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find Keyword "重症肌无力" 64 results
  • 血浆置换辅助重症肌无力危象患者撤机的观察

    目的 回顾血浆置换辅助治疗重症肌无力危象伴撤机困难患者的疗效。方法 选择2000 ~2005 年因撤机困难入住广州呼吸疾病研究所ICU的重症肌无力患者11 例, 均需有创呼吸机辅助通气。其中接受了血浆置换( PE) 治疗7 例, 称PE 组; 仅接受常规治疗4 例, 称对照组。比较两组患者的住院天数、总通气天数、ICU 天数、用PE 前( 后) 的机械通气天数, 以及PE 前和撤机时的许氏临床相对评分。结果 血浆置换组用PE 前的通气天数( 19. 7 ±21. 3) d, 接受PE 后的通气天数为( 2. 57 ±5. 94) d, 总通气天数( 27 ±23) d; 对照组的总通气天数( 26 ±22) d, 两组的住院天数、总通气天数、ICU 天数均无显著差异。PE 组治疗前许氏绝对评分平均为44 分,4 次PE 治疗后许氏绝对评分平均为18 分, 相对计分为0. 59。结论 对这类撤机困难的重症肌无力危象患者应用PE 治疗4 次后, 在不增加治疗总费用的前提下, 能在较短时间内协助患者尽快撤机; 对急性呼吸衰竭机械通气的重症肌无力患者, 在综合治疗前提下, 机械通气7 d 以上仍难以撤机者, 可考虑应用血浆置换。

    Release date:2016-09-14 11:23 Export PDF Favorites Scan
  • 自身免疫性多腺体综合征Ⅳ型一例

    Release date:2021-03-19 07:10 Export PDF Favorites Scan
  • Clinical pathological characteristics and prognosis of 468 thymoma patients

    ObjectiveTo assess the correlation of WHO pathological classification and Masaoka stage of thymomas with its prognosis.MethodsA total of 468 patients with thymomas who received surgeries during 2009-2019 in Huashan Hospital, Fudan University, were collected. There were 234 males and 234 females with an average age of 21-83 (49.6±18.7) years. A total of 132 patients underwent video-assisted thoracic surgery (VATS) and 336 patients underwent thymectomy with median sternal incision. The follow-up time was 5.7±2.8 years. The clinical data of the patients were analyzed.ResultsThe amount of intraoperative bleeding was 178.3±133.5 mL in the median sternal incision group, and 164.8±184.1 mL in the VATS group (P=0.537). The operative time was 3.3±0.7 h in the median sternal incision group and 3.4±1.2 h in the VATS group (P=0.376). Postoperative active bleeding, phrenic nerve injury and chylothorax complications occurred in 8 patients, 9 patients and 1 patient in the VATS group, respectively, and 37 patients, 31 patients and 7 patients in the median sternal incision group, respectively. There was no statistical difference between the two groups (P=0.102, 0.402, 0.320). The 5-year cumulative progression free survival (PFS) rates of patients with WHO type A, AB, B1, B2, B3 and C thymomas were 100.0%, 100.0%, 95.7%, 81.4%, 67.5% and 50.0%, respectively (P<0.001). The 5-year PFS rates of patients with Masaoka stageⅠ-Ⅳ thymomas were 96.1%, 89.2%, 68.6% and 19.3%, respectively (P<0.001). The 5-year PFS rate was 87.3% in patients with myasthenia gravis (MG) and 78.2% in patients without MG (P<0.001). The 5-year PFS rates of patients with different surgeries were 82.4% and 83.8%, respectively (P=0.904). ConclusionWHO pathological classification and Masaoka stage have significant clinical prognosis suggestive effect. Thymoma patients combined with MG have better prognosis, which suggests early diagnosis and treatment of thymoma are important.

