• Journal of Chest Surgery
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• 제20권1호
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• pp.112-127
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• 1987

Myasthenia gravis is a neuromuscular transmission function disorder characterized by fatigue and weakness of voluntary muscles. This muscular weakness is intensified by activity and stress, and improved by the use of anticholinesterase compounds. It was initially described by Erb in 1879 and later named myasthenia gravis by Jolly in 1895. Although the pathogenesis is Known to be an autoimmune related reduction in the number of available acetylcholine receptors at neuromuscular junctions, the role of thymus in myasthenia gravis is still unclear and under investigation. Thymectomy in the management of myasthenia gravis has become increasingly important since Dr. Blalock observed in 1939 that some patients with thymic tumors and myasthenia gravis improved following thymectomy. A clinical study of 102 cases of myasthenia gravis was performed at Yonsei University College of Medicine. Seoul, Korea from Jan. 1976 to Jun. 1986. In order to determine which factors are of prognostic significance, attention is focused upon pre-operative patient evaluation, problems in operative and post-operative care, and long-term follow-up observations. The results were as follows: 1. The sex distribution was 67 females and 35 males, the mean age of onset was 28.95*1.69 years, and the maximal incidence occurred between 21 and 40 years of age [56 cases: 54.9%]. 2. Clinical manifestations of ocular symptoms were seen to 70 patients [68.6%] extremities weakness in 33 [32.3%], bulbar weakness in 29 [28.4%], and dyspnea in 13 [12.7%]. 3. Study cases more than two thirds were classified as mild types [MG 1 and MG 11A] and 6 cases as grave [MG 1V] based on the modified Osserman`s classification system, 4. Thymectomy was performed in 19 cases which presented in severe myasthenia symptoms and showed no improvement with cholinergic drugs. Histologic examination of the excised thymus glands revealed no abnormalities in 4 cases, thymic hyperplasia in 5, benign thymoma in 5, and malignant thymoma in 5. 5. Immediate post-operative complications included 2 cases of pneumothorax which were treated by tube thoracostomies, there was no operative mortality. 6. The response to cholinergic drugs in 36 cases younger than 20 years old and in 27 cases older than 40 years was relatively poor, while that in 35 cases between the ages of 21 and 40 years old was good. 7. Thirty of 39 cases in groups IIB, III & IV improved markedly with medical or surgical management while only 16 of 59 cases in the mild groups [I and IIA] improved, almost all surgical cases improved in all categories. 8. There were 5 deaths. occurring between 7 months and 3 years 3 months of treatment of myasthenia gravis. The causes of death were myasthenic crisis in 2 cases, respiratory failure due to candidiasis & radiation pneumonitis in one case, cerebral hemorrhage due to high blood pressure in two case.

• 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.

• 대한기관식도과학회지
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• 제13권2호
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• pp.34-39
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• 2007

Background: The mediastinum is the anatomic space which is restricted and the clinical aspect varies according to location, size and type of neoplasm. Diagnostic and therapeutic approaches to mediastinal neoplasms have changed over and over. We presented our recent therapeutic experiences with these neoplasms. The object and method: The 36 patients were treated in operation from 2000 until 2006. The male patients were 20 and the female patients were 16. Age of the patients ranged from 5 to 70, and the median age was $46.4{\pm}21.9$. The child patients were two. Result: The most prevalent anatomic distribution of the neoplasm was anterosuperior mediastinum. The most common neoplasm was thymoma, followed by thymic cyst, teratoma, ganglioneuroma. The complete excison of neoplasm was accomplished in 96% of patient group except thymoma patient group. One patient underwent total thymectomy, and the other 12 patients underwent extended thymectomy from 13 thymoma patient group. In the malignant neoplasm, 7 patients were received additional treatment after operation. There was short-term death of 1 person and late death of 1 person. Conclusion: Our results except clinical manifestation are compared favorably with other reports. Surgery is the management of choice for patients with mediastinal neoplasm and early curative resection is considered to optimize clinical outcome for patients.

• Journal of Chest Surgery
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• 제39권10호
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• pp.795-798
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• 2006

쿠싱증후군과 연관된 흉선 carcinoid종양은 드문 질병으로 그 예후가 나쁜 것으로 알려져 있다. 그중 부신피질자극 호르몬 분비 촉진 호르몬을 생산하는 흉선 carcinoid 종양은 훨씬 더 드물다. 우리는 거대한 전방 종격동 종양을 갖고 있는 58세 여자 환자에 대해 보고하려고 한다. 환자는 흉선제거술 5개월 후 전반적인 부종과 호흡곤란으로 다시 입원하였다. 환자는 재발과 전이가 발견되었고 쿠싱증후군으로 진단 받았다.

