Cases reported "Behcet Syndrome"

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1/13. Clonally accumulating T cells in the anterior chamber of Behcet disease.

    PURPOSE: To clarify the nature of infiltrating T cells into the anterior chamber of a patient with Behcet disease. methods: aqueous humor was obtained from a patient with ocular Behcet disease by paracentesis. rna isolated from the cells in aqueous humor was reverse transcribed into complementary dna. Complementary dna encoding the variable (V) diversity (D) joining (J) (junctional) region of T-cell receptor beta chain V domain (TCR BV) chain was amplified by T-cell receptor BV family polymerase chain reaction. RESULTS: polymerase chain reaction Southern blot analysis showed that T-cell receptor BV3, BV5, and BV7 were dominantly expressed on ocular T cells of this patient. In addition, dna sequencing revealed that monoclonal or oligoclonal T-cell accumulation was found in T-cell receptor BV3( ), BV5( ), and BV7( ) T cells. CONCLUSION: These findings suggest that some T cells infiltrating into the anterior chamber of a patient with ocular Behcet disease expand by antigen-driven stimulation, indicating the pivotal role of T cells in the pathogenesis of ocular Behcet disease.
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2/13. Renal Behcet's disease: a cumulative analysis.

    OBJECTIVE: To analyze cumulated data about renal involvement in Behcet's disease (BD) and to report on 6 patients with BD and renal problems. methods: We found reports of 159 patients (including our patients) with BD and specific renal disease (amyloidosis 69, glomerulonephritis [GN] 51, renal vascular disease 35, and interstitial nephritis 4) in our survey. RESULTS: The frequency of renal problems among BD patients has been reported to vary between 0% to 55%. male gender is a risk factor for all types of renal BD. nephrotic syndrome was present in 83% of patients with amyloidosis, and renal failure was common at the time of diagnosis. The mean interval between the initial manifestation of BD and diagnosis of amyloidosis was shorter in men than in women (P =.02). AA-type amyloid fibrils were shown in all cases studied. Vascular involvement was common in the patients with amyloidosis (60%). The renal findings in GN show a wide spectrum, from asymptomatic hematuria and/or proteinuria to rapidly progressive GN. Several types of glomerular lesions ranging from minor glomerular changes to crescentic glomerulonephritis are observed in BD. The common types of glomerular lesions among the reported cases are crescentic GN, proliferative GN, and immunoglobulin a (IgA) nephritis. Aneurysms may be located throughout the renal artery, from the orifice of the main artery to intrarenal microaneurysms. Another type of renal disease (amyloidosis or GN) and other major vascular involvement were present in all cases with renal vein thrombosis. hypertension is common among patients with renal artery aneurysm or stenosis. Microscopic vascular disease was described in 4 patients. CONCLUSIONS: Based on data in the literature, we suggest that renal involvement in BD is more frequent than has been recognized, although it is most often mild in nature. amyloidosis is one of the prognostic factors affecting survival. patients with vascular involvement carry high risk for amyloidosis, and administration of colchicine to these patients may be beneficial. More evidence is needed to accept interstitial nephritis as a manifestation of BD. In spite of some difficulties, hemodialysis and renal transplantation are safe treatment options in BD-related uremia.
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3/13. Intracranial hemorrhage in neuro-Behcet's syndrome.

    OBJECTIVE: Most cerebrovascular disturbances in Behcet's syndrome are occlusive in nature, while hemorrhage is rare. In this paper, we report three cases of neuro-Behcet's syndrome presenting with intracerebral hemorrhaging, and discuss the possible causes as they relate to cyclosporine treatment. patients: Three cases of neuro-Behcet's syndrome presented with intracranial hemorrhage. One patient had been taking cyclosporine, and the other two patients had never taking cyclosporine. RESULTS: Together with previous reports, these cases suggest that there are two types of intracranial hemorrhage in neuro-Behcet's syndrome. One type occurs in the center of a lesion and during the acute phase of the disease, while the other occurs in the peripheral lesion and during the subacute phase. CONCLUSIONS: It appears that the intracranial hemorrhages in neuro-Behcet's syndrome can be divided into two groups. It is possible that the vascular pathologies caused by Behcet's syndrome and by cyclosporine conspire to induce CNS hemorrhaging in some cases.
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4/13. Endovascular aneurysm exclusion along a femorodistal venous bypass in active Behcet's disease.

