• 대한화학회지
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• 제50권2호
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• pp.123-128
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• 2006

혈관염이 특징적인 질환으로 구강궤양, 음부궤양, 안구염증과 관절, 피부, 중추신경계, 위장관 등 여러 장기를 침범할 수 있는 만성 염증성 질환이다. 베체트병의 발병원인이나 기전에 대해 확실히 밝혀지지는 않았으나 유전적인 소인이 있는 사람에서 감염 등 환경적인 요인이 면역 반응에 이상을 일으켜 질병의 여러 증상이 발현된다. 주조직복합체 (major histocompatibility complex, MHC)와 non-MHC gene등 다양한 유전자들이 베체트병의 병인으로 관여한다. 이 연구에서는 IL-6의 유전적 다형성이 한국인 베체트병의 감수성에 관여하는지를 확인하였다. 유전자 다형성은 VNTR (variable number of tandem repeat), RFLP (restriction fragment length polymorphism), DHPLC (denaturing high performance liquid chromatography) 방법을 이용하여 확인하였다. 실험 결과, 베체트 환자와 대조군에서 IL-6prom의 유전적 다형성은 관련성이 없었지만 IL-6vntr에서는 유전형과 대립형질의 빈도가 다르게 나타났다. IL-6vntr*C 대립유전자가 한국인 베체트병과 연관성이 높게 나타났다. 이런 결과를 확인하기 위해 앞으로 여러 집단과 다른 유전자의 연구가 필요하다.

• Journal of Chest Surgery
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• 제25권4호
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• pp.412-417
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• 1992

Pulmonary artery aneurysm is Behcet`s disease is rare and can be fatal due to rupture. We experienced a case of pulmonary artery aneurysm in Behcet`s disease. The patient was 21 year old woman who was adimitted with three month history of dyspnea, fever and cough. On examination, she had aphthous ulcer in the mouth and erythema nodosum on the left popletial fossa and forearm, but didn`t have any lesion at eyes and genitalia. The latex fixation test for rheumatoid factor, VDRL test for syphillis, antinuclear antibody and LE cell test were all negatives. The third and fourth components of complement in the serum, serum immunoglobulin concentrations[IgG, IgM, IgA] were within normal range. The chest radiography revealed a 5x6cm sized radiopaque mass density in the left hilar region. Two months later, the mass was enlarged to 6x7cm. The IV-DSA showed a single aneurysm at the proximal part of left lower lobe artery with lingular segment artery and no distal perfusion by thombotic obstruction. The steroid therapy was done for a month, but symptoms not improved. We performed resection of lingular segment and lower lobe including the aneurysm. The microscopic findings of the operative specimen were intimal hyperplasia and fragmentation of the internal elastic fibers. She was improved without remarkable event, except infection of the operative wound.

• Journal of Chest Surgery
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• 제32권7호
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• pp.660-664
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• 1999

현재 베체트병은 여러 기관을 침범하는 질환으로 인식되고 있으며 구강 및 음부 궤양, 안병변과 피부병변 을 주증상으로 하고 심혈관계, 호흡기계, 소화기계, 중 颯키麗\ulcorner 비뇨기계 등을 침범하여 다양한 증상을 나타 내고 있다. 베체트병에서의 폐동맥의 침범은 드물지만 다량의 객혈이 발생될 수 있으며 동맥류의 파열에 의 한 사망의 위험성이 높다. 29세의 남자 환자가 6개월간 지속된 객혈과 동반된 호흡곤란을 주소로 내원하였 다. 우측폐하엽에 종괴가 발견되어 우측 중엽 및 하엽 절제술을 시행 받았다. 수술소견상 우측 폐하엽에 4$\times$ 5$\times$4 cm의 박동 원형종괴가 있었으며 우중엽 및 우하엽의 폐동맥이 종괴와 연결이 되어 있었다. 병리소견과 병력상 베체트병에 의한 폐동맥류를 경험하였기에 문헌 고찰과 함께 보고하고자 한다.

