• Childhood Kidney Diseases
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• v.5 no.2
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• pp.125-135
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• 2001

Purpose : Efforts to predict long-term outcome of focal segmental glomerulosclerosis(FSCS) have been made but have yielded conflicting results. Reports are rare especially in Pediatric patients. In this study, we reviewed the predictable prognostic factors in patients of FSGS Method : Fifty children who diagnosed as biopsy-proven FSGS at department of pediatrics at Yonsei university were studied retrospectively. Based on medical records, response to treatment and pathologic slides, we compared normal renal function group and decreased renal function group, assessed the factors affecting renal survival and progression to renal failure. Results : The mean age at onset was 8 1/12 years, sex ratio was 2.3 : 1, and the mean duration of follow-up was 7 1/12 years. The overall renal survival rate was $34\%$ at 5 years, $8\%$ at 10 years Five-year survival rate was $74\%$ in normal renal function group and $27\%$ in decreased renal function group. Between the two groups, there were no significant differences in age at onset, sex ratio, amount of proteinuria, incidence of hematuria and hypertension, mesangial hypercellularity. Decreased renal function group showed higher serum creatinine level, poor response to treatment, higher percent of glomeruli with sclerosis, moderate to severe tubulointerstitial change and vascular change(P<0.05). The prognostic factors of renal survival rate were same as above and incidence of hypertension also affected renal survival( P<0.05). The progression rate to renal failure did not show statistically significant factor. Conclusion : We reviewed the factors affecting long-term outcome of FSGS. Serum creatinine level, steroid responsiveness, and the degree of glomerulosclerosis were significant prognostic factors. (J Korean Soc Pediatr Nephrol 2001 ;5 : 125-35)

• Journal of Chest Surgery
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• v.39 no.8 s.265
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• pp.626-632
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• 2006

Background: Pulmonary hypertension caused by chronic pulmonary embolism is underrecognized and carries a poor prognosis. Medical therapy is generally unsatisfactory and palliative. With the improvement of operative technique and postoperative management, pulmonary endarterectomy has been the treatment of choice for this condition. Material and Method: Between January 2001 and December 2005, eleven patients were received pulmonary endarterectomy. All patients had chronic dyspnea and exercise intolerance. Diagnosis was made with cardiac echocardiography, lung perfusion scan and computed tomography. Before the operation, Greenfield vena cava filter were placed in all patient except one. Deep hypothermic circulatory arrest was used for the distal-most portion of the endarterectomy procedure. More than moderate degree of tricuspid reguirgitation was repaired during operation. Result: There was no early and late death. Right ventricular systolic pressure was reduced significantly after operation from $91{\pm}21$ mmHg to $40{\pm}17$ mmHg on echocardiography (p=0.001). NYHA class and tricuspid reguirgitaion were improved postoperatively. Although mild reperfusion injury in three case and postoperative delirium in one case were observed, all of them recovered without complication. Conclusion: Pulmonary thromboendarterctomy offers to patient an acceptable morbidity rate and anticipation of clinical improvement. This method is safe and effective operation for pulmonary hypertension caused by chronic pulmonary thromboembolism.

• Journal of Hospice and Palliative Care
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• v.8 no.1
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• pp.30-36
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• 2005

Purpose: Regardless of treatment, brain metastases are associated with a poor prognosis. We aimed to investigate the clinical characteristics of cancer patients with brain mestastases they after admission into a hospice unit and knowing they have received appropriate hospice and palliative care. Methods: We retrospectively reviewed the medical records in 40 cancer patients with brain metastases they after admission into a hospice unit from March in 2003 to March in 2005. Results: There were 20 males (50%) and 20 females (50%), and the median age of the patients was 64 years. The most common cause of brain metastases was lung cancer (22 patients, 55%), followed by stomach cancer (5, 13%) and breast cancer (3, 8%). Sixteen patients (40%) have never been teated and 20 patient have received the chemotherapy for the primary cancer. The most common symptom of cancer patients with brain metastases is headache (12 patients, 30%), followed by mental change (10, 25%), focal weakness (9, 23%) and convulsion (4, 10%). The most prevalent cause for admission was mental change (13 patients, 33%), followed by pain (9 patients, 23%). The reasons for hospice and palliative care were through recommendation of physician (1 patient, 3%), patient and family self (14, 35%) and the others (25, 62%). Twenty five of the others has been referred to hospice and palliative care during conservative management after referral and enrolled at hospice unit. The median hospitalization was 19 days and median survival in hospice and palliative care was 41 days. The median survival was 87 days from the day when the cancer patients have been diagnosed as brain metastases to death. Conclusion: The duration of hospice and palliative care was not enough to care the cancer patients with brain metastases. We suggest physicians, patients and family need the education and promotion for effective hospice and palliative care.

