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Susac syndrome is a rare disease affecting mainly young women, characterized by a microangiopathy limited to the cerebral, retinal, and cochlear vessels. Although the pathophysiology of Susac syndrome is not yet fully elucidated, recent advances favour a primitive vasculitis affecting the cerebral, retinal and cochlear small vessels. Susac syndrome must be recognized in the presence of the pathognomonic clinical triad associating: 1/subacute encephalopathy with unusual headache and pseudopsychiatric features associated with diffuse white matter, grey matter nuclei and specifically corpus callosum lesions on brain MRI; 2/eye involvement that may be pauci-symptomatic, with occlusions of the branches of the central artery of the retina at fundoscopy and arterial wall hyperfluorescence on fluorescein angiography; and 3/cochleo-vestibular damage with hearing loss predominating at low frequencies on the audiogram. Relapses are frequent during an active period lasting approximately 2 years. Eventually, the disease resolves but isolated retinal arterial wall hyperfluorescence without new occlusions may recur, which should not lead to treatment intensification. First-line treatment consists of a combination of anti-aggregants and high dose corticosteroids. In refractory patients or in case of relapse, immunomodulatory molecules such as intravenous immunoglobulins or immunosuppressive drugs such as mycophenolate mofetil, cyclophosphamide or rituximab should be started. Unfortunately, sequelae-mostly hearing loss- remain frequent in these young patients.  相似文献   

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Aim

To estimate the prevalence of the co-infection by the human immunodeficiency virus (HIV) and the hepatitis C virus (HCV) from a patients?? representative sample of patients infected by the HIV.

Methods

Retrospective study over 5 years (2004?C2009) carried out in the ??Turiho Center?? of the National Association of Support for HIV-Seropositive and AIDS-patients. We included patients HIV positive who had HCV antibodies dosed and results clearly recorded in the medical files.

Results

A total of 1053 patients had HCVantibodies dosage (722 were females, 331weremales).Among the 1053 patients, the co-infection was found at 112 patients that is to say the prevalence rate of 10,6% CI95 % = [0,09; 0,13]. The middle age of the co-infected patients was 48 years. In the feminine population, the rate of co-infection was 9,4% IC95%= [0,07;0,12], and among men, it was 13,2% IC95%= [0,10;0,17]. No significant difference: X2 = 2.51, p = 0,15

Conclusion

This high co-infection prevalence strengthens the recommendation of a systematic screening of the HCV in patients infected by the HIV.  相似文献   

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Rhythm control or rate control are the 2 therapeutic strategies for atrial fibrillation (AF) management. Despite strong physiological and epidemiological data to support a rhythm control strategy, the results of the prospective studies, especially AFFIRM and RACE trials, did not demonstrate any superiority of a rhythm control versus a rate control strategy. The AF-CHF trial conducted in heart failure patients led to the same conclusions. In clinical practice, the therapeutic management is only driven by the patient symptoms. For rhythm control, antiarrhythmic drugs are still the first step before considering, in selected patients, ablative techniques. Treatment algorithms proposed in 2006 by the European society of cardiology are the references for patient management, treatment being individually optimized.  相似文献   

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The authors describe the case of a 16-year-old male without any medical history who developed anaphylactic shock a few minutes after the injection of the intradermal vaccine BCG Pasteur. Chronology of events, lack of any other drug intake and the dramatic increase of serum tryptase level account for the anaphylactic reaction consecutive to vaccine injection. A few weeks after the accident, the allergic data donˈt reveal much. At this time, serum anti dextran IgG are significantly high (8 mg/L), suggesting the involvement of dextran in this severe adverse reaction.  相似文献   

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