Ryanodine receptor dysfunction causes senescence and fibrosis in Duchenne dilated cardiomyopathy.

Background: Duchenne muscular dystrophy (DMD) is an X-linked disorder characterized by progressive muscle weakness due to the absence of functional dystrophin. DMD patients also develop dilated cardiomyopathy (DCM). We have previously shown that DMD (mdx) mice and a canine DMD model (GRMD) exhibit abnormal intracellular calcium (Ca) cycling related to early-stage pathological remodelling of the ryanodine receptor intracellular calcium release channel (RyR2) on the sarcoplasmic reticulum (SR) contributing to age-dependent DCM.

NTPDase1/CD39 Ectonucleotidase Is Necessary for Normal Arterial Diameter Adaptation to Flow.

NTPDase1/CD39, the major vascular ectonucleotidase, exerts thrombo-immunoregulatory function by controlling endothelial P2 receptor activation. Despite the well-described release of ATP from endothelial cells, few data are available regarding the potential role of CD39 as a regulator of arterial diameter. We thus investigated the contribution of CD39 in short-term diameter adaptation and long-term arterial remodeling in response to flow using male mice.

An immuno-lipidomic signature revealed by metabolomic and machine-learning approaches in labial salivary gland to diagnose primary Sjögren's syndrome.

Introduction: Assessing labial salivary gland exocrinopathy is a cornerstone in primary Sjögren's syndrome. Currently this relies on the histopathologic diagnosis of focal lymphocytic sialadenitis and computing a focus score by counting lym=phocyte foci. However, those lesions represent advanced stages of primary Sjögren's syndrome, although earlier recognition of primary Sjögren's syndrome and its effective treatment could prevent irreversible damage to labial salivary gland.

Infantile-onset parkinsonism, dyskinesia, and developmental delay: do not forget polyglutamine defects!

We present the phenotype of an infant with the largest ATN1 CAG expansion reported to date (98 repeats). He presented at 4 months with developmental delay, poor eye contact, acquired microcephaly, failure to thrive. He progressively developed dystonia-parkinsonism with paroxysmal oromandibular and limbs dyskinesia and fatal outcome at 17 months.

Veno-arterial extracorporeal membrane oxygenation for circulatory failure in COVID-19 patients: insights from the ECMOSARS registry.

Objectives: The clinical profile and outcomes of patients with Coronavirus Disease 2019 (COVID-19) who require veno-arterial extracorporeal membrane oxygenation (VA-ECMO) or veno-arterial-venous extracorporeal membrane oxygenation (VAV-ECMO) are poorly understood. We aimed to describe the characteristics and outcomes of these patients and to identify predictors of both favourable and unfavourable outcomes.

Methods: ECMOSARS is a multicentre, prospective, nationwide French registry enrolling patients who require veno-venous extracorporeal membrane oxygenation (ECMO)/VA-ECMO in the context of COVID-19 infection (652 patients at 41 centres).

Ten-year Experience of Surgical Management of Paget-Schroetter Syndrome.

Background: Paget-Schroetter syndrome (PSS) or effort-induced thrombosis is an acute (<14 days) venous thrombosis of the axillosubclavian vein. Early catheter-directed thrombolysis (CDT) is required to improve patency rate and avoid postthrombotic syndrome. This study aimed to report the management of PSS in our center across 10 years and compare it to the established guidelines.

Association between age-related hearing loss and gait disorders in older fallers.

Introduction: Falls are associated with hearing loss, which might be explained by the onset of gait disorders. The objective of this study was to examine the association between Age-Related Hearing Loss (ARHL) and gait disorders assessed with GAITrite walkway in a population of fallers aged 75 and over while accounting for the vestibular function.

Methods: We examined data from 53 older patients (mean 84.

Accurate stratification between VEXAS syndrome and differential diagnoses by deep learning analysis of peripheral blood smears.

Objectives: VEXAS syndrome is a newly described autoinflammatory disease associated with somatic mutations and vacuolization of myeloid precursors. This disease possesses an increasingly broad spectrum, leading to an increase in the number of suspected cases. Its diagnosis via bone-marrow aspiration and -gene sequencing is time-consuming and expensive.

Placental protein 13 dilation of pregnant rat uterine vein is endothelium dependent and involves nitric oxide/calcium activated potassium channels signals.

Introduction: Accumulating evidence demonstrates the importance of the galectin protein Placental Protein 13 (PP13) in predicting Preeclampsia (PE), a gestational disorder that has no cure and is associated with a compromised uterine vascular adaptation to pregnancy. Uterine vasculature undergoes significant remodeling (growth in length and in circumference) during normal pregnancy to accommodate the increased blood volume to the feto-placental unit. The aim of this study was to demonstrate the role of PP13 on the uterine veins (UVs).

Estrogen Receptor and Vascular Aging.

Cardiovascular diseases remain an age-related pathology in both men and women. These pathologies are 3-fold more frequent in men than in women before menopause, although this difference progressively decreases after menopause. The vasculoprotective role of estrogens are well established before menopause, but the consequences of their abrupt decline on the cardiovascular risk at menopause remain debated.

