Objective To describe the clinical features and genetic susceptibility of the familial myasthenia gravis (FMG) patients. Methods The clinical data of 47 FMG patients and 2953 sporadic myasthenia gravis (SMG) patients from 1977 to 2003 were analyzed retrospectively, and 15 FMG patients, 36 SMG patients and 47 healthy controls were investigated for human leukocyte antigen (HLA)-DQA1 genotyping by polymerase chain reaction-sequence specific primers. Results FMG was an autoimmune disorder disease and immunosuppressive therapy was as highly effective in FMG as in SMG. In all patients, 11(23.4%) cases improved significantly, 14(29.8%) cases relieved with only 5 mg prednidone, and 20(42.6%) cases relieved completely. Most FMG occurred in siblings and familial members within 2 generations, and no sexual difference was found. These suggested it should be atypical Mendel’s rules. Onset and clinical features of patients within the same family often had little difference. As compared with the SMG patients, the frequence of DQA1 *0301 allele was higher in FMG patients, especially in the ocular form, and the differences had statistical significance(40.0% vs 19.4%, P

Objective To improve the diagnosis and treatment of liver trauma. Methods We retrospectively analyzed different therapeutic means on liver trauma including 79 cases with operative treatment and 27 cases with nonoperative treatment. Results In nonoperative treatment group, 25 cases were cured, 2 cases died. In operative treatment group, 73 cases were cured, 6 cases died. Cure rate was 92.5%(98/106), mortality was 7.5%(8/106). 5 cases died of major blood vessels rupture, 3 cases died on multiple organ function failure. Postoperative complications included 3 cases of subphrenic infection, 10 cases of hepatic abscess, 8 cases of pleural hydrops, 7 cases of incisional infection. Conclutions Type I of liver trauma can be treated by nonoperation, type Ⅱ～Ⅳ of blunt liver trauma can be treated by nonoperation on the condition of intensive monitoring. Type Ⅱ～ Ⅵ of liver trauma should be operated on emergently in case of massive intraabdominal bleeding and combined organ injury.

0.05),but the duration of the peaking period, the time for improving one grade, the time for reaching 2 grade, and the hospitalization was significantly shorter in patients treated with steroid than without steroid(P0.05). (3) The major side effects included Cushing's reaction and the infection of the upper respiratory tract.Conclusion Steroids can quicken the recovery of GBS, and shortened average hospitalization in patients with GBS, but steroid doesn't alter the incidence rate of the sequel and the severity of the disability at 1 year in the course.It has no side reaction to use steroid reasonably.

Objective To investigate the clinical features of myasthenia gravis (MG) in elderly people. Methods Elderly patients among all MG patients observed from February 1977 to February 2003 were respectively reviewed. Results Of 3010 MG patients,232(7.7%) were identified with the onset of the disease after or at the age of 60,and males were more than females with the ratio of 1.3∶1. The first and most commonly seen symptom in the elderly people after MG onset were ocular manifestation,occurring in 79.3%. Bulbar symptoms(12.9%) such as dysarthria and swallowing difficulties were the following symptoms. The systemic form (62.9%) were significantly more than ocular form (37.1%). There were less MG patients with autoimmunity diseases such as thyroid diseases associated with MG than that with the other senile diseases,and the myasthenia crisis were even less in the elderly patients with MG. However,it was found that more MG patients with thymoma than with other thymus abnormality. Conclusions The elderly MG patients had its distinctive clinical features. It is beneficial for diagnosing and treating the MG patients in the elderly people by understanding these clinical features.

Objective To evaluate the long-term outcomes of immunosuppressive agents used for myasthenia gravis(MG) patients with disease onset after or at 60. Methods Long-term outcome was evaluated in 99 cases with following-up longer than 1 year. Remission, pharmacological remission, and marked improvement were considered as good results or good long-term outcome. Results All good results were recorded in 79 MG patients (79.8%) receiving immunosuppressive treatments. 48 patients had been treated with glucocorticoids (GC) alone, of them, 35(72.9%) achieved good results. 8 of them having a complete remission for 18 to 67 months (averaging (54.8?19.8) months). 16 patients got pharmacological remission for 7 to 37 months (averaging (15.7?8.0) months). MG patients in different gender had different responses to GC. The good long-term outcome was found in 83.3% (25/30) males and only 55.6% (10/18) in females with GC therapy. There was a rate statistically higher in male than in female patients (P

