30 research outputs found

    Basement membrane components are key players in specialized extracellular matrices

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    More than three decades ago, basement membranes (BMs) were described as membrane-like structures capable of isolating a cell from and connecting a cell to its environment. Since this time, it has been revealed that BMs are specialized extracellular matrices (sECMs) with unique components that support important functions including differentiation, proliferation, migration, and chemotaxis of cells during development. The composition of these sECM is as unique as the tissues to which they are localized, opening the possibility that such matrices can fulfill distinct functions. Changes in BM composition play significant roles in facilitating the development of various diseases. Furthermore, tissues have to provide sECM for their stem cells during development and for their adult life. Here, we briefly review the latest research on these unique sECM and their components with a special emphasis on embryonic and adult stem cells and their niches

    Treatment of Progressive Multifocal Leukoencephalopathy With Mirtazapine

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    International audienceBACKGROUND AND OBJECTIVES: Progressive multifocal leukoencephalopathy (PML) is a rare, JC-virus-mediated, demyelinating disease with a high mortality rate. As no recommended treatment exists, mirtazapine, a potential blocker of virus entry into cells, has been empirically used. METHODS: We analysed existing data on mirtazapine's efficacy to treat PML by systematically reviewing the literature since 2005, when it was first used. RESULTS: Searches in PubMed, EBSCO, SCOPUS and Google Scholar between January 2005 and December 2015, identified five cohort studies and 74 case reports. No statistically significant effect of mirtazapine on PML outcome was observed in the cohort studies. From studying the case reports, mortality rate for PML was associated with the underlying circumstances, such as an older age, the use of an immunosuppressant, or PML occurring in patients with a haematological malignancy or a transplant. CONCLUSIONS: Except for natalizumab-associated PML, we did not highlight any potential benefit of mirtazapine on disease outcomes. Further interventional studies are needed to confirm that 5-HT2AR inhibition is relevant to treat PM

    The spectrum of opportunistic diseases complicating sarcoidosis.

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    Sarcoidosis is an inflammatory disease marked by a paradoxical immune status. The anergic state, which results from various immune defects, contrasts with the inflammatory formation of granulomas. Sarcoidosis patients may be at risk for opportunistic infections (OIs) and a substantial number of cases have been reported, even in untreated sarcoidosis. It is not clear how OIs in patients with sarcoidosis are different from other groups at risk. In this review, we discuss the most common OIs: mycobacterial infection (including tuberculosis), cryptococcosis, progressive multifocal leukoencephalopathy, and aspergillosis. Unlike peripheral lymphocytopenia, corticosteroids are a major risk factor for OIs but the occurrence of Ols in untreated patients suggests more complex predisposing mechanisms. Opportunistic infections presenting with extrapulmonary features are often misdiagnosed as new localizations of sarcoidosis. Aspergillomas mostly develop on fibrocystic lungs. Overall, physicians should be aware of the possible occurrence of OIs during sarcoidosis, even in untreated patients

    Sarcoidosis-related mortality in France: a multiple-cause-of-death analysis

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    International audienceWe evaluated mortality rates and underlying causes of death among French decedents with sarcoidosis from 2002 to 2011.We used data from the French Epidemiological Centre for the Medical Causes of Death to 1) calculate sarcoidosis-related mortality rates, 2) examine differences by age and gender, 3) determine underlying and nonunderlying causes of death, 4) compare with the general population (observed/expected ratios), and 5) analyse regional differences.1662 death certificates mentioning sarcoidosis were recorded. The age-standardised mortality rate was 3.6 per million population and significantly increased over the study period. The mean age at death was 70.4 years (versus 76.2 years for the general population). The most common underlying cause of death was sarcoidosis. Sarcoidosis decedents were more likely to be males when aged \textless65 years. When sarcoidosis was the underlying cause of death, the main other mentions on death certificates were chronic respiratory and cardiovascular diseases. The overall observed/expected ratio was \textgreater1 for infectious disease, tuberculosis and chronic respiratory disease, and \textless1 for neoplasms. We observed a north-south gradient of age-standardised mortality ratio at the country level.Despite the limitation of possibly capturing the more severe cases of sarcoidosis, this study may help define and prioritise preventive intervention

    Les infections opportunistes au cours de la sarcoïdose

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    International audienceOpportunistic infections (OI) are uncommon in sarcoidosis (1 to 10%) and mostly occur in patients with previously diagnosed disease or can rarely be the presenting manifestation. The most common OIs are, in descending order: aspergillosis, cryptococcosis, and mycobacterial infections. Treatment with corticosteroids is the most frequent risk factor for OI occurrence during sarcoidosis but immunosuppressive drugs and therapy with anti-TNFalpha are also risk factors. Overall, clinical presentation, treatment, and outcome are identical to that occur in other conditions complicated with the occurrence of OIs. However, some atypical presentations of OIs can mimic sarcoidosis exacerbation and misdiagnosis may lead clinicians to increase immunosuppression, causing worsening of the OI. The meticulous collection of patient's history along with factors differentiating OI from sarcoidosis exacerbation is key factor to optimally manage these patientsLes infections opportunistes (IO) sont rares en cas de sarcoïdose (1 à 10 %) et surviennent le plus souvent au cours de sarcoïdose connue ou, plus rarement, la révèlent. Les IO les plus fréquentes sont par ordre décroissant : l’aspergillose pulmonaire, la cryptococcose et les infections mycobactériennes. La corticothérapie systémique représente le facteur de risque le plus fréquent d’IO au cours de la sarcoïdose mais les immunosuppresseurs et les anti-TNFα peuvent aussi être en cause. Le plus souvent, la présentation clinique, le traitement et le devenir des patients ne diffèrent pas des autres conditions favorisant la survenue d’une IO. Néanmoins, certaines présentations atypiques d’IO peuvent mimer une exacerbation de la sarcoïdose et la confusion risque de conduire le clinicien à une intensification de l’immunosuppression, entraînant l’aggravation de l’IO. Le recueil minutieux des antécédents ainsi que la connaissance des indices permettant de différencier une IO d’une exacerbation de la sarcoïdose sont donc des facteurs clés dans la prise en charge de ces patients

    What is the cost of endoscopic submucosal dissection (ESD)? A medico-economic study.

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    Endoscopic submucosal dissection (ESD) is the gold-standard treatment for superficial lesions of the digestive tract. No medico-economic study has been conducted in Europe. A monocentric study was conducted including all patients undergoing ESD between January 2015 and December 2017. The global cost of hospital stays was measured by microcosting, and revenue was based on the diagnosis-related group (DRG) system. The primary objective was to assess the cost/revenue balance. A medico-economic comparison with surgery was performed as a secondary outcome. A total of 193 patients were prospectively included. The cost per procedure was €3463.79, subtracted from a €2726.84 revenue, with a deficit of -€736.96 per stay. Presence of comorbidities/complications increasing DRG value was the only predictive factor for a positive budgetary balance in a multivariate analysis (odds ratio 49.21, 95% confidence interval 11.3-214.25, p < 0.0001). In comparison with surgery, ESD was associated with shorter length of stay (11 vs 2 days; p < 0.0001) and lower morbidity (28% vs 14%; p = 0.061), lower cost (€8960 vs €1770; p < 0.0001). The ESD cost/revenue balance is negative in 80% of cases. Given the benefits of ESD in terms of patient morbidity and financial savings compared with surgery, the implementation of a specific ESD reimbursement is warranted
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