• Journal of Chest Surgery
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• 제10권1호
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• pp.98-105
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• 1977

A 36 year old blindman, engineer was admitted with chief complaints of hemoptysis, recurrent sore throat, pyoderma in genital organ, uveitis and thrombophlebitis for 10 years. Above the chief complaints were remission or exacerbation during hospitalization. Physicalexamination showed that left radial, ulnar & brachial pulse was not palpable. No bruit or murmur was obtained over the mass. Neurologic examination revealed no significant finding.On admission, chest P-A showed hen egg sized round & oval compact hazy density on left upper lung field. Bronchogram revealed no pathological finding and Lt. tomogram showed well define large,ovoid mass density in the superior mediastinum. Fluoroscopy finding showed nonpulsatile on left upper lung field. Pre-op. aortography was not taken, under the impression of lung Ca. rule out .sortie aneurysm, exploratory operation was performed through the 2nd intercostal space, Lt. It was performed that the mass was ascending sortie aneurysm of saccular type. Direct aneurysmectomy with multiple figure of eight suture were done without any prosthetic graft. Post-op. control I.V.C graphy showed completely obstruction sign. Postopcontrol aortography revealed good surgical result. Final, histopathological answered non-specific sortie aneurysm, saccular type. Post-op. courses were uneventful except mild neurologic disturbance with subclavian steal syndrome and associated with both lower leg pitting edema due to inferior vena cava obstruction. After op, 3 month later, discharged to home, with big systemic problem. Behcet`s syndrome reviewed with related literatures. The coexistence of mouth and genital ulceration with hypopyon mentioned by hippocrates and described by various workers in the early part of this century was first defined as a syndrome by Behcet in 1937. In 1937 Behcet described a chronic relapsing triple symptom complex of oral ulceration, genital ulceration, and ocular inflammation. The place of the syndrome as part of a systemic disorder in now clearer, and the under lying pathology appears to be a vasculitis. The disease runs a- chronic course, blindness being the greatest disability and control nervous system involvement a cause of death. Thrombophlebitis is fairly frequent, france et al [1951] giving an incidence of 25% and Dowling [1961] 12%, superficial thrombophlebitis migrans and thrombosis of large veins, including venae cavae [Thomas, 1947: Boolukos 1960] are recorded. Little attention has been paid to arterial involvement. Mishima et al. [1961] described resection cf an aortic aneurysm in a 38 year old man with Behcet`s syndorme. Mounsey in a clinicopathological conference described a case [Brit, med. J., 1966] of ruptured aortic aneurysm in Bechcet`s syndrome treated by aorto-iliac graft. Also, Shikano and Oshima et al [1963] recorded two aneyrysm of smaller arteries. Unfrequently, aortic aneurysm was presumed to be secondary to osteomyelitis of the lumber spine, though the possible association between aortic aneurysm and Behcet`s syndrome was raised. A further case is reported here, in which ascending aortic aneurysm with Behcet`s Ds. appeared to form part of this generalized disease. This is a case report of surgical experience of Behcet`s Ds. with ascending aortic aneurysm which had nearly all the typical clinical features. Above mentioned and was reviewed with related literatures.

• Journal of Korean Neurosurgical Society
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• 제52권5호
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• pp.488-490
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• 2012

Behçet's disease (BD) is an inflammatory systemic disorder with oral and genital ulcers, as well as ophthalmologic and cutaneous symptoms. Neurological manifestations in BD represent between 2.2% to 50% of the cases. The 25-year-old male patient, diagnosed with BD three years earlier, was admitted to our clinic with complaints of recurrent headaches. Tumor-like-parenchimal involvement was detected on a cranial magnetic resonance imaging. The lesion was removed surgically and then he suffered from right hemiparesis and epilepsy. Pathological examination of the lesion noted a demyelinating non-tumoural etiology. A neuro-Beh$\check{g}$et's case with parenchymal involvement has been examined in light of the literature, in terms of a tumor and a demyelinating disease differential diagnosis.

• Tuberculosis and Respiratory Diseases
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• 제67권5호
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• pp.454-457
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• 2009

Infliximab, a TNF-${\alpha}$ antagonist, has been used to treat refractory rheumatoid arthritis, ankylosing spondylitis, Crohn's disease and Behcet's disease. Tuberculosis (TB) is a well-known opportunistic infection in patients receiving infliximab. Therefore, patients should be screened and treated for latent or active TB infection before being administered infliximab. Recently, we encountered a case of military TB during infliximab therapy in a patient suffering from Behcet's disease and uveitis. We report this case with a review of the relevant literature.

