重症肌无力患者胸腺切除术后发生肌无力危象的危险因素分析

目的:分析重症肌无力患者胸腺切除术后发生肌无力危象的危险因素。方法:对实施胸腺切除术的306例重症肌无力(MG)患者的临床资料进行回顾性分析,利用Logistic回归从多种相关因素(例如年龄、分型、术前术后处理、手术方式、预后等)中筛选出危险因素。结果:术后(半年内)发生肌无力危象与患者发病年龄、手术时间、病程、术前分型、术前是否累及吞咽肌、术前是否发生危象、是否确定为胸腺瘤。结论:术前吞咽肌受累、术前发生危象、确诊胸腺瘤是预测胸腺切除术后肌无力危象发生的独立危险因素。     

老年晚发型重症肌无力患者术后肌无力危象的危险因素

目的探讨老年晚发型重症肌无力(LOMG)患者术后发生肌无力危象的危险因素。方法回顾分析该院2008年6月至2014年6月LOMG合并胸腺瘤行扩大胸腺切除术患者的临床资料,采用Logistic回归分析术后发生肌无力危象的易感因素。结果术后共发生肌无力危象15例(25.9%)。单因素分析术后肌无力危象发生与术前溴吡斯的明剂量较大(P0.001),术前发生过肌无力危象、Osseman分型高、手术方式、术后出现肺感染及术后未使用激素冲击有关(均P0.05)。术前Osseman分型高(OR=5.544,P=0.042)和手术方式(OR=14.727,P=0.049)是发生肌无力危象的独立因素。术后使用激素冲击(OR=0.083,P=0.048)是预防肌无力危象的保护因素。结论 Osseman分型高、胸骨劈开术式、术后未使用激素冲击易发生肌无力危象。     

重症肌无力术后早期危象的预测因素

重症肌无力(MG)最严重的临床表现即病变累及呼吸肌引起的肌无力危象[1].肌无力危象致死率高,需给予机械通气等呼吸支持治疗及重症监护[2].目前,在MG的综合治疗方案中,胸腺切除术仍占有重要地位[3,4].胸腺切除术的远期疗效及预后影响因素、肌无力危象的诱发因素多为之前研究关注热点.但对胸腺切除术后肌无力危象预测因素的分析却报道较少,且争论较大.本研究回顾性分析2002年7月至2012年7月在我科行胸腺切除术的MG患者的临床资料,以探索影响术后肌无力危象发生的危险因素,为临床工作中防治术后肌无力危象提供依据.     

胸腺瘤切除术的围手术期处理的改进和预防重症肌无力的发生

胸腺瘤的主要治疗手段是手术,但手术会引起肌无力危象的发生。改进围手术期处理,可以明显降低肌无力危象的发生。术前、术中、术后使用肾上腺糖皮质激素及吡啶斯的明可以有效防止胸腺瘤病人围手术期出现肌无力危象。     

胸腺瘤伴重症肌无力外科治疗的研究进展

胸腺瘤是一种起源于胸腺上皮细胞的肿瘤,发病率约为0.2/100 000。重症肌无力是其常见的伴随症状,此类患者可发生肌无力危象进而导致死亡。胸腺切除术已经成为胸腺瘤伴重症肌无力常见的治疗方法。该文针对胸腺瘤伴重症肌无力外科治疗的术前检查、临床诊断与分期、外科手术方式与入路选择和预后等进行综述。     

重症肌无力胸腺瘤的围术期处理

目的总结重症肌无力（myasthenia gravis,MG）胸腺瘤的围术期处理经验。方法回顾分析本科1990年7月～2008年6月连续手术治疗胸腺瘤合并MG65例患者的临床资料。结果危象预测积分〈12分者,无危象发生;12～17分者危象发生率50.0%;18～23分者危象发生率71.4%;≥24分者危象发生率75.0%。术前危象预测积分≥12分者,术后24h拔除气管插管和气管切开的患者分别为85.7%和61.4%,显著高于危象预测积分〈12分者的20.5%和6.8%。术后肌无力症状完全缓解23例、改善25例,有效率85.7%,改善不明显或加重8例。结论胸腺瘤切除是治疗重症肌无力胸腺瘤的一种较安全而有效的方法。围手术期治疗的重点是防治MG危象。     