Exercise Oximetry Correlates Better With Exercise-Induced Lactate Increase, than Ankle Brachial Index or Walking Time, in Vascular Claudicants.

In claudication, the correlation between walking-induced biomarkers and indices of clinical severity (e.g., walking distance or ankle brachial index (ABI)), is fair.

Metabolomic Profiling of Angiotensin-II-Induced Abdominal Aortic Aneurysm in Ldlr Mice Points to Alteration of Nitric Oxide, Lipid, and Energy Metabolisms.

Aneurysm is the second-most common disease affecting the aorta worldwide after atherosclerosis. While several clinical metabolomic studies have been reported, no study has reported deep metabolomic phenotyping in experimental animal models of aortic aneurysm. We performed a targeted metabolomics study on the blood and aortas of an experimental mice model of aortic aneurysm generated by high-cholesterol diet and angiotensin II in Ldlr mice.

Masseter muscle pressure injury: First report of a prone position complication in patients with COVID-19.

We report the case of a 59-year-old, obese woman who underwent prolonged prone position during the medical management of an acute respiratory distress syndrome induced by SARS-CoV-2 infection, complicated by a masseter muscle pressure injury. Such side effect may be underestimate in intensive care units and should be prevent by prophylactic dressings on facial weight-bearing sites. The understanding of facial deep tissue injury is essential to guide clinical detection and management of such a complication in COVID-19 patients.

Relationship Between the Severity of Exercise Induced Ischaemia and the Prevalence of Exercise Induced Calf Symptoms During Treadmill Testing With Transcutaneous Oximetry.

Objective: It was hypothesised that there is a linear relationship between the severity of exercise induced calf ischaemia and the prevalence of calf claudication on a treadmill until a plateau is reached. It was expected that no pain would be present in the absence of ischaemia and all severely ischaemic calves would be symptomatic.

Methods: This was a retrospective analysis of a cross sectional acquired database recording.

Vitamin D and Calcium Supplementation Accelerate Vascular Calcification in a Model of Pseudoxanthoma Elasticum.

Unlabelled: Arterial calcification is a common feature of pseudoxanthoma elasticum (PXE), a disease characterized by mutations, inducing a deficiency in pyrophosphate, a key inhibitor of calcium phosphate crystallization in arteries.

Methods: we analyzed whether long-term exposure of Abcc6 mice (a murine model of PXE) to a mild vitamin D supplementation, with or without calcium, would impact the development of vascular calcification. Eight groups of mice (including Abcc6 and wild-type) received vitamin D supplementation every 2 weeks, a calcium-enriched diet alone (calcium in drinking water), both vitamin D supplementation and calcium-enriched diet, or a standard diet (controls) for 6 months.

Distinction between clonal and paraclonal cutaneous involvements in VEXAS syndrome.

VEXAS (vacuoles, E1 enzyme, X-linked, auto-inflammatory, somatic) syndrome is an inflammatory disorder with hematological and systemic features. A recent study demonstrated that the dermal infiltrate in neutrophilic dermatosis from VEXAS patients is derived from the pathological UBA1-mutated myeloid clone. Neutrophilic dermatosis is, however, only one of the various skin involvements observed in VEXAS syndrome.

Discordant Predictions of Extraglandular Involvement in Primary Sjögren's Syndrome According to the Anti-SSA/Ro60 Antibodies Detection Assay in a Cohort Study.

Electrophoresis-derived techniques for anti-SSA/Ro60 KDa (anti-SSA) antibodies detection have been progressively replaced by methods using non-native antigens. We aimed to compare the patients' phenotypes and the occurrence of extraglandular manifestations in primary Sjögren's syndrome according to the method used to detect anti-SSA antibodies. Sera from patients with a diagnosis of pSS according to ACR/EULAR 2016 criteria between 2008 and 2017 were tested for anti-SSA antibodies using methods with non-native antigens (magnetic bead multiplex assay; line immunoassays) and one with native antigens (counterimmunoelectrophoresis (CIE)).

Postoperative prophylactic intermittent noninvasive ventilation versus usual postoperative care for patients at high risk of pulmonary complications: a multicentre randomised trial.

Background: Pulmonary complications are an important cause of morbidity and mortality after surgery. We evaluated the clinical effectiveness of noninvasive ventilation (NIV) in preventing postoperative acute respiratory failure.

Methods: This is an open, multicentre randomised trial that included patients at high risk of postoperative pulmonary complications after elective or semi-urgent surgery with an Assess Respiratory Risk in Surgical Patients in Catalonia (ARISCAT) score ≥45.

Comparison between conventional duplex ultrasonography and the dual-gate Doppler mode for hemodynamic measurements of the carotid arteries.

Purpose: This study investigated the correlations of hemodynamic parameters measured to quantify stenosis between the gold-standard duplex ultrasonography and the dual-gate Doppler mode.

Methods: Patients examined due to suspicion of carotid artery stenosis or for surveillance of known stenosis were invited to participate in this prospective single-center study. Upon acceptance, the hemodynamic characteristics of the carotid arteries were determined successively in standard duplex and dual-gate Doppler modes.