• Title/Summary/Keyword: joint disease

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Soft Tissue Malignant Myoepithelioma in the Extremities (사지에 발생한 연부 조직 악성 근상피종)

  • Kong, Chang-Bae;Lee, Jung-Wook;Koh, Jae-Soo;Song, Won Seok;Cho, Wan Hyeong;Jeon, Dae-Geun;Lee, Soo-Yong
    • The Journal of the Korean bone and joint tumor society
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    • v.20 no.2
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    • pp.54-59
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    • 2014
  • Purpose: We report the diagnosis, treatment outcomes and prognosis of the patients with soft tissue malignant myoepithelioma in the extremities. Materials and Methods: We retrospectively reviewed 6 patients with soft tissue malignant myoepithelioma in the extremities who were treated at our institution between 2008 and 2014. Two patients received unplanned excision at another hospital and remaining 4 patients underwent the biopsy procedures and received wide excision at our hospital. Results: There were 3 men and 3 women with mean age of 41 (33-54) years. The average follow up was 28 (9-45) months. Among the 6 patients, only 4 patients underwent biopsy procedures under the impression of malignant soft tissue sarcoma. Surgical margins for these 4 patients were negative. Two patients who had unplanned excision received another re-excision and one of them showed no residual tumor in the resected specimen. Local recurrences were developed in all patients and distant metastasis in 4 patients. All 4 patients who developed distant metastasis died due to disease progression. Among the 2 patients who developed local recurrence only, one patient has another local recurrence after re-operation and remaining one patient is no evidence of disease for 2 years after resection of locally recurred mass. Conclusion: Soft tissue malignant myoepithelioma in the extremities is a rare disease and shows an aggressive behavior. Appropriate biopsy under the impression of soft tissue malignancy is necessary and complete surgical resection with wide margins is the recommended treatment of choice.

Diagnostic Value of MRI in Schwannoma (신경초종 진단에 있어 자기공명영상촬영(MRI)의 진단적 가치)

  • Kim, Jeung Il;Kim, Um Ji;Moon, Tae Yong;Lee, In Sook;Song, You Seon;Choi, Kyung Un
    • The Journal of the Korean bone and joint tumor society
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    • v.20 no.2
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    • pp.60-65
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    • 2014
  • Purpose: To diagnose soft tissue tumor, such as lipoma and Schwannoma, magnetic resonance imaging (MRI) is sufficient in most cases. However, various characteristics are found in MRI images of Schwannoma, thus other type of tumors are often misdiagnosed as Schwannoma with MRI images. In this study, we evaluate the diagnostic value of specific MRI findings of Schwannoma. Materials and Methods: From January 2002 to May 2013, 104 patients who are suspected as Schwannoma rith MRI images are included in data, and the final diagnosis is confirmed with biopsy. Patients are divided into group 1 and group 2 who are confirmed as Schwannoma and other disease with biopsy, respectively. Results: 92 patients were diagnosed as Schwannoma (group 1) and 12 patients were diagnosed as other disease (group 2). We investigate the diagnostic value of specific MRI findings of Schwannoma. 41 patients of group 1 (45%) and 0 patients of group 2 (0%) showed target sign, 47 patients of group 1 (51%) and 2 patients of group 2 (17%) showed fascicular sign, 44 patients of group 1 (48%) and 5 patients of group 2 (42%) showed fat split sign, 28 patients of group 1 (30%) and 1 patients of group 2(9%) showed nerve entering and exiting sign, and 8 patients of group 1 (9%) and 6 patients of group 2 (50%) showed none of four specific findings on their MRI images. 52 patients of group 1 (57%) and 5 patients of group 2 (42%) have tumors on the pathway of nerve. Target sign could be considered as the best diagnostic value of the sign we investigate (p<0.05). Conclusion: Although specific MRI findings have powerful diagnostic value, patients are often misdiagnosed as Schwannoma with MRI findings. Therefore, if patients who are suspected as Schwannoma based on MRI findings have no target sign on their MRI images, we should consider the possibility of other disease.

