• Title/Summary/Keyword: Behcet s disease

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Analysis of Interleukin-6 Gene Polymorphisms in Behcet’s Disease Using RFLP and DHPLC (RFLP와 DHPLC를 이용한 베체트병 환자에서 Interleukin-6 유전자 다형성의 분석)

  • Jang, Won-Cheol;Park, Sang-Beom;Nam, Yun-Hyeong;Lee, Jae-Sik
    • 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.

A Neuro-Behçet's Case Operated with the Intracranial Mass Misdiagnosis

  • Tokgoz, Osman Serhat;Akpinar, Zehra;Guney, Figen;Seyithanoglu, Abdullah
    • 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.

A Case of Miliary Tuberculosis in a Patient with Behcet's Disease and Uveitis Receiving Infliximab (베체트병 포도막염 환자에서 Infliximab 사용 중 발생한 속립성 결핵 1예)

  • Yoo, Jung-Wan;Roh, Jae Hyung;Park, Jin Wook;Kim, Yong Giun;Jang, Ji Woong;Na, Soo Young;Shim, Tae Sun
    • 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.

The Prevalence of Human Leucocyte Antigen(HLA)-B51 in Patients with Behcet's Disease (베체트 질환자로부터 Human Leucocyte Antigen(HLA)-B51 발생빈도)

  • Cho, Seang-Sig
    • 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).

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Homograft Aortic Root Replacement for Aortic Regurgitation with Behcet's Disease (Behcet씨 병과 동반된 대동맥판막 폐쇄부전에서 동종이식편을 이용한 대동맥근부 치환술)

  • Baek, Man-Jong;Na, Chan-Young;Kim, Woong-Han;Oh, Sam-Se;Kim, Soo-Cheol;Lim, Cheong;Ryu, Jae-Wook;Kong, Joon-Hyuk;Lee, Young-Tak;Moon, Hyun-Soo;Park, Young-Kwan;Kim, Chong-Whan
    • 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.

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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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.

Surgical Management of Aortic Insufficiency in Behcet's Disease (베체트씨병에 의한 대동맥판 폐쇄부전의 수술적 치료)

  • Kim, Kyung-Hwan;Kim, Ki-Bong;Kim, Won-Gon;Kim, Joo-Hyun;Ahn, Hyuk
    • 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.

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