重症肌无力伴胸腺瘤术后多次发生肌无力危象的护理

重症肌无力伴胸腺瘤在围术期常可由于手术、感染等因素而加重病情,呼吸肌无力维持正常的呼吸功能,出现肌无力危象。我科近期收治1例重症肌无力伴胸腺瘤患者,围手期多次发生肌无力危象,由于密切观察病情及切实加强护理确保了治疗和抢救成功。报告如下。1临床资料患者男性,40岁。2004年8月,感冒后次日出现眼睑下垂、咀嚼无力,并有抬头困难及四肢乏力。嗣后上述症状逐渐加重,2005年1月出现复视、呼吸费力。CT检查,发现前上纵隔内有4×4×3.5厘米软组织块影,肌电图检查:肌电位波幅明显递减。予溴吡斯的明治疗后,症状明显改善。诊断为“重症肌无…     

胸腺瘤切除术的围手术期处理的改进和预防重症肌？…

胸腺瘤的主要治疗手段是手术，但手术会引起肌无力危象的发生。改进围手术期处理，可以明显降低肌无力危象的发生。术前、术中、术后使用肾上腺糖皮质激素及吡啶期的可以有效防止胸腺瘤病人围手术期出现肌无力危象。     

全身型重症肌无力患者胸腺切除术后肌无力危象的影响因素分析

［摘要］　目的　分析全身型重症肌无力（MG）患者胸腺切除术后肌无力危象（POMC）的影响因素。方法　回顾性分析2018年1月至2022年6月于首都医科大学宣武医院胸外科接受胸腺切除术的116例全身型MG患者的临床资料，根据术后是否出现POMC分为POMC组38例和非POMC组78例。对其临床特点进行单因素和多因素分析，确定POMC的影响因素。结果　POMC组的机械通气时间、住ICU时间显著长于非POMC组（P<0.001）。POMC组和非POMC组在改良Osserman分型、合并胸腺瘤、手术方式、用力肺活量（FVC）、第一秒用力呼气容积（FEV₁）以及血清白蛋白与球蛋白比值（AGR）等方面比较差异均有统计学意义（P<0.05）。多因素logistic回归分析结果显示，Osserman分型、FVC、FEV₁以及AGR是POMC发生的影响因素（P<0.05）。结论　术前Osserman分型、FVC、FEV₁以及AGR是全身型MG患者发生POMC的影响因素，可为临床工作提供参考。     

胸腺瘤及其伴重症肌无力的外科治疗

本文报告38例单纯性胸腺瘤和21例伴发重症肌无力病人,指出胸部X线检查是诊断本病的重要方法,经皮穿刺有助于细胞学检查,确诊依据术后病理。主要根据术中所见肿瘤是否包膜完整、有无侵袭生长及镜下细胞间变与否来区分良、恶性。胸腺瘤有潜在恶性倾向,术后应辅以放疗或化疗。对伴发重症肌无力者,术前准备、麻醉选择、手术操作和术后处理更应审慎。对出现重症肌无力危象者,应及早行气管切开。     

Factors influencing improvement and remission rates after thymectomy for myasthenia gravis.

BACKGROUND: Thymectomy has become an accepted option in the treatment of myasthenia gravis (MG). However, the optimal selection of patients for surgery remains controversial. OBJECTIVE: The objective of the study is to define factors influencing improvement and remission rates after thymectomy for MG. METHODS: We retrospectively reviewed our experience in the surgical management of patients with MG over the last 20 years, and analyzed if patient's age, sex, preoperative Osserman Stage, thymic histology, duration of symptoms, or radiological findings influenced outcome. RESULTS: There were 25 women and 10 men. Most of them presented with Osserman stage IIA (n = 12) and stage IIB (n = 17). Hyperplasia of the thymus was observed in 15 patients (43%), thymoma in 12 (34%), and atrophic or normal thymus in 8 (23%). Computed tomography (CT) had a positive predictive value of 91% in detecting thymoma and of 80% in detecting thymic hyperplasia. Over a mean follow-up of 96 months, MG improved markedly in 26 patients. Postoperative improvement was significantly greater in patients with advanced MG (stage IIB, III and IV), whereas a higher rate of remission occurred in patients with mild MG (stage I and IIA), and in those with thymic hyperplasia. No relation was observed between outcome and age, sex, duration of symptoms and abnormal CT findings. CONCLUSIONS: The beneficial effects of thymectomy are characterized by postoperative clinical improvement in most patients with moderate to severe preoperative myasthenic involvement and by a higher remission rate in patients with thymic hyperplasia and/or mild myasthenic symptoms.     

