Objective To summarize experiences of surgical treatment and long-term results of myasthenia gravis (MG). Methods Two hundred thirty-six patients underwent thymectomy for MG in our department from Jan.1978 to Dec. 2002. The perioperative management, relative factors of postoperative crisis and long-term results were analysed. Results In 236 patients postoperative crisis took place in 44 cases accounted for 18.6%. The occurrence of postoperative crisis was related to preoperative management, modified Osserman clinical classification and combination with thymoma. Three cases died in the postoperative periods. Among them, one died of acute respiratory distress syndrome induced by aspiration and the other died of crisis. The effective rate in 1, 3, 5 years was 84.6%, 91.0% and 89.0% respectively. Conclusions Thymectomy for MG is safe and effective. Delayed extubation could decrease the needs of tracheotomy in patients with high risk factors for postoperative crisis. The partial sternotomy approach is less traumatic but the long-term effects of surgery are identical to those reported by the most authors.
ObjectiveA two-sample Mendelian randomization analysis was used to explore the causal associations between four basic body indices (basal metabolic rate, body fat percentage, BMI and hip circumference) and myasthenia gravis (MG). MethodsPooled gene-wide association study (GWAS) data were obtained from large publicly searchable databases, and four basic body indices were selected as the exposure factors and myasthenia gravis as the outcome factors, and single nucleotide polymorphisms (SNPs), which were strongly correlated with the phenotype of the exposure factors, were screened as the instrumental variables, and two-sample Mendelian randomization analyses were performed in order to assess the potential causal relationship between the exposure and the disease. ResultsInverse variance weighting (IVW) analysis showed that increased basal metabolic rate (OR=1.39, 95%CI 1.00 to 1.93, P=0.047), body fat percentage (OR=1.61, 95%CI 1.06 to 2.44, P=0.024), and hip circumference (OR=1.67, 95%CI 1.29 to 2.17, P<0.001) increased the risk of MG. But there was no significant causal relationship between BMI and MG. ConclusionBasal metabolic rate, body fat percentage and hip circumference have a positive causal relationship with MG, while BMI does not have a significant causal relationship with MG.
Myasthenia gravis is an autoimmune neuromuscular junction disorder primarily mediated by autoantibodies against the acetylcholine receptor (AChR). It is now widely recognized that the total titer of anti-AChR antibodies does not correlate directly with clinical severity and shows significant interindividual variability. This review focuses on the structure of the AChR, the three major pathogenic mechanisms mediated by anti-AChR antibodies, the pathogenic differences associated with distinct antigenic epitopes, the characteristics of various immunoglobulin subclasses, and the limitations of current antibody detection methods. It further explores future directions in antibody profiling and functional assessment. By systematically analyzing the complexity and heterogeneity of anti-AChR antibodies, this article underscores the critical role of precision medicine in the management of myasthenia gravis.
ObjectiveTo assess the long-term efficacy of extended thymectomy in the treatment of myasthenia gravis(MG) and the factors influencing the results. MethodsWe retrospectively analyzed the clinical data of 45 MG patients who underwent extended thymectomy in our hospital from January 2005 through December 2010. There were 11males and 34 females at age of 32.6(16-50) years. The overall effective rate was calculated. Univariate analysis was used to analyze the risk factors including sex, age, Osserman classification, and pathological type for outcomes. ResultsAll the patients were followed up for a mean of 58.3 months(ranged from 36 to 108 months). The overall effective rate was 80.0%(36/45) and rose with the extention of time. Age(P=0.019), pathological type(P=0.024) of thymus and Osserman classification(P=0.043) of MG were the main factors influencing the outcomes of the treatment except sex(P=0.666). ConclusionExtended thymectomy in the treatment of MG has better outcomes. And the overall effective rate rises with the extention of time.