• 한국동물학회:학술대회논문집
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• 한국동물학회 2003년도 한국생물과학협회 학술발표대회
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• pp.206.3-206
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• 2003

No Abstract, See Full Text

• 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.

• 대한임상검사과학회지
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• 제38권3호
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• pp.189-195
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• 2006

Behcet's disease (BD) is a chronic, multisystemic disorder which is more frequently seen in the Mediterranean basin, Middle East, and Far East. The causes and pathogenesis of BD are unknown although many possibilities are being investigated. The diagnosis of BD is based on clinical manifestations because there are no pathognomonic laboratory tests. So, the purpose of this study was to examine the prevalence of HLA-B51 in patients with BD. We used the whole blood of 33 patients diagnosed with BD at Chosun University Hospital from August 2003 to January 2006. For the HLA-B51 test, we extracted the DNA from the whole blood of 33 BD patients, and we investigated it through the nested PCR method. Data were analyzed using the SPSS/PC 10.0. The frequencies of gender of the 33 cases diagnosed as BD were male 13 (39.4%) and female 20 (60.6%). The frequencies of age group of the 33 cases diagnosed as BD were 20 yrs 8 (24.2%), 30 yrs 12 (36.4%), 40 yrs 8 (24.2%), 50 yrs 1 (3.0%), and 60 yrs and 70 yrs 2 (6.1%), respectively. The frequencies of HLA-B51 of the 33 cases diagnosed as BD were HLA-B51-negative 18 (54.5%) and HLA-B51-positive 15 (45.5%). In conclusion, BD occurred more often in women than men (1: 1.53), and the mean age of the BD patients was 39.8 years old. HLA-B51 was positive in 45.5% of patients with BD, and was statistically significant in age (p<0.05).

• Journal of Chest Surgery
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• 제35권4호
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• pp.274-282
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• 2002

배경: Behcet씨 병과 동반된 대동맥판막 폐쇄부전에서 인공 대동맥판막 치환술 후 발생하는 인공판륜 주위 누출이나 가성동맥류는 가장 심각한 합병증의 하나로써, 재수술을 필요로 한다. 저자들은 Behcet씨 병과 동반된 대동맥판막 폐쇄부전 환자에서 동종이식편을 이용한 대동맥근부 치환술 결과를 알아보고자 하였다. 방법 및 대상: 1992년 1월부터 2001년 12월까지 저자들은 6명의 Behcet씨 병 환자에서 동종이식편을 이용한 근부치환술 7례와 1례의 Ross 술식을 시행하였다. 남자 5명, 여자가 1명이었으며 수술 당시 평균 연령은 37$\pm$9세(27~51세)였다. 2명은 1차 수술에서 동종이식편을 이용한 근부치환술을 시행하였고 다른 4명은 대동맥판막 치환술 후 발생한 인공판륜 주위 누출로 동종이식편을 이용하여 근부치환술을 시행하였다. 인공판막치환술 후 재수술까지 평균 기간은 21 $\pm$29개월(5 ~73.3개월, 중앙값, 7.6 개월)이였다. 결과: 동종이식편을 이용한 근부치환술 후 조기 사망은 없었다. 6명에서 평균 18.9$\pm$24.0개월(1.9~68.9개월, 중앙값, 8.4개월)을 추적 관찰하였다. 판막치 환술 후 동종이식편을 이용한 근부치환술 환자 4명중 2명에서 상행대동맥에 발생한 가성동맥류와 폐동맥 동종이식편의 기능 부전으로 재수술이 필요하였다. 1명은 대동맥 동종이식편을 이용하여 대동맥근부, 상행대동맥 및 부분 대동맥궁을 재치환하였으며 다른 1명은 Ross수술을 시행하였다. 결론: 본 연구 결과 Behcet씨 병과 동반된 대동맥판막 폐쇄부전에서 동종이식편이나 폐동맥 자가이식편을 이용한 대동맥근부.치환술은 양호한 조기 수술 결과를 보이며, 인공판막 치환술 후 발생할 수 있는 인공판륜 주위 누출이나 가성동맥류의 발생을 줄일 수 있다. 그렇지만 Behcet씨 병의 정확한 진단과 수술 전후 적절한 내과적인 치료 및 수술시 염증 조직의 완전한 제거가 장기 결과의 향상을 위해 매우 중요할 것으로 사료된다.