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Clinical Characteristics of Pulmonary Histiocytosis X (폐조직구증식증의 임상적 특징)

  • Hwang, Yong-Ii;Park, Gun-Min;Yim, Jae-Joon;Yoo, Chul-Gyu;Lee, Choon-Taek;Kim, Young-Whan;Han, Sung-Koo;Shim, Young-Soo
    • Tuberculosis and Respiratory Diseases
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    • v.51 no.4
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    • pp.346-353
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    • 2001
  • Background : Pulmonary Langerhans cell histiocytosis forms part of a spectrum of diseases that are characterized by a monoclonal proliferation and infiltration of organs by Langerhans cells. Several organ systems may be involved in Langerhans cell histiocytosis, including the lungs, bone, skin, pituitary gland, liver, lymph nodes and thyroid. Pulmonary histiocytosis X represents 2.8% of interstitial lung disease. Here we present the clinical, radiological, therapeutic aspects of pulmonary histiocytosis X. Method : Fourteen cases of biopsy-proven pulmonary histiocytosis X patients who were diagnosed in Seoul National University Hospital during the period from January 1990 to December 1998 were analyzed retrospectively. Result : There were 12 men and 2 women in this study. The initial presenting symptoms were dyspnea, cough, chest pain, which was associated with the pneumothorax, and chest radiography abnormalities. Only 8 patients (57%) were smokers. There were 5 patients with extra-pulmonary histiocytosis (pituitary, bone, skin). Eight patients had received the chemotherapy. There were no mortalities and only one patient experienced an aggravation of symptom during the follow-up period. Conclusion : In contrast to previous reports from other countries, the patients with pulmonary histiocytosis X in this study presented with several different clinical characteristics, such as a male predominance, relatively low smoker's rate, and a better prognosis.

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The Correlation between the Radiological Changes and the Level of Transforming Growth Factor-β1 in Patients with Pulmonary Tuberculosis (폐결핵의 영상학적 진행과 Transforming Growth Factor-β1 농도와의 관련성)

  • Cho, Yongseon;Lee, Yang Deok;Cho, Wook;Na, Dong Jib;Han, Min Soo
    • Tuberculosis and Respiratory Diseases
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    • v.60 no.3
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    • pp.297-303
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    • 2006
  • Background : Pulmonary tuberculosis is frequently accompanied with complications such as bronchiectasis, cavities, fibrosis and a deterioration of the lung function. However, there is little information available on the pathogenesis of these complications in pulmonary tuberculosis. Among the many factors involving in tissue remodeling, transforming growth factor-${\beta}1$ ($TGF-{\beta}1$) is a potent stimulus of the extracellular matrix fomation and a mediator of potential relevance for airway wall remodeling. Therefore, this study examined the relationship between the radiological changes and the $TGF-{\beta}1$ level in patients with pulmonary tuberculosis. Methods : Serum and bronchoalveolar lavage fluid (BALF) were collected from total of 35 patients before treating them for active pulmonary tuberculosis, and the $TGF-{\beta}1$ levels were measured using an enzyme-linked immunosorbent assay (ELISA). The BALF levels were recalculated as the epithelial lining fluid (ELF) levels using the albumin method. pulmonary function test (PFT) and high resolution computed tomography (HRCT) were performed before and after treatment. Results : There was a strong correlation between the serum $TGF-{\beta}1$ level and the presence of cavities (r=0.404, p=0.006), even though the BAL $TGF-{\beta}1$ level showed a weak correlation with complications. In addition, there was no correlation between the $TGF-{\beta}1$ levels before treatment and the changes in the PFT and HRCT during treatment. Conclusion : There is a correlation between the serum $TGF-{\beta}1$ level and cavity formation in pulmonary tuberculosis before treatment. However, further study will be needed to confirm this.

