colonisation in severe community-acquired pneumonia: not just a mere colonisation.

https://bit.ly/46PMk1f.

Identification of the first homozygous intragenic deletion in the YY1AP1 gene in a consanguineous family: New insights into the phenotypic variability associated with Grange syndrome.

Grange syndrome (GRNG-MIM#135580) is a rare recessive disorder associating variable features including diffuse vascular stenosis, brachysyndactyly, osteopenia with increased bone fragility, cardiac malformations, and variable developmental delay. Since its first description in 1998, only 15 individuals from 10 families have been reported, carrying homozygous or compound heterozygous frameshift or nonsense variants in YY1AP1. In a patient with cutaneous and bone syndactyly and a hemorrhagic stroke at the age of 16 months, consistent with a clinical diagnosis of GRNG, we performed exome sequencing after negative array-CGH and congenital limb malformation panel results.

Pneumococcal pneumonia and endotoxemia: An experimental and clinical reappraisal.

Background: Circulating endotoxins could result from bacterial digestive translocation during sepsis, thus contributing to uncontrolled systemic inflammation, leading in turn to organ dysfunction. We addressed this issue in the setting of severe pneumococcal pneumonia.

Methods: Endotoxemia was measured in a clinically relevant rabbit model of ventilated pneumococcal pneumonia and in 110 patients with bacteraemic pneumonia, using a patented mass spectrometry (LC-MS/MS) method for detection of 3-OH fatty acids (C10, C12, C14, C16 and C18), which are molecules bound to the lipid A motif of LPS.

Effect of norepinephrine on the vascular waterfall and tissue perfusion in vasoplegic hypotensive patients: a prospective, observational, applied physiology study in cardiac surgery.

Background: Norepinephrine is a commonly used drug for treating vasoplegic acute circulatory failure in ICU. The prediction of norepinephrine macro- and micro-circulatory response is complicated by its uneven receptors' distribution between the arterial and the venous structures, and by the presence of a physiological vascular waterfall (VW) that disconnects the arterial and the venous circulation in two pressure systems. The objectives of this study were to describe the VW in patients with arterial hypotension due to vasodilatory circulatory shock, and its behavior according to its response to norepinephrine infusion.

Factors Affecting Health-Related Quality of Life among Survivors of Non-Hodgkin Lymphoma: A Population-Based Study.

Purpose: To describe Health-Related Quality of Life (HRQoL) and to identify the association between sociodemographic, clinical and psychosocial factors, and self-reported HRQoL among NHL survivors.

Methods: The data of the cancer registry specialized in hematological malignancies in Côte d'Or (France) were used to identify all patients diagnosed with follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL) from 2010 to 2017. Patients were invited to complete SF-12 and other questionnaires.

Regulatory T-cell dysfunctions are associated with increase in tumor necrosis factor α in autoimmune hemolytic anemia and participate in Th17 polarization.

Warm autoimmune hemolytic anemia (wAIHA) is a rare acquired autoimmune disease mediated by antibodies targeting red blood cells. The involvement of CD4 T-helper cells has been scarcely explored, with most findings extrapolated from animal models. Here, we performed quantification of both effector T lymphocytes (Teff) and regulatory T cells (Treg), associated with functional and transcriptomic analyses of Treg in human wAIHA.

Healing Treatments in COVID-19 Patients: A Narrative Review.

Since December 2019, many drugs have been evaluated or advocated as potential treatments of SARS-CoV-2 induced disease (COVID-19), including many repositioned drugs and some others specifically developed for these diseases. They can be roughly classified into three categories according to their main mechanism of action (passive immunization, direct antivirals, and anti-inflammatory treatments), and their use depends on the stage of the disease. Despite often promising preclinical data, most of the treatments evaluated failed to show a significant clinical benefit.

Dynamic full-field optical coherence tomography for live-cell imaging and growth-phase monitoring in .

Introduction: The diagnosis of cutaneous manifestations of deep mycoses relies on both histopathological and direct examinations. Yet, the current diagnostic criteria cannot prevent missed cases, including invasive aspergillosis, which requires the development of a novel diagnostic approach and imaging tools. We recently introduced the use of dynamic full-field optical coherence tomography (D-FF-OCT) in fungal diagnostics with a definition approaching that of conventional microscopy and the ability to return metabolic information regarding different fungal species.

The biological sample collection of the OFSEP French MS registry: An essential tool dedicated to researchers.

Today's medicine strives to be personalized, preventive, predictive and participatory. This implies to have access to multimodal data to better characterize patients groups and to combine clinical and imaging data with high-quality biological samples. Collecting such data is one of the objectives of the Observatoire français de la sclérose en plaques (OFSEP), the French MS registry.

Associated Factors to Efficacy and Tolerance of Immunotherapy in Older Patients with Cancer Aged 70 Years and Over: Impact of Coprescriptions.

Background: Immunotherapy with immune checkpoint blockers (ICB) significantly improves the prognosis for an increasing number of cancers. However, data on geriatric populations taking ICB are rare.