• Journal of Chest Surgery
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• 제18권4호
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• pp.872-880
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• 1985

Myasthenia gravis is a neuromuscular transmission disorder characterized by fatigue and weakness of voluntary muscles. Although the pathogenesis is known as reduction of available acetylcholine receptors at neuromuscular junctions by autoimmune attack, the thymic role in myasthenia gravis is still unclear and under investigation. But thymectomy in the management of myasthenia gravis has become increasingly important since the first successful operation with remission of symptoms in 1939 by Blalock. From January 1983 to June 1985, authors performed 17 thymectomies for patients with myasthenia gravis. Among them, 12 patients were free from thymoma [Croup A] and 5 were coupled with thymoma [Group B]. The results were as follows: 1] Sex distribution was 11 females and 6 males. Mean age of the patients was 32.2 year old. Sex and age distribution by the Group A and B are shown Table 1. 2] Clinical manifestations of ocular symptoms were seen in 5 patients [88.2%], extremity weakness in 13 patients, bulbar weakness in 12 patients and dyspnea in 6 patients. According to the Osserman`s classification, 5 patients were in group IIA, 6 in IIB and 6 in IIC. 3] Pre-operatively, all patients were positive response to the anti-cholinesterase test and 12 patients [92.3%] revealed positive findings in electromyography [EMC] which was done in 13 patients. 4] The postoperative complications were respiratory distress in 3 patients, myasthenic crisis in 2 patients and wound disruption in one patients. 5] Pathologic examination of the thymus showed hyperplasia in 10 patients [90%] and thymoma in 5 patients, of which 4 were mixed type with invasion to the adjacent tissues and one lymphocytic type without invasion. Normal thymus was noticed in only 2 patients. 6] In postoperative evaluations, among the 12 patients c free from thymoma [Group A], complete remission of symptoms was noticed in 3 patients and improvement in 7 patients. But among the 5 patients coupled with thymoma [Group B], only one patients showed improvement [Table 8]. Therefore, remission and clinical improvement were noticed in 11 patients [64.7%] of the all and complete remission was noticed in 3 patients [17.6%].

• Journal of Chest Surgery
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• 제45권3호
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• pp.199-201
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• 2012

Castleman's disease is a rare disorder characterized by benign tumors that may develop in the lymph node tissue throughout the body. Castleman's disease associated with myasthenia gravis is an especially rare disease. Only less than 10 cases have been reported in the world literature. The cause of Castleman's disease is associated with immune mediated reaction, and myasthenia gravis also develops due to an antibody-mediated process. The cause of myasthenia gravis is the immune activity of Castleman's disease, which may be the promoter of the antibody-mediated process. We report here a case of Castleman's disease, which was incidentally found in a patient diagnosed with myasthenia gravis.

• Journal of Chest Surgery
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• 제50권2호
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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.

• Journal of Chest Surgery
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• 제54권3호
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• pp.228-231
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• 2021

Herein, we report a case in which thoracomyoplasty was performed to manage chronic postlobectomy empyema (PLE). A 54-year-old male patient with a surgical history of right upper lobectomy and thymectomy 35 years previously who had undergone adjuvant radiotherapy presented with purulent discharge on the anterior chest wall. The patient was diagnosed with chronic PLE with ascending infection and concurrent osteonecrosis of the parasternum. Proper drainage was performed for local infection control and the dead spaces were successfully closed with muscle flaps. There have been no complications to date.

• Radiation Oncology Journal
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• 제17권2호
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• pp.146-150
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• 1999

중증근무력증은 수의근을 침범하는 대표적인 자가면역성 질환으로 매우 드물게 발생한다. 흉선종과 중증근무력증이 함께 발생한 환자에서 고식적인 치료방법후 증상의 악화를 보여 전신 방사선치료를 시행한 경험을 보고하고자 한다. 가톨릭대학교 의과대학 성모병원 치료방사선과에서는 1994년 이후 2례의 흉선종을 동반한 중증근무력증 환자에서 흉선절제술, 면역요법과 호르몬치료 등에도 불구하고 증상의 악화를 보여 전신 방사선치료를 시도하였다. 전신 방사선치료는 6MV 선형가속기를 이용하여 10cGy씩, 주 3회로 5$\~$6주에 걸쳐서 150$\~$180cGy 를 조사하였다. 전신 방사선조사 중 구토 이외의 특이한 부작용은 관찰되지 않았다. 증상의 변화를 추적 관찰한 결과, 전신 방사선 치료 후 4$\~$5주부터 증상의 완화를 보이기 시작하여 18개월과 42개월까지 증상의 악화없이 지속적인 증상의 완화를 관찰하였다. 고식적인 치료방법에 효과가 없었던 흉선종을 동반한 중증근무력증 환자에서 전신 방사선치료를 시행하여 비교적 안전하고 좋은 임상 효과를 경험하였기에 보고하는 바이다.

• Journal of Chest Surgery
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• 제50권4호
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• pp.312-315
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• 2017

In the embryo, the thymus originates from the third and fourth pharyngeal pouches and migrates from the superior neck to the mediastinum. Ectopic cervical thymoma (ECT) is an extremely rare tumor that originates from ectopic tissue, and is caused by the aberrant migration of the embryonic thymus. Our patient was a 30-year-old woman who had a nodular lesion in the neck for several years. Ultrasonography and computed tomography were performed. She underwent surgery, and a histological examination resulted in a diagnosis of type AB thymoma. Herein, we report a case of ECT that was resected through a transcervical approach.