• Title/Summary/Keyword: Behcet%27s disease

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Pulmonary Involvement in Behcet's Disease -A Case Report- (폐동맥류를 동반한 Behcet's Disease)

  • 백효채
    • Journal of Chest Surgery
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    • v.27 no.5
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    • pp.402-406
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    • 1994
  • Behcet`s disease is a rare systemic disease of unknown cause and it is a progressive disorder with episodes of activity and remission. The major features are oral and genital ulceration and skin and eye lesions. Pulmonary involvement is rare, and we have experienced a 38 year old female patient who had undergone right lower lobe lobectomy due to extensive, rapidly growing pulmonary thromboemboli and pseudoaneurysm in pulmonary artery.

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A Study on the Effects of Local Factors on the Oral Ulcers Observed in Behcet s Disease (베체트 병의 구강궤양에 대한 국소인자의 영향에 관한 연구)

  • Myoung-Chan Kim;Chong-Youl Kim
    • Journal of Oral Medicine and Pain
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    • v.17 no.2
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    • pp.75-86
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    • 1992
  • Of the many first-occuring major manifestations of Behcet's disease, the oral ulcer is most frequently observed, shows the highest mean recurrence rate, and the longest mean duration period. A study of the relationship between mechanical irritation buy local factors and the recurrence of oral ulcers was carried out to see if there is a correlation. The subjects chosen for the study were 81 patients who were referred to the Department of Oral Diagnosis at Yonsei University Dental Hospital from the Behcet clinic of Severace Hospital after being diagnosed as Behcet's disease patients. 1. By Shimuzu's classification, possible type was the most numerous with 55.55% ; while suspected type was 19.76% ; incomplete type, 17,28% ; and complete type, 7.41% were observed in that order. The male to female ration was 1 to 1.61, and the average age of onset was 17.63 years. 2. Oral ulcers developed most frequently on the tongue (48.28%), and lip(23.15%), buccal mucosa (20.69%), palate\pharynx(4.43%), and gingiva(3.45%) also showed ulceration. 3. 38.27% of the patients were HSV positive : 27.16% were CRP positive ; 12.34% were ASO positive ; 9.87% were RF positive ; and 3.7% were ANA positive. 4. According to the answers to the survey, fatigue(85.18%) was most frequently associated with Behcet's diseage. Trauma by tooth brushing (22.22%), un specified reasons (20.98%), hard food chewing (12.35%), and irritation by dentition and/or prosthesis (13.58%) were observed in association with Behcet's disease. 23.46% had a history of tonsilitis. 5. Oral ulcers on the anterior part of tongue and buccal mucosa were associated with local irritatants. 6. Oral ulcers on the lip and posterior portion of tongue were not associated with local irritants. Local irritation by dentition and oral ulcer on the anterior part of tongue and buccal mucosa was observed to coexit with one another : in other words, lo9cal factors can act to cause of recurrence of an oral ulcer and to delay the healing process in Behcet's disease. Therefore, elimination of local factors along with systemic therapy must be recommended when treating oral ulcers.

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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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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 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.

Reoperations on the Aortic Root and Ascending Aorta (대동맥근부 혹은 상행대동맥의 재수술)

  • Baek, Man-Jong;Na, Chan-Young;Kim, Woong-Han;Oh, Sam-Se;Kim, Soo-Cheol;Lim, Cheong;Ryu, Jae-Wook;Kong, Joon-Hyuk;Kim, Wook-Sung;Lee, Young-Tak;Moon, Hyun-Soo;Park, Young-Kwan;Kim, Chong-Whan
    • Journal of Chest Surgery
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    • v.35 no.3
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    • pp.188-198
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    • 2002
  • Background: Reoperations on the aortic root or the ascending aorta are being performed with increasing frequency and remain a challenging problem. This study was performed to analyze the results of reoperations on the ascending aorta and aortic root. Material and Method: Between May 1995 and April 2001, 30 patients had reoperations on the ascending aorta and aortic root and were reviewed retrospectively. The mean interval between the previous repair and the actual reoperation was 56 months(range 3 to 142 months). Seven patients(23.3%) had two or more previous operations. The indications for reoperations were true aneurysm in 7 patients(23.3%), prosthetic valve endocarditis in 6(20%), false aneurysm in 5(16.7%), paravalvular leak associated with Behcet's disease in 4(13.3%), malfunction of prosthetic aortic valve in 4(13.3%), aortic dissection in 3(10%), and annuloaortic ectasia in 1(3.3%). The principal reoperations performed were aortic root replacement in 17 patients(56.7%), replacement of the ascending aorta in 8(26.7%), aortic and mitral valve replacement with reconstruction of fibrous trigone in 2(6.6%), patch aortoplasty in 2(6.6%), and aortic valve replacement after Bentall operation in 1 (3.3%). The cardiopulmonary bypass was started before sternotomy in 7 patients and the hypothermic circulatory arrest was used in 16(53.3%). The mean time of circulatory arrest, total bypass, and aortic crossclamp were 20$\pm$ 12 minutes, 228$\pm$56 minutes, and 143$\pm$62 minutes, respectively Result: There were three early deaths(10%). The postoperative complications were reoperation for bleeding in 7 patients(23.3%), cardiac complications in 5(16.7%), transient acute renal failure in 2(6.6%), transient focal seizure in 2(6.6%), and the others in 5. The mean follow-up was 22.8 $\pm$20.5 months. There were two late deaths(7.4%). The actuarial survival was 92.6$\pm$5.0% at 6 years. One patient required reoperation for complication of reoperation on the ascending aorta and aortic root(3.7%). The 1- and 6-year actuarial freedom from reoperation was 100% and 83.3$\pm$15.2%, respectively. One patient with Behcet's disease are waiting for reoperation due to false aneurysm, which developed after aortic root replacement with homograft. There were no thromboembolisms or anticoagulant related complications. Conclusions: This study suggests that reoperations on the ascending aorta and aortic root can be performed with acceptable early mortality and morbidity, and adequate surgical strategies according to the pathologi conditions are critical to the prevention of the reoperation.