Treatment and Survial Rate of Malignant Peripheral Nerve Sheath Tumors (악성 말초신경막 종양의 치료와 생존율)

  • Lee, Jong-Seok;Jeon, Dae-Geun;Cho, Wan-Hyung;Lee, Soo-Yong;Oh, Jung-Moon;Kim, Jin-Wook
    • The Journal of the Korean bone and joint tumor society
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    • v.9 no.2
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    • pp.131-138
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    • 2003
  • Purpose: We analyzed our malignant peripheral nerve sheath tumor (MPNST) cases to find out their oncologic results following by each treatment modalities. Materials and Methods: Thirty four patients with MPNST were registered in Korea Cancer Center Hospital from Feb. 1986 to Nov. 1996. Seventeen cases were male and 17, female. Average age was 41 years (range 18 to 74). Location of the tumor was as follows; 17 in lower extremity, 11 upper extremity, 4 trunk, and 2 retroperitoneum. Following the AJC classification, stage IA were 2 cases, stage IIA 2, stage IIB 6, stage III 16 and stage IV 8. Twenty six patients took operations and adjuvant chemotherapy and/or radiation therapy, 3 operation only and 3 adjuvant chemotherapy or radiation therapy. Average follow up period was 33.5 months (5.6 to 146.1). Kaplan-Meiyer method was done for survival curve, and log rank test for comparison analysis. Results: Fourteen cases were continuous disease free, 2 no evidence of disease, 2 alive with disease and 14 dead of disease states at final follow up. Actual 5-year and 10-year survival rates were 53.5%, 35.7%. Local recurrence rate after operation was 24.1%. 5-year survival rates of stage I/II/III were 100/85.7/55.9% and 2-year survival rate of stage IV was 14.3% (p=0.04). In 21 cases operated with stage II-III, wide margin (15cases) had 76.0% 5-year survival rate, and marginal or intralesional marigin (6cases) had 40.0%. The actual 5-year survival rate of the group which were done 4 or more cycles chemotherapy (8cases) was 71.4% and the actual 3-year survival rate less than 4cycles chemotherapy (6cases) was 83.3% (p=0.96). In 19 cases operated with stage II-III and which had no radiotherapy, marginal or intralesional margin (5cases) had 3 cases of local recurrences (60.0%), though wide margin (14cases) had 4 cases recurrences (28.6%). There was no local recurrence in 8cases which had pre-or post-operative radiotherapy. Conclusions: Surgical margin is an important factor in local recurrence. Resection margin has a tendency to influence the survival despite insufficient statistical significance. Conventional chemotherapy has no defnite statistical sigficance in the effect on local control and survival. Preoperative and postoperative radiotherapy has some positive effect on local control.

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The Reconstruction with Extracorporeal Irradiated Autograft for Osteosarcoma of Extremities (사지 골육종에서 체외 방사선 조사를 이용한 재건술)

  • Kim, Jae-Do;Lee, Gun-Woo;Chung, So-Hak
    • The Journal of the Korean bone and joint tumor society
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    • v.16 no.1
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    • pp.1-7
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    • 2010
  • Purpose: With advances in various treatment modalities, limb salvage surgery has been commonly used in osteosarcoma of extremities. An alternative method for skeletal reconstruction is reimplantation of the tumor bearing bone following extracorporeal irradiation (ECI). We report the long-term results of ECI autograft in aspect of the oncological and functional outcomes, and complications. Materials and Methods: We retrospectively reviewed 31 osteosarcoma patients who underwent reconstruction with ECI between July 1995 and January 2006. There were 24 males and 7 females with a mean age of 24 (7-74 years) and a mean follow-up of 117 months (17-177 months). Twenty-five cases were reconstructed with ECI autograft, 6 cases with ECI autograft-prosthesis composite. The pathologic subtypes were conventional in 29 cases, periosteal in 1 case, and parosteal in 1 case. The most common location of tumor was distal femur (15 cases) followed by humerus (3), proximal fibula (3) and proximal tibia (3). Musculoskeletal Tumor Society (MSTS) score was used for functional evaluation. The overall survival rate, local recurrence, complications were analyzed. Results: The overall survival rate was 80.6% and the disease-free survival rate was 64.5%. Five patients died of distant metastasis. One patient required above-knee amputation due to local recurrence. All of them, twenty-three complications occurred, which included nonunion in 7 cases, deep infection in 5 cases, joint instability in 4 cases, metal failure in 2 cases, Limb-length discrepancy (LLD) in 2 cases, periprosthetic fracture in 1 case, epiphyseal collapse in 1, local recurrence in 1 case. The mean MSTS functional score was 62.5%. Conclusion: Extracorporeal irradiated autograft can be achieved relatively good result in aspect of oncological and functional aspect, but is needed to be additional research about occurring many complications. The reconstruction with ECI after intercalary or fragmentary resection is effective reconstruction in aspect of oncological and functional result, complications.