Objective To compare three surgical treatments for mediastinal mass with myasthenia gravis. Methods Retrospective analysis was performed on the clinical data of 53 patients who underwent extended thymectomy between January 2010 and December 2017 in our hospital. There were 29 males and 24 females, aged 17-73 years. Patients were divided into three groups according to the surgical methods: a group A (video-assisted thoracoscopic surgery with the da Vinci robotic system, n=22), a group B (video-assisted thoracoscopic surgery, n=12) and a group C (median sternotomy, n=19). The gender distribution, age, intraoperative blood loss, operation time, postoperative extubation time, postoperative hospital stay, Osserman classification of myasthenia gravis, postoperative myasthenic remission rate, etc were compared in three groups. Results No perioperative death was observed in 53 patients. One patient in the group C suffered from postoperative myasthenic crisis and improved after active treatment. One patient with video-assisted thoracoscopic surgery was converted to median sternotomy due to the intraoperative injury of the left brachiocephalic vein. Compared with the group B and group C, the group A had shorter operation time, less intraoperative blood loss and drainage on the first postoperative day and fewer days of extubation. Postoperative hospital stay was less in the group A than that in the group C (P<0.05). The postoperative myasthenic remission rate was higher in the group A than that in the other two groups, but there was no statistical difference. Conclusion Because of the robot’s unique minimally invasive advantage, in this study, the outcome of patients with myasthenia gravis treated with Da Vinci robots and thymectomy is better than that of the remaining two groups in terms of perioperative outcomes and myasthenic remission rate. But long-term results and a large of number matching experiments are needed to confirm. However, it is undeniable that robotic surgery must be the future of the minimally invasive surgery.
Abstract: Objective To investigate the clinical characteristics of thymoma and thymoma with myasthenia gravis(MG). Methods From Oct.1979 to July 2004,185 patients with thymoma were surgically treated. Among these patients, comparative analysis was made between 94 cases of thymoma (thymoma group) and 91 cases of thymus tumor with MG(thymoma with MG group).155 patients underwent radical operation (83.8%),16 patients underwent palliative operation (8.6%),and 14 patients underwent exploratory operation (7.6%). Clinical characteristics was analyzed in two groups. The factors affecting prognosis was analyzed by Masaoka’s stage system, with the lifttable method. Results Five patients died after operation, others had complete remission or symptomatic improvement. There was statistically difference of Masaoka’s stage system in two groups (χ2=53.14, P＜0.05). There were no statistically difference in pathological type of thymoma and clinical type of MG and pathologic period (χ2=8.21, P＞0.05). 57 cases of thymoma group were followed up, the duration of follow-up was 1 to 10 years, average follow-up was 40.7 months, and the patients with 1-, 3- and 5-year survival rates were 70.2% (40/57), 66.7% (22/33), 593% (16/27) respectively. 55 cases of thymoma with MG group were followed up. The patients’ survival rates were 98.2% (54/55), 86.4% (38/44), 81.6% (31/38) at 1-, 3-and 5-year respectively. There was no statistically difference of survival rates in two groups (χ2=0.83, P＞0.05). Totally, 112 patients were followed up in two groups, by Masaoka’s stage system, the 5-year survival rates were 93.7% for stage Ⅰ, 79.2% for stageⅡ, 51.4% for stage Ⅲ and 0% for stage Ⅳ respectively. Result of asaoka’s stage system evidence was statistically significant (χ25-year=51.62, P＜0.01). Conclusions Pathological type of thymoma isn’t related to modified Osserman’s classification, prognosis of thymoma is obviously related to Masaoka’s stage and isn’t related to MG. Generalized MG is the major type in MG patients accompanied by thymomas, and the major pathological type is lymphocytic. Chest CT can increase the accuracy early diagnosis of thymoma. The principal treatment is to resect the tumor as completely as possible, and proper administration of postoperative radiotherapy or chemotherapy according to the surgical status. Operative program and tumor stage are the most important prognostic factors.
Thymectomy is a major surgical procedure for patients with non-thymomatous myasthenia gravis，and can enhance their symptomatic remission rate and cure rate. There is still much controversy about appropriate surgical approach and extent of resection of thymectomy. The majority of thoracic surgeons believe that the completeness of thymectomy is closely associated with clinical symptom improvement，and perform complete resection of encapsulated thymus and surroun-ding fat tissues via mid-sternotomy. But minimally invasive thymectomies are often more acceptable by patients. On the contrary，in view of common existence of ectopic thymus tissue，some thoracic surgeons advocate a combination of cervical incision and sternotomy in order to further completely remove all thymus tissue.
