1,720,972 research outputs found

    Eosinophilic granulomatosis with polyangiitis (EGPA) and PRES: a case-based review of literature in ANCA-associated vasculitides

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    Eosinophilic granulomatosis with polyangiitis (EGPA) is a small-sized vessel systemic necrotizing vasculitis and belongs to the family of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. The involvement of central nervous system in this condition is pretty rare. Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiological entity described for the first time by Hinchey et al. (N Engl J Med 334(8):494-500, 1996) and characterized by MRI findings of reversible subcortical vasogenic edema predominantly in the white matter of posterior cerebral lobes. There are few case reports describing the concurrence of PRES with ANCA-associated vasculitides. We describe a case of PRES in a patient with a diagnosis of EGPA with a concise review of the literature. The exact cause of this syndrome is unknown. It has been related to eclampsia, drug-induced hypertension, renal insufficiency and also to rheumatologic diseases. Endothelial injury, hypertension and immunosuppressive medications can compromise the regulation of cerebral blood flow. In ANCA-associated vasculitides, patients presenting with symptoms of PRES represent a challenge to treatment with immunosuppressive medications. However, since an inflammatory process might be implicated, judicious use of these agents along with tight control of blood pressure and a supportive therapy may contribute to the resolution of the encephalopathic syndrome treating at the same time other manifestation related to the rheumatologic disease. Larger clinical studies are warranted to optimize the management of vasculitis-associated PRES

    In systemic sclerosis skin perfusion of hands is reduced and may predict the occurrence of new digital ulcers

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    Systemic sclerosis (SSc) patients are at high risk for the development of ischemic digital ulcers (DUs). The aim of this study was to assess in SSc patients a correlation between skin perfusion evaluated by LDPI and DUs and to evaluate the prognostic value of skin perfusion to predict the new DUs occurrence. Fifty eight (47 female, 11 male) SSc patients were enrolled. Skin perfusion of hands and region of interest (ROIs) was measured by Laser Doppler perfusion Imager (LDPI). The proximal-distal gradient (PDG) was present when the perfusion mean difference between ROI1 and ROI2 was > 30 pU. The skin perfusion of hands is lower in SSc patients than in healthy controls. The skin perfusion decreased with severity of capillaroscopic damage. Both mean perfusion of hand and PDG are significantly (p < 0.01 and p < 0.0001, respectively) lower in SSc patients with new DUs than in SSc patients without DUs. Only 2 of 11 SSc patients (18.2%) with PDG developed new digital ulcers, conversely 36 of 47 (76.6%) SSc patients without PDG developed new digital ulcers (p < 0.001). The ROC curves demonstrated a good accuracy of new DUs prediction for PDG (0.78, p < 0.0001). Using this cut-off value of 30 pU, RR for new DUs development in SSc patients without PDG is 4,2 (p < 0.001). LDPI indices could be used in association to the capillaroscopic and clinical findings or serological tests in the identification of patients at high risk of developing DU

    ENDOCARDITIS AND LYMPHOMA: WHAT IS THE THERAPY ?

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    A 68 year old man presented with fever, anemia (6.8gr%), pancytopenia and weight loss. The fever, of 2 months duration, was characterized by daily intermittent peaks, accompanied by chills which did not respond to antimicrobial treatment. Chest X-ray was normal. Viral and serological investigations (Vidal-Writh and Weil-Felix, tests for leishmania and malaria) were negative. Blood cultures were positive for Staphilococcus Epidermidis. A transesophageal echocardiography revealed valvular vegetations. Abdominal ultrasonography and total body CT scan demonstrated splenomegaly and enlarged lymph nodes. A bone marrow biopsy was performed showing a non-Hodgkin large B cells lymphoma (HL) grade IV. Additionally the patient was HBV positive. The Immunologist was consulted and he prescribed combined treatment with Gentamicin, Rifampicin and Vancomicin for 6 weeks and once echocardiography resulted normal, to start antineoplastic treatment. Instead, the haematologist recomended only steroid treatment followed by one infusion of Vincristin and once healed endocarditis, to give four cycles of R-CHOP, combined with Lamuvidin. From a review of the literature, we decided to begin therapy with antibiotics, steroids, Vincristine and Rituximab in four doses and prophylaxis with Lamivudine [1,2]. The patient continued chemotherapy with a complete response after six months

    Miglior Poster

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    A 49- year-old woman had a clinical history that began at age 35, characterized by the presence of anemia, increased C-reactive protein (CRP) and Erythrocyte sedimentation rate (ESR), hypergammaglobulinemia, symmetric polyarthritis involving the small joints in the hands and wrists, treated which resulted with improvement of symptoms. She was hospitalized in March because of fever and dyspnea. The chest CT scanning revealed interstitial pneumonia and pleural effusion, she was treated with antibiotic therapy for about 10 days. A medical examination showed fever, chest pain and cardipalmo and she was again hospitalized. At the clinical examination, she appeared unwell, febrile at 39 oC, with a widespread vasculitic rash was present and most prominent on the legs. At the physical exam of the chest: dullness to percussion at the right base, with increased tactile fremitus and bilateral basal crackles. No evidence of lymphadenopathy was observed and the rest of the examination was unremarkable. During the hospitalization she presented pancitopenica, serum C3 level was decreased and antinuclear antibody, anti-dsDNA antibody, anti-SS-A, anti-SS-B, anti-Sm and anti-RNP antibody were positive. Futhermore she showed hyperferritinemia, hypofibrinogenemia, liver dysfunction with hypertriglyceridemia and raised liver enzymes, splenomegaly and bone marrow aspirate showed hemophagocytosis. Repeated urine and blood cultures were negative. Sputum was negative for bacterial and fungal cultures. A thorough infection screen, including a viral panel for Herpes Simplex, Herpes Zoster, Epstein-Barr, Cytomegalovirus, Hepatitis B, Hepatitis C, HIV, Echo and Coxsackie viruses was negative. She was diagnosed having Systemic lupus erythematosus (SLE) associated with Macrophage activation syndrome (MAS). She was treated only with corticosteroids (metilprednisolone) with improvement of symptoms

    Going Beyond Counting First Authors in Author Co-citation Analysis

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    The present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed

    Variations on the Author

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    “Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
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