• Journal of Chest Surgery
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• 제27권4호
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• pp.303-309
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• 1994

Eighteen patients with thymoma have undergone surgical treatment at Pusan National university Hospital from 1983 to 1993. The mean age of the patients was 47.3$\pm$13.3 years old and male to female ratio was 1.6 : 1. Eleven of the patients[61.1%] with thymoma had myasthenia gravis; 12 cases[66.7%] were encapsulated and 6[33.3%] invasive. Operation was complete resection in 14[77.8%] of the patients, partial resection with residual tumor in 3[16.7%], and simple biopsy in one[5.5%]. Adjuvant radiotherapy was administered in 5 of 6 invasive thymoma. Postoperatively one patient died for excessive bleeding and sepsis. A tumor relapse was observed in one patient who had invasive thymoma and underwent partial resection followed by postoperative radiotherapy. The overall 5-year survival rate was 88.2$\pm$ 7. 9%.The presence of myasthenia gravis is not considered as an adverse factor for survival but it contributes to early discovery of associated thymoma.

• Journal of Chest Surgery
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• 제28권1호
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• pp.78-80
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• 1995

We report the thoracoscopic resection of thymus and pericardial fat tissue in a patient who was in the setting of late severe myasthenia gravis[Osserman`s Group II-C-2 and previous tracheostomy state. The patient was 33-year-old female. She had been supported with mechanical ventilator for 88 days and suffered from recurrent pneumonia. Our first aim was to weaning her from mechanical ventilator. Traditional methods such as median sternotomy or transcervial thymectomy or transsternal sternotomy were difficult because of the anticipating complications of mediastinitis or morbidity, especially chest pain, following thoracotomy. We could wean her from the mechanical ventilator at postoperative 9 days. So, we concluded that video-assisted thoracoscopic thymectomy is a useful alternative tool in this case.

• Journal of Chest Surgery
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• 제21권3호
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• pp.454-461
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• 1988

Myasthenia gravis is a functional neuromuscular disorder with characteristic voluntary muscle weakness. The role of thymus in pathogenesis of this disorder has become apparent that thymectomy in treatment has gained acceptance. Between January 1976 and June 1987, twenty patients underwent thymectomy for myasthenia gravis at the Department of Thoracic and Cardiovascular Surgery, College of Medicine, Korea University. A clinical study that is focused on the factors affecting the operative results was analyzed and the following results were obtained. Among the 20 patients, male to female ratio was 8:12 and the age of onset was ranged from 3 years to 67 years. The chief complaints in order of frequency were as follows; ocular symptoms such as ptosis and diplopia[7 cases], general weakness[4 cases], swallowing difficulty[3 cases], dyspnea[3 cases], dysphasia[1 case], headache[1 case] and dizziness[1 case]. The severity of disease was classified by modified Osserman`s method that Group IIa was 8 cases, Group IIb; 7 cases, Group IIc; 3 cases and Group I; 2 cases. In histopathology of thymus, the most frequent finding was hyperplasia[11 cases] followed by thymoma[4 cases], normal tissue[3 cases] and malignant` thymoma[2 cases]. There were two cases of postoperative complications; one case was wound infection and the other was mediastinitis. One case of malignant thymoma died due to respiratory failure with pulmonary metastasis. There was 16 cases[80%] of improvement after thymectomy as follows; complete remission was 4 cases[20%], marked improvement was 9 cases[45%] and subjective improvement only was 3 cases[15%]. The effect of severity and duration of disease on operative result has statistically significant. The effect of thymus histopathology on operative result was not statistically significant. But there were comparable results between thymoma cases and non-thymoma cases.

• 한국임상수의학회지
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• 제22권4호
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• pp.392-395
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• 2005

체중 32kg의 3년령 암컷 독일셰퍼드가 식이역류와 구토로 내원하였다. 신체검사에서 체중감소, 음성변화, 유연 및 후구유약이 관찰되었다. 방사선검사에서 거대식도가 관찰되었다. Neostigmine challenge test는 음성이었으며 acetyle\choline receptor에 대한 항체가는 1.58nmol/L로 양성이었다. 따라서 후천성 중증 근육무력증의 만성형으로 진단하였다. Pyridostigmine bromide 1mg/kg을 1일 2회 투여하여 치료하여 후구의 유약 증상은 현저히 개선되었고, 정상보행을 보였다. 치료과정중 이물성 폐렴이 발생하였고, 항생제 치료를 실시하였으나 관리의 어려움과 경제적 문제로 인한 보호자의 요구로 안락사 시켰다.

