• Journal of the Korean Chemical Society
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• v.50 no.2
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• pp.123-128
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• 2006

Behcet's disease (BD) is a systemic vasculitis characterized by recurrent oral and genital ulcers, and ocular inflammation, and which may involve the joints, skin, central nervous system and gastrointestinal tract. 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 IL-6 gene polymorphisms are associated with susceptibility to Behcet's disease in Koreans. Gene polymorphisms were typed by VNTR (variable number of tandem repeat), RFLP (restriction fragment length polymorphism), DHPLC (denaturing high performance liquid chromatography).There were no evidences for genetic association conferred by the IL-6prom polymorphism. However, significant differences in the IL-6vntr genotype and allele frequencies were found between patients with BD and controls. The IL-6vntr*C allele appeared to be an additional susceptibility gene to Korean BD. Further studies in other populations and gene are required to confirm these results.

• Journal of Chest Surgery
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• v.25 no.4
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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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• v.32 no.7
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• pp.660-664
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• 1999

Behcet's disease is classically described as featuring recurrent aphthous ulcers in the mouth and genital organs and as having relapsing iritis. Now it is being recognized as a mul tisystem disorder that involves of the skin, gastrointestinal system, cardiovascular system, lung and the central nervous system as well as the joints, blood vessels and urologic systems. Large vessel diseases are unusual but aneurysm may occur in which the pulmonary circulation may give rise to the massive and often fatal hemoptysis. A 29 year-old man who complained of having dyspnea and hemoptysis during six months visited our hospital. He received right bilobectomy for a mass located in the right lower lobe. He underwent right bilobectomy. The final pathologic diagnosis was a pulmonary artery aneurysm which origina ted from the pulmonary artery.

• Journal of Chest Surgery
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• v.10 no.1
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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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• v.52 no.5
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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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• v.67 no.5
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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.

• Proceedings of the Zoological Society Korea Conference
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• 2003.08a
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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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• v.33 no.5
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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.

• Korean Journal of Clinical Laboratory Science
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• v.38 no.3
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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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• v.35 no.4
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• pp.274-282
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• 2002

Background: Paravalvular leakage or false aneurysm developed after isolated aortic valve replacement(AVR) for aortic regurgitation(AR) associated with Behcet's disease is one of the most serious complications, and requires subsequent reoperations. We describe the surgical result of homograft aortic root replacement(ARR) for AR associated with Behcet's disease. Material and Method: From January 1992 to December 2001, 6 patients with AR associated with Behcet's disease underwent 7 ARR with homograft and 1 Ross operation. Five patients were male and one was female. The grafts used for ARR were 5 aortic and 2 pulmonic homografts. Ages at operation ranged from 27 to 51 years(mean, 37$\pm$9 years). Two patients underwent ARR with aortic homograft at the first operation. In the remaining 4 patients, ARR using a homograft was performed for paravalvular leakage that developed after AVR, and the mean interval from AVR to ARR was 21 $\pm$29 months(range, 5 to 73.3 moths, median, 7.6 months). Result: There was no early death. All patients were followed up for an average of 18.9$\pm$24.0 months(range, 1.9 to 68.9 months, median, 8.4 months). Two of 4patients who had undergone ARR after AVR required subsequent reoperations for false aneurysm of the ascending aorta and failure of pulmonary homograft. One patient underwent re-replacement of the aortic root, ascending aorta and partial aortic arch with an aortic homograft, the other underwent Ross operation. Conclusion: This study suggests that aortic root replacement using a homograft in aortic regurgitation with Behcet's disease may provide good clinical results and decrease the incidence of paravalvular leakage or false aneurysm after aortic valve replacement. However, the adequate perioperative management and complete removal of the inflarrunatory tissue at operation were also important for the good long-term results.