• Journal of Chest Surgery
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• 제33권5호
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• pp.391-397
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• 2000

Background: Cardiac involvement of Behcets disease is very rate, however, the prognosis of Behcet disease depends on cardiovascular complications. In this article, we described surgical treatment of aortic insufficiency with Behcets disease. Material and Method: From March 1986 to February 1998, we operated on 10 patients of aortic insufficiency with Behcets disease. Male to female ratio was 8 to 2, and age ranged from 21 to 40 years(mean 32.8 years). There were 8 patients with evidence of Behcets disease and another 2 patients had some suspicious findings of Behcets disease(i.e., prosthetic value dehiscence, hypertrophied aortic wall). Adequate preoperative medical treatment for Behcets disease was done in 3 patients. Result: We performed 24 open heart surgeries in 10 patients. Redo value replacements using prosthetic valves were done in 4 patients. Among them, 2 patients were operated on for a second redo valve replacement and one of them operated on for a 4th and 5th operation because of recurrent paravalvular leakage. These 4 patients expired. 1 patient who had undergons tissue value replacement is alive. 1 patient who underwent Cabrol operation expired dut to rupture of graft anastomosis site. We used homografts in 3 patients. In 2 of them, we performed aortic root replacement and subcoronary valve replacement in another patient. The patient who underwent subcoronary valve insertion had remnant aortic insufficiency, so we are closely observing him. We also performed Ross operation in a 24 year old female who suffered severs aortic insufficiency and endocarditis after aortic valvuloplasty. 5 patients are alive and mean follow up duration is 49.0 months. Among them, we used homografts or sutografts in 4 patients. We could observe excellent clinical results in the patients who underwent aortic root replacement using homograft and they were treated medically for Behcets disease. Conclusion: We concluded that adequate preporative diagnosis, clinical suspicion, and periopertive medical treatment for Behcets disease are very important for the result of surgical management of aortic insufficiency with Behcets disease. The use of homograft or autograft was helpful for the healing of anastomosis site and we should carefully observe the long term follow up results.

• Clinical and Experimental Pediatrics
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• 제49권1호
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• pp.107-111
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• 2006

크론병은 매우 드문 질환이나 지난 10년간 발생률이 꾸준히 증가하고 있다. 저자들은 골수 이형성 증후군을 가진 환아에서 크론병이 발병한 2례를 소아에서는 최초로 보고하는 바이다. 첫번째 환아는 3세에 골수 이형성 증후군으로 진단받았고, 수 년간 지속된 반복적인 복통 및 설사, 혈변, 성장 부전이 있어 8세에 크론병으로 진단받았다. 대장 내시경 검사에서는 맹장에서 오름 결장에 걸쳐 조약돌상 점막과 표재성 궤양 및 염증성 삼출이 있었으며, 조직 소견은 궤양 사이에 정상 점막을 포함하고 있으면서 림프구 침윤을 보였다. Mesalazine과 deflazacort로 치료 후 증상은 호전을 보였다. 두 번째 환아는 9세에 골수 이형성증후군으로 진단받았으며, 13세에 반복되는 혈변과 복통, 구토, 발열로 크론병으로 진단받았다. 대장 내시경 검사에서 크고 깊은 경화성 궤양이 회맹판에서 맹장 쪽 주위에서 발견되었다. 이 외의 부위에는 병변이 없어 병변을 절제하였고, 조직은 경벽 염증과 림프구 집합을 동반한 궤양 소견을 보였다. 절제술 후 증상은 호전을 보였다.

• Journal of Gastric Cancer
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• 제21권3호
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• pp.268-278
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• 2021

Purpose: While several prognostic models for the stratification of death risk have been developed for patients with advanced gastric cancer receiving first-line chemotherapy, they have seldom been tested in the Chinese population. This study investigated the performance of these models and identified the optimal tools for Chinese patients. Materials and Methods: Patients diagnosed with metastatic or recurrent gastric adenocarcinoma who received first-line chemotherapy were eligible for inclusion in the validation cohort. Their clinical data and survival outcomes were retrieved and documented. Time-dependent receiver operating characteristic (ROC) and calibration curves were used to evaluate the predictive ability of the models. Kaplan-Meier curves were plotted for patients in different risk groups divided by 7 published stratification tools. Log-rank tests with pairwise comparisons were used to compare survival differences. Results: The analysis included a total of 346 patients with metastatic or recurrent disease. The median overall survival time was 11.9 months. The patients were different into different risk groups according to the prognostic stratification models, which showed variability in distinguishing mortality risk in these patients. The model proposed by Kim et al. showed relative higher predicting abilities compared to the other models, with the highest χ² (25.8) value in log-rank tests across subgroups, and areas under the curve values at 6, 12, and 24 months of 0.65 (95% confidence interval [CI]: 0.59-0.72), 0.60 (0.54-0.65), and 0.63 (0.56-0.69), respectively. Conclusions: Among existing prognostic tools, the models constructed by Kim et al., which incorporated performance status score, neutrophil-to-lymphocyte ratio, alkaline phosphatase, albumin, and tumor differentiation, were more effective in stratifying Chinese patients with gastric cancer receiving first-line chemotherapy.