• Annals of Clinical Neurophysiology
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• 제7권2호
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• pp.93-96
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• 2005

Background: Myasthenia gravis (MG) and systemic lupus erythematosus (SLE) are well recognized to coexist and have some similarities in immunologic, clinical and serologic findings. Despite several reports of the association with autoantibodies and thymectomy in these disorders, the pathomechanism of coexistence remains to be elucidated. Objective: We aimed to investigate the relationship of MG and SLE through overall features of patients with both disorders;: clinical, laboratory, and electrophysiological findings. Materials and Methods: We reviewed the medical records of 6 consecutive patients with MG and SLE (2 men, 4 women, ages 17-51, mean 30.5 years, Seoul National University Hospital, from 1998 to 2005). Results: Three patients who developed SLE first, had ocular type of MG and 2 were children showing much severe and recurrent SLE features and only 1 patient had thymic hyperplasia. The other 3 developed MG first and they were generalized type and none underwent thymectomy. In addition, the development of MG or SLE was not coincident with remission or improvement of another disorder. Conclusion: The coexistence of SLE and MG may support the hypothesis of two different antibody populations modulated by thymus in the opposite extremesThis report suggests that the systemic and extensive autoimmune response in preceding MG or SLE may effect the development of the other disorder followed, while. the coexistence of two disorders cannot be explained by the hypothesis of two different antibody populations modulated by thymus in the opposite extremes The role of thymectomy and the theorectical subsequent effect on the development of SLE have been debated with controversy. However, SLE occurred without thymectomy in MG and these disorders did not develop in the quiescent period of another disorder. Therefore, the other pathomechanism for the coexistence of MG and SLE should be elucidated.

• Journal of Yeungnam Medical Science
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• 제13권1호
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• pp.86-96
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• 1996

1985년 8월부터 1996년 1월까지 중증 근무력증으로 진단받은 환자 52례에 대한 임상적 고찰을 통하여 다음과 같은 결론을 얻었다. 1. 전체 환자 중 남자가 19례(35.8%), 여자가 33례(64.2%)이었고 11세에서 20세 사이가 12례 (23%)로 가장 높은 빈도를 보였다. 2. 환자의 초기 증상으로는 안검하수, 복시 등의 안구증상이 37례(71.2%)로 가장 많았고 modified Osserman의 분류에 따라서도 가장 증상이 가볍고 약물치료에 잘 반응하는 I군이 36례(69.3%)로 가장 많았다. 3. 환자가 호소하는 중상의 비특이성과 회복과 재발을 거듭하는 질병의 특성으로 인해 발병 이후 진단될 때까지의 시간은 비교적 오래 걸렸고 1년이상 경과하며 진단된 경우도 16례(30.8%)나 되었다. 4. 자가면역질환이 7.6%에서 동반되었는데 갑상선기능 항진증이 2례(3.8%), 인슐린의존형 당뇨 2례(3.8%)였고 종격동 전산화 단층촬영소견에서 흉선의 비대가 의심되었던 환자는 6례(11.5%), 흉선종이 발견되었던 경우는 3례였다. 5. 환자의 치료는 대부분 항콜린에스테라제나 부선피질호르몬을 병용하는 내과적 치료를 하였으며 약물의 치료에 반응이 미비하거나 악화되는 경우 혹은 종격동 전산화 단층촬영 상에서 흉선의 이상 소견이 보인 경우에 2례(3.9%)에서는 흉선절제술이, 1례(1.9%)에서는 혈장여과요법이 추가되었고 흉선절제술과 혈장여과요법이 같이 병합 치료된 경우도 3례(5.9%) 있었다. 6. 전체 환자중 2명에서 자연치유의 경과를 보였으며 이들은 모두 I군이였다. 그리고 흉선절제술과 부신피질 호르몬 투여로 완치된 예가 1례였었다.

