Chagas Disease, France

Chagas Disease, Franc

Anomalies électrocardiographiques d'origine neurologique centrale (revue de la littérature à propos de deux cas )

Les connections anatomiques végétatives entre cœur et cerveau sont étroites. Ainsi, des lésions du système nerveux central peuvent entraîner des perturbations de l'électrocardiogramme ( troubles du rythme, de la conduction et de la repolarisation ). Le rôle du cortex insulaire semble prépondérant. L'hypothèse pathogénique avancée dans les accidents vasculaires cérébraux, les crises d'épilepsie, les tumeurs cérébrales et dans les traumatismes crâniens, est l'hypothèse " neurogène ". Le marqueur histopathologique au niveau du cœur est la myocytolyse. En pratique, les études ont permis d'affiner la prise en charge de la fibrillation auriculaire inaugurale à la phase aiguë d'un accident cérébral. L'utilisation des beta-bloquants dans ces pathologies semble également prometteuse.NANTES-BU Médecine pharmacie (441092101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Épidémiologie des cancers du sein dans le Finistère entre 2000 et 2009 à partir d'une base de données anatomopathologiques . [Breast cancer in Finistère, France: epidemiological description and tendencies over a 10-year period (2000-2009) according to pathology data].

International audienceUNLABELLED: The aim of this study was the description of breast carcinoma over a 10-year period according to pathology data. METHOD: Descriptive epidemiological study based on data collection of pathological code ADICAP (injury, organ, and applied technical), histological, hormonal, node and administrative data. From January 1st 2000 to December 31st 2009, 6186 women living in Finistère have had a diagnosis of invasive breast carcinoma. The incidence rate involved from 125 per 100,000 women to 136 in 2009. Average age to the first diagnosis was 61.4 ± 13.6; class of age with the more important incidence rate was for the 50-74 years old. The different histological subtypes varied over the period (P<0.0001). Tumour's size was notified for more than 75% in the whole period of the study. The average size evolved significantly over the period (P<0.0001 from 23.5mm [± 18.4] in 2000 to 21.02 [± 16.2] in 2009, particularly after 2003 [P<0.0002]). The grade status (SBR, MSBR and Elston Ellis) showed a trend to the gravity decrease over the period (respectively P=0.03 [r(2)=-0,04]; P<0.0001 [r(2)=-0.10]; P<0.0001 [r(2)=-0.08]). CONCLUSION: Our results confirm the interest of pathology database for the description of invasive breast cancer

Efficacy of lenalidomide in POEMS syndrome: a retrospective study of 20 patients.

International audiencePOEMS syndrome is a rare disorder characterized by polyneuropathy, monoclonal gammopathy, multiorgan involvement, and elevated vascular endothelial growth factor levels. Localized bone lesions require irradiation, whereas young patients with disseminated disease receive intensive treatment with stem cell support. Treatment of older and non responding patients is not yet standardized. We report the use of a combination of lenalidomide and dexamethasone in 20 patients with POEMS syndrome. Four patients were newly diagnosed, and 16 had relapsed or progressed after treatment. All but one of the patients responded: clinical improvements were noted in neuropathies (16/20) organomegaly (13/13), peripheral edema (14/15), and pulmonary hypertension (5/5). At least a very good partial response was noted in 68% of patients, with partial responses in 26%. Serum VEGF levels fell markedly in all 17 patients with available values. Twelve patients had 18-FDG-PET/CT at diagnosis (11 with positive findings), and nine patients during follow-up. The number of lesions fell markedly in five cases and remained stable in two cases, while two patients became negative. During a median follow-up of 22 months, four patients relapsed. Toxicity, predominantly hematological, was mild and manageable. Lenalidomide thus appears to be effective in POEMS syndrome, inducing high rate of clinical and biological responses

Long-term effectiveness and safety of interleukin-1 receptor antagonist (anakinra) in Schnitzler's syndrome: A French multicenter study.

