle Aged , Myasthenia Gravis/mortality , Myasthenia Gravis/therapy , Retrospective Studies , Treatment Outcome
14.
World J Surg Oncol ; 15(1): 150, 2017 Aug 08.
Article in English | MEDLINE | ID: mdl-28789662

ABSTRACT

BACKGROUND: Thymectomy is the primary approach for the treatment of myasthenia gravis (MG). This retrospective study aimed to identify the clinical and demographical features that may impact the duration of mechanical ventilation (DMV), the long-term survival, and the quality of life (QOL) in patients with post-thymectomy myasthenic crisis (PTMC). METHODS: We reviewed the patients who suffered from PTMC from June 2008 to November 2015. Cox proportional hazard regression analysis was used to identify potential prognostic factors that may impact DMV and long-term survival. Spearman bivariate correlation analysis was used to analyze the relationship between DMV and QOL. Statistical powers were calculated. RESULTS: In total, 70 patients with PTMC were enrolled. Alcohol abuse, high scores of Myasthenia Gravis Foundation of America (MGFA) classification and Clavien-Dindo classification were critical factors that remarkably delayed early extubation. High scores of Osserman's classification, MGFA classification, and Clavien-Dindo classification predicted a poor prognosis in PTMC patients. Occupational skills and job status were observed to be negatively affected in PTMC patients. CONCLUSIONS: To decrease the duration of mechanical ventilation, we suggest alcohol abstinence before the operation, appropriate preoperative treatment to decrease MGFA classification, and greater attention to the treatment of postoperative complications.


Subject(s)
Alcohol Drinking/adverse effects , Myasthenia Gravis/surgery , Postoperative Complications/epidemiology , Quality of Life , Respiration, Artificial/statistics & numerical data , Thymectomy/adverse effects , Adult , Female , Follow-Up Studies , Humans , Length of Stay/statistics & numerical data , Male , Middle Aged , Myasthenia Gravis/mortality , Postoperative Complications/etiology , Preoperative Care/methods , Prognosis , Retrospective Studies , Time Factors , Treatment Outcome
15.
Neurol India ; 65(3): 518-524, 2017.
Article in English | MEDLINE | ID: mdl-28488612

ABSTRACT

OBJECTIVE: To analyze the mortality and potential risk factors for death in myasthenia gravis (MG) patients. MATERIALS AND METHODS: A total of 2195 adult patients with MG (aged older than 18 years) diagnosed during the period between 2003 and 2013 were followed-up and retrospectively reviewed. RESULTS: During the 10-year follow-up, 129 patients died and the total mortality rate was 5.88%. The risk factors associated with MG-related deaths were duration of the disease, occurrence of myasthenic crisis, severity of disease that included the Myasthenia Gravis Foundation of America (MGFA) grade III and IV at onset, elevation of acetylcholine receptor antibody (AchR-abs) titers, presence of thymic pathology, and failure of administrating immunosuppressants (P < 0.05). In addition, the non-MG related factors, including the history of preceding strokes, and the presence of chronic obstructive pulmonary disease (COPD), diabetes mellitus, atrial fibrillation, hyperlipidemia, myocardial infarction, and malignant tumors, were closely linked with death in the MG population (the hazard ratios [HRs] were 3.251, 4.173, 3.738, 3.886, 1.945, 2.177, and 14.7, respectively; P< 0.05). CONCLUSIONS: The severity of disease at entry, presence of AchRabs, thymic pathology, and duration of the disease predict a higher risk for death. Systemic illnesses including stroke, COPD, diabetes mellitus, atrial fibrillation, hyperlipidemia, myocardial infarction, and malignant tumor, which may also increase the risk of death, should be carefully monitored and managed.


Subject(s)
Myasthenia Gravis/complications , Myasthenia Gravis/mortality , Adolescent , Adult , Age Distribution , Aged , Atrial Fibrillation/etiology , Autoantibodies/blood , Diabetes Mellitus/etiology , Female , Humans , Longitudinal Studies , Male , Middle Aged , Myasthenia Gravis/epidemiology , Myasthenia Gravis/therapy , Pulmonary Disease, Chronic Obstructive/etiology , Receptors, Cholinergic/immunology , Retrospective Studies , Risk Factors , Statistics, Nonparametric , Stroke/etiology , Thymectomy , Thymus Gland/pathology , Thymus Gland/surgery , Young Adult
16.
Pediatr Surg Int ; 33(6): 683-694, 2017 Jun.
Article in English | MEDLINE | ID: mdl-28401300