• 대한기관식도과학회지
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• 제10권2호
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• pp.17-21
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• 2004

Background Insertion of tracheal stent in the treatment of benign tracheal & bronchial disease has increased since the introduction of expandable metallic stent. Material & Methods : Between Jan, 1995 and Feb. 2004, eight patients who had benign tracheo-bronchial disease underwent insertion of expandable metallic tracheal stent. We retrospectively analyzed stent insertion indications, complications, and following the result. Results : Surgical indications were post-intubation tracheal stenosis (1 case), tracheal stenosis following tracheal surgery (2 cases), tracheo-esophageal fistula (2 cases), broncho-pleural fistula(1 case), left main bronchus stenosis following bronchoplasty (1 case), and left main bronchus stenosis due to mediastinal repositioning (1 case). Expandable metallic tracheal stent was inserted in five patients to resolve dyspnea caused by airway obstruction, and to prevent recurrent pneumonia in three patients. The complication developed in 6 patients $75\%$; 3 cases of distal stenosis due to growth of granulation tissue, and one case each of tearing of posterior membrane, aggravation of tracheo-esophageal fistula, and airway partial obstruction due to stent migration. The stent was removed in 5 patients and tracheal surgery (tracheal resection and end to end anastomosis with primary repair of esophagus, pericardial patch tracheo-bronchoplasty, tracheal repair and omental wrapping) was performed in 3 patients. Conclusion Insertion of self expandable metallic stent in benign tracheo-bronchial disease is an effective means of relieving dyspnea for only a short period, and it did not increase the long term survival. Better means of treatment of benign tracheo-bronchial stenosis in necessary.

• Journal of Korean Neurosurgical Society
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• 제58권1호
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• pp.54-59
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• 2015

Objective : The aim of this prospective study was to evaluate the efficacy of bone cement-augmented percutaneous short segment fixation for treating Kummell's disease accompanied by severe osteoporosis. Methods : From 2009 to 2013, ten patients with single-level Kummell's disease accompanied by severe osteoporosis were enrolled in this study. After postural reduction for 1-2 days, bone cement-augmented percutaneous short segment fixation was performed at one level above, one level below, and at the collapsed vertebra. Clinical results, radiological parameters, and related complications were assessed preoperatively and at 1 month and 12 months after surgery. Results : Prior to surgery, the mean pain score on the visual analogue scale was $8.5{\pm}1.5$. One month after the procedure, this score improved to $2.2{\pm}2.0$ and the improvement was maintained at 12 months after surgery. The mean preoperative vertebral height loss was $48.2{\pm}10.5%$, and the surgical procedure reduced this loss to $22.5{\pm}12.4%$. In spite of some recurrent height loss, significant improvement was achieved at 12 months after surgery compared to preoperative values. The kyphotic angle improved significantly from $22.4{\pm}4.9^{\circ}$ before the procedure to $10.1{\pm}3.8^{\circ}$ after surgery and the improved angle was maintained at 12 months after surgery despite a slight correction loss. No patient sustained adjacent fractures after bone cement-augmented percutaneous short segment fixation during the follow-up period. Asymptomatic cement leakage into the paravertebral area was observed in one patient, but no major complications were seen. Conclusion : Bone cement-augmented percutaneous short segment fixation can be an effective and safe procedure for Kummell's disease.

• Asian Pacific Journal of Cancer Prevention
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• 제14권5호
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• pp.3305-3308
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• 2013

Background: The role of inflammation in prostate diseases is suggested by the presence of inflammatory cells within the prostate in benign prostatic hyperplasia (BPH) and prostate cancer (PCa) patients. In addition, bacterial and viral infection may lead to chronic and recurrent inflammation of the prostate. The human papillomaviruses (HPVs) are a family of sexually transmitted viruses which have been implicated in the aetiology of cervical cancer and several other malignancies. This study evaluated the frequency of HPV infection in individuals with prostatic disease in Iran. Materials and Methods: The study included formalin fixed paraffin- embedded tissue samples of 196 primary prostate cases, including 29 PCa and 167 BPH samples. HPV DNA was purified and amplified through MY09/MY11 and GP5+/GP6+ primers with nested PCR. All patients were interviewed using a questionnaire to collect demographic information. Results: Nested PCR showed that HPV DNA was found in 17.2 percent of PCa samples and 4.8 percent of BPH samples (not significant). Conclusions: Our data do not support a significant role of HPV infection in prostatic disease in Iranian patients, but demographic data indicated a probable association between presence of HPV DNA and risk of inflammation in prostate tissue which might lead to prostate carcinoma. Further studies are required to elucidate any roles of HPV infection in prostatic disease.