    Release date:2021-12-27 11:31 Export PDF Favorites Scan
  • 视神经脊髓炎谱系疾病合并重症肌无力、干燥综合征及桥本甲状腺炎首例报告

    Release date:2018-03-07 04:35 Export PDF Favorites Scan
  • Comparison of perioperative outcomes of robotic trans-subxiphoid and video-assisted thoracoscopic extended thymectomy in the treatment of myasthenia gravis complicated with thymoma

    ObjectiveTo compare the perioperative outcomes of subxiphoid robot-assisted extended thymectomy (SRAET) and video-assisted thoracoscopic extended thymectomy (VATET) for myasthenia gravis complicated with thymoma.MethodsRetrospective analysis of 61 patients with myasthenia gravis combined with thymoma who were admitted to the Department of Thoracic Surgery, West China Hospital, Sichuan University from January 2017 to June 2019 was performed. All patients underwent extended thymectomy, and the patients were divided into a SRAET group and a VATET group. There were 26 patients in the SRAET group, including 11 males and 15 females, with an average age of 42.20±13.20 years. There were 35 patients in the VATET group, including 14 males and 21 females, with an average age of 45.00±13.00 years. The perioperative outcomes of the two groups including gender, age, operation time, intraoperative blood loss, conversion rate, postoperative drainage, tube removal time, drainage volume, visual analogue scale, hospital stay and postoperative complications were compared.ResultsThere was no conversion to thoracotomy, death or myasthenia crisis in both groups. The operation time (111.42±28.60 min vs. 103.71±26.20 min, P=0.845), intraoperative blood loss (32.31±23.84 mL vs. 63.57±132.22 mL, P=0.239), visual analogue scale at postoperative 24 h (2.46±0.76 vs. 2.40±0.74, P=0.751) and postoperative 48 h (2.12±0.77 vs. 2.26±0.56, P=0.407), complication rate (3.8% vs. 2.9%, P=0.675), drainage volume (206.85±130.09 mL vs. 276.86±173.46 mL, P=0.089) and hospital stay (5.81±2.52 d vs. 5.29±2.17 d, P=0.642) were not significantly different between the two groups. The visual analogue scale of the SRAET group at postoperative 72 h (1.12±0.65 vs. 1.86±0.91, P=0.001) was significantly lower than that of the VATET group.ConclusionSRAET is a safe and feasible method with less postoperative short-term pain, which is an alternative surgical treatment for myasthenia gravis complicated with thymoma.

    Release date:2020-12-07 01:26 Export PDF Favorites Scan
  • Value of ice test in diagnosis of myasthenia gravis

    Objective To investigate the value of ice test in the diagnosis of ptosis of myasthenia gravis(MG). Methods A total of 32 patients with myasthenic ptosis and 33 with nonmyasthenic ptosis underwent ice and rest test which were performed alternately twice within 1 day on each patient. Besides, neostigmine test was performed on the patients with myasthenic ptosis after ice and rest test . Two observers who didnrsquo;t know the clinical diagnosis were asked to evaluate the improvement of eyelid elevation by measuring the width between the midpoints of upper and lower eyelid with a 20mm steel rule (precision of 0.5 mm). The average of margin of palpebral fissure width after double ice or rest tests subtrac ted from the one before the tests in one patient was the standard of the improve ment of eyelid elevation. Results Ice and rest test improved myasthenic ptosis but not nonmyasthenic ptosis with the specificity of 100% in both of the tests. In addition, ice test improved myasthenic ptosis more effectively with a higher sensitivity of 78%, and it could also improve the palpebral fissure width in pa i tents with complete myasthenic ptosis apparently. Compared with the neostigmine test, ice test had lower sensitivity, cost shorter time, didnt need injection which avoided the discomfort, and had no side effects. Conclusion Ice test is a simple and safe means with high sensitivity and specificity to diagnose myasthenic ptosis, which is valuable in clinical application. (Chin J Ocul Fundus Dis, 2006,22:382-384)

    Release date:2016-09-02 05:51 Export PDF Favorites Scan
  • Evaluation of Risk Factors Affecting the Postoperative Prognosis in Patients with Myasthenia Gravis

    Objective To evaluate the risk factors affecting the prognosis in patients with myasthenia gravis after thymeetomy. Methods Therapeutic effects were evaluated with Relative Counting Method in 136 cases with myasthenia gravis who receivingthymectomy. Six clinical factors including sex, age, preoperative course, et al. were converted into quantitative parameters and used for analysis. Cumulative logit model for ordinal response was employed to investigate the therapeutic effects of various factors. Results Sex, age and preoperative course exerted significant effects on postoperative prognosis in patients with myasthenia gravis, while clinical subtype, pathological changes and preoperative administration of cholinesterase inhibitors had no significant impact on therapeutic effects. Conclusion Some factors may lead to the remission and improvement in postoperative patients with myasthenia gravis, therefore, they should be included for the evaluation of prognosis. Early diagnosis and early thymeetomy may improve the therapeutic effects and shorten the duration of amelioration.