Retrospective analyses of clinical features and therapeutic outcomes in thymectomized patients with myasthenia gravis at Shinshu University

OBJECTIVES: Thymectomy has become recognized as an integral element in the treatment of patients with myasthenia gravis (MG). Although the incidence of elderly-onset MG has recently been increasing, there is little data demonstrating the efficacy and complications of thymectomy in this population. To clarify this point, we divided the thymectomized patients with MG into young and elderly groups, and retrospectively examined their clinical features and therapeutic outcomes. PATIENTS AND METHODS: We reviewed 57 MG patients who had been admitted to our hospital between 1988 and 2002. The patients were classified into young (younger than 60) and elderly (60 or older) groups according to the age of onset, and the therapeutic outcomes of thymectomy were evaluated using myasthenic severity scales and the duration from operation to discharge. RESULTS: Myasthenic severity scales significantly improved after thymectomy in the elderly group both with (p<0.005) and without thymoma (p<0.05) compared with before. With regard to the duration from thymectomy to discharge, no significant difference could be found between the young and elderly groups, irrespective of associated thymoma. There were no serious complications during and after thymectomy in either the young or the elderly group. CONCLUSIONS: Despite various possible complications due to aging, thymectomy should be actively considered also in the treatment of elderly MG patients because it can reliably and safely improve myasthenic symptoms in combination with immunosuppressive agents, including corticosteroid.     

Clinical Utility of Videofluorography with Concomitant Tensilon Administration in the Diagnosis of Bulbar Myasthenia Gravis

Myasthenia gravis (MG) classically presents with ocular, bulbar, and predominantly proximal muscle weakness. Isolated bulbar symptoms occur in less than 25% of cases and can mimic stroke (1–3). If left untreated, MG can lead to significant morbidity and mortality, including myasthenic crisis and recurrent aspiration pneumonia. We describe a case of a 68-year-old man who presented with isolated bulbar symptoms. We used a novel approach to diagnosis which included a videofluorographic swallow study with concomitant Tensilon (edrophonium) injection.     

La myasthénie du côté de l’interniste

Myasthenia gravis is an autoimmune disease due to specific antibodies inducing a neuromuscular transmission defect causing muscle fatigability. If onset of the disease may be at any age, myasthenia gravis concerns mostly young adults, in majority females. The disease characteristic features are the following: ocular symptoms (ptosis or diplopia) as main initial manifestation, extension to other muscles in 80 % of the cases, variability of the deficit, effort induced worsening, successive periods of exacerbation during the disease course, severity depending on respiratory and swallowing impairment (if rapid worsening, a myasthenic crisis is to be suspected), association with thymoma in 20 % of patients and with other various autoimmune diseases, most commonly hyperthyroidism and Hashimoto's disease. Diagnosis relies on the clinical features, improvement with cholinesterase inhibitors, detection of specific autoantibodies (anti-AChR or anti-MuSK), and significant decrement evidenced by electrophysiological tests. The points concerning specifically the internist have been highlighted in this article: diagnostic traps, associated autoimmune diseases, including inflammatory myopathies that may mimic myasthenia gravis, adverse effects of medications commonly used in internal medicine, some of them inducing myasthenic syndromes. The treatment is well codified: the treatment is well codified: (1) respect of adverse drugs contra-indications, systematically use of cholinesterase inhibitors, (2) thymectomy if thymoma completed with radiotherapy if malignant, (3) corticosteroids or immunosuppressive agent in severe or disabling form, (4) intensive care unit monitoring, plasmapheresis or intravenous immunoglobulins for patients with myasthenic crisis.     

Clinical characteristics and prognosis of myasthenia gravis in older people

OBJECTIVES: To investigate the characteristics of myasthenia gravis (MG) in older people and to evaluate the benefits of immunosuppressive treatments at this age. BACKGROUND: Myasthenia gravis in older adults has not been extensively studied. In patients with disease onset after the age of 60, treatment mainly relies on medical therapy because thymectomy is generally not performed unless a thymoma is present. METHODS: Of 837 myasthenic patients followed since 1978, we identified 172 cases with onset after age 60. All patients were treated with anticholinesterases. In the decade from 1978 to 1988, immunosuppressive therapy was performed mainly with corticosteroids (prednisone); since 1989, azathioprine alone or, more often, associated with prednisone, has been increasingly used in MG patients. Long-term outcome was evaluated in 149 cases with follow-up longer than 1 year. Remission, pharmacological remission, and marked improvement with reduction in drug dosage were considered good results. RESULTS: Patients older than age 60 at onset of the disease were 20.5% of our series, male/female ratio was 1.9, age at onset ranged from 61 to 86 years, 87.2% patients had generalized disease, thymoma was detected in 37 patients (21.5%). Of 149 cases with sufficient follow-up data, 9 were in remission, 111 achieved good results, 3 died of MG, and 120 required immunosuppressive therapy at some time. Sixty-seven patients had been treated with prednisone for 0.5-16 years (mean, 5 years); good results were recorded in 51 patients (76.1%) and severe side effects in 12 (17.9%). Forty-six patients had received combined therapy with prednisone and azathioprine for 1 to 12 years (mean, 3.9 years); good results were recorded in 41 patients (89.1%) and severe side effects in six (19.5%). Seven patients had been treated with azathioprine alone for 1 to 4 years (mean, 2.3 years) with good results in five and with no side effects. CONCLUSIONS: The prognosis of MG in older people seems to be favorable, although full remission is rare and MG weakness, treatment side effects, and associated thymoma can contribute to mortality rate. In our experience, the combined therapy with prednisone and azathioprine was more effective than prednisone alone, and steroid-related side effects were more frequent than those related to azathioprine.     