The Significance of Sentinel Node Biopsy in Malignant Melanoma and Squamous Cell Carcinoma of Lower Extremities (하지에 발생한 악성흑색종 및 편평상피세포암에서 소속 림프절 생검의 의미)

  • Kim, Jae-Do;Lee, Gun-Woo;Kwon, Young-Ho;Chung, So-Hak
    • The Journal of the Korean bone and joint tumor society
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    • v.16 no.2
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    • pp.69-73
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    • 2010
  • Purpose: Sentinel lymph node (SLNB) is the first confronted lymph node from primary lesion of tumor through lymphatic drainage, which is important for determining early metastasis and setting guidelines for treatment. We reported significant of sentinel lymph node biopsy in malignant melanoma (MM) and squamous cell carcinoma (SCC) of lower extremities. Materials and Methods: Twenty-five cases of surgically treatment and being possible for follow up more than 1 year among the patients who were diagnosed as MM and SCC in this institution from Sep. 2005 to Jan. 2009, and 10 cases of them were performed SLNB. Average age was 64 years old, and 15 cases of male and 10 cases of female were in this group. Results: 3 years overall survival rate was 100% and 3 years disease-free survival rate was 76%. Metastasis occurred in total 6 patients, 4 cases of inguinal lymph nodes, 1 case of soft tissue around knee, 1 case of left achilles tendon. In 15 cases of not performing SLNB, overall survival rate was 93.3% and disease-free survival rate was 73.3%. In 10 cases of performing SLNB, overall survival rate was 100% and disease-free survival rate was 90%. And only 1 case showed positive finding in the biopsy, and none of the 10 cases showed metastasis in follow-up. Conclusion: SLNB leads simpler and less complications compared to prior elective lymph node dissection, and shows high degree of accuracy. Throughout the SLNB, setting guidelines for treatment by accurate staging is thought to be helpful for increasing the survival rate in the patient with MM and SCC.

A Case Report of Novel Mutation in GNPTAB in Two Siblings with Mucolipidosis Type III Alpha/beta (GNPTAB 유전자에서 새로운 돌연변이가 확인된 뮤코지방증 III형 남매)

  • Kim, Min-Sun;Park, Esther;Song, Ari;Im, Minji;Park, Hyung-Doo;Cho, Sung Yoon;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.18 no.3
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    • pp.99-106
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    • 2018
  • Mucolipidosis type III (pseudo-Hurler polydystrophy) is a mucolipids degrading disorder caused by a mutation in the GNPTAB gene and is inherited by autosomal recessive. It is diagnosed by examining highly concentrated mucolipids in blood and the diagnosis can be confirmed by genetic testing. Mucolipidosis type III is a rare and progressive metabolic disorder. Its initial signs and symptoms usually occur around 3 years of age. Clinical manifestations of the disease include slow growth, joint stiffness, arthralgia, skeletal abnormalities, heart valve abnormalities, recurrent respiratory infection, distinctive facial features, and mild intellectual disability. Here, we are presenting two siblings of mucolipidosis type III, a 4-year-old female and a 2 years and 7 months old male with features of delayed growth and coarse face. The diagnosis was confirmed by [c.2715+1G>A(p.Glu906Leufs*4), c.2544del(p.Glu849Lysfs*22)] mutation in targeted gene panel sequencing. In this case, c.2544del is a heterozygote newly identified mutation in mucolipidosis type III and was not found in the control group including the genome aggregation database. And it is interpreted as a pathogenic variant considering the association with phenotype. Here, we report a Korean mucolipidosis type III patients with novel mutations in GNPTAB gene who have been treated since early childhood. Owing to recent development of molecular genetic techniques, it was possible to make early diagnosis and treatment with pamidronate was initiated appropriately in case 1. In addition to these supportive therapies, efforts must be made to develop fundamental treatment for patients with early diagnosis of mucolipidosis.

