• Childhood Kidney Diseases
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• 제2권1호
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• pp.73-76
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• 1998

Behcet syndrome is a multisystem disease complex of unknown cause. It is usually manifested by aphthous oral and genital ulcers, uveitis and skin eruption. Less frequently, CNS involvement, colitis, large vessel vasculitis, and myocarditis occur. Recently, several studies have reported renal manifestations of amyloidosis and focal necrotizing glomerulonephritis in Behcet syndrome. We describe a patient with Behcet syndrome who experienced nephrotic syndrome with focal effacement of epithelial foot process in glomeruli. A brief review of literature ensues.

• Journal of Chest Surgery
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• 제21권5호
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• pp.899-904
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• 1988

In Behcet syndrome, cardiac involvements are rare and have been reported pericarditis, myocarditis, right heart endocardial fibrosis, right ventricle mural thrombus with pulmonary embolism, active endocarditis, granulomatous endocarditis, conduction disturbance, acute aortic insufficiency, mitral valve prolapse. Our three patients underwent AVR because of aortic insufficiency and ascending aorta enlargement combined with Behcet syndrome. Two patients had mitral regurgitation too. So one underwent MAP and the other underwent MVR concomitantly. One who underwent AVR have been well for 50 months. Another who underwent AVR+MAP and redo AVR due to aortic paravalvular leakage was died of congestive heart failure. The other who underwent AVR+MVR and repeated AVR three times because of aortic paravalvular leakage is in condition of aortic paravalvular leakage. Paravalvular leakage is considered to recur due to progressive dilatation and fragility of aortic root that is the result of pathologic change of Behcet syndrome in it. If Open heart surgery is needed in Behcet`s syndrome during inflammatory reaction is active, postoperative complications such as paravalvular leakage or suture line rupture may be prevented with pre- and postoperative anti-inflammatory management.

• 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 Yeungnam Medical Science
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• 제29권1호
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• pp.38-41
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• 2012

Behcet's disease is a rare multisystemic disorder whose main pathological defectis vasculitis, and superior vena cava (SVC) syndrome without thrombosis is a very rare manifestation of the disease. These authors encountered a case of SVC syndrome without thrombosis caused by Behcet's disease. A 33-year-old man visited the hospital for aggravated dyspnea without any related medical and familial history. He had a threeday history of abrupt swelling of the face, neck, and right arm. He suffered from recurrent oral ulcer, and there were acneiform nodules on his face as well as redness and swelling at the site of the intravenous injection. On the multi-detected computed tomography (CT) chest angiograms (chest angio MDCT), the SVC narrowed without thrombosis. Venogram was carried out, and percutaneous transluminal balloon angioplasty of the SVC stenotic site was performed. The following day, the swelling was found to have subsided. The details of the case are reported herein.

• Journal of Chest Surgery
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• 제47권2호
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• pp.178-180
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• 2014

Pulmonary and/or bronchial aneurysm with systemic thrombophlebitis can be seen in the case of both Behcet's disease and Hughes-Stovin syndrome. These two diseases may be indistinguishable since the clinical, radiological, and histopathological findings are similar in both cases. In particular, from the perspective of pulmonary involvement, hemoptysis is the leading cause of death in both conditions and is followed by aneurysmal rupture. Here, we report a case in which surgical resection was performed for a patient presenting recurrent hemoptysis as an atypical presentation of Behcet's disease.

• Journal of Chest Surgery
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• 제48권5호
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• pp.364-367
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• 2015

Cardiovascular involvement in cases of Behcet's disease is a rare but life-threatening condition, and prosthetic valve detachment is a frequent and serious complication attributable to Behcet's disease following the surgical repair of aortic regurgitation. We report the case of a patient with Behcet's disease presenting with contained aortic rupture around the aortic root. The patient had previously undergone aortic valve surgery three times due to recurrent prosthetic valve detachment. An emergency operation was performed, consisting of aortic root replacement (ARR) using a composite valved conduit and the replacement of the hemiarch. ARR may be an appropriate surgical option for patients with Behcet's disease in order to prevent recurrence of the disease.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제33권1호
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• pp.49-54
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• 2011

A case of Behcet's syndrome found in a 22 years old male was is reported along with the histological and immunohistochemical studiesfindings. He The patient had had received prolonged medical therapy of anti-inflammatory agents for the conjunctival ulcer, genital ulcer, oral ulcer, and intestinal ulcers since the first onset of the Behcet's syndrome about approximately 7 years agoearlier. Recently, he felt the athickening of both corners of his lip causing that caused mouth opening difficultydifficulties. A plastic rReconstructive surgery was performed to enlarge the size of the oral orifice by multiple Z-plasty incisions, and finallywhich resulted in proper enlargement of the circumferential length of the lip. During the operation, a scar-like thick fibrous tissue was obtained and examined pathologically. In theThe microscopic observation the revealed the submucosal lesion was to be diffusely fibrosed with the a distribution of sclerotic collagen bundles. Particularly, sSeveral foci of collagen degeneration were found observed in the deep connective tissue, and the degenerating collagen bundles were gradually lost their fibrillar appearance. In the immunohistochemical observations, the foci of collagen degeneration was were strongly positive for IgK, but almost negative for TNF${\alpha}$ lysozyme, and MMP-3. Taken together, it was presumed that tThe submucosal fibrosis was presumed to have firstly presented in this study was probablybeeninduced by the prolonged anti-inflammatory therapy, which may inhibit the removal of sclerosed collagen bundles by the cell-mediated immunity and proteolytic digestion of macrophages, and that it was secondarily aggravated by the deposition of immunoglobulins derived from an autoimmune origin. Therefore, even after the successful plastic surgery of the lip to ameliorate the mouth opening difficulty, the recurrent submucosal fibrosis of lip should be carefully managed in the follow-up treatment.

• 대한치과의사협회지
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• 제35권10호통권341호
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• pp.724-726
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• 1997

• 보험의학회지
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• 제31권1호
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• pp.19-28
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• 2012

Nowadays, Rheumatic diseases are increasing more and more. So, it's important knowing the pathophysiology and extra-risk of each rheumatic disease so as to do sound underwriting. Here is brief review and long-term prognosis of some rheumatic diseases such as rheumatoid arthritis, systemic lupus erythematosus, Sj$\ddot{o}$gren syndrome, antiphospholipid syndrome, systemic sclerosis, ankylosing spondylitis, Takayasu's arteritis, and Behcet syndrome.

• Journal of Chest Surgery
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• 제29권2호
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• pp.219-222
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• 1996

34세 남자가 복수 및 복부 팽 만감을 주소로 입원하였다. 단층촬영 및 하대정맥 조영술상 간정맥 직상부의 하대 정맥폐색이 의심되는 Budd-Chiari 증후군으로 진단되었다. 대증요법으로 간문맥고혈압과 하대정맥폐색증상이 호전이 안되어, 체외순환, 중등도저체온 및 정 상심박동하에 폐색부위절제술 및 하대정맥성형술을 10mm인조혈관을 사용하여 시행하였다. 술후 환자는 구강및 피부와 이학적 검사상 구강궤양, 피하 혈전성 정맥염, 모낭염성 병변, 피부의 자극성 항진 및 포도막염이 관찰되었고 상대정맥 폐색증상이 병발하였으며 Behcet씨 병으로 진단되었다.