Long-term Result after Repair of Sinus Valsalva Aneurysm Rupture (발살바동류 및 파열의 수술 후 장기 성적)

  • Lim, Sang-Hyun;Chang, Byung-Chul;Joo, Hyun-Chul;Kang, Meyun-Shick;Hong, You-Sun
    • Journal of Chest Surgery
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    • v.38 no.10 s.255
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    • pp.693-698
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    • 2005
  • Background: Sinus valsalva aneurysm (SVA) is a rare disease, and it is frequently accompanied by ventricular septal defect and aortic valve regurgitaion. For treatment of SVA, several surgical mordalities were applied, but there was no report on the long-term result after surgical repair in Korea. We reviewed our 28 years of experiences and analyzed the long-term results after treatment of sinus valsalva aneurysm with or without rupture. Material and Method: Between March 1974 and February 2002, 81 patients were operated under the impression of sinus valvsalva aneurysm or sinus valsalva aneurym rupture. Retrospectively we reviewed the patients' record. Mean age of patients was $29.2\pm11.5$ and there were 49 males. Accompanyng diseases were as follows: VSD in 50, PDA in 2, Behcet's disease in 2, TOF in 1, RVOTO in 1, AAE in 1. Seventy-seven $(95\%)$ patients had sinus valsalva rupture and in 14 patients, subacute bacterial endocarditis was accompanied. Degree of aortic valve regurgitation was as follows: grade I: 8, II: 10, III: 9, IV: 4. Most common rupture site was right coronary sinus (66 patients, $81\%$) and most common communication site was right ventricle (53 patients). In repair of sinus valsalva rupture, patch was used in 37 patients, and direct suture was done in 38 patients. Result: There was one surgical death $(1.2\%)$. Follow up was done in 78 patients $(97.5\%)$, mean follow up period was $123.3\pm80.9(3\~330\;months)$. During the follow up period, 3 patients died $(3.8\%)$. One patient died of heart failure, another patient died of arrhythmia and the other one died of unknown cause. In two patients, complete atrio-ventricular block was developed during follow up period, and there was no operation related event or complication. Kaplan-Meier survival analysis revealed $92.5\pm3.5\%$ survival at 15 and 27 years and it seems to be satisfactory. Conclusion: Long-term surgical results and survival is satisfactory after repair of sinus valsalva aneurysm with or without rupture.

Early Results of Aortic Valve-sparing Procedures in Patients with Annuloaortic Ectasia (대동맥륜대동맥확장(Annuloaortic Ectasia) 환자에서 대동맥판막을 보존하면서 시행된 대동맥근부 및 상행대동맥 치환술의 단기 성적)

  • Sung Kiick;Park Kay-Hyun;Lee Young Tak;Jun Tae-Gook;Yang Ji-Hyuk;Kim Su Wan;Kim Jin Sun;Cho Sung Woo;Kim Si Wook;Choi Jin Ho;Park Pyo Won
    • Journal of Chest Surgery
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    • v.38 no.7 s.252
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    • pp.483-488
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    • 2005
  • Aortic valve-sparing procedures could reduce valve-related morbidity, but may increase operative risks; therefore, these procedures could not be performed routinely. We attempted to find out the early results while focusing on the operative risks associated with these procedures in our hospital. Material and Method: From May 1996 to July 2003, 26 patients underwent these procedures including 15 patients with Marfan syndrome and 1 patient with Behcet disease. There were 17 men and 9 women with mean age of $37.9\pm19.2$ years (range: 6 months-74 years). Ten patients had ascending aortic dissection, 18 patients had more than moderate degree of aortic valve insufficiency (AI). Two types of valve-sparing procedures were performed: valve reimplantation in 14 and root remodeling in 12 patients. Associated procedures were performed as follows: aortic valve plasty in 6, mitral valve plasty in 5, hemi-arch replacement in 4, total arch replacement in 2, coronary artery bypass surgery in 1 and Maze procedure in 1 patient(s). Result: In four patients, valve-sparing procedures were converted to Bentall procedures during operation. Including these patients, there was no operative deaths, 3 patients underwent re-operation due to bleeding, 1 patient had permanent pacemaker. The median duration of ICU stay was 45.5 hours, the median duration of hospital stay was 10.5 days. In 22 patients excluding 4 converted patients, intraoperative transesophageal echocardiogram (TEE) showed less than mild degree of AI in all except one who had not received intra-operative TEE in the beginning and showed moderate degree of AI at discharge. The mean duration of follow-up was $21.2\pm27.4$ months. All patients were alive except one who died during other departmental surgery. In 3 patients, more than moderate degree of AI was recurred, but there were no reoperation. Conclusion: Aortic valve-sparing procedures could be performed relatively safely in selected patients who had annuloaortic ectasia.