• Title/Summary/Keyword: Behcet Disease

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Homograft Aortic Root Replacement (동종이식편을 이용한 대동맥 근부 치환술)

  • Kim Jae Hyun;Oh Sam Sae;Lee Chang-Ha;Baek Man Jong;Kim Chong Whan;Na Chan-Young
    • Journal of Chest Surgery
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    • v.38 no.3 s.248
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    • pp.197-203
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    • 2005
  • Homograft aortic valve replacement (AVR) has many advantages such as excellent hemodynamic performance, faster left ventricular hypertrophy regression, resistance to infection and excellent freedom of thromboembolism. To find out the results of homograft AVR, we reviewed our surgical experiences. Material and Method: Eighteen patients (male female=16 : 2, mean age=39.3$\pm$16.2 years, range: 14$\~$68 years) who underwent homo-graft aortic valve replacement between May 1995 and May 2004 were reviewed. The number of homografts was 20 (17 aortic and 3 pulmonic homografts) including two re-operations. Ten patients had a history of previous aortic valve surgery. Indications for the use of a homograft were native valve endocarditis (n=7), prosthetic valve endocarditis (n=5), or Behcet's disease (n=8). The homograft had been implanted predominantly as a full root except in one patient in the subcoronary position. Result: Mean follow-up was 41.3 $\pm$ 26.2 months. There was one operative mortality. Postoperative complications included postoperative bleeding in 3 patients, and wound infection in 1. There was no late death. Three patients underwent redo-AVR. The etiology of the three reoperated patients was Behcet's disease (p=0.025). Freedom from reoperation was $87.5\pm8.3\%$, $78.8\pm11.2\%$ at 1, 5 years respectively, In patients with infective endocarditis, there was no recurrence of endocarditis. There was no thromboembolic complication. Conclusion: Although longer term follow-up with larger numbers of patients is necessary, the operative and mid-term results for homograft AVR was good when we took into account the operative risks of Behcet's disease or infective endocarditis. Behest's disease was a risk factor for reoperation after the homograft AVR. We think homograft AVR is the procedure of choice, particularly in patients with infective endocarditis.

A Case of Neuro-Behest's Disease: Comparison of Neurological Symptoms with PET, SPECT, and MRI Findings (신경 베체트병 1례: 신경학적 증상과 뇌 PET, SPECT, MRI 소견의 비교)

  • Kim, Jin-Wook;An, Min;Kim, So-Yon;Kim, Young-Jung;Cho, Min-Koo;Lee, Gwon-Jun;Lim, Sang-Moo;Hong, Sung-Woon;Choi, Chang-Woon
    • The Korean Journal of Nuclear Medicine
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    • v.32 no.6
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    • pp.534-541
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    • 1998
  • We describe a 27-year-old man who developed gait disturbance and dysarthria 2 years after the onset of cardinal symptoms of Behcet's disease. Positron emission tomography with $^{18}F$-fluorodeoxyglucose revealed severe hypometabolisrn in the cerebellum, in accordance with cerebellar symptoms and signs of the patient. However, single-photon emission tomography with $^{99m}Tc$-HMPAO and $^{99m}Tc$-ECD did not disclose significant perfusion abnormalities in the brain. Routine brain magnetic resonance imaging did not show signal abnormalities. The findings of imaging studies compared with neurological manifestations of the patient are discussed.

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Budd-Chiari Syndrome Complicating Behcet's Disease -Report of one case- (Behcet's 병을 동반한 Budd-Chiari 증후군 -1례 보고-)

  • O, Bong-Seok;Kim, Bo-Yeong;Kim, In-Gwang
    • Journal of Chest Surgery
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    • v.29 no.2
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    • pp.219-222
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    • 1996
  • A 34-year-old man was admitted to the hospital because of ascites, abdominal fullness. computed tomography and cavography revealed inferior vena cavil occlusion just above the hepatic vein and diagnosed as Budd-Chiari syndrome. conservative medical therapy failed to control the symptoms produced from both portal hypertension and versa caval stasis. Therefore, under extracorporeal circulation with moderate hypothermia and normal cardiac contraction, membranoto y and inferior vena casa venoplasty with Gore-tex (10mm) was performed. Postoperatively, physical examination revealed oral ulceration, subcutaneous thrombophlebitis, folliculitic lesions. uveitis And increased reactivity of the skin to needle punctures. 10 month later, superior vena ciiva obstruction symptom was found. Hehcet's disease was diagnosed.

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Review of rheumatic diseases in terms of insurance medicine (주요 류머티스 질환의 보험의학적 이해)

  • Lee, Sin-Hyung
    • The Journal of the Korean life insurance medical association
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    • v.31 no.1
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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.

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Oral ulcerative and vesicular diseases (구강궤양 및 수포성 질환)

  • Byun, Jin-Seok
    • The Journal of the Korean dental association
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    • v.55 no.9
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    • pp.634-639
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    • 2017
  • Oral ulcerative and/or vesicular diseases have similar characteristics of clinical phenotypes. Detailed and careful history taking is the cornerstone of the diagnosis of oral mucosal disease. Moreover, complete screening of review of system for patient is important. Through this article, the simple ulcerative, recurrent ulcerative, acute multiple ulcer, chronic multiple ulcerative diseases will be discussed. Clinicians have to keep in mind its differential diagnosis and management.