Objective To explore the clinicopathological characteristics of early gastric cancer (EGC),surgical treatment, and prognosis in elderly patients. Methods The clinicopathological and follow-up data in 83 elderly patients with EGC treated by gastrectomy from 1989. 8-2004. 8 were studied retrospectively. Results All of 83 patients were resectable, and there was no operative death nor surgical complications. The 5-, 10-, and 15- year survival rate was 98%,95%,and 92%, respectively. Sixty-one cases (74%) were mucosal cancer without lymph node metastasis, 22 cases were submucosal cancer with node metastasis in 4 cases. 7 cases were multiple primary carcinoma. Conclusions Local resection and partial gastrectomy are not suitable for multiple primary carcionoma in elderly patieats with EGC. Subtotal gastrectomy, and total gastrectomy should be the procedure of choice when foci were scattered about. Lymph node metastasis,multiple primary cancer foci and invasion of submucosal layer are all among factors impacting postoperative prognosis.

Objective To evaluate the long-term outcome of thymectomy combined with steroid therapy for myasthenia gravis (MG) with thymoma.Methods Fifty-two MG patients with thymoma (35 males, 17 females) treated with thymectomy combined with steroid therapy underwent a retrospective study during a follow-up period of 1 to 20 years (mean 4.5±3.6) after thymectomy in order to evaluate the long-term effective rate and survival rate. Five cases had ocular MG, and 47 had generalized MG (90.3%). The patients at onset from 8 to 61 years (mean 38.5±12.7). All patients were given steroid therapy before and after thymectomy. At the start of therapy, prednisone was given 30 mg to 60 mg daily. This dose was maintained until obvious improvement was found, after which prednisone dosage was gradually tapered to 10 mg to 20 mg daily as a maintenance dose and continued for 1 to 2 years. In all these patients the extended thymectomy was performed by median sternotomy, if possible, a complete resection of the thymoma and the thymic gland, including perithymic fat tissue was given. The survival rate after thymectomy was calculated employing the Kaplan-Meier's method.Results The long-term effective rate was 88.5% (46/52). In the 46 survivors, 8 of them (15.4%)obtained complete remission (patients asymptomatic without any therapy for at least 6 months), 29 of them (55.8%) had pharmacological remission (patients asymptomatic still on low dose corticosteroids or anticholinesterases), 9 of them (17.3%) had marked improvement (stable improvement of myasthenic signs corresponding to a decrease of 2 points on the functional scale of Oosterhuis et al). Six patients (11.5%) died during the observation period. Two of them died in one year after thymectomy; 4 of them died during the follow-up period in from 1 to 20 years. The causes of death after surgery were: thymoma relapse and metastasis in 2 patients, heart failure after operation in 1, myocardial infarction in 1, myasthenic crisis in 1 and digestive tract hemorrhage due to high dose steroid in 1. The mortality of patients with invasive thymoma was 23% (3/13); the mortality of patients with noninvasive thymoma was 7.7% (3/39). The 1-year survival rate after operation was 96.2% (50/52), 3-year survival rate 91.4% (32/35), the overall 5-year survival rate 85.7% (18/21), the overall 7-year survival rate 77.8% (14/18) and the overall 10-year survival rate 33.3% (3/9).Conclusions The long-term therapeutic results of thymectomy combined with steroid therapy were good for myasthenia gravis patients with thymoma. Our retrospective study results showed that thyectomy combined with steroid therapy played an important role in decreasing mortality and increasing remission rate.

Objective To discuss the effectiveness and safety of Ommaya sar implantation through neuronavigation in treatment of intracranial cysts. Methods Twenty-eight patients with intracranial cysts (cystic glioma, cystic metastases, radioactive encephalopathy cystic necrosis), admitted to our hospital from January 2007 to December 2014, were chosen in our study; these patients accepted Ommaya sar implantation through neuronavigation. The clinical data, disease courses, CT scan results, operation efficacies and postoperative complications of these patients were retrospectively analyzed. Results In the 8 patients with cystic gliomas, improvement of clinical symptoms and activity of daily living was noted in 6 patients; imaging re-check indicated focus shrink<50%in 6 patients, enjoying an effective rate of 75%. In the 12 patients with cystic metastases, the improvement of clinical symptoms and activity of daily living was noted in 9 patients; imaging re-check indicated focus shrink<50% in 9 patients, enjoying an effective rate of 75%. In the 8 patients with radioactive encephalopathy cystic necrosis, the improvement of clinical symptoms and activity of daily living was noted in 7 patients;imaging re-check indicated focus shrink<50%in 7 patients, enjoying an effective rate of 87.5%. No antidromic intracranial infection was noted in these 28 patients. Conclusion Implantation of Ommaya sar through neuronavigation is an effective treatment in intracranial cysts, enjoying minimally invasiveness.

