Objective To evaluate the frequency, distribution and clinical significance of the antibodies to the fetal and/or adult acetylcholine receptor （AChR） in patients with myasthenia gravis （MG）. Methods AChR antibodie...Objective To evaluate the frequency, distribution and clinical significance of the antibodies to the fetal and/or adult acetylcholine receptor （AChR） in patients with myasthenia gravis （MG）. Methods AChR antibodies were detected by cell-based assay in the serum of ocular MG （OMG） （n = 90） and generalized MG （GMG） patients （n = 110）. The fetal-type （2α： β： γ： δ） and adult-type （2α： β： ε： δ） AChR were used as antigens, and their relevance to disease presentation was assessed. Results The overall frequencies of anti-adult and anti-fetal AChR antibodies were similar in all 200 patients examined, with 14 having serum specific to the AChR-γ subunit, and 22 to the AChR-ε subunit. The overall sensitivity when using the fetal and adult AChR antibodies was higher than that when using the fetal AChR antibody only （P = 0.015）. Compared with OMG patients, the mean age at disease onset and the positive ratio of antibodies to both isoforms of the AChR were significantly higher in patients who subsequently progressed to GMG. Older patients and patients with both anti-fetal and anti-adult AChR antibodies had a greater risk for developing generalized disease [odds ratio （OR）, 1.03; 95% confidence interval （CI）, 1.01–1.06 and OR, 5.09; 95% CI, 2.23–11.62]. Conclusion Using both fetal-and adult-type AChRs as the antigens may be more sensitive than using either subtype. Patients with serum specific to both isoforms are at a greater risk of progressing to GMG. Patients with disease onset at an advanced age appear to have a higher frequency of GMG conversion.展开更多
Although respiratory failure commonly occurs during the course of myasthenia gravis (MG),it is rarely described as the first presentation in patients with previously unrecognized MG.Here we reported one case of MG in ...Although respiratory failure commonly occurs during the course of myasthenia gravis (MG),it is rarely described as the first presentation in patients with previously unrecognized MG.Here we reported one case of MG in a 76-year-old man with the isolated respiratory failure as the first symptom.As illustrated by this case,it is important to consider neuromuscular disorders in cases of unexplained respiratory failure.展开更多
Anti-acetylcholine receptor antibodies （AAR） are considered pathognomonic and pathogenetic for myasthenia gravis （MG）. AAR detection confirms clinical diagnosis of MG. However, AAR is rarely detected in patients w...Anti-acetylcholine receptor antibodies （AAR） are considered pathognomonic and pathogenetic for myasthenia gravis （MG）. AAR detection confirms clinical diagnosis of MG. However, AAR is rarely detected in patients without MG. The underlying pathophysiological mechanisms in a normal subject without MG have not been adequately addressed in previous studies. The present study reports on a case study of a healthy, elderly man with high AAR titers for 14 years. Pathophysiological mechanisms could be due to AAR heterogeneity in specificity, affinity, and multiform, and to muscle variability in response to AAR.展开更多
Background Thymectomy is considered the most effective treatment in patients with myasthenia gravis. This study aimed to explore the predictors of postoperative myasthenic crisis in patients with myasthenia gravis aft...Background Thymectomy is considered the most effective treatment in patients with myasthenia gravis. This study aimed to explore the predictors of postoperative myasthenic crisis in patients with myasthenia gravis after thymectomy.Methods Clinical records of 243 patients with myasthenia gravis who underwent thymectomy were reviewed retrospectively. The following factors were analyzed in relation to the occurrence of myasthenic crisis after thymectomy：gender, age, duration of symptoms, Osserman stage, history of myasthenic crisis, concomitant diseases, preoperative pyridostigmine dose, preoperative steroid therapy, operation approach, operation time, presence of thymoma, major postoperative complications.Results Forty-four patients experienced postoperative myasthenic crisis during the first month after thymectomy.Univariate analysis revealed that Osserman stage （RR=0.0976, P=0.000）, history of myasthenic crisis （RR=0.2309,P=0.012）, preoperative pyridostigmine dose （RR=0.4349, P=0.016）, thymoma （RR=0.0606, P=0.000）, and major postoperative complications （RR=0.1094, P=0.000） were significantly related to postoperative myasthenic crisis.Multivariate Logistic regression analysis showed that Osserman stage （Ⅱb＋Ⅲ＋Ⅳ） （RR=0.0953, P=0.000）, thymoma （RR=0.0294, P=0.000）, and major postoperative complications （RR=0.0424, P=0.000） independently predict postoperative myasthenic crisis.Conclusion Osserman stage （Ⅱb＋Ⅲb＋Ⅳ）, thymoma and major postoperative complications are independent predictors of postoperative myasthenic crisis in patients with myasthenia gravis who underwent thymectomy.展开更多
文摘制备了适合于二甲基硅油合成的固体超强碱催化剂,首次开发了以固体超强碱Na-KOH3/r-Al2O3为催化剂,以八甲基环四硅氧烷和六甲基二硅氧烷为原料合成二甲基硅油的新工艺.考察了催化剂用量、反应温度、反应时间和脱低沸物时间等因素对产品粘度和闪点的影响.实验结果表明,在原料配比n(八甲基环四硅氧烷)∶n(六甲基二硅氧烷)=18∶1下,合成二甲基硅油较佳的工艺条件为:催化剂用量为反应原料总质量的1%、反应温度170℃、反应时间5 h、脱低沸物时间3 h.