ABSTRACT

BACKGROUND: The role of thymectomy in the treatment of juvenile myasthenia gravis (JMG) is poorly defined. The objective of this systematic review was to evaluate the effect of thymectomy on survival, disease severity, and peri-operative complications for patients with JMG. METHODS: A search of MEDLINE, EMBASE, and the Cochrane Library (1/1/2000-3/1/2016) identified all English language, human studies of thymectomy for JMG. The population was patients with JMG age ≤18 years who underwent thymectomy (comparator group was unexposed to thymectomy). Outcomes included survival, disease severity, and post-operative complications. Data extraction was performed by independent reviewers. RESULTS: Sixteen retrospective studies included 1131 participants with JMG and 488 (43%) underwent thymectomy. Post-operative improvement in JMG severity occurred for 77% (n = 376/488). Comparisons of thymectomy to non-operative management were mixed. Post-operative complications were poorly recorded. Power to compare surgical approaches was limited. Outcomes specific to antibodies, surgical pathology findings, severity of JMG, and timing of thymectomy were sparse. CONCLUSIONS: Existing data regarding thymectomy for JMG are limited and entirely retrospective. The majority of patients who underwent thymectomy had improvement in disease severity and post-operative complications were rare. Prospective, multicenter study of thymectomy for JMG is warranted.


Subject(s)
Myasthenia Gravis/surgery , Thymectomy , Humans , Myasthenia Gravis/complications , Myasthenia Gravis/mortality , Severity of Illness Index , Thymectomy/adverse effects
17.
BMC Neurol ; 16(1): 225, 2016 Nov 17.
Article in English | MEDLINE | ID: mdl-27855632

ABSTRACT

BACKGROUND: We have previously reported using two-step cluster analysis to classify myasthenia gravis (MG) patients into the following five subtypes: ocular MG; thymoma-associated MG; MG with thymic hyperplasia; anti-acetylcholine receptor antibody (AChR-Ab)-negative MG; and AChR-Ab-positive MG without thymic abnormalities. The objectives of the present study were to examine the reproducibility of this five-subtype classification using a new data set of MG patients and to identify additional characteristics of these subtypes, particularly in regard to response to treatment. METHODS: A total of 923 consecutive MG patients underwent two-step cluster analysis for the classification of subtypes. The variables used for classification were sex, age of onset, disease duration, presence of thymoma or thymic hyperplasia, positivity for AChR-Ab or anti-muscle-specific tyrosine kinase antibody, positivity for other concurrent autoantibodies, and disease condition at worst and current. The period from the start of treatment until the achievement of minimal manifestation status (early-stage response) was determined and then compared between subtypes using Kaplan-Meier analysis and the log-rank test. In addition, between subtypes, the rate of the number of patients who maintained minimal manifestations during the study period/that of patients who only achieved the status once (stability of improved status) was compared. RESULTS: As a result of two-step cluster analysis, 923 MG patients were classified into five subtypes as follows: ocular MG (AChR-Ab-positivity, 77%; histogram of onset age, skewed to older age); thymoma-associated MG (100%; normal distribution); MG with thymic hyperplasia (89%; skewed to younger age); AChR-Ab-negative MG (0%; normal distribution); and AChR-Ab-positive MG without thymic abnormalities (100%, skewed to older age). Furthermore, patients classified as ocular MG showed the best early-stage response to treatment and stability of improved status, followed by those classified as thymoma-associated MG and AChR-Ab-positive MG without thymic abnormalities; by contrast, those classified as AChR-Ab-negative MG showed the worst early-stage response to treatment and stability of improved status. CONCLUSIONS: Differences were seen between the five subtypes in demographic characteristics, clinical severity, and therapeutic response. Our five-subtype classification approach would be beneficial not only to elucidate disease subtypes, but also to plan treatment strategies for individual MG patients.


Subject(s)
Myasthenia Gravis/mortality , Severity of Illness Index , Adolescent , Adult , Aged , Aged, 80 and over , Child , Cluster Analysis , Female , Humans , Japan , Kaplan-Meier Estimate , Male , Middle Aged , Myasthenia Gravis/complications , Myasthenia Gravis/drug therapy , Myasthenia Gravis/immunology , Myasthenia Gravis/pathology , Reproducibility of Results , Thymoma/complications , Thymus Neoplasms/complications , Young Adult
18.
Zhongguo Fei Ai Za Zhi ; 19(7): 418-24, 2016 Jul 20.
Article in Chinese | MEDLINE | ID: mdl-27339718