• Clinical and Experimental Pediatrics
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• v.48 no.7
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• pp.731-736
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• 2005

Purpose : Acute pyogenic osteomyelitis is uncommon in children. Delayed diagnosis and inappropriate treatment are leading to growth failure and deformation. We review the clinical manifestations and treatment of acute osteomyelitis in children according to age. Methods : A retrospective analysis was made of 32 patients who underwent antibiotic management or operation between Aug 1989 and Dec 2003 for acute pyogenic osteomyelitis in age from 0 to 15 years old. Results : The study group was composed of 21 boys and 11 girls. The subjects were divided into four groups according to age : 0-1 yr(n=6), 1-5 yr(n=11), 6-10 yr(n=8), and 11-15 yr(n=7). Nineteen cases were diagnosed in Winter. Femur was the most common infected site(37.5%). There were no predisposing factor in 17 patients, and 7 of 15 patients had trauma history. Sepsis was important predisposing factor in neonates. The chief complaints were pain, swelling and fever. S. aureus(61%) is the most common organism. Twenty-nine patients were treated with operation and concomitant antibiotics. Two cases had sequelae in follow-up period : One is avascular necrosis of femur and the other is discrepancy of leg length. Conclusion : In our review, because of poor prognosis in septic neonates, we recommend to treat actively neonatal sepsis and prevent or detect osteomyelitis early. Because most of patients were diagnosed and treated in orthopedic surgery, the rate of operation was too high. So, protocol for further evaluation and management of acute osteomyelitis in pediatric patients were needed.

• Clinical and Experimental Reproductive Medicine
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• v.33 no.4
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• pp.237-243
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• 2006

Objective: The aim of this study was to evaluate the efficacy of frozen-thawed ET in poor prognosis patients such as the old age (38~44 years; OA group) and the patients who did not achieve clinical pregnancy with the first fresh ET cycle (non-pregnant patients; NP group). Methods: Laboratory and clinical data were collected from fresh and frozen-thawed ET cycles of OA and NP group. Controlled ovarian hyperstimulation (COH) and conventional insemination or ICSI, in vitro culture and ET were performed by routine procedures. Supernumerary embryos were frozen by the slow freezing method, and frozen embryos were thawed by the rapid thawing method. Embryo development, pregnancy and implantation rates were statistically analyzed by Student t-test and chi square test Results: Mean ages were similar between fresh ET ($40.0{\pm}1.8$ years, n=206) and frozen-thawed ET ($39.9{\pm}1.9$ years, n=69) cycles in OA group. However, the clinical pregnancy and implantation rate of subsequent frozen-thawed ET significantly higher than those of fresh ET cycles (29.0% and 11.2% vs. 16.5% and 7.0%, p<0.05). In NP group, there was no difference in the mean age between fresh ET ($31.2{\pm}2.3$ years, n=40) and frozen-thawed ET ($31.9{\pm}3.1$ years, n=119) in subsequent cycles. The clinical pregnancy and implantation rates were similar between the subsequent fresh ET (42.5% and 22.6%) and the frozen-thawed ET (40.3% and 18.8%). Conclusion: In old age patients, higher pregnancy rate of frozen-thawed ET compared to fresh ET cycles in this study. It may be related that better uterine environments for implantation in frozen-thawed ET cycles than that of non-physiological hormonal condition in uterus of fresh COH cycles.