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An Analysis on Factors Affecting Local Control and Survival in Nasopharvngeal Carcinoma (비인두암의 국소 종양 치유와 생존율에 관한 예후 인자 분석)

  • Chung Woong-Ki;Cho Jae-Shik;Park Seung Jin;Lee Jae-Hong;Ahn Sung Ja;Nam Taek Keun;Choi Chan;Noh Young Hee;Nah Byung Sik
    • Radiation Oncology Journal
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    • v.17 no.2
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    • pp.91-99
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    • 1999
  • Propose : This study was performed to find out the prognostic factors affecting local control, survival and disease free survival rate in nasopharyngeal carcinomas treated with chemotherapy and radiation therapy. Materials and Methods : We analysed 47 patients of nasopharyngeal carcinomas, histologically confirmed and treated at Chonnam University Hospital between July 1986 and June 1996, retrospectively. Range of patients' age were from 16 to 80 years (median; 52 years). Thirty three (70$\%$) patients was male. Histological types were composed of 3 (6$\%$) keratinizing, 30 (64$\%$) nonkeratinizing squamous cell carcinoma and 13 (28$\%$) undifferentiated carcinoma. Histoiogicai type was not known in 1 patient (2$\%$). We restaged according to the staging system of 1997 American Joint Committee on Cancer Forty seven patients were recorded as follows: 71: 11 (23$\%$), T2a; 6 (13$\%$), T2b; 9 (19$\%$), 73; 7 (15$\%$), 74: 14 (30$\%$), and NO; 7 (15$\%$), Nl: 14 (30$\%$), N2; 21 (45%), N3: 5 (10%). Clinical staging was grouped as follows: Stage 1; 2 (4$\%$), IIA: 2 (4$\%$), IIB; 10 (21$\%$), III; 14 (30$\%$), IVA; 14 (30$\%$) and IVB; 5 (11$\%$). Radiation therapy was done using 6 MV and 10 MV X- ray of linear accelerator. Electron beam was used for the Iymph nodes of posterior neck after 4500 cGy. The range of total radiation dose delivered to the primary tumor was from 6120 to 7920 cGy (median; 7020 cGy). Neoadjuvant chemotherapy was performed with cisplatin +5-fluorouracil (25 patients) or cisplatin+pepleomycin (17 patients) with one to three cycles. Five patients did not received chemotherapy. Local control rate, survival and disease free suwival rate were calculated by Kaplan-Meier method. Generalized Wilcoxon test was used to evaluate the difference of survival rates between groups. multivariate analysis using Cox proportional hazard model was done for finding prognostic factors. Results: Local control rate was 81$\%$ in 5 year. Five year survival rate was 60$\%$ (median survival; 100 months). We included age, sex, cranial nerve deflicit, histologic type, stage group, chemotherapy, elapsed days between chemotherapy and radiotherapy, total radiation dose, period of radiotherapy as potential prognostic factors in multivariate analysis. As a result, cranial none deficit (P=0.004) had statistical significance in local control rate. Stage group and total radiation dose were significant prognostic factors in survival (P=0.000, P=0.012), and in disease free survival rates (P=0.003, P=0.008), respectively. Common complications were xerostomia, tooth and ear problems. Hypothyroidism was developed in 2 patients. Conclusion : In our study, cranial none deficit was a significant prognostic factor in local control rate, and stage group and total radiation dose were significant factors in both survival and disease free survival of nasopharyngeal carcinoma. We have concluded that chemotherapy and radiotherapy used in our patients were effective without any serious complication.

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Long-Term Results of 2-Dimensional Radiation Therapy in Patients with Nasopharyngeal Cancer (이차원방사선치료를 시행한 코인두암 환자의 장기 추적 결과 및 예후인자 분석)