ObjectiveTo investigate the effect of noninvasive ventilation (NIV) in patients with myasthenic crisis after thymectomy. Methods31 myasthenic crisis patients after thymectomy who initially used NIV,admitted in the First Affiliated Hospital of Guangzhou Medical University between January 2011 and June 2013,were analyzed retrospectively.They were assigned to two groups according to the successful application of NIV or not,with 13 patients in the NIV success group and 18 patients in the NIV failure group.The related factors including gender,age,APACHEⅡ score when admitted to ICU,the results of blood gas analysis before NIV,thymoma or not,the history of myasthenic crisis,the history of chronic lung disease,and minute ventilation accounted for the largest percentage of predicted value (MVV%pred)were analyzed. ResultsThere were no significant differences in age,gender,or APACHEⅡ score between two groups (P>0.05).The PaCO2 in the NIV success group was lower than that in the NIV failure group.The preoperative MVV%pred in the NIV success group was higher than that in the NIV failure group.There were no significant differences between two groups in pH,PO2,thymoma or not,the history of myasthenic crisis,or the history of chronic lung disease (P>0.05).If using the 45 mm Hg as the cut-off value of PaCO2 and 60% as the cut-off value of MVV%pred,the incidence of PaCO2<45 mm Hg and the incidence of MVV%pred>60% were higher in the NIV success group than those in the NIV failure group (84.6% vs.33.3%, P<0.05;100% vs. 55.6%,P<0.05).Logistic regression analysis revealed that PaCO2<45 mm Hg was an independent influence factor for successful application of NIV in patients with myasthenic crisis after thymectomy. ConclusionPaCO2<45 mm Hg can be a predictor of successful application of NIV in patients with myasthenic crisis after thymectomy.For the patients underwent NIV whose PaCO2<45 mm Hg or MVV%pred<60%,the clinician should predict the possibility of failure and prepared for intubation.
ObjectiveTo analyze the risk factors for post-thymectomy myasthenic crisis (PTMC) and prolonged mechanical ventilation, in myasthenia gravis patients who underwent extended thymectomy. MethodsWe retrospectively analyzed the clinical data of 79 patients including 38 males and 41 females who experienced PTMC and required mechanical ventilation in Daping Hospital between June 2008 and November 2014. Single factor analysis and multivariate analysis were conducted. ResultsMorbidity of PTMC was 20.6% (79/384). Result of single-factor analysis showed that postoperative pneumonia was one of the main reasons of prolonged mechanical ventilation (P < 0.05). Result of multiple-factor analysis showed that the operation time was positively correlated with mechanical ventilation time (P < 0.05). The risk factor of prolonged mechanical ventilation time in PTMC was not associated with sex, age, disease history, myasthenic crisis history, Osserman classification, dosage of pyridostigmine before and after the operation, surgical approach, bleeding volume, other therapies besides mechanical ventilation (P > 0.05). ConclusionMechanical ventilation is one the main therapy of PTMC, operation time, and postoperative pneumonia are the main factors to prolong mechanical ventilation time. In order to decrease morbidity of PTMC and shorten mechanical ventilation time, the operation time should be controlled and pulmonary infection should be avoided.
ObjectiveTo analyze the clinical outcomes of extended thymectomy for myasthenia gravis (MG) patients under different surgical approaches, and to determine the factors affecting the prognosis of MG. MethodsThe MG patients who underwent extended thymectomy from January 2014 to March 2021 in our hospital were retrospectively collected. According to the surgical approach, they were divided into a subxiphoid group and an intercostal group, and the perioperative results and prognosis were compared between the two groups. A “good outcome” was defined as complete stable remission (CSR), pharmacological remission (PR) or minimal manifestations state (MMS); a “poor outcome” was defined as outcomes worse than MMS. Univariate and multivariate logistic regression analyses were performed to assess the factors associated with the good outcomes. ResultsA total of 187 MG patients were included in the study, including 82 males and 105 females, with a median age of 50 (36, 60) years. There were 134 patients in the intercostal group and 53 patients in the subxiphoid group. Compared with the intercostal group, although the operation time of the subxiphoid group was longer [200.0 (172.0, 232.0) min vs. 141.0 (118.0, 169.0) min, P<0.001], the intraoperative blood loss was less [10.0 (10.0, 20.0) mL vs. 20.0 (10.0, 50.0) mL, P<0.001], the postoperative hospital stay was shorter [3.0 (2.5, 4.0) d vs. 5.0 (3.0, 7.0) d, P<0.001], and the incidence of complications was lower [1 (1.9%) vs. 26 (19.4%), P=0.001]. A total of 159 (85.0%) patients were followed up for a median period of 46 (13, 99) months, with a good outcome rate of 90.6% and CSR rate of 33.3%. There were no statistical differences in PR, MMS or overall good outcome rates between the two groups (P>0.05). Multivariate logistic analysis showed that age≤50 years was an independent predictor for "good outcome" of MG patients. ConclusionExtended thymectomy via subxiphoid for MG is a safe, feasible and effective surgical approach.