Conflicts of interest: DL received a grant from Sobi (< 10,000 USD). EH received lecture fees from Sobi (< 10,000 USD). The other authors have no conflict of interest to disclose. Ethics approval: According to French legislation regarding retrospective observational studies using anonymous data, Ethics Committee approval was not required, which was confirmed by the chair of our Institutional Ethics Committee. The study was performed in accordance with the Declaration of Helsinki's guidelines for research in humans.International audienceThe aim of this study is to assess the long-term effectiveness and safety of IL1Ra in Schnitzler syndrome (SchS). Between 2010 and 2012, we performed a nationwide survey among French internal medicine departments to identify SchS patients. We retrospectively analyzed the long-term efficacy and safety of IL1Ra and the outcome of patients that did not receive this treatment. Forty-two patients were included in the study, 29 of whom received IL1Ra. The mean age at disease onset was 59.9years. Disease manifestations included urticaria (100%), fever (76%), bone/joint pain (86%), bone lesions (76%), anemia (67%), and weight loss (60%). The monoclonal gammopathy was overwhelmingly IgM kappa (83%). The mean follow-up was 9.5years (range: 1.6-35). Two patients developed Waldenström's macroglobulinemia and one developed AA amyloidosis. All of the 29 patients who received IL1Ra responded dramatically. After a median follow-up of 36months (range: 2-79), the effectiveness remained unchanged. All patients remained on anti-IL-1 therapy. Twenty-four patients (83%) went into complete remission and five (17%) into partial remission. Three patients experienced grade 3-4 neutropenia. Six patients developed severe infections. No lymphoproliferative diseases occurred while on IL1Ra. When last seen, all patients without anakinra had an active disease with variable impact on their quality of life. Their median corticosteroids dosage was 6mg/d (range: 5-25). IL1Ra is effective in SchS, with a sharp corticosteroid-sparing effect. Treatment failures should lead to reconsider the diagnosis. Long-term follow-up revealed no loss of effectiveness and a favorable tolerance profile. The long-term effects on the risk of hemopathy remain unknown

Comparison of idiopathic (isolated) aortitis and giant cell arteritis-related aortitis. A French retrospective multicenter study of 117 patients

International audienceObjectives: The aim of the study was to compare clinical/imaging findings and outcome in patients with idiopathic (isolated aortitis, IA) and with giant cell arteritis (GCA)-related aortitis.Methods: Patients from 11 French internal medicine departments were retrospectively included. Aortitis was defined by aortic wall thickening &gt; 2 mm and/or an aortic aneurysm on CT-scan, associated to inflammatory syndrome. Patients with GCA had at least 3 ACR criteria. Aortic events (aneurysm, dissection, aortic surgeries) were reported, and free of aortic events-survival were compared.Results: Among 191 patients with non-infectious aortitis, 73 with GCA and 44 with IA were included. Patients with IA were younger (65 vs 70y, p = 0.003) and comprised more past/current smokers (43 vs 15%, p = 0.0007). Aortic aneurisms were more frequent (38% vs 20%, p = 0.03) and aortic wall thickening was more pronounced in IA. During follow-up (median = 34 months), subsequent development of aortic aneurysm was significantly lower in GCA when compared to IA (p = 0.009). GCA patients required significantly less aortic surgery during follow-up than IA patients (p = 0.02). Mean age, sex-ratio, inflammatory parameters and free of aortic aneurism-survival were equivalent in patients with IA ≥ 60y when compared to patients with GCA-related aortitis.Conclusions: IA is more severe than aortitis related to GCA, with higher proportions of aortic aneurism at diagnosis and during follow-up. IA is a heterogeneous disease and its prognosis is worse in younger patients &lt; 60y. Most patients with IA ≥ 60y share many features with GCA-related aortitis

Germline CYBB mutations that selectively affect macrophages in kindreds with X-linked predisposition to tuberculous mycobacterial disease

Germline mutations in CYBB, the human gene encoding the gp91(phox) subunit of the phagocyte NADPH oxidase, impair the respiratory burst of all types of phagocytes and result in X-linked chronic granulomatous disease (CGD). We report here two kindreds in which otherwise healthy male adults developed X-linked recessive Mendelian susceptibility to mycobacterial disease (MSMD) syndromes. These patients had previously unknown mutations in CYBB that resulted in an impaired respiratory burst in monocyte-derived macrophages but not in monocytes or granulocytes. The macrophage-specific functional consequences of the germline mutation resulted from cell-specific impairment in the assembly of the NADPH oxidase. This \u27experiment of nature\u27 indicates that CYBB is associated with MSMD and demonstrates that the respiratory burst in human macrophages is a crucial mechanism for protective immunity to tuberculous mycobacteria

Pouvoir local, régionalismes, décentralisation

En Amérique latine, depuis le début des années quatre-vingt, deux phénomènes majeurs, l'approfondissement de la crise financière-économique et l'évolution démocratique, ont renouvelé les termes et les enjeux d'un débat désormais généralisé sur les rapports entre pouvoirs centraux, pouvoirs régionaux et pouvoirs locaux. En France, dans la même période, mais dans un contexte politique différent, la discussion puis l'application des lois de décentralisation ont répondu à la volonté des sociétés locales de prendre une plus grande part dans la détermination de leur destin et de celui de leur territoire. Démocratisation et modernisation, modernisation et démocratisation sont ainsi devenues, tant en France (en Europe devrait-on dire) qu'en Amérique latine les mots-clés, les références incontournables pour toute réflexion sur les voies et moyens permettant d'affronter les mutations imposées par la compétition internationale