• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.12 no.2
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• pp.177-182
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• 2009

Purpose: Colitis is a condition associated with a spectrum of altered morphologic changes and cellular adhesion. E-cadherin plays a key role in the establishment and maintenance of epithelial tissue structure and cell-cell adhesion. The purpose of this study is to evaluate E-cadherin expression in colonic epithelium of various colitis in children. Methods: The expressions of E-cadherin were examined in 39 cases of colonic mucosal biopsy specimen using immunohistochemical staining. When more than 50 percent of cells exhibited uniformly the same intensity and pattern of immunostaining as the adjacent normal mucosa, the antigen expression was considered normal. Abnormal expression was defined when less than 50 percent of cells stained, when cells showed a heterogeneously weak or altered distribution, or when complete absence of staining was observed. Results: Fifteen cases with non-specific colitis (38.5%), 7 cases of with Crohn's disease (17.9%), 5 cases of infectious colitis and milk protein sensitive proctocolitis (12.8%), 3 cases of ulcerative colitis (7.7%), 2 cases of Henoch-Schonlein purpura colitis (5.1%), one case of Behcet's disease and ischemic colitis (2.6%) were included in this study. E-cadherin expression was decreased in all kinds of colitis. Reduced expression of E-cadherin was observed in 77 percent of cases. E-cadherin was weaker or no expression in reparative epithelium and "ulcer associated cell lineage". Conclusion: Altered expression of E-cadherin occurs during mucosal inflammation in any kinds of colitis. These changes may be involved in promoting cell migration during epithelial restitution of the gastrointestinal mucosa.

• Journal of Chest Surgery
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• v.41 no.2
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• pp.239-246
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• 2008

Background: Aortic surgery for high risk patients has high mortality and morbidity rates, and the necessity of performing aortic surgery in cancer patients is questionable because of their short life expectancy. Endovascular repair of aneurysm repair can be considered for high risk patients and cancer patients because it has relatively lower invasiveness and shorter recovery times than aortic surgery does. Especially, percutaneous endovascular stent graft treatment is more useful for high risk patients because it does not require general anesthesia. Material and Method: From July 2003 to September 2007, twelve patients who had inoperable malignancy or who had a high risk of complication because of their combined diseases during aortic surgery underwent endovascular aortic aneurysm repair. he indications for endovascular repair were abdominal aortic aneurysm in 5 patients, descending thoracic aortic aneurysm in 6 patients and acute type B aortic dissection in one patient. The underlying combined disease of these patients were malignancy in 3 patients, respiratory disease in 6 patients, old age with neurologic disease in 6 patients, Behcet's iseae in one patient and chronic renal failure in one patient. Result: Stent grafts were inserted percutaneously in all cases. There were 4 hospital deaths and there were 3 delayed deaths during the follow-up periods. There were no deaths from aortic disease, except one hospital death. There were several complications: a mild cerebrovascular accident occurred in one patient, acute renal failure occurred in 2 patients and ischemic bowel necrosis occurred in one patient. Mild type I endoleak was observed in 2 patients and type II endoleak was observed in a patient after stent graft implantation. Newly developed type I endoleak was observed in a patient during the follow-up period. Conclusion: Percutaneous endovascular stent graft insertion is relatively safe procedure for high risk patients and cancer patients. Yet it seems that its indications and its long term results need to be further researched.

• Journal of Chest Surgery
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• v.35 no.3
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• pp.188-198
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• 2002

Background: Reoperations on the aortic root or the ascending aorta are being performed with increasing frequency and remain a challenging problem. This study was performed to analyze the results of reoperations on the ascending aorta and aortic root. Material and Method: Between May 1995 and April 2001, 30 patients had reoperations on the ascending aorta and aortic root and were reviewed retrospectively. The mean interval between the previous repair and the actual reoperation was 56 months(range 3 to 142 months). Seven patients(23.3%) had two or more previous operations. The indications for reoperations were true aneurysm in 7 patients(23.3%), prosthetic valve endocarditis in 6(20%), false aneurysm in 5(16.7%), paravalvular leak associated with Behcet's disease in 4(13.3%), malfunction of prosthetic aortic valve in 4(13.3%), aortic dissection in 3(10%), and annuloaortic ectasia in 1(3.3%). The principal reoperations performed were aortic root replacement in 17 patients(56.7%), replacement of the ascending aorta in 8(26.7%), aortic and mitral valve replacement with reconstruction of fibrous trigone in 2(6.6%), patch aortoplasty in 2(6.6%), and aortic valve replacement after Bentall operation in 1 (3.3%). The cardiopulmonary bypass was started before sternotomy in 7 patients and the hypothermic circulatory arrest was used in 16(53.3%). The mean time of circulatory arrest, total bypass, and aortic crossclamp were 20$\pm$ 12 minutes, 228$\pm$56 minutes, and 143$\pm$62 minutes, respectively Result: There were three early deaths(10%). The postoperative complications were reoperation for bleeding in 7 patients(23.3%), cardiac complications in 5(16.7%), transient acute renal failure in 2(6.6%), transient focal seizure in 2(6.6%), and the others in 5. The mean follow-up was 22.8 $\pm$20.5 months. There were two late deaths(7.4%). The actuarial survival was 92.6$\pm$5.0% at 6 years. One patient required reoperation for complication of reoperation on the ascending aorta and aortic root(3.7%). The 1- and 6-year actuarial freedom from reoperation was 100% and 83.3$\pm$15.2%, respectively. One patient with Behcet's disease are waiting for reoperation due to false aneurysm, which developed after aortic root replacement with homograft. There were no thromboembolisms or anticoagulant related complications. Conclusions: This study suggests that reoperations on the ascending aorta and aortic root can be performed with acceptable early mortality and morbidity, and adequate surgical strategies according to the pathologi conditions are critical to the prevention of the reoperation.