BACKGROUND: A number of clinical trials addressing olfactory ensheathing cells for the treatment of chronic spinal cord injury have been conducted in the world, but the efficacy and safety are still controversial. OBJECTIVE: To evaluate the safety and efficacy of olfactory ensheathing cell transplantation for chronic spinal cord injury, and to further compare its short-and long-term efficacy. METHODS: PubMed, Cochrane Library, EMBASE, CNKI and WanFang databases were searched at July 23, 2018 for retrieval of clinical trials addressing olfactory ensheathing cells in the treatment of chronic spinal cord injury. Types and cases of adverse events during the safety trial should be recorded in detail. In the enrolled studies, American Spinal Injury Association scale was used to assess the motor, light touch, and pinprick scores of spinal cord injury patients before and after cell transplantation. The follow-up time was recorded. Systematic evaluation of efficacy data was performed using Review Manager 5.3. RESULTS AND CONCLUSION: Both short-and long-term follow-up data showed that the neurological function of patients was significantly improved after olfactory ensheathing cell transplantation (P < 0.05) , and the results were homogeneous (I2 < 50％ and P> 0.1). However, the long-term efficacy was not as good as the short-term efficacy, which may be related to chronic rejection and olfactory ensheathing cell survival. The overall adverse event rate was 8.99％, and no complications associated with olfactory ensheathing cells occurred. These findings show that olfactory ensheathing cell transplantation is effective and safe in the treatment of chronic spinal cord injury, but it is still necessary to explore more minimally invasive approaches to reduce surgical complications. In addition, a large number of high-quality experiments and clinical trials are warranted to confirm factors affecting the long-term efficacy of olfactory ensheathing cell transplantation.

Objective:To assess the occurrence, prognosis and possible early risk factors of jaundice in polytrauma patients.Methods:This study was a single-center, prospective study. Polytrauma patients (age>18 years) admitted to Tongji Trauma Center from October 2020 to January 2023 were enrolled. The patients with liver, biliary tract or pancreatic traumatic injury, previously suffered from chronic liver disease were excluded. The clinical characteristics of patients, laboratory test results, imaging examination results, Injury Severity Score (ISS), Glasgow Coma Score and APACHEⅡ score were collected. The incidence of jaundice, the classification of jaundice or the severity of jaundice after multiple injuries, the mortality rate of polytrauma patients with jaundice, and the early independent risk factors of jaundice in polytrauma were analyzed. The differences between the groups were compared by Student’s t test or χ2 test. The independent risk factors of jaundice were analyzed by Logistic regression analyzed. Results:A total of 742 polytrauma patients were included, 34.09% polytrauma patients were accompanied by jaundice, and the ratio of both moderate and severe jaundice were as high as 32.41%. The main type of jaundice was intrahepatic cholestatic jaundice (47.03%). The mortality rate of polytrauma patients accompanied by jaundice was significantly higher than that of polytrauma patients without jaundice (12.25% vs. 3.47%, P<0.001). Logistic regression analysis showed that ISS score ( OR=3.405, 95% CI: 1.962-7.438, P=0.026), plasma lactate ( OR=2.216, 95% CI: 1.203-4.862, P=0.017), interleukin-6 levels ( OR=2.431, 95% CI: 1.424-3.793, P=0.007), the overall duration of parenteral nutrition ( OR=3.011, 95% CI: 1.624-5.041, P=0.022), and the total duration of mechanical ventilation ( OR=3.572, 95% CI: 1.497-4.601, P=0.031) were the early independent risk factors for jaundice in patients after polytrauma. Conclusions:Polytrauma patients are prone to developing jaundice after injury, which is more harmful, especially for intrahepatic cholestatic jaundice after injury. Early identification and early intervention of risk factors associated with jaundice after injury should be strengthened.