• Title/Summary/Keyword: Myasthenia gravis

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Surgical Treatment and Assessment of Prognostic Factors of Thymoma (흉선종의 수술적 치료 및 예후인자의 평가)

  • 정경영;김길동
    • Journal of Chest Surgery
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    • v.29 no.7
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    • pp.734-740
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    • 1996
  • The relative importance of various factors influencing the prognosis and survival in the treatment of thymoma is still controversial. Sixty ave patients operated on for thymoma from Jan. 1981 to Dec. 1994 were evaluated, 28 patients (43.1 %) with myasthenia gravis and 37 patients (56.9%) without. Masaoka staging revealed stage I disease in 28 patie ts(4).1%) , stage ll in 1) patients(20.0%), stage 111 In 22 patients(33.8%), stage IVa in 1 patients(1.5%), and stage IVb in 1 patient(1.5%). There was no operative mortality. A complete resection was performed in 48 patients (73.8%) patients, associated in 10 patients (15.4%) with postoperative adjuvant treatment(radiotherapy 5; chemotherapy 1: radio- and chemotherapy 4). Thymomas were found to be predominantly of the epithelial type in 16 patients(24.6%), predominantly Iymphocytic type in 18 patients(27.7%), and mlxed in 22 patients (33.9%). The overall 5- and 10-year survival rates were 87% and 82%, respectively, Factors indicating a poor prognosis included local invasion, incomplete excision, thymic carcinoma, advanced staging and myasthenia gravis. The de- gree of tumor invasion turned out to be the main prognostic factor, and treatment should be planned ac- cordingly. The prognosis is best predicted by the stage of the tumor as determined intraoperatively and is poorer in patie ts with incomplete resection than in those with complete resection of the thynoma. No recurrence developed In patients with stage I disease.

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Meta-Analysis of Limited Thymectomy versus Total Thymectomy for Masaoka Stage I and II Thymoma

  • Pulle, Mohan Venkatesh;Asaf, Belal Bin;Puri, Harsh Vardhan;Bishnoi, Sukhram;Kumar, Arvind
    • Journal of Chest Surgery
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    • v.54 no.2
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    • pp.127-136
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    • 2021
  • Background: This meta-analysis aimed to evaluate the incidence of tumor recurrence, postoperative myasthenia gravis, postoperative complications, and overall survival after limited versus total thymectomy for Masaoka stage I and II thymoma. Methods: A systematic search of the literature was conducted using the PubMed, Embase, MEDLINE, and Cochrane databases to identify relevant studies that compared limited and total thymectomy in Masaoka stage I-II patients. The quality of the included observational studies was assessed using the Newcastle-Ottawa Scale. The results of the meta-analysis were expressed as log-transformed odds ratios (log ORs), with 95% confidence intervals (CIs). Results: Seven observational studies with a total of 2,310 patients were included in the meta-analysis. There was an overall non-significant difference in favor of total thymectomy in terms of tumor recurrence (pooled log OR, 0.40; 95% CI, -0.07 to 0.87; p=0.10; I2=0%) and postoperative myasthenia gravis (pooled log OR, 0.12; 95% CI, -1.08 to 1.32; p=0.85; I2=22.6%). However, an overall non-significant difference was found in favor of limited thymectomy with respect to postoperative complications (pooled log OR, -0.21; 95% CI, -1.08 to 0.66; p=0.64; I2=36.1%) and overall survival (pooled log OR, -0.01; 95% CI, -0.68 to 0.66; p=0.98; I2=47.8%). Conclusion: Based on the results of this systematic review and meta-analysis, limited thymectomy as a treatment for stage I and II thymoma shows similar oncologic outcomes to total thymectomy.

A Clinical Analysis of Surgically Treated Myasthenia Gravid (중증 근무력증환자에서 흉선절제술의 효과 및 임상적 고찰)