Cystic Fibrous Dysplasia in the Kong Bone (낭종성 섬유성 이형성증)

  • Bahk, Won-Jong;Rhee, Seung-Koo;Kang, Yong-Koo;Lee, An-Hi;Park, Jeong-Mi;Chung, Yang-Guk;Choi, Kwang-Cheon
    • The Journal of the Korean bone and joint tumor society
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    • v.13 no.1
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    • pp.22-30
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    • 2007
  • Purpose: We describe clinical, radiographic, MRI and pathologic findings as well as final outcome after simple curettage and bone graft of cystic fibrous dysplasia (FD) in the long bone, which has been rarely documented in the literature. Materials and Methods: Clinical records, radiographs, MRI and histologic slides of 11 patients with cystic FD in the long bone were retrospectively analyzed. Results: Six patients complained pain for several months, 4 patients presented pain after trivial injury event, and 1 patient suffered pathologic fracture. The mode of involvement was monostotic in 10 patients and polyostotic in l patient. The femur was affected in 7 patients, the humerus in 3, and the radius in 1. Radiography showed prominent, expansive lysis associated with ground-glass density of FD. MRI revealed 2 different signals of FD and cyst. Microscopic examination revealed classic findings of FD and non-specific cystic degeneration. The final outcome was satisfactory in every patient. Local recurrence was not observed. Conclusion: Cystic FD in the long bone seems not as rare as the scarcity of reported cases would indicate. MRI features provide a basis for differential diagnosis between benign cystic change and malignant transformation. Cystic FD would be an indication for surgery and simple curettage with allo-chip-bone graft is effective.

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Impact of Hemodialysis on Left Ventricular Performance: A Doppler Echocardiographic Study (혈액투석 유지요법 환자에서 투석 전후의 좌심실 Doppler 심초음파를 이용한 기능지표의 변화)

  • Kang, Dong-Oh;Lee, Du-Ha;Kim, Hyun-Seo;Kim, Hyun-Su;Kim, Sung-Rok;Park, Jong-Seon
    • Journal of Yeungnam Medical Science
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    • v.16 no.2
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    • pp.309-317
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    • 1999
  • Background: Left ventricular diastolic filling is an important determinant for maintenance of cardiac output during hemodialysis. Few investigators have studied the influence of hemodialysis on diastolic function. To evaluate the change of left ventricular systolic and diastolic function. we performed M-mode and Doppler echocardiographic studies before and after hemodialysis. Methods: The study population consisted of 30 patients(15 patients were male, mean age $45{\pm}10$ years) with CRF on maintenance hemodialysis. They have normal left ventricular systolic function(Fractional shortening>30%) and no evidence of valvular heart disease or regional wall motion abnormalities. The ejection fraction (EF) was measured using M-mode echocardiography and Doppler indices such as peak E velocity, peak A velocity, isovolumetric relaxation time(IVRT), deceleration time(DT). and left ventricular ejection time(LVET) obtained from Doppler echocardiography. The index of myocardial performance (IMP) was calculated from each of the Doppler velocity indices. Results: The weight reduction after hemodialysis was $2.1{\pm}1.0kg$(p<0.0001), After hemodialysis, there was some decrease in blood pressure(p<0.05), but no significant change in heart rate, EF and fractional shortening, mean VCF, peak A velocity, and DT. And significant reduction in peak E velocity, E/A ratio(p<0.0001. p<0.001), and significant increase in IVRT and IMP(p<0.05, p<0.0001) were noted. Conclusion: In conclusion, preload reduction is the main mechanism that accounts for changes in Doppler diastolic indices after hemodialysis. And an increased IMP suggests that diastolic function may be aggravated after hemodialysis, and that implies impaired left ventricular filling and disturbed left ventricular compliance.