• Asian Pacific Journal of Cancer Prevention
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• v.14 no.12
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• pp.7367-7369
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• 2013

Background: Inoperable and metastatic hepatocellular carcinoma (HCC) is associated with a poor prognosis and low chemotherapeutic efficiency. Sorafenib is an oral multi-kinase inhibitor exerting its effects via the RAF/MEK/ERK pathway, vascular endothelial growth factor receptor (VEGFR) and platelet derived growth factor receptor beta (PDGFR-${\beta}$) tyrosine kinases. Randomized studies have shown a significant contribution of sorafenib to life expectancy and quality of life of cancer patients. The aim of the present study is to evaluate the efficacy and side effects of sorafenib therapy in Turkey. Materials and Methods: Data for 103 patients (82 males, 21 females) receiving sorafenib therapy in 13 centers from February 2008 to December 2012 were evaluated. Median age was 61 years and median ECOG performance status was 1 (range: 0-2). 60 patients (58%) had hepatitis B, 15 patients (15%) had hepatitis C infection and 12 patients (12%) had a history of alcohol consumption. All of the patients had Child scores meeting the utilization permit of the drug in our country (Child A). Results: A total of 571 cycles of sorafenib therapy were administered with a median of four per patient. Among the evaluable cases, there was partial response in 15 (15%), stable disease in 52 (50%), and progressive disease in 36 (35%). Median progression-free survival was 18 weeks and median overall survival was 48 weeks. The dose was reduced only in 6 patients and discontinued in 2 patients due to grade 3-4 toxicity, 18 patients (17%) suffering hand-foot syndrome, 7 (7%) diarrhea, and 2 (2%) vomiting. Conclusions: This retrospective study demonstrated better efficacy of sorafenib therapy in patients with advanced HCC compared to the literature while progression-free survival and overall survival findings were comparable. The side effect rates indicate that the drug was tolerated well. In conclusion, among the available treatment options, sorafenib is an efficient and tolerable agent in patients with inoperable or metastatic HCC.

• Clinical and Experimental Pediatrics
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• v.45 no.3
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• pp.370-375
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• 2002

Purpose : In most cases, myelodysplastic syndrome(MDS) transforms into a more aggressive state or acute myelogenous leukemia; it's prognosis is very poor. It is believed that hematopoietic stem cell transplantation(HSCT) is the only curative treatment of MDS, but available data in children are very sparse. In this report, the short term outcome of HSCT in childhood MDS was analyzed. Methods : Ten children with MDS(CMMoL 5, RAEB 3, RAEBt 2) underwent HSCT(HLA-matched sibling transplantation 4, HLA-matched unrelated transplantation 4, cord blood transplantation 1, HLA-mismatched familial transplantation 1) between November 1995 and January 2001 at St. Mary's Hospital. Median follow-up duration was 11 months. Results : Engraftment was successful in all cases and 8 patients are alive without disease. Three cases of VOD were observed and improved without complication. Four cases of grade II and 1 case of grade III acute GVHD were observed and well controlled with treatment. Three patients relapsed after transplantation. One patient is alive without disease after cytoreduction with allogenic stem cell rescue and 2 patients died of relapse. Conclusion : HSCT is a curative strategy of MDS and the survival rate is relatively higher than that of adults. But there is an obvious need for more studies because of the small number of patients and the short duration of the follow-up.

• Journal of Korean Neurosurgical Society
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• v.30 no.3
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• pp.263-271
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• 2001

Objective : Glioblastomas, the most common type of primary brain tumors, are highly invasive and cause massive tissue destruction at both the tumor invading edges and in areas that are not in direct contact with glioma cells. As a result, patients with high-grade gliomas are faced with a poor prognosis. Such grim statistics emphasize the need to better understand the mechanisms that underlie glioma invasion, as these may lead to the identification of novel targets in the therapy of high grade gliomas. Protein kinase C(PKC) is a family of serine/threonine kinases and an important signal transduction enzyme that conveys signals generated by ligand-receptor interaction at the cell surface to the nucleus. PKC appears to be critical in regulating many aspects of glioma biology. The purpose of this study was to assess accurately the role of PKC in the invasion regulation of human gliomas based on hypothesis that protein kinase C(PKC) is functional in the process of glial tumor cell invasion. Method : To test this hypothesis, U-87 malignant glioma cell line intracellular PKC levels were up and down regulated and their invasiveness was tested. Intracellular PKC level was characterized using PKC activity assays. Invasion assays including barrier migration and spheroid confrontation were used to study the relationship between PKC concentration and invasiveness. Result : The cell line which were treated by PKC inhibitor tamoxifen and hypericin exhibited decreased PKC activity and decreased invasive abilities dose dependently both in matrigel invasion assay and tumor spheroid fetal rat brain aggregates(FRBA) confrontation assay. However, the cell line that was treated by PKC activator 12-O-tetradecanylphorbol-13acetate(TPA) did not exhibit increases in either PKC activity or invasive ability. Conclusion : These studies suggest that PKC may be a useful molecular target for the chemotherapy of glioblastoma and other malignancies and that a therapeutic approach based on the ability of PKC inhibitors may be helpful in preventing invasion.