基金supported by the National Natural Science Foundation of China (31170864 and 81171183)
文摘Objective To evaluate the frequency, distribution and clinical significance of the antibodies to the fetal and/or adult acetylcholine receptor （AChR） in patients with myasthenia gravis （MG）. Methods AChR antibodies were detected by cell-based assay in the serum of ocular MG （OMG） （n = 90） and generalized MG （GMG） patients （n = 110）. The fetal-type （2α： β： γ： δ） and adult-type （2α： β： ε： δ） AChR were used as antigens, and their relevance to disease presentation was assessed. Results The overall frequencies of anti-adult and anti-fetal AChR antibodies were similar in all 200 patients examined, with 14 having serum specific to the AChR-γ subunit, and 22 to the AChR-ε subunit. The overall sensitivity when using the fetal and adult AChR antibodies was higher than that when using the fetal AChR antibody only （P = 0.015）. Compared with OMG patients, the mean age at disease onset and the positive ratio of antibodies to both isoforms of the AChR were significantly higher in patients who subsequently progressed to GMG. Older patients and patients with both anti-fetal and anti-adult AChR antibodies had a greater risk for developing generalized disease [odds ratio （OR）, 1.03; 95% confidence interval （CI）, 1.01–1.06 and OR, 5.09; 95% CI, 2.23–11.62]. Conclusion Using both fetal-and adult-type AChRs as the antigens may be more sensitive than using either subtype. Patients with serum specific to both isoforms are at a greater risk of progressing to GMG. Patients with disease onset at an advanced age appear to have a higher frequency of GMG conversion.
文摘Although respiratory failure commonly occurs during the course of myasthenia gravis (MG),it is rarely described as the first presentation in patients with previously unrecognized MG.Here we reported one case of MG in a 76-year-old man with the isolated respiratory failure as the first symptom.As illustrated by this case,it is important to consider neuromuscular disorders in cases of unexplained respiratory failure.
文摘Anti-acetylcholine receptor antibodies （AAR） are considered pathognomonic and pathogenetic for myasthenia gravis （MG）. AAR detection confirms clinical diagnosis of MG. However, AAR is rarely detected in patients without MG. The underlying pathophysiological mechanisms in a normal subject without MG have not been adequately addressed in previous studies. The present study reports on a case study of a healthy, elderly man with high AAR titers for 14 years. Pathophysiological mechanisms could be due to AAR heterogeneity in specificity, affinity, and multiform, and to muscle variability in response to AAR.
文摘Background Thymectomy is considered the most effective treatment in patients with myasthenia gravis. This study aimed to explore the predictors of postoperative myasthenic crisis in patients with myasthenia gravis after thymectomy.Methods Clinical records of 243 patients with myasthenia gravis who underwent thymectomy were reviewed retrospectively. The following factors were analyzed in relation to the occurrence of myasthenic crisis after thymectomy：gender, age, duration of symptoms, Osserman stage, history of myasthenic crisis, concomitant diseases, preoperative pyridostigmine dose, preoperative steroid therapy, operation approach, operation time, presence of thymoma, major postoperative complications.Results Forty-four patients experienced postoperative myasthenic crisis during the first month after thymectomy.Univariate analysis revealed that Osserman stage （RR=0.0976, P=0.000）, history of myasthenic crisis （RR=0.2309,P=0.012）, preoperative pyridostigmine dose （RR=0.4349, P=0.016）, thymoma （RR=0.0606, P=0.000）, and major postoperative complications （RR=0.1094, P=0.000） were significantly related to postoperative myasthenic crisis.Multivariate Logistic regression analysis showed that Osserman stage （Ⅱb＋Ⅲ＋Ⅳ） （RR=0.0953, P=0.000）, thymoma （RR=0.0294, P=0.000）, and major postoperative complications （RR=0.0424, P=0.000） independently predict postoperative myasthenic crisis.Conclusion Osserman stage （Ⅱb＋Ⅲb＋Ⅳ）, thymoma and major postoperative complications are independent predictors of postoperative myasthenic crisis in patients with myasthenia gravis who underwent thymectomy.