• Tuberculosis and Respiratory Diseases
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• v.43 no.4
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• pp.630-636
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• 1996

Bronchiolitis obliterans organizing pneumonia(BOOP) is a pathologic entity characterized by the formation of plugs of fibrous tissue in bronchioles and alveolar ducts. It clad been described in association with several connective tissue diseases, i.e. systemic lupus erythematosus, Behcet's disease, polymyositis-dermatomyositis, polymyalgia rheumatica and Sjogren's syndrome. Recently, there were few additional reports on BOOP, anteceded by rheumatoid arthritis. We clad experienced a case of 60 years old female patient who clad been admitted for acute respiratory difficulty and abnormal chest roentgenographic findings. She was diagnosed as having rhematoid arthritis 20 years ago. On the course of our treatment, she did not respond to antibiotics. Moreover, no microorganism grew in the sputum and blood. Thus, the bronchoscopic biopsy was done. The patient showed a dramatic response to steroid therapy, and the pathologic bindings of the bronchoscopic biopsy confirmed as BOOP. To our knowledge, this is the first reported case of BOOP anteceded by rheumatoid arthritis in Korea.

• The Korean Journal of Blood Transfusion
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• v.23 no.2
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• pp.173-179
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• 2012

We report on two cases of anti-$Jk^a$, whose reactivity disappeared on an antibody identification test using enzyme-treated red cells. One of two patients was a 72-year-old female with cirrhosis of the liver and colon cancer, and the other was a 55-year-old female with known MDS and incomplete Behcet's disease. Results of an antibody identification test using a LISS/Coombs gel card (DiaMed AG) showed negative to one positive with red cells having the $Jk^a$ antigen; however, all reactions using the enzyme-treated cells showed negative results, which was unexpected. The patients' RBC phenotype was Jk(a-b+). We obtained positive results in reactions of enzyme-treated $Jk^a+$ cells and EDTA using a patient's serum and proved that the cause of the negative reaction might be complement-related.

• Journal of Chest Surgery
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• v.38 no.10 s.255
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• pp.693-698
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• 2005

Background: Sinus valsalva aneurysm (SVA) is a rare disease, and it is frequently accompanied by ventricular septal defect and aortic valve regurgitaion. For treatment of SVA, several surgical mordalities were applied, but there was no report on the long-term result after surgical repair in Korea. We reviewed our 28 years of experiences and analyzed the long-term results after treatment of sinus valsalva aneurysm with or without rupture. Material and Method: Between March 1974 and February 2002, 81 patients were operated under the impression of sinus valvsalva aneurysm or sinus valsalva aneurym rupture. Retrospectively we reviewed the patients' record. Mean age of patients was $29.2\pm11.5$ and there were 49 males. Accompanyng diseases were as follows: VSD in 50, PDA in 2, Behcet's disease in 2, TOF in 1, RVOTO in 1, AAE in 1. Seventy-seven $(95\%)$ patients had sinus valsalva rupture and in 14 patients, subacute bacterial endocarditis was accompanied. Degree of aortic valve regurgitation was as follows: grade I: 8, II: 10, III: 9, IV: 4. Most common rupture site was right coronary sinus (66 patients, $81\%$) and most common communication site was right ventricle (53 patients). In repair of sinus valsalva rupture, patch was used in 37 patients, and direct suture was done in 38 patients. Result: There was one surgical death $(1.2\%)$. Follow up was done in 78 patients $(97.5\%)$, mean follow up period was $123.3\pm80.9(3\~330\;months)$. During the follow up period, 3 patients died $(3.8\%)$. One patient died of heart failure, another patient died of arrhythmia and the other one died of unknown cause. In two patients, complete atrio-ventricular block was developed during follow up period, and there was no operation related event or complication. Kaplan-Meier survival analysis revealed $92.5\pm3.5\%$ survival at 15 and 27 years and it seems to be satisfactory. Conclusion: Long-term surgical results and survival is satisfactory after repair of sinus valsalva aneurysm with or without rupture.