• Journal of Chest Surgery
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• v.43 no.4
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• pp.375-380
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• 2010

Background: Primary cardiac tumors are extremely rare. The most common type are benign myxomas, and these are almost completely curable with early surgery. Malignant tumors, however, such as sarcomas, are difficult to remove surgically, and their prognosis is known to be poor. In this study, data on patients who had undergone surgical treatment of cardiac tumor in the authors' hospital were collected and analyzed. Material and Method: The subjects included 28 patients who had undergone surgical treatment of cardiac tumor from August 1993 to December 2008. Their medical records were reviewed and retrospectively analyzed. Result: The patients were aged from 20 to 76 years (mean age: $54.2{\pm}15.6$), and 11 were male (39%) and 17 female (61%). Fifteen of them (54%) underwent emergency surgery to improve heart failure symptoms. The most common preoperative symptom was dyspnea (15 cases, 54%). Preoperative echocardiography was performed on all the patients. The average size of the tumor as measured during the operation was $7.0{\pm}6.9cm$ (the average length of the long axis was 2∼40 cm), and the sites of tumor attachment were the interatrial septum (18 cases, 64%), the left atrium (9 cases, 32%), the mitral valve annulus (2 cases, 7%), and the left ventricle (2 cases, 7%). The operation was performed with an incision through both atria in all the patients, and a complete excision was made in 25 cases (89%). According to the biopsy results, there were 4 cases of sarcoma (14%), 1 case of lipoma (4%), and 23 cases of myxoma (82%). The three cases in which the tumors were not completely excised were sarcomas. No operative deaths occurred after the operations. Outpatient follow-up was possible for 24 cases (86%), with a mean follow-up period of $46.8{\pm}42.7$ months. Late death occurred in 3 of the 24 patients; each of these patients had sarcomas. Of these patients, the first had undergone two repeat surgeries, the second had metastatic sites removed, and the last had only chemotherapy. The average recurrence time was $12.7{\pm}10.8$ months, and the average metastasis time was $20.5{\pm}16.8$ months. Conclusion: Most cardiac tumors are benign myxomas. In principle, they should be surgically treated because they can create risks such as embolism, and can be radically treated when surgically removed. In most cases, however, malignant sarcomas are already considerably advanced with severe infiltration into the neighboring tissues at the time of diagnosis. The surgical removal of malignant sarcomas is known to be difficult because of the advanced stage and degree of infiltration. We suggest that excision of the removable portion of the tumor sites to alleviate symptoms such as heart failure can improve quality of life.

• Tuberculosis and Respiratory Diseases
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• v.39 no.1
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• pp.15-23
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• 1992

Background: The prognosis of lung cancer remains poor and early detection and curative surgery is still the most effective treatment for many. In the early detection of lung cancer, sputum cytology and simple chest x-ray are used, but both of these tests are far from being perfect. So we studied the characteristics of patients diagnosed as lung cancer without demonstrable mass lesion on simple chest x-ray to help in the early diagnosis of lung cancer. Methods: We conducted a retrospective study on 11 subjects who were diagnosed as lung cancer at Seoul National University Hospital between August 1986 and June 1989 and had no demonstrable mass lesion on simple chest x-rays. Results: Ten of 11 patients were male, 8 had a history of smoking, most frequent symptoms were sputum, cough, and hemoptysis, and 3 patients either had wheezing or stridor. In 3 of the cases, although there were no mass lesion, there were ill-defined infiltration, major fissure thickening, and fibrostreaky density mimiking tuberculosis where the tumor was eventually found and in one patient tumor was masked by a rib shadow. Also in one case, lateral chest film demonstrated a retrocardiac mass. Both bronchoscopy and computed tomogram were useful in the localization of the tumor. Seven of 11 had relatively early disease (less than StageII). Four of 11 are still alive without any evidence of recurrence between 2 and a half and 4 years after the operation. Conclusion: We conclude that in patients with respiratory symtoms in whom cancer cannot be ruled out, sputum cytology and lateral chest x-rays should be taken and that if necessary further studies like computed tomogram and bronchoscopy should be done to aid in the early diagnosis of lung cancer.