Postoperative respiratory care after transsternal thymectomy in myasthenia gravis. A 3-year experience in 53 patients

During a three-year period, 53 patients with myasthenia gravis underwent transsternal thymectomy by a partial sternum-splitting technique at our institution. This procedure was carried out (1) because an x-ray film or a computed tomography scan had suggested the presence of a thymoma or (2) to manage symptoms of myasthenia gravis. In 41 patients the endotracheal tube was removed in the post-anesthesia recovery room. The remaining 12 patients were extubated in the Respiratory Care Unit--in five, after prolonged mechanical ventilation. From our experience, the only factor useful for predicting the probable need for prolonged postoperative mechanical ventilation is the degree of bulbar involvement. Patients in Osserman classification groups 3 and 4 have an extremely high incidence of postoperative respiratory failure. Consideration should be given to the use of preoperative plasmapheresis in myasthenia gravis patients who have significant bulbar symptoms.     

Plasma Exchange for Treatment of Myasthenia Gravis: Pathophysiologic Basis and Clinical Experience

Abstract: Myasthenia gravis is an autoimmune disease characterized by production of antibodies to acetylcholine receptors located at the motor end plate in skeletal muscles. The antibodies bind and subsequently induce degeneration of these receptors. Loss of acetylcholine receptors results in inadequate contraction of muscle fibers in response to acetylcholine released from nerve terminals and clinically apparent muscle weakness. Plasma exchange removes the circulating antibodies in myasthenic patients with short‐term clinical improvement. Plasma exchange may be indicated in patients with acute exacerbation of neuromuscular weakness with bulbar or respiratory compromise, preoperative optimization prior to thymectomy, and postoperative deterioration following thymectomy or other surgical procedures. Long‐term, intermittent plasma exchange for patients who do not adequately respond to standard treatment is another evolving indication.     

Resolution of autoimmune oophoritis after thymectomy in a myasthenia gravis patient

Myasthenia gravis (MG) is an autoimmune disorder characterized by autoantibodies against acetylcholine receptors. MG is generally an isolated disorder but may occur concomitantly with other autoimmune diseases. We describe an eighteen-year-old girl with MG who was admitted to our clinic with secondary amenorrhea and diagnosed as autoimmune oophoritis. Since her myasthenic symptoms did not resolve with anticholinesterase therapy, thymectomy was performed. After thymectomy, her menses have been regular without any hormonal replacement therapy. To our knowledge, this is the first report on a patient with autoimmune ovarian insufficiency and MG in whom premature ovarian insufficiency resolved after thymectomy, without hormonal therapy.     

Microscopic-sized "microthymoma" in patients with myasthenia gravis

BACKGROUND: In 2005, Cheuk et al reported two patients with microscopic-sized thymomas and proposed the term microthymoma to distinguish it from the nodular hyperplasia of thymic epithelium, so-called microscopic thymoma. Here, we present microthymomas that were found in 196 patients with myasthenia gravis (MG) who had undergone thymectomy. MATERIALS AND METHODS: Thymic tissues in 196 patients with MG who underwent thymectomy or thymothymomectomy were examined. Of these patients, 73 patients had thymoma indicated by CT before surgery, and the other 123 patients had no mediastinal tumors. From the resected thymic tissues, an average of 14 hematoxylin-eosin-stained sections (range, 4 to 55 sections) were prepared for microscopic examination. The histologic type of the thymoma was classified according to the World Health Organization (WHO) classification. RESULTS: From the 196 patients, we found three microthymomas in 3 patients (1.5%). While these three tumors could not be seen grossly in pathology section, they were found microscopically (range, 2 to 4 mm). The histologic subtype according to the WHO classification system was B1 in one patient and B2 in two patients. CONCLUSION: Microthymoma was found in 3 of 196 patients (1.5%) with MG. Microthymoma might exist in thymus of patients with MG, even in patients who have no thymoma indicated by CT.