  • Lee, Nam-Kwon;Park, Young-Je;Yang, Dae-Sik;Yoon, Won-Sup;Lee, Suk;Kim, Chul-Yong
    • Radiation Oncology Journal
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    • v.28 no.4
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    • pp.193-204
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    • 2010
  • Purpose: To analyze the treatment outcomes, complications, prognostic factors after a long-term follow-up of patients with nasopharyngeal carcinoma treated with radiation therapy (RT) alone or concurrent chemoradiation therapy (CCRT). Materials and Methods: Between December 1981 and December 2006, 190 eligible patients with non-metastatic nasopharyngeal carcinoma were treated at our department with a curative intent. Of these patients, 103 were treated with RT alone and 87 patients received CCRT. The median age was 49 years (range, 8~78 years). The distributions of clinical stage according to the AJCC 6th edition included I: 7 (3.6%), IIA: 8 (4.2%), IIB: 33 (17.4%), III: 82 (43.2%), IVA: 31 (16.3%), IVB: 29 (15.3%). The accumulated radiation doses to the primary tumor ranged from 66.6~87.0 Gy (median, 72 Gy). Treatment outcomes and prognostic factors were retrospectively analyzed. Acute and late toxicities were assessed using the RTOG criteria. Results: A total of 96.8% (184/190) of patients completed the planned treatment. With a mean follow-up of 73 months (range, 2~278 months; median, 52 months), 93 (48.9%) patients had relapses that were local 44 (23.2%), nodal 13 (6.8%), or distant 49 (25.8%). The 5- and 10-year overall survival (OS), disease-free survival (DFS), and disease-specific survival (DSS) rates were 55.6% and 44.5%, 54.8% and 51.3%, in addition to 65.3% and 57.4%, respectively. Multivariate analyses revealed that CCRT, age, gender, and stage were significant prognostic factors for OS. The CCRT and gender were independent prognostic factors for both DFS and DSS. There was no grade 4 or 5 acute toxicity, but grade 3 mucositis and hematologic toxicity were present in 42 patients (22.1%) and 18 patients (9.5%), respectively. During follow-up, grade 3 hearing loss in 9 patients and trismus in 6 patients were reported. Conclusion: The results of our study were in accordance with findings of previous studies and we confirmed that CCRT, low stage, female gender, and young age were related to improvement in OS. However, there are limitations in the locoregional control that can be achieved by CCRT with 20 conventional radiation therapy. This observation has led to further studies on clarifying the efficacy of concurrent chemotherapy by intensity modulated radiation therapy.

The Study on the investigation of oriental medical theraphy(oriental medical theraphy by symptoms and signs and Sasang constitutional medicine)and the each effect of oriental medicine, occidental medicine and both joint control (뇌졸중(腦卒中)에 대(大)한 한방치료법(韓方治療法) 연구(硏究)(증치의학(證治醫學)과 사상의학(四象醫學)) 및 한방(韓方), 양방(洋方), 양(洋)·한방(韓方) 협진치료(協診治療) 효과(效果)에 관(關)한 연구(硏究))