  • 민경석;조유원
    • Journal of Chest Surgery
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    • v.29 no.1
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    • pp.73-78
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    • 1996
  • From June 1989 to May 1994, 23 patients with myasthenia gravis underwent thymectomy in the Department of Thoracic Surgery, Asan Medical Center. For the evaluation of the effect of thymectomy, clinical analysis including age and sex distribution, preoperative disease severity, duration of the symptoms, thymic pathology, and postoperative clinical improvement was performed. The mean follow-up period was 15 mon hs(range : 5∼60mon1hs). The effectiveness of thymectomy according to the postoperative symptoms and drug dose was graded as follow : a) complete remission in 5 cases (21.7%), b) clinical improvement with dose reduction in II cases (47.9%), c) clinical improvement without dose reduction in 3 cases(13.0%), 4) unchanged in 2 cases (8.8%), e) early death in 1 case(4.3%) and f) recurrence in 1 case(4.3%). According to the preoperative disease stage by Modified Osserman classification, 6(66.7%) of the 9 patients in stage I and 13 (92.9%) of 14 patients in stage II were clinically improved after thymectomy. although there was no significant statistical differences between these two groups (p> 0.05). According to the thymic pathology, 4 (100%) of 4 cases with normal thymus were clinically improved. Ten (90.9%) of 11 cases with thymic hyperplasia and 5 (62.5%) of 8 cases with thymoma were clinically improved, but there was no significant statistical differences in these three groups (p> 0.05 . The thymectomy resulted in dramatic clinical improvement in 19 (82.5%) out of 23 patients. According to the preoperative disease stage by Modified Osserman classification or the thymic pathology, there was no significant statistical differeneces in the effect of thyinectomy (p> 0.05).

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Total Body Irradiation for MyastHenia Gravis with Thvmoma - Case Report - (흉선종을 동반한 중증근무력증 환자에서 전신 방사선치료)

  • Kang, Ki-Mun;Choi, Ihl-Bohng;Kim, In-Ah
    • Radiation Oncology Journal
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    • v.17 no.2
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    • pp.146-150
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    • 1999
  • Myasthenia gravis (MG) is relatively rare ouuring as one of important autoimmune disease to affect neuromuscular junction. This study was clinically to evaluate total body irradiation (TBI) against two patients including 33-year and 39-year females for chronic MG with thymoma who hospitalized in the St. Mary's Hospital, Catholic University since 1994 as well as who showed no response by thymectomy, immunotherapy and hormonal therapy. TBI designed by the dose of 150$\~$180 cGy consisting of 10 cGy per fraction, three times a week, for 5~e weeks using linear accelerator of 6 MV. During the treatment of TBI, they did complain acute side effect such as vomiting and also appear improved physical condition from 4$\~$6 weeks after TBI. Through the follow-up period of 18 or 42 months after TBI, they did not have any symptomatic recurrence. Consequently, the results suggest that TBI can be used as an alternative tool for the patients concurrently for MG with thymoma who had been refractory to various conventional therapies like thymectomy, immunotherapy and hormonal therapy.

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Surgical Treatment of Thymoma (흉선종의 수술치료)

  • 홍치욱;박주철
    • Journal of Chest Surgery
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    • v.30 no.1
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    • pp.61-66
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    • 1997
  • Between January 1986 and December 1995, 24 patients were treated surgically for thymoma. There were 17 males and 7 females, and their ages ranged from 23 to 69 years old and mean age was 49 years. Thymomas ere associated with fourteen myasthenia gravid, and classified histologically as Iymphocytic in 12 patients, mixed in 8, epithelial in 4, and classified clinically as stage I in 11, stage II in 4, stage III in 8 and stage VI in 1 patient. Eleven patients with non-invasive thymoma had received surgical resection, and 10 out of 13 patients with invasive thymoma were able to undergo complete resection. A partial resection or tissue biopsy followed by radiation or chemotherapy was done with the remaining three patients. Three died, four had improvement of symptom, two had relapse and fifteen had no symptom during follow up ranged from 25 days to 60 months. In fourteen cases of thymoma with myasthenia gravid, one died due to myasthenic crisis, two showed symptom aggravation, six had less medical treatment and five patients had medical treatment as same as dosage received preoperatively.

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Surgical Treatment of Thymoma (흉선종의 외과적 치료)

  • Jo, Gyu-Cheol;Jo, Gyu-Seok;Park, Ju-Cheol
    • Journal of Chest Surgery
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    • v.28 no.3
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    • pp.303-307
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    • 1995
  • We experenced 18 patients with surgically treated thymoma from January 1986 to December 1993. There were 13 male and 5 female ranged from 23 to 69 years of age. Among them Myasthenia gravis was present in 8 patients (44%) The predominant cell type was lymphocytic(11 patients), followed by epithelial (3) and mixed (4), and had no value in predicting prognosis. Treatment consisted of complete resection in 15 patients, partial resection in 2 patients and 1 patient was performed biopsy. Only, and then adjuvant radiation therapy was done in 7 patients and 3 patients needed adjuvant chemotherapy. Invasion of the adjacent tissue in thymoma was the most improtant prognostic value. There were 6 non-invasive tumors and 12 invasive tumors. Two patients with invasive thymomas resulted in death and one of 6 patients with non-invasive thymomas died during follow up ranged from 25 day to 60 months. The causes of death were myasthenic crisis in 1 patient, C. N. S. problem in 1 patient and pulmonary & mediastinal metastasis in 1 patient.