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The Effect of Long-term Steroid Therapy Combined with Azathioprine in Henoch-$Sch{\ddot{o}}nlein$ Nephritis (Henoch-$Sch{\ddot{o}}nlein$(HS) 신염에서 장기 스테로이드와 Azathioprine의 병합치료 효과)

  • Moon, Kyoung-Sang;Jin, So-Young;Kim, Eun-Mi
    • Childhood Kidney Diseases
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    • v.2 no.2
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    • pp.118-124
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    • 1998
  • Purpose : There is no specific treatment guidelines for Henoch-$Sch{\ddot{o}}nlein$(HS) nephritis. Therefore we performed this study to observe the effect of long term steroid therapy combined with azathioprine Methods : Treatment protocols; 1) Steroid pulse therapy: methylprednisolon 30 mg/kg/dose, maximum 1 gm, intravenolisly 6 times for alternate day. 2) Oral steroid was given 2 mg/kg/day for 1 month, 1 mg/kg/day for following 1 month and alternate day oral steroid combined with azathioprine 2 mg/kg/day for 2 years. Results : Time period from HSP to onset of HS nephritis was between 2 weeks to 5 months with mean $7.4{\pm}7.4$ weeks. Clinical remission were seen in 4 cases out of 5 ($80\%$). Mean time period with disappearance of proteinuria and microscopic hematuria were $5{\pm}2.4$ month and $13.3{\pm}2.9$ month respectively. On pathologic findings by ISKDC, 3 cases were grade IIIb, 2 cases were grade IV in first kidney biopsies and showed pathologic improvement in follow up tidneybiopsiesafterlyearstreatment. Conclusion : As there is no definitive treatment for HS nephritis so far, our study of long term oral steroid therapy with azathioprine was effective in clinical and histologic aspect. Therefore further study in HS nephritis with in a large group will be needed in the future.

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Radiographic Status of the Visited Patients at University Hospital Emergency Room (한 대학병원 응급실 내원환자의 방사선촬영 실태)

  • Ahn, Byeoung-Ju
    • Journal of the Korean Society of Radiology
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    • v.5 no.2
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    • pp.81-92
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    • 2011
  • This study was carried out to improve service efficiency and to cope with a emergency situation in emergency radiography, through analysis of the radiographic distriution and literature cited about emergency care. Data collection of radiographic distribution was surveyed for 1270 emergency outpatients who visit during JAN, 2009at ER of the general hospital in Gwang city. The results is as follows : Emergency radiography rate of simple radiography was 56.6%, special radiography 2. 5%, CT 34.2%, and ultrasonography 6.7%, In simple radiography rate. a high rate was distributed on male(63.6%), thoracicsurgery part(90.0%), admission patient(77.9%), and long stayed patient at ER. In special raiography rate, a high rate was obsurved in urologic part(28.6%), and in CT rate, observed neurosurgerty part(49.2%) and neurologic part(36.7%). Ultrasonography rate was high for female(8.8%) and internal medicine part(15.9%). There are distributed regional radiography rate in radio-graphic type that chest(55.3%) is high in the simple radiography, urinary system(1.2%) in the special study, and brain(40.0%) in the CT. Regional radiography rate according to diagnostic department also was showed highly for head(64.6%) in neuro surgery, chest(90.0%) in thoracic surgery, abdomen(58.0%) in general surgery, spine(40.0%) in neuro surgery, and pelvis(15.9%), upper extrimity(20.5%), and lower extrimity(31.8%) in orthopedic surgery each. Mean radiographic case number per patient of simple radiography was sinificant on sex, age, transfer relation in both total and radiopraphic patients(p<0.05). Mean radiographic case number was highly distributed on male(2.2 case number) in sex, on thirties(2.7) in age, transferred patient(2.7) in patient type, and on nurosurgery(3.4) in diagnostic charged part. Total radiographic case number in regional party was highly distributed on chest(998 case number.) Considering the above results, emergency radiographer should take care of the elder patient in emergency radiography and get hold of injury mechanism to decrease possible secondary injury during radiography. Because of high radiography rate of urinary system in special study, related instrument. All radiographer who take charge emergency patient should cope with a emergency situation during radiography. Because head trauma patients is very important in patient care, especilly in CT at night, charged doctor should be always sitted with CT room and monitoring patient. Radiography was reqested by many diagnostic department in ER. Considering that rate of simple radiography is high, special room for emergency radiopraphy should be established in ER area, and the radioprapher of this room should be stationed radiologic technician who is career and can implement emergency patient care and The disposition of men which is appropriate with emergency patient increase is necessary.