ABSTRACT

BACKGROUND: It is so far not clear that how myasthenia gravis (MG) affected the prognosis of thymoma patients. The aim of this assay is to compare the postoperative survival between patients with thymoma only and those with both thymoma and MG. METHODS: The Chinese Alliance for Research in Thymomas (ChART) registry recruited patients with thymoma from 18 centers over the country on an intention to treat basis from 1992 to 2012. Two groups were formed according to whether the patient complicated MG. Demographic and clinical data were reviewed, Patients were followed and their survival status were analyzed. RESULTS: There were 1,850 patients included in this study, including 421 with and 1,429 without MG. Complete thymectomy were done in 91.2% patients in MG group and 71.0% in non-MG group (P<0.05). There were more percentage of patients with the histology of thymoma AB, B1, or B2 (P<0.05) in MG group, and more percentage of patients with MG were in Masaoka stage I and II. The 5 year and 10 year OS rates were both higher in MG group (93% vs 88%; 83% vs 81%, P=0.034) respectively. The survival rate was significantly higher in patients with MG when the Masaoka staging was III/IV (P=0.003). Among patients with advanced stage thymoma (stage III, IVa, IVb), the constituent ratios of III, IVa, IVb were similar between MG and Non-MG group. Histologically, however, there were significantly more proportion of AB/B1/B2/B3 in the MG group while there were more C in the non-MG group (P=0.000). Univariate analyses for all patients showed that MG, WHO classification, Masaoka stage, surgical approach, chemotherapy and radiotherapy and resectability were significant factors, and multivariate analysis showed WHO Classification, Masaoka stage, and resectability were strong independent prognostic indicators. CONCLUSIONS: Although MG is not an independent prognostic factor, the survival of patients with thymoma was superior when MG was present, especially in late Masaoka stage patients. Possible reasons included early diagnosis of the tumor, better histologic types, an overall higher R0 resection and less recurrence.
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Subject(s)
Myasthenia Gravis/mortality , Thymoma/mortality , Thymus Neoplasms/mortality , Adult , Aged , China , Databases, Factual , Humans , Male , Middle Aged , Myasthenia Gravis/complications , Myasthenia Gravis/pathology , Myasthenia Gravis/surgery , Neoplasm Staging , Postoperative Period , Prognosis , Retrospective Studies , Survival Rate , Thymoma/complications , Thymoma/pathology , Thymoma/surgery , Thymus Neoplasms/complications , Thymus Neoplasms/pathology , Thymus Neoplasms/surgery
19.
Ann Thorac Cardiovasc Surg ; 22(1): 1-5, 2016.
Article in English | MEDLINE | ID: mdl-26822625

ABSTRACT

Robotic surgeries have developed in the general thoracic field over the past decade, and publications on robotic surgery outcomes have accumulated. However, controversy remains about the application of robotic surgery, with a lack of well-established evidence. Robotic surgery has several advantages such as natural movement of the surgeon's hands when manipulating the robotic arms and instruments controlled by computer-assisted systems. Most studies have reported the feasibility and safety of robotic surgery based on acceptable morbidity and mortality compared to open or video-assisted thoracic surgery (VATS). Furthermore, there are accumulated data to indicate longer operation times and shorter hospital stay in robotic surgery. However, randomized controlled trials between robotic and open or VATS procedures are needed to clarify the advantage of robotic surgery. In this review, we focused the literature about robotic surgery used to treat lung cancer and mediastinal tumor.


Subject(s)
Lung Neoplasms/surgery , Mediastinal Neoplasms/surgery , Myasthenia Gravis/surgery , Robotic Surgical Procedures , Thoracic Surgery, Video-Assisted/methods , Thymectomy/methods , Clinical Competence , Humans , Learning Curve , Length of Stay , Lung Neoplasms/mortality , Lung Neoplasms/pathology , Mediastinal Neoplasms/mortality , Mediastinal Neoplasms/pathology , Myasthenia Gravis/diagnosis , Myasthenia Gravis/mortality , Operative Time , Postoperative Complications/etiology , Risk Factors , Robotic Surgical Procedures/adverse effects , Robotic Surgical Procedures/mortality , Thoracic Surgery, Video-Assisted/adverse effects , Thoracic Surgery, Video-Assisted/mortality , Thymectomy/adverse effects , Thymectomy/mortality , Time Factors , Treatment Outcome
20.
Muscle Nerve ; 53(1): 73-7, 2016 Jan.
Article in English | MEDLINE | ID: mdl-25914186

ABSTRACT

INTRODUCTION: In previous studies of myasthenia gravis (MG), increased mortality has been reported. The aim of this study was to estimate mortality in patients with acetylcholine receptor antibody-positive (AChR-Ab-seropositive) MG in a nationwide population-based, long-term follow-up study. METHODS: All AChR-Ab-seropositive MG patients, diagnosed between 1985 and 2005, were identified. Defined by age at diagnosis (≤ 50 or >50 years), patients were classified as having early- or late-onset MG. For comparison, 10 non-MG individuals from the general population were matched with each patient. All patients and controls were followed until January 1, 2009. Mortality rates and estimated mortality rate ratios (MRRs) were calculated. RESULTS: Of 702 AChR-Ab-seropositive MG patients, 302 died during follow-up. Overall mortality was higher for patients with MG (MRR = 1.41, range 1.24-1.60). In late-onset women and men, the MRRs were 1.64 (1.36-1.99) and 1.22 (1.02-1.46), respectively. Total MRR was highest during the first 5 years after diagnosis. CONCLUSIONS: MG diagnosis is still associated with increased mortality.


Subject(s)
Myasthenia Gravis/epidemiology , Myasthenia Gravis/mortality , Adolescent , Adult , Aged , Aged, 80 and over , Antibodies/blood , Community Health Planning , Denmark/epidemiology , Female , Follow-Up Studies , Humans , Kaplan-Meier Estimate , Male , Middle Aged , Proportional Hazards Models , Receptors, Cholinergic/immunology , Retrospective Studies , Young Adult
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