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Pure Red Cell Aplasia Associated with Good Syndrome

  • Okui, Masayuki;Yamamichi, Takashi;Asakawa, Ayaka;Harada, Masahiko;Horio, Hirotoshi
    • Journal of Chest Surgery
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    • v.50 no.2
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    • pp.119-122
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    • 2017
  • Pure red cell aplasia (PRCA) and hypogammaglobulinemia are paraneoplastic syndromes that are rarer than myasthenia gravis in patients with thymoma. Good syndrome coexisting with PRCA is an extremely rare pathology. We report the case of a 50-year-old man with thymoma and PRCA associated with Good syndrome who achieved complete PRCA remission after thymectomy and postoperative immunosuppressive therapy, and provide a review of the pertinent literature.

Channelopathies

  • Kim, June-Bum
    • Clinical and Experimental Pediatrics
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    • v.57 no.1
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    • pp.1-18
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    • 2014
  • Channelopathies are a heterogeneous group of disorders resulting from the dysfunction of ion channels located in the membranes of all cells and many cellular organelles. These include diseases of the nervous system (e.g., generalized epilepsy with febrile seizures plus, familial hemiplegic migraine, episodic ataxia, and hyperkalemic and hypokalemic periodic paralysis), the cardiovascular system (e.g., long QT syndrome, short QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia), the respiratory system (e.g., cystic fibrosis), the endocrine system (e.g., neonatal diabetes mellitus, familial hyperinsulinemic hypoglycemia, thyrotoxic hypokalemic periodic paralysis, and familial hyperaldosteronism), the urinary system (e.g., Bartter syndrome, nephrogenic diabetes insipidus, autosomal-dominant polycystic kidney disease, and hypomagnesemia with secondary hypocalcemia), and the immune system (e.g., myasthenia gravis, neuromyelitis optica, Isaac syndrome, and anti-NMDA [N-methyl-D-aspartate] receptor encephalitis). The field of channelopathies is expanding rapidly, as is the utility of molecular-genetic and electrophysiological studies. This review provides a brief overview and update of channelopathies, with a focus on recent advances in the pathophysiological mechanisms that may help clinicians better understand, diagnose, and develop treatments for these diseases.

Intravenous Immunoglobulin Therapy in Peripheral Neuropathy (말초신경병증에 대한 정맥내 면역글로불린 요법)

  • Kim, Nam Hee;Park, Kyung Seok
    • Annals of Clinical Neurophysiology
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    • v.8 no.1
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    • pp.6-15
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    • 2006
  • Intravenous immunoglobulin (IVIg) is the treatment of choice for many autoimmune neuropathic disorders such as Guillain-Barre syndrome (GBS), chronic inflammatory Demyelinating neuropathy (CIDP), and multifocal motor neuropathy (MMN). IVIg is preferred because the adverse reactions are milder and fewer than the other immune-modulating methods such as steroid, other immunosuppressant such as azathioprine, and plasmapheresis. IVIg also has been used in other autoimmune neuromuscular disorders (inflammatory myopathy, myasthenia gravis, and Lambert-Eaton myasthenic syndrome) and has been known as safe and efficient agent in these disorders. Since IVIg would get more indications and be used more commonly, clinicians need to know the detailed mechanism of action, side effects, and practical points of IVIg.

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Adverse Events Associated with Intravenous Immunoglobulin Therapy in Neuromuscular Disorders (신경근질환의 정맥 내 면역글로불린 치료와 연관된 유해사례)

  • Na, Sang-Jun;Choi, Young-Chul
    • Annals of Clinical Neurophysiology
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    • v.8 no.1
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    • pp.48-52
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    • 2006
  • Background: Intravenous immunoglobulin (IVIg) has been administered for various immune-mediated neurological diseases such as autoimmune neuropathy, inflammatory myopathies, and other autoimmune neuromuscular disorders. The purpose of this study is to investigate side effects and complications of IVIg therapy in neuromuscular disorders. Methods: We enrolled 29 patients (age 8~63 years) with IVIg therapy for various neurological diseases including Guillain-Barre syndrome, myasthenia gravis, dermatomyositis, polymyositis, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. IVIg therapy was used at a dose of 0.4 g/kg body weight/day for 5 consecutive days. Results: 10 patients (34%) had adverse events. There are adverse events in 16 courses (11%) among total 145 courses. The majority of patients presented with mild side effects, mostly asymptomatic laboratory changes. Rash or mild headache occurred in 3 patients. One patient showed a serious side effect of deep vein thrombosis. Conclusions: IVIg therapy is safe for a variety of immune-mediated neurological diseases in our study.

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