Hypofractionated Radiation Therapy for Early Glottic Cancer - Preliminary Results - (초기 성문암 환자에서의 소분할 조사법을 이용한 방사선치료 - 예비적 결과 -)

  • Wu Hong-Gyun;Hong Semie;Shin Seong Soo;Park Charn Il
    • Radiation Oncology Journal
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    • v.19 no.4
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    • pp.301-305
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    • 2001
  • Purpose : This study was peformed for the evaluation of the feasibility and toxicity of hypofractionated radiation therapy for early glottic cancer Methods and Materials : From February 1999 to February 2000, 20 patients with Histologically confirmed Stage I, II glottic cancer were enrolled into this study. There were 18 males and 2 females, the median age of the patients was 59 years. The distribution of stage distribution was as fellows; T1aN0-16 patients, T1bN0-1 patient, T2N0-3 patients. Eighteen patients underwent laryngomicroscopic biopsy only, and two patients underwent laser cordectomy. All patients received radical radiation therapy (2.5 Gy per fraction, 24 fractions, total 60 Gy). Median duration of treatment was 36 days (range $31\~45\;days$). Results : Radiation therapy were well tolerated. Most common acute reactions were odynophagia and hoarseness, and these reactions resolved after radiation therapy. There were one case of RTOG grade 3 odynophagia $(5\%)$, six cases of grade 3 hoarseness $(30\%)$. Response of radiation therapy was evaluated one month after completion of treatment. All patients revealed complete response. During follow up, total three cases of treatment failure were detected. two cases were local recurrence in 10 and 13 months of radiation therapy and one case was local recurrence and distant metastasis in 2 months of radiation therapy. Conclusion : This hypofractionated radiation therapy schedule was feasible and effective for control of early glottic cancer But longer follow up time would be required to assess the long-term disease control and the late complication by shortening radiation therapy duration.

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Thrombolytic Therapy for Thrombosis of Prosthetic Mitral Valve - A Case Report - (인공 승모판막에 생긴 혈전의 혈전용해 치료 - 1례 보고 -)

  • Kang, Shin-Kwang;Kim, Si-Wook;Won, Tae-Hee;Ku, Kwan-Woo;Na, Myung-Hoon;Yu, Jae-Hyun;Lim, Seung-Pyung;Lee, Young;Jeong, Jin-Ok
    • Journal of Chest Surgery
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    • v.35 no.11
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    • pp.826-830
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    • 2002
  • Prosthetic valve thrombosis(PVT) may be a life-threatening complication requiring prompt intervention. This is a case report of thrombolytic therapy for thrombosis of prosthetic mitral valve. A 47 year-old male admitted to the emergency room for abrupt onset of dyspnea. He had undergone mitral valve replacement(On-Ⅹ valve, 29mm) for mitral stenosis 8 months ago. The patient's international normalized ratio(INR) on admission was 1.09. The mechanical clicks were muffled and rales were heard in both lung fields. A transesophageal echocardiography(TEE) revealed prosthetic valve thrombosis with increased transvalvular pressure gradient(34 mmHg). The patient's condition needed to intubation for mechanical ventilation due to hemodynamic compromise, however his wife and relatives refused the surgical intervention due to financial problems. The patient was transferred to the cardiac care unit and we decided to perform thrombolytic therapy. A bolus of 1,500,000 IU of urokinase was given, followed by a drip of 1,500,000 IU for 1 hour. The patient did not improved hemodynamically; therefore, we gave 100 mg of tissue plasminogen activator(t-PA) for over 2 hours. During that time mechanical clicks were audible and hemodynamics of the patient improved progressively. A TEE showed disappearance of thrombus and decreased pressure gradient(1.7 mmHg) after 6 hours of thrombolytic therapy. The patient was recovered without any neurologic sequale and was discharged with administration of warfarin.