  • Kim, Jong-won;Kim, Young-kyun;Kim, Beob-young;Lee, In-seon;Lee, In-seon;Jang, Kyung-jeon;Gwon, Jeong-Nam;Lee, Won-oe;Song, Chang-won;Park, Dong-il
    • Journal of Sasang Constitutional Medicine
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    • v.10 no.2
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    • pp.351-429
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    • 1998
  • The Purpose of Study 1. Inspection of clinical application on TCD to CVA 2. Objective Comparement and analysis about treatment effect of Western-Medicine, Korean Medicine, Cooperative consultation of Korean and Western medicice for CVA The Subject of Study We intended for the eighty six patient of CVA who had been treated in the Oriental Medical Hospital at Dong Eui Medical Center from 1997. 8. I to 1998. 7. 31 1. View of CT, MRI : the patient of Cb infarction 2. Attack Time : The patient who coming hospital falling ill within the early one week The method of study 1. Treat four group of Korean medicine, Constitution medicine, Western medicine, cooperative consultation of Korean medicine and Western medicine. 2. Application of TCD Check the result for three times, immediatly after the attack, two months later, four months later 3. Comparative analysis of each treatment effect by clinical symptoms and pathologic examination 4. The Judgement of the patient The Result From 8/1/1997 to 7/31/1998, We have the following result by clinical analysis intended for CVA 86 patients who had been treated in the Oriental Medical Hospital at Dong Eui Medical Center from 1997. 8. 1 to 1998. 7. 31 in 1. Analysis according to Age The first stage of thirties, forties, seventies is heavier than forties, fifties in improvement and Index of improvement of symptom 2. Analysis according to sex We have no special relation in an average of symptom and improvement, Index improvement 3. Analysis according to Family History We have the better result in first stage and improvement, index improvement when no family history. 4. Analysis according to Past History We have no special relation in past history like hypertension, DM, heart problem 5. Analysis devided two group, above group and under group on the basis of the average in first stage of all patient. We have the better result when the first stage is light, that the first score of barthel index and CNS is high. 6. Analysis of the effect of treatment about Korean medical treatment, Western medical treatment, cooperative treatment. In this study, the highest group of rate of treatment at four contrast groups (Korean medicine, Constitution medicine, Western medicine, cooperative treatment according to dyagnosis and range of treatment was the patient group of doing dyagnosis and method of treatment based on constitution medicine theory. This is that of doing demostation, A-Tx, po-herb-medication according to dyagnosis and treat method of constitution of Lee Je-ma In case of left, the case of dyagnosis any disease according to doctor view but, normal in TCDwas 22-beginning of attack, 20- two weeks later, 11 case-four weeks later in case of right, 15-beginning of attack, 12-two weeks later, 9 case four weeks later. So left vessel compares to right vessel is more interference, in fact more than a 1/2 of the patients of MCA disease can't do dyagnosis. In rate of imparement, the state of pacient improved but there isn't the improved case of result in TCD. 7. In TCD dyagnosis, between the case of inconsus the doctor view specially MCA in brain blood vessel is in large numbers and in total 86's patient, impossible case of dyagnosis according to interferiance of temporal is 21 case. 7. Result study about application of Kreaan medical treatment 1) The impossible patient of observation MCA blood vescular for interference temporal bone happened in large numbers. 2) There is the case having difference result to CT,MRI, MRA result. 3) Because individual difference is large, excluding to ananalogy of symptom. This is normal numerical value that has possibility of being checked as abnormal numerical value 4) there are a lot of cases that the speed of normal part is as similarly measured as that of abnormal part. It means that we cannot judge the disease by this measure 5) It is rare that this measure represent degree of improvement in patient's condition of disease. When we observe patient's condition become better, but we have no case that the result of TCD test better. 6) The result could be appear differently by the technique of the tester or by the experience of the tester 7) In the TCD test, abnormal symptoms is checked at 0 week, but at 2th week, normal symptoms is checked, again at 4th week abnormal is checked. According to the above result, CVA diagnosis is difficult only with TCD, as it appear in diagnosis error check which is suggested in the problem connected to project, for the aged persons who have the worst hardening of part of the cranium (1998. 5. 26 77 of 83 patients is 50s) there is a lot of cases that the measurement is impossible by TCD and the correction of measurement numerical value is decreased, as the age of cerebral infarction is high, TCD is inappropriate to diagnosis equipment through this study.

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The Clinical Aspects of Acute Septic Arthritis and Acute Osteomyelitis in Children (소아기 급성 화농성 관절염과 급성 골수염의 임상적 고찰)

  • Kim, Jin-Man;Lee, So-Yeon;Kim, Young-Ho;Shin, Eon-Woo;Oh, Phil-Soo;Kim, Kwang-Nam
    • Pediatric Infection and Vaccine
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    • v.12 no.2
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    • pp.149-156
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    • 2005
  • Purpose : Acute septic arthritis and acute osteomyelitis are not rare diseases in pediatric population. But when the diagnosis is delayed or inappropriate treatments are given, permanent disabilities of joint or bone can be followed. We analysed clinical manifestations, laboratory findings, X-ray findings, causative microorganisms and antibiotic susceptibility results of the two diseases in children. Methods : During January 1992 and May 2002, we conducted a retrospective study of 103 children who were diagnosed as acute septic arthritis and acute osteomyelitis. We selected out 34 children who had positive culture results in the blood or involved sites. Results : 19 cases were diagnosed as acute septic arthritis and 15 cases were acute osteomyelitis. These diseases were most common in preschool children and next in neonates. Hip joints and tibia were the most common sites in each disease. X-ray findings showed abnormalities in 6 cases(36%) of acute septic arthritis and 7 cases(50%) of acute osteomyelitis on admission. The most common microorganism isolated from the involved sites was Staphylococcus aureus; 12 out of 14 cases in acute septic arthritis and 6 out of 13 cases in acute osteomyelitis. Conclusion : It is difficult to make a clear initial diagnosis of the two diseases. We could not find any differences between these two diseases on clinical manifestations such as fever, swelling, tenderness and limitation of movements in joint and bone. The most common microorganism was Staphylococcus aureus.