Objective: This study aimed to identify factors associated with the efficacy and tolerance of ICB in an older population.

Delayed diagnosis of pneumonia in the emergency department: factors associated and prognosis.

Introduction: Whether a delayed diagnosis of community-acquired pneumonia (CAP) in the emergency department (ED) is associated with worse outcome is uncertain. We sought factors associated with a delayed diagnosis of CAP in the ED and those associated with in-hospital mortality.

Methods: Retrospective study including all inpatients admitted to an ED (Dijon University Hospital, France) from 1 January to 31 December 2019, and hospitalized with a diagnosis of CAP.

Tandem versus single haematopoietic stem cell transplant and BV maintenance in relapsed/refractory Hodgkin lymphoma: A matched cohort analysis from the LYSA.

Autologous hematopoietic stem cell transplant (ASCT) is the standard curative treatment for patients with high-risk relapsed/refractory Hodgkin lymphoma (R/R HL). The AETHERA study showed survival gain with Brentuximab Vedotin (BV) maintenance after ASCT in BV-naive patients, which was recently confirmed in the retrospective AMAHRELIS cohort, including a majority of BV-exposed patients. However, this approach has not been compared to intensive tandem auto/auto or auto/allo transplant strategies, which were used before BV approval.

Intestinal Candida albicans overgrowth in IgA deficiency.

Background: Secretory IgA interacts with commensal bacteria, but its impact on human mycobiota ecology has not been widely explored. In particular, whether human IgA-deficiency is associated with gut fungal dysbiosis remains unknown.

Objectives: Our goal was to study the impact of IgA on gut mycobiota ecology.

Automatic classification of patients with myocardial infarction or myocarditis based only on clinical data: A quick response.

Background: In acute cardiovascular disease management, the delay between the admission in a hospital emergency department and the assessment of the disease from a Delayed Enhancement cardiac MRI (DE-MRI) scan is one of the barriers for an immediate management of patients with suspected myocardial infarction or myocarditis.

Objectives: This work targets patients who arrive at the hospital with chest pain and are suspected of having a myocardial infarction or a myocarditis. The main objective is to classify these patients based solely on clinical data in order to provide an early accurate diagnosis.

Effectiveness of mogamulizumab in patients with Mycosis Fungoides or Sézary syndrome: A multicentre, retrospective, real-world French study.

Background: Efficacy and safety of mogamulizumab, a monoclonal antibody directed against C-C chemokine receptor 4, were demonstrated in a previous multinational clinical trial conducted in patients with previously treated cutaneous T-cell lymphoma (CTCL): Sézary syndrome (SS) or Mycosis Fungoides (MF).

Objectives: The real-world French OMEGA study aimed to describe effectiveness and tolerability of mogamulizumab in adult patients with CTCL, overall and according to the disease (SS or MF).

Methods: In this retrospective study, patients treated with mogamulizumab for SS or MF were included from 14 French expert centres.

Locomotor Strategy to Perform 6-Minute Walk Test in People with Multiple Sclerosis: A Prospective Observational Study.

Two-thirds of people with Multiple Sclerosis (PwMS) have walking disabilities. Considering the literature, prolonged tests, such as the 6 min walk test, better reflect their everyday life walking capacities and endurance. However, in most studies, only the distance traveled during the 6MWT was measured.

YWHAE loss of function causes a rare neurodevelopmental disease with brain abnormalities in human and mouse.

Purpose: Miller-Dieker syndrome is caused by a multiple gene deletion, including PAFAH1B1 and YWHAE. Although deletion of PAFAH1B1 causes lissencephaly unambiguously, deletion of YWHAE alone has not clearly been linked to a human disorder.

Methods: Cases with YWHAE variants were collected through international data sharing networks.

A new cure model that corrects for increased risk of non-cancer death: analysis of reliability and robustness, and application to real-life data.

Background: Non-cancer mortality in cancer patients may be higher than overall mortality in the general population due to a combination of factors, such as long-term adverse effects of treatments, and genetic, environmental or lifestyle-related factors. If so, conventional indicators may underestimate net survival and cure fraction. Our aim was to propose and evaluate a mixture cure survival model that takes into account the increased risk of non-cancer death for cancer patients.

Coronary-subclavian steal syndrome causing myocardial infarction after arteriovenous fistula creation: a case report.

Coronary subclavian steal syndrome (CSSS) caused by left subclavian artery (LSA) stenosis is a rare cause of myocardial infarction in patients having coronary artery bypass grafting (CABG), and it has also been observed after an arteriovenous fistula (AVF) was made. A 79-year-old woman who had undergone CABG years earlier and an AVF creation 1 month before experienced a non-ST-elevation myocardial infarction (NSTEMI). While selective catheterization of the left internal thoracic artery graft was impossible, a computed tomography scanner showed patency of all bypasses and proximal subocclusive LSA stenosis, and the digital blood pressure measurements objectified a haemodialysis-induced distal ischaemia.