    Release date:2016-08-30 06:26 Export PDF Favorites Scan
  • 96例重症肌无力患者的外科治疗

    目的 总结重症肌无力(MG)患者的外科治疗和围术期处理经验,以提高手术疗效。 方法 2002年1月至2007年6月,对96例MG患者行胸腺切除加前纵隔脂肪组织清扫术,根据临床相对记分、服药量改变及生活能力于术后3个月评估临床疗效。 结果 无围术期死亡。术后发生MG危象8例(8.3% ),其中术前未服用糖皮质激素6例(18.75%),服用糖皮质激素2例(3.13%),经相应的治疗治愈。术后发生其他并发症9例(9.4%),其中肺部感染7例,切口感染2例,均经相应的治疗治愈。随访96例,随访时间3~18个月,其中32例临床痊愈,30例基本痊愈,15例显效,12例好转,7例无效。 结论 MG患者经内科治疗效果不佳或无效时,均应考虑手术治疗,无论是否有胸腺增生,特别是对合并有胸腺瘤者,应限期手术。若决定行手术治疗,术前除继续服用抗胆碱酯酶药物外,口服糖皮质激素15d左右可增加手术安全性,提高手术疗效。

    Release date:2016-08-30 06:04 Export PDF Favorites Scan
  • Causal association between basic body mass index and myasthenia gravis: a two-sample Mendelian randomization study

    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.

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  • Risk Factors of Myasthenic Crisis after Thymectomy for Patients with Myasthenia Gravis: A Meta-analysis

    ObjectiveTo explore the risk factors of myasthenic crisis after thymectomy (MCAT) for patients with myasthenia gravis (MG). MethodsWe searched PubMed, EMbase, The Cochrane Library (Issue 8, 2015), Web of Knowledge, CBM, CNKI and WanFang Data from inception to August 31, 2015, to collect case-control studies and retrospective cohort studies about the MCAT for patients with MG. Two reviewers independently screened literature, extracted data and assessed the risk of bias of included studies. Then meta-analysis was performed using Stata 13.0 software. ResultsA total of 17 studies involving 394 patients with myasthenic crisis and 1642 controls were included. Of the 17 studies, 11 were retrospective cohort studies and 6 were case-control studies. The results of meta-analysis showed that:a) univariate analysis indicated that history of myasthenic crisis (OR=8.05, 95%CI 5.80 to 11.15, P<0.01), bulbar symptoms (OR=5.10, 95%CI 3.01 to 8.67, P<0.01), preoperative severity of gravis (Osserman-stage) (OR=10.55, 95%CI 7.28 to 15.30, P<0.01), postoperative pulmonary infection (OR=10.77, 95%CI 3.88 to 29.95, P<0.01), thymoma (OR=2.37, 95%CI 1.50 to 3.75, P<0.01), dose of pyridostigmine (MD=0.45, 95%CI 0.29 to 0.62, P<0.01), AChRAb level >100 nmol/L (OR=12.14, 95%CI 4.80 to 30.73, P<0.01) and operation time (MD=0.57, 95%CI 0.26 to 0.88, P<0.01) were the risk factors of MCAT; b) multivariate analysis showed that, history of myasthenic crisis (OR=5.06, 95%CI 2.30 to 11.14, P<0.01), bulbar symptoms (OR=5.21, 95%CI 2.62 to 10.35, P<0.01), preoperative severity of gravis (Osserman-stage) (OR=5.82, 95%CI 2.60 to 13.04, P<0.01) and AChRAb level >100 nmol/L (OR=8.38, 95%CI 3.31 to 23.08, P<0.01) were the independent risk factors of MCAT. ConclusionThe independent risk factors of MCAT for patients with MG are history of myasthenic crisis, bulbar symptoms, preoperative severity of gravis (Osserman-stage) and AChRAb level >100 nmol/L.

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