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Limb Salvage in the Treatment of the Upper Extremity Bone Tumors (상지에 발생한 악성 및 침윤성 양성골종양에 대한 사지 구제술)

  • Hahn, Soo-Bong;Shin, Kyoo-Ho;Kim, Bum-Soo
    • The Journal of the Korean bone and joint tumor society
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    • v.1 no.2
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    • pp.154-163
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    • 1995
  • With the development of anticancer chemotherapy and improved radiographic imaging studies, limb salvage operation became possible in the treatment of malignant and aggressive benign tumors. High grade sarcomas of the shoulder or the upper extremity can be surgically treated with a forequarter amputation, shoulder disarticulation or limb salvage surgery such as Tikhoff-Linberg procedure, segmental resection and replacement with endoprosthesis, segmental resection and replantation, or segmental resection and free vascularized bone graft. Among them the limb salvage surgery showed not only preservation of the remained upper extremity but also the excellent functional results. When comparing amputation and limb salvage operation while performing anticancer chemotherapy in both cases, 5 year survival rate, local recurrence, and distant metastasis did not show much difference. We studied 13 cases of limb salvage for the malignant and aggressive benign bone tumor of the upper extremity from March 1986 to December 1993 at Severance hospital. The summarized results were as follows. 1. There were 21 cases of malignant bone tumors and 5 cases of benign aggressive ones. 2. Of the 26 cases of malignant and benign aggressive bone tumors, limb salvage procedures such as Tikhoff-Linberg operation(8 cases), endoprosthetic replacement(2 cases), segmental resection and replantation(2 cases), and segmental resection and free vascularized fibular graft(l case) were done in 13 cases. 3. In 13 patient on whom the limb salvage procedure was performed, there were 3 osteosarcomas, 4 chondrosarcomas, 3 giant cell tumors, 1 Ewing's sarcoma, 1 leiomyosarcoma and 1 chondroblastoma. 4. In 13 patients, there was no local recurrence nor distant metastasis except one who had a segmental resection of the entire humerus part including glenoid and then postoperative anticancer chemotherapy for the treatment of the Ewing's sarcoma of the entire shaft of the humerus with pathological fracture. Local recurrence occurred 2 years and 6 months postoperatively in this Ewing's sarcoma patient, so forequarter amputation was performed and the irradiation and the anticancer chemontherapy were performed, but multiple bony metastasis developed and died of the disease 22 months after local recurrence. 5. The patients were followed-up for I year to 7 years and 5 months(average 4 years 5 months). 6. In 8 cases in which Tikhoff-Linberg procedure was performed, the function of the hand was almost normal. 7. Segmental resection and endoprosthetic replacement was performed in 2 cases, and the function of the remained upper extremity was good with no evidence of aseptic loosening or nerve palsy. 8. In 1 case of segmental resection and free vascularized fibular graft for the patient of the chon drosarcoma in the humerus, the function of the shoulder, elbow and hand was nearly normal. 9. In I case of leiomyosarcoma which involved both forearm muscles and bone near wrist joint, segmental resection and replantation was performed, and the patient has useful hand function.

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Evaluation of Quality of Life according to Temporomandibular Disorder Symptoms in Dental Hospital Worker (치과병원 종사자에서 측두하악관절장애 증상에 따른 삶의 질 평가)

  • Kim, Dong-Kook;Lim, Hyun-Dae;Lee, You-Mee
    • Journal of Oral Medicine and Pain
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    • v.37 no.1
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    • pp.61-72
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    • 2012
  • Temporomandibular disorder(TMD) is relatively prevalent disease, and quality of life may be impaired in TMD patients. Like general population, dental hospital workers are also exposed to the risk of TMD. But, many of them tend to overlook or tolerate their symptoms for lack of time and interest. Therefore, problems may become more serious, causing interference of performing task and decrease of quality of life. The aim of this study were to obtain data for TMD prevalence in dental hospital workers and to evaluate quality of life according to TMD symptoms. Subjects were recruited from Wonkwang University Dental Hospital. After consent, subjects completed quality of life questionnaire and were evaluated for subjective and objective signs and symptoms of TMD. Subjects were classified into 4 groups : (1) normal group (2) joint disorder group, (3) local myalgia group, and (4) myofascial pain group. The result of the study indicated that TMD negatively influences the quality of life in dental hospital worker. TMD symptoms can deteriorate quality of life in dental hospital worker. Future effort to make protocol for proper management is needed.