• Tuberculosis and Respiratory Diseases
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• v.43 no.5
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• pp.763-773
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• 1996

Background : Behçet's syndrome is a chronic multisystemic disease affecting many organs such as skin, mucosa, eye, joint, central nervous system and blood vessels. Lung involvement occurs in 5% of Behçet's syndrome and is thought to be due to the pulmonary vasculitis leading to thromboembolism, aneurysm and arteriobronchial fistula. Pulmonary vasculitis in Behçet's syndrome is a unique clinical feature, differing from other vasculitis affecting the lung and is one of the major causes of death. Therefore, we examined the incidence, the clinical features, the radioloic findings and the clinical courses of the lung involvement in Behçet's syndrome. Methods: We retrospectively reviewed the medical records and radiologic studies of 10 cases of the lung involvement in Behçet's syndrome diagnosed at Yongdong Severance Hospital and Severance Hospital from 1986 to 1995. We analysed the clinical features, the radiological findings, the treatment modalities and the clinical courses. Results: 1) The incidence of the lung involvement in Behçet's syndrome was 2%(10/487). The male to female ratio was 8 : 2 and the mean age was 34 years. The presenting symptom was hemoptysis in 5 of 10 cases, and massive hemoptysis was noted in 2 cases. Other pulmonary symptoms were cough(6/10), dyspnea(4/10), and chest pain(2/10). Other manifestations were oral ulcers(10/10), genital ulcers(9/10), skin lesions(7/10), and eye lesions(6/10). 2) The laboratory findings were nonspecific. The posteroanterior views of chest radiographies showed multiple infiltrates(6/10), nodular or mass-like opacities(4/10), or normal findings(2/10). The chest CT scans showed multifocal consolidations(6/8), and aneurysms of the pulmonary aneries(4/8). The pulmonary angiographies were performed in 3 cases, and showed pulmonary artery aneurysms in 2 cases. The ventilation-perfusion scans in 2 cases of normal chest x-ray showed multiple mismatched findings. 3) The patients were treated with combination therapy consisting of corticosteroids, cyclophosphamide, and colchicine or anticoagulant agents. Surgical resection was performed in one case with a huge aneurysm. 4) We have followed up nine of ten cases. Three cases are well-being with medical therapy, two cases are severely disabled now and four cases died due to massive hemoptysis, massive pulmonary embolism, or sepsis. Conclusion : Pulmonary vasculitis is a main feature of the lung involvement of Behçet's syndrome, causing hemorrhage, aneurysmal formation, and/or thromboemboism. The lung involvement of Behçet's syndrome is uncommon but is one of the most serious prognostic factors of the disease. Therefore, an aggressive diagnostic work-up for early detection and proper treatment are recommended to improve the clinical course and the survival.

• Journal of Chest Surgery
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• v.38 no.7 s.252
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• pp.483-488
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• 2005

Aortic valve-sparing procedures could reduce valve-related morbidity, but may increase operative risks; therefore, these procedures could not be performed routinely. We attempted to find out the early results while focusing on the operative risks associated with these procedures in our hospital. Material and Method: From May 1996 to July 2003, 26 patients underwent these procedures including 15 patients with Marfan syndrome and 1 patient with Behcet disease. There were 17 men and 9 women with mean age of $37.9\pm19.2$ years (range: 6 months-74 years). Ten patients had ascending aortic dissection, 18 patients had more than moderate degree of aortic valve insufficiency (AI). Two types of valve-sparing procedures were performed: valve reimplantation in 14 and root remodeling in 12 patients. Associated procedures were performed as follows: aortic valve plasty in 6, mitral valve plasty in 5, hemi-arch replacement in 4, total arch replacement in 2, coronary artery bypass surgery in 1 and Maze procedure in 1 patient(s). Result: In four patients, valve-sparing procedures were converted to Bentall procedures during operation. Including these patients, there was no operative deaths, 3 patients underwent re-operation due to bleeding, 1 patient had permanent pacemaker. The median duration of ICU stay was 45.5 hours, the median duration of hospital stay was 10.5 days. In 22 patients excluding 4 converted patients, intraoperative transesophageal echocardiogram (TEE) showed less than mild degree of AI in all except one who had not received intra-operative TEE in the beginning and showed moderate degree of AI at discharge. The mean duration of follow-up was $21.2\pm27.4$ months. All patients were alive except one who died during other departmental surgery. In 3 patients, more than moderate degree of AI was recurred, but there were no reoperation. Conclusion: Aortic valve-sparing procedures could be performed relatively safely in selected patients who had annuloaortic ectasia.