    PURPOSE: To report the endovascular repair of dual aneurysms along a femorodistal venous bypass graft in a patient with Behcet's disease. CASE REPORT: A 55-year-old man of middle European ancestry with Behcet's disease had dual aneurysms evolve along the proximal segment of a femorodistal venous bypass that had been implanted 2.5 years earlier for recurrent false aneurysm formation. Owing to the lack of suitable venous conduits and the active nature of the disease, the aneurysms were successfully excluded with overlapping Hemobahn and Jostent endografts; the immunosuppressive therapy was intensified. rupture of the aneurysms was successfully prevented, but the stent-grafts thrombosed 6 weeks later owing to exacerbation of the underlying disease. CONCLUSIONS: Endovascular exclusion of aneurysm in venous bypass grafts in Behcet's disease is feasible. Although the stent-grafts thrombosed, they did prevent rupture of the aneurysms.
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5/13. myocardial infarction and deep venous thrombosis in a young patient with Behcet disease.

    Behcet disease (BD) is a chronic relapsing systemic vasculitic disorder affecting the arteries, veins, and vessels of any size. Vascular lesions in BD usually represent an occlusive nature suggesting a hypercoagulable/ prothrombotic state. Coronary arteries are rarely involved in BD. In this report, a 27-year-old male patient in whom myocardial infarction developed secondary to coronary arterial thrombosis together with deep venous thrombosis was presented. This is a review of the pathologic hemostasis and the prothrombotic state of BD.
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6/13. Behcet's syndrome with childhood onset.

    Behcet's syndrome is a multisystemic disease of vasculitic nature. Main symptoms consist of oral and genital ulceration, uveitis and skin lesions, mainly erythema nodosum. Other features may evolve: arthritis, thrombophlebitis, gastrointestinal, cardiovascular and neurologic lesions. The disease usually manifests in early adulthood, childhood onset is rare. There exists a male preponderance and a genetic disposition. The course of Behcet's syndrome in 5 patients with childhood onset is described.
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7/13. Post-surgical pyoderma gangrenosum of the vaginal vault associated with ulcerative colitis and Behcet's disease; a case report.

    A case is described in which a patient with ulcerative colitis developed chronic vaginal ulceration around the incision lines after cone biopsy. The ulcers persisted at the vaginal vault after abdominal hysterectomy and removal of a cuff of vagina. Non-specific histological features compatible with pyoderma gangrenosum were found on biopsy. The auto-immune nature of this chronic ulcer is further supported by its rapid response to steroid therapy and the subsequent development of Behcet's syndrome.
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8/13. colitis in Behcet's syndrome. Two new cases.

    Two new cases of Behcet's disease with colitis are presented and the radiographic features described. The changes in the colon resemble Crohn's disease more closely than ulcerative colitis but show some features which are distinct from both. One patient had perforation of a cecal ulcer and severe bleeding, stressing the serious nature of this form of colitis.
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9/13. Behcet's disease: a case with hemoptysis, pseudotumor cerbri, and arteritis.

    A case of Behcet's diease with massive hemoptyis is described. The hemoptysis occurred during disease exacerbation with extensive oropharyngeal and laryngeal ulcerations, and responded to high-dose corticosteroid therapy. Other unusual manifestations included a light sensitive dermatitis, pseudotumor cerebri, and right bundle branch block. Also, arteritis and subsequent occlusion of the femoral and popliteal arteries occurred. Although uncommon, pumonary involvement may be life-threatening and should be treated with corticosteroids. Both pulmonary and neurologic involvement tend to occur with active aphthosis and respond to corticosteroids. This case underscores the protean nature of the organ system involvement in Behcet's disease and supports the concept that vasculitis is a primary pathophysiologic event.
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10/13. Arterial aneurysms in Behcet's disease.

    Six cases of arterial aneurysms, due to "Behcet's disease" located in various arteries are presented. Surgical reconstruction included: Resection of the lesions and replacement with corresponding grafts with satisfactory results. In spite of the macroscopic similarity of the aneurysms found in "Behcet's disease" as compared to those of mycotic origin, the presence of any microorganism could not be detected in various cultures or pathological specimens. Meticulous diagnosis on the nature of the aneurysm, close follow-up and careful postoperative anticoagulant treatment, is imperative for satisfactory immediate and late results.
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