Increased aortic stiffness and blood pressure in non-classic Pompe disease

Vascular abnormalities and glycogen accumulation in vascular smooth muscle fibres have been described in Pompe disease. Using carotid-femoral pulse wave velocity (cfPWV), the gold standard methodology for determining aortic stiffness, we studied whether aortic stiffness is increased in patients with Pompe disease. Eighty-four adult Pompe patients and 179 age- and gender-matched volunteers participated in this cross-sectional case-controlled study. Intima media thickness and the distensibility of the right common carotid artery were measured using a Duplex scanner. Aortic augmentation index, central pulse pressure, aortic reflexion time and cfPWV were assessed using the SphygmoCor® system. CfPWV was higher in patients than in volunteers (8.8 versus 7.4 m/s, p < 0.001). This difference was still present after adjustment for age, gender, mean arterial blood pressure (MAP), heart rate and diabetes mellitus (p = 0.001), and was shown by subgroup analysis to apply to the 40-59 years age group (p = 0.004) and 60+ years age group (p = 0.01), but not to younger age groups (p = 0.99). Except for a shorter aortic reflexion time (p = 0.02), indirect indicators of arterial stiffness did not differ between patients and volunteers. Relative to volunteers (20 %), more Pompe patients had a history of hypertension (36 %, p = 0.005), and the MAP was higher than in volunteers (100 versus 92 mmHg, p < 0.001). This study shows that patients with non-classic Pompe disease have increased aortic stiffness and blood pressure. Whether this is due to glycogen accumulation requires further investigation. To reduce the potential risk of cardiovascular diseases, we recommend that blood pressure and other common cardiovascular risk factors are monitored regularly

An Automated Platform for Cell Dielectric Spectroscopy

International audienceThis paper focuses on the development of a new automated platform dedicated to cell manipulation and characterization by dielectrophoretic methods. Microelectrode structures powered with computercontrolled AC generators are used to impose several forms of electric field to cells such as non-uniform, rotating or travelling wave, over a wide frequency range. The resulting induced motion is analysed to deduce cell electric parameters. As an illustration of the system capabilities, several electrorotation spectra of single viable and non-viable yeast cells have been measured at a medium conductivity of 1.1 mS.m-1

Automated cell characterization platform: Application to yeast protoplast study by electrorotation

IDS Number: BHU55 ISBN: 978-989-8111-17-3International audienceThis paper is about the development of a new automated platform dedicated to cell manipulation and characterization by dielectrophoretic methods. We illustrate its possibilities by studying yeast protoplasts and yeast cells electrorotation spectra, obtained using polynomial microelectrode structures powered by computer-controlled generators. Measurements were made over the frequency range 100 kHz to 80MHz, mostly in a suspending medium of conductivity 50 mS/m inside the rotation chamber. The rotation rate of yeast protoplasts was inferior to that of whole yeast cells. To understand such behavioral differences, yeast protoplasts were modelled as single-shell spheres in a first approach

Selecting an electrode structure for cell sorting by differential dielectric affinity

ISBN: 978-1-4244-0787-3International audienceThis paper focuses on the application of dielectrophoresis to on-chip cell sorting. Differential dielectric affinity separation is a "binary" technique, dividing a cell mixture into two distinct sub-populations. The principle and efficiency of this method are illustrated by potential energy plots of cells exposed to negative and positive dielectrophoresis. This paper aims at comparing several microelectrode structures, either bipolar or quadrupolar, in order to guide the choice of a geometry facilitating the sorting operation. This comparison relies on a 3D finite-elements calculation of the potential energy profiles obtained for each electrode shape

Electromagnetic characterization of biological cells

Papier complet disponible sur : http://doi.editoracubo.com.br/10.4322/rbeb.2012.016International audienceThis paper presents the most commonly used method to characterize individual biological cells on a dielectric point of view. It is a force based technique which lays on dielectrophoresis and/or electrorotation. First the principle of these phenomena are described and analyzed with an extension to magnetic forces at the micrometric scale level. Secondly we present an experimental setup which permits to acquire the dielectrophoretic spectrum which is a dielectric signature of a cell. The main dielectric parameters can be deduced by fitting the theoretical response of the cell issued from a dielectric model and the experimental data. At the end we present an improved fitting method which takes advantage of a sensitivity analysis based on a probabilistic approach

Objective evaluation of clinical actionnability for genes involved in myopathies: 51 promising genes

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NAITRE study on the impact of conditional cash transfer on poor pregnancy outcomes in underprivileged women: protocol for a nationwide pragmatic cluster-randomised superiority clinical trial in France

International audienceIntroduction Prenatal care is recommended during pregnancy to improve neonatal and maternal outcomes. Women of lower socioeconomic status (SES) are less compliant to recommended prenatal care and suffer a higher risk of adverse perinatal outcomes. Several attempts to encourage optimal pregnancy follow-up have shown controversial results, particularly in high-income countries. Few studies have assessed financial incentives to encourage prenatal care, and none reported materno-fetal events as the primary outcome. Our study aims to determine whether financial incentives could improve pregnancy outcomes in women with low SES in a high-income country.Methods and analysis This pragmatic cluster-randomised clinical trial includes pregnant women with the following criteria: (1) age above 18 years, (2) first pregnancy visit before 26 weeks of gestation and (3) belonging to a socioeconomically disadvantaged group. The intervention consists in offering financial incentives conditional on attending scheduled pregnancy follow-up consultations. Clusters are 2-month periods with random turnover across centres. A composite outcome of maternal and neonatal morbidity and mortality is the primary endpoint. Secondary endpoints include maternal or neonatal outcomes assessed separately, qualitative assessment of the perception of the intervention and cost-effectiveness analysis for which children will be followed to the end of their first year through the French health insurance database. The study started in June 2016, and based on an expected decrease in the primary endpoint from 18% to 14% in the intervention group, we plan to include 2000 women in each group.Ethics and dissemination Ethics approval was first gained on 28 September 2014. An independent data security and monitoring committee has been established. Results of the main trial and each of the secondary analyses will be submitted for publication in a peer-reviewed journal.Trial registration number NCT02402855; pre-results

Objective evaluation of clinical actionnability for genes involved in myopathies: 51 promising genes

International audienc