• Journal of Gastric Cancer
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• 제15권2호
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• pp.113-120
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• 2015

Purpose: Although several clinical trials have proven the efficacy of adjuvant S-1 treatment in gastric cancers, it is still unclear which patients receive the most benefit. In this study, we prospectively recruited patients with locally advanced gastric cancer who had undergone curative resection followed by adjuvant S-1 administration to investigate which factors affect the outcomes. Materials and Methods: Between July 2010 and October 2011, we enrolled 49 patients who underwent curative resection for stage II or III gastric cancer and who subsequently received adjuvant S-1 treatment for 1 year. Results: Twenty-nine patients (59.2%) continued S-1 treatment for 1 year, and 12 patients (24.5%) experienced recurrent disease during the follow-up period. Patients with continuation of S-1 for 1 year had significantly increased rates of disease-free survival (P<0.001) and overall survival (P=0.001) relative to the patients who discontinued S-1 during year 1. Multivariate analysis indicated poor outcomes for patients with stage III disease and those who discontinued S-1 treatment. Excluding patients who discontinued S-1 due to cancer progression (n=7), adjuvant treatment with S-1 still demonstrated a significant difference in the disease-free survival rate between the patients who continued treatment and those who discontinued it (P=0.020). Conclusions: S-1 is tolerated as adjuvant treatment in gastric cancer patients. However, discontinuing S-1 treatment may be an unfavorable factor in the prevention of recurrence. S-1 adjuvant treatment should be continued for 1 year if possible through the proper management of toxicities.

• Journal of Medicine and Life Science
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• 제17권3호
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• pp.65-73
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• 2020

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a single-gene disease of the cerebral small blood vessels caused by mutations in the NOTCH3 gene on chromosome 19. Although CADASIL was known as a rare disease, recent research has suggested that the NOTCH variants could be found frequently even in the general population. The main clinical features included recurrent stroke, migraine, psychiatric symptoms, and progressive cognitive decline. On brain magnetic resonance imaging, patients with CADASIL showed multifocal white matter hyperintensity lesions, lacunar infarcts, microbleeds, and brain atrophy. Among them, lacunar infarcts and brain atrophy are important in predicting the clinical outcomes of patients with CADASIL. In the Jeju National University Hospital, we have diagnosed 213 CADASIL patients from 2004 to 2020. Most NOTCH3 mutations were located in exon 11 (94.4%), and p.Arg544Cys was the most common mutation. The mean age at diagnosis was 61.0±12.8 years. The most common presenting symptoms were ischemic stroke (24.4%), followed by cognitive impairment(15.0%), headache (8.9%), and dizziness(8.0%). Although the exact prevalence of CADASIL in Jeju is still unknown, the disease prevalence could be as high as 1% of the population considering the prevalence reported in Taiwan. Therefore, it is necessary to discover efficient biomarkers and genetic tests that can accurately screen and diagnose patients suspected of having CADASIL in this region. Ultimately, it is urgent to explore the exact pathogenesis of the disease to identify leading substances of treatment potential, and for this, multi-disciplinary research through active support from the Jeju provincial government as well as the national government is essential.

• Pediatric Infection and Vaccine
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• 제29권3호
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• pp.166-172
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• 2022

블라우증후군(Blau syndrome)은 nucleotide-binding oligomerization domain protein 2 (NOD2) 유전자의변이에 의해 발생하는 질환으로 육아종성 피부염 및 만성 포도막염, 관절염을 특징으로 한다. 증상이 비특이적이고 동시다발적으로 발생하지 않아 진단이 어려운 경우가 많다. 반복되는 피부 발진 및 발열에 대해 두 차례 가와사키병으로 오진되어 면역글로불린과 전신 스테로이드로 치료받은 바 있는 13개월 남자 환자에서 블라우 증후군을 진단한 증례를 보고하고자 한다.

• 대한안과학회지
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• 제59권11호
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• pp.1071-1076
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• 2018

목적: 기존 안와 황색육아종이 진단되었던 환자에서 재발된 피부 황색육아종을 통하여 면역글로불린G4관련안질환이 동반 진단된 증례를 보고하고자 한다. 증례요약: 양안의 눈꺼풀 부종을 주소로 38세 남성이 내원하였다. 전안부 및 후극부에서 특이 소견이 관찰되지 않았다. 조직검사상 포말조직구 및 토우튼 거대세포가 관찰되며 S100 및 CD1 염색에 음성 소견을 보여 성인황색육아종 진단하 경구 아자씨오프린과 프레드리솔론 치료를 받았다. 4년 뒤 양안의 노란색 눈꺼풀 종괴를 주소로 재내원하였다. 자기공명영상에서 양안 안와 상외측의 대칭적인 연부조직 부종이 관찰되었고, 혈액검사상 immunoglobulin G (IgG) 및 IgG4 수치가 상승되었다. 조직검사 후 면역화학염색에서 IgG4+/IgG+ 10-20%, IgG4+ 22/고배율시야가 확인되었으며 과거 조직을 재염색하여 IgG4+/IgG+ 40-50%, IgG4+ 59/고배율시야를 확인하여 전형적인 면역글로불린G4관련안질환으로 새롭게 진단하였다. 결론: 비특이적인 눈꺼풀 부종환자에서 면역글로불린G4관련안질환을 고려해야 하며, 기존 황색육아종 환자에서도 면역글로불린G4관련안질환이 동반될 수 있다.