Mortality In Adult Myasthenia Gravis Patients And Humanized SCID Mice Of Myasthenia Gravis And Marker

Objective:To analyze the mortality and the potential risk factors for death in MG patients. Methods:2195 adult patients with myasthenia gravis (aged older than 18 years) diagnosed before 2013 at Tongji hospital have been followed up and retrospectively reviewed. Results:During 10-year follow-up,129 patients died and the total fatality rate was 5.88%. The causes of death were various, including cancers, myasthenia gravis, heart attacks, pulmonary diseases, strokes, gastrointestianl bleeding and suicide.65.9% of death happened at hospitals and (34.1%) in residential communities.The risk factors associated with MG-related death were the disease course, occurrence of myasthenic crisis, the severity of disease in MGFA types III and IV at onset, elevation of AchR-abs titers, thymic pathology, and failure of administrating immunosuppressants (p<0.05). In addition, the non-MG related factors including the preceding strokes, chronic obstructive pulmonary disease (COPD), diabetes, atrial fibrillation, hyperlipidemia, myocardial infarction and malignant tumors, were closely linked with death in the MG population (HR were 3.251,4.173,3.738,3.886,1.945,2.177and 14.7 respectively, p<0.05). However, Peripheral vascular disease (PVD), heart failure, hypertension, chronic nephrology, and smoking were not significantly related to death (p>0.05).Conclusion: Death in MG patients remains a major concern. The severity of disease at entry, presence of AchRabs, thymic pathology and duration of the disease predict a higher risk for death. Systemic illness such as strokes, COPD, diabetes, atrial fibrillation, hyperlipidemia, myocardial infarction and malignant tumors which will increase the risk of death should be carefully monitored and managed.Objective:T cell in patients with myasthenia gravis crisis were injected into the SCID mice to induce mice model of myasthenia gravis and detection of cytokines.Methods:32 male SCID mice bred in SPF animal laboratory. All mice were intraperitoneal injected of cyclophosphamide (CTX) according to 40mg/kg for four consecutive days. After 5 days, Cultured 2 × 106 lymphocytes were injected to SCID mice every week, and the control group was given PBS. Clinical scores, Grip strength, Inverted screen test, electromyography were recorded, ELISA method to detect the acetylcholine receptor antibody, IL-6 and IL-4.Results:Six mice of the experimental group were identified as myasthenia gravis, Clinical scores, Grip strength, Inverted screen test were worse than the control group,6 mice of electromyography showed positive, acetylcholine receptor antibody detection of experimental group is higher than the control group, and it is no different about IL-6 or IL-4 between two groups.Conclusion: T cell proliferation in patients with myasthenia gravis can induce humanized SCID mouse model, and maybe a foundation for the personalized treatment of myasthenia gravis crisis in the future.Objective:To research the results of diffusion tensor imaging (DTI) and magnetic resonance spectroscopy (MRS) in amyotrophic lateral sclerosis (ALS) patients.Methods:During October 2013 to July 2014, nineteen ALS patients and thirteen age-matched healthy controls underwent MRS and DTI Tongji hospital. N-acetylaspartate (NAA),Fractional anisotropy (FA), choline (Cho), creatine (Cr), and apparent diffusion coefficient (ADC) were collected as the quantitative outcome of DTI or MRS. Patient disability assessment were evaluated by disease progression rate and the ALS functional rating scale-revised(ALSFRS-R) The DTI and MRS results between ALS patients and healthy controls were compared. The relationship was analyzed between disability assessment and imaging study results.Results:NAA/Cr in the motor cortex and FA in the corticospinal tract (CST) of both sides were significantly lower in patients than controls. There was no significant difference between the two groups in Cho/Cr, tract length, tract volume, ADC or NAA. No relationship was found between ALSFRS-R and FA (r=0.243, p=0.316) in the right CST; NAA (r=0.095, p=0.699) or NAA/Cr (r=0.172,p=0.481) in the left motor cortex; or NAA (r=0.320,p=0.182) or NAA/Cr (r=0.193,p=0.492) in the right motor cortex. There was no relationship between the disease progression rate and FA, NAA, or NAA/Cr on either side.Conclusion:NAA/Cr and FA on each side can help diagnose ALS. Only regional brain NAA/Cr and FA values could not assess disease progression rate or the ALSFRS-R.