• Journal of Chest Surgery
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• 제43권5호
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• pp.557-561
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• 2010

중증 근무력증의 치료법으로서 흉선절제술은 효과가 입증된 표준 치료법 중 하나로 받아들여지고 있다. 흉선절제 수술법은 종래에 시행되던 흉골절개나 혹은 다른 개흉 수술법에 반해 최근 최소 침습 수술법이 시도되고 있다. 증례는 28세 남자로, 양손의 위약감 및 피로감을 호소하여 검사 결과 흉선 과형성을 동반한 중증 근무력증으로 진단하였고, da Vinci 로봇을 이용하여 흉선절제술을 시행하였다. 수술은 좌우 흉강을 통해 양측으로 접근하여, 우측에서 흉선의 2/3를 절제하고 좌측에서 나머지를 절제하였으며, 소요시간은 각각 1시간, 30분이었다. 환자는 술후 8일째 합병증 없이 퇴원하였다. 최소 침습 흉선절제술로서 da Vinci 로봇을 이용한 수술법이 국외에서 시도되고 있지만 국내에는 아직 보고된 바가 없어, 본원에서 수행한 성공적인 경험을 보고하는 바이다.

• Journal of Microbiology and Biotechnology
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• 제10권5호
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• pp.707-713
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• 2000

Myasthenia gravis (MG) is caused mainly by autoantibodies directed against acetylcholine receptors located in the postsynaptic muscle cell membrane. Using in vitro selection techniques, we isolated an RNA containing 2'-fluoro pyrimidines that can specifically and avidly ($K_d$ ~25 nM) bind rat monoclonal antibody called mAb198, which recognizes the main immunogenic region on the acetylcholine receptors. This RNA can act as a very effective decoy and block mAb198 binding to the receptors in vitro. Furthermore, this RNA decoy can prevent the antigenic modulation of the acetylcholine receptor caused by mAb198 in human muscle cell cultures with and $IC_{50} $of approximately $2.4{\mu}M$. These results indicate that the RNA selected in this study is a more potent decly inhibitor of myashthenic antibodies than the previously identified RNA with 2'-amino pyrimidines [11]. Moreover, this RNA cross-reacts with autoantibodies from patients with MG and can protect human cells from the effects of these antibodies. These observations have important implications for developing an antigen-specific treatment of autoimmune diseases including MG, which is based on decoy RNAs selected in vitro.

• Journal of Chest Surgery
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• 제23권4호
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• pp.715-719
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• 1990

Thymoma is defined as a neoplasm of the epithelialreticular framework cells of the thymus. The treatment of choice and the prognosis for patients with thymoma are still controversial subjects due to lack of a uniform histological classification and standardized criteria for typing thymic tumors. Between June 1985 and May 1989, eight patients underwent thymomectomy at the Department of Thoracic and Cardiovascular Surgery, Korea University Medical Center. A clinical analysis was performed and the following result was obtained. In histologic cell type, epithelial type was 2 cases, lymphocytic type 3 cases, and mixed type was 3 cases. The clinical stage was classified by Masaoka’s classification that Stage I was 4 cases, Stage II; 2 cases, Stage III; 2 cases and Stage IV was none. Myasthenia Gravis was associated with thymoma in 5 patients. In 8 patients, thymomectomy were performed. Adjuvant radiotherapy was performed in one patient with Stage I due to suspicious tumor remnant and in Stage II, III patients routinely. Combined chemotherapy was performed in one patient with Stage III due to local recurrence. We concluded that the most important prognostic factor of thymoma is extent of tumor invasion. And the presence of Myasthenia Gravis is no more prognostic factor. Surgical resection of thymoma is treatment of choice, If local invasion or distant dissemination is present, postoperative radiotherapy and /or combined chemotherapy is recommended.

• Journal of Genetic Medicine
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• 제16권1호
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• pp.31-38
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• 2019

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a maternally inherited mitochondrial disorder of which m.3243A>G is the most commonly associated mutation, resulting in an inability to meet the energy requirements of various organs. MELAS poses a diagnostic challenge owing to its multiple organ involvement and great clinical variability due to its heteroplasmic nature. We report three cases from a family who were initially misdiagnosed with myasthenia gravis or undiagnosed. Although there is no optimal consensus treatment approach for patients with MELAS because of the disease's heterogeneity, our 21-year-long therapy regimen of ${\text\tiny{L}}-arginine$, ${\text\tiny{L}}-carnitine$, and coenzyme Q10 supplementation combined with dietary management appeared to provide noticeable protection from the symptoms and complications. Prompt early diagnosis is important, as optimal multidisciplinary management and early intervention may improve outcomes.