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EARLY DETECTION OF BEHCET'S DISEASE (베체트병의 조기 진단)

  • Kim, Seung-Hye;Song, Je-Seon;Choi, Hyung-Jun;Son, Heung-Kyu;Lee, Jae-Ho
    • Journal of the korean academy of Pediatric Dentistry
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    • v.36 no.4
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    • pp.575-579
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    • 2009
  • Behcet's disease(BD) is a multisystem vasculitis that almost any organ in the body may be involved. Its diagnosis bases on clinical criteria in the absence of any specific laboratory test to confirm it. The clinical criteria proposed for the diagnosis for BD include recurrent oral and genital ulceration, skin lesion, ocular changes, and positive response to pathergy test. Besides these essential features, BD may manifest itself on the gastrointestinal tract, central nervous system, and other systemic involvements. Among these clinical features, recurrent oral ulcers are present in most BD patients, often as the initial symptom, preceding other manifestations by many years. Therefore, dentists are in the important position for the early detection and diagnosis of BD. In this case report, we are presenting a case of 6 year-old girl, who came to the pediatric dentistry department for evaluation and treatment of pain and dysphagia caused by severe recurrent oral ulcers. Through taking detailed dental and medical history and clinical examination, we reported the possibility of BD to the medical team and prescribed topical lidocaine to relief pain during swallowing. The medical team, which involved pediatrics, ophthalmology, dermatology, and gastroenterology, finally confirmed BD through various examinations. Detection of the possibility of BD in the dental field helped early diagnosis and proper management by the medical team.

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A Case of Behçet's Disease with Superior Vena Cava Syndrome (상대정맥증후군을 유발한 Behçet병 1예)

  • Kim, Young-Jee;Kim, Sang-Hun;Lee, Sang Moo;Ahn, Youngsoo
    • Tuberculosis and Respiratory Diseases
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    • v.56 no.6
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    • pp.657-663
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    • 2004
  • As a chronic multisystemic inflammatory disorder, Behçet's disease may manifest vascular, cardiac, neurological and gastrointestinal abnormalities. However, involvement of large veins, such as thrombosis of the superior or inferior vena cava, is a very rare complication. Herein, a case of superior vena cava syndrome, due to the thrombotic obstructions of the subclavian and brachiocephalic vein, is reported in a 27-year old woman with chronic Behçet's disease.

Neuro-Behçet disease presented diplopia with hemiparesis following minor head trauma

  • Choi, Ja-Yun;Park, Sun-Young;Hwang, In-Ok;Lee, Young-Hwan
    • Clinical and Experimental Pediatrics
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    • v.55 no.9
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    • pp.354-357
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    • 2012
  • Behçet disease (BD) is rare in childhood. We report a 9-year-old boy with neuro-Behçet disease who presented diplopia and weakness on the left side after a cerebral concussion. Brain magnetic resonance imaging (MRI) revealed hyperintensity of the right mesodiencephalic junction on T2-weighted and fluid attenuated inversion recovery images. Prednisolone administration resulted in complete remission and normalization of abnormal MRI finding. Brain MRI is a useful diagnostic tool when the neurological sign is the first symptom of subclinical BD.

Hughes-Stovin Syndrome as an Outcome of Behçet Disease or as a Different Entity

  • Demirkan, Serkan;Gultekin, Yildirim
    • Journal of Chest Surgery
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    • v.51 no.1
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    • pp.64-68
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    • 2018
  • Hughes-Stovin syndrome is a rare disorder of unknown etiology. Although the association between multiple pulmonary artery aneurysms and venous thrombosis of the lower limbs was reported by Beattie and Hall in 1911, it was not until 1962 that the eponym "Hughes-Stovin syndrome" was formally introduced in the medical literature. We describe 2 patients with Hughes-Stovin syndrome who presented with pulmonary artery aneurysm, thrombophlebitis, hemoptysis, and oral ulcers, review the manifestations of the disease, and compare its similarities with and differences from Behçet disease.

Molecular Genetic Analysis of Behcet's Disease in Korean (한국인 베체트 환자의 분자유전학적 연구)

  • Park, Sang-Bum;Nam, Youn-Hyoung;Park, Su-Min;Lee, Sang-Hyun;Ahn, Young-Chang;Cho, Min-Ho;Kim, Jong-Gyu;Choi, Jae-Gu;Kim, Seong-Kyu;Jang, Won-Cheoul
    • Journal of the Korean Chemical Society
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    • v.51 no.6
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    • pp.536-542
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    • 2007
  • Behcet's disease (BD) is a chronic inflammatory disorder, involving several organs. Inflammation in the disease is thought to be mediated by cytokines derived from T-helper type 1 (Th1) lymphocytes. Although the exact pathogenesis for BD is not completely understood, it has been suggested that the disease is triggered in genetically susceptible individuals by environmental factors, such as microbial agents. It is noted that multiple genes, including MHC (major histocompatibility complex) and non-MHC genes, are implicated in the pathogenesis of BD. This study tries to determine whether HLA-B51, IL-18, SLC11A1 and TNF-α polymorphisms are associated with susceptibility to Behcet's disease in Koreans. As a results, HLA-B51 was a genetic factor with the strongest association with BD. But it is still uncertain whether this HLA molecule is directly involved in the pathogenesis of BD. Although the IL-18 gene polymorphisms were not associated with a susceptibility to BD in the Korean population, the patients carrying the GG genotype at position 137 had a higher risk of developing the ocular lesions. This study suggests that the allele 3 and the genotype allele 3 / allele 3 of 5'-promoter (GT)n polymorphism in the SLC11A1 gene may have a protective effect for the development of BD in the Korean population. There were no evidences for genetic association conferred by the TNF-α gene with respect to susceptibility to BD.