A transcriptomic continuum of differentiation arrest identifies myeloid interface acute leukemias with poor prognosis.

Classification of acute lymphoblastic and myeloid leukemias (ALL and AML) remains heavily based on phenotypic resemblance to normal hematopoietic precursors. This framework can provide diagnostic challenges for immunophenotypically heterogeneous immature leukemias, and ignores recent advances in understanding of developmental multipotency of diverse normal hematopoietic progenitor populations that are identified by transcriptional signatures. We performed transcriptional analyses of a large series of acute myeloid and lymphoid leukemias and detected significant overlap in gene expression between cases in different diagnostic categories.

Child-Adult Transition in Sarcoidosis: A Series of 52 Patients.

(1) Background: Pediatric sarcoidosis is a rare and mostly severe disease. Very few pediatric series with a prolonged follow-up are reported. We aimed to evaluate the evolution of pediatric sarcoidosis in adulthood.

The effect of exposure to long working hours on stroke: A systematic review and meta-analysis from the WHO/ILO Joint Estimates of the Work-related Burden of Disease and Injury.

Background: The World Health Organization (WHO) and the International Labour Organization (ILO) are developing joint estimates of the work-related burden of disease and injury (WHO/ILO Joint Estimates), with contributions from a large network of individual experts. Evidence from mechanistic data and prior studies suggests that exposure to long working hours may cause stroke. In this paper, we present a systematic review and meta-analysis of parameters for estimating the number of deaths and disability-adjusted life years from stroke that are attributable to exposure to long working hours, for the development of the WHO/ILO Joint Estimates.

Increased Prevalence of Q703K Variant Among Patients With Autoinflammatory Diseases: An International Multicentric Study.

The inflammasome has been recognized as one of the key components of innate immunity. Gain-of-function mutations in the exon 3 of gene have been implicated in inflammatory diseases suggesting the presence of functionally important sites in this region. Q703K (c.

Allostatic load, emotional hyper-reactivity, and functioning in individuals with bipolar disorder.

Objectives: Diagnosis and management of bipolar disorder (BD) are limited by the absence of available biomarkers. Allostatic load (AL) represents the strain that stress, including the effects of acute phases and inter-episode chronic mood instability, exerts on interconnected biological systems. This study aimed to operationalize an AL index and explore whether it could be relevant to better characterize BD patients with and without emotional hyper-reactivity particularly those at higher risk of immune-cardiometabolic dysregulation and functional impairment.

Early and very early-onset schizophrenia compared with adult-onset schizophrenia: French FACE-SZ database.

Objective: To compare the clinical symptomatology in patients with Early-Onset Schizophrenia (EOS, N = 176), especially the subgroup Very Early Onset Schizophrenia (VEOS) and Adult Onset Schizophrenia (AOS, N = 551).

Method: In a large French multicentric sample, 727 stable schizophrenia patients, classified by age at onset of the disorder, were assessed using standardized and extensive clinical and neuropsychological batteries: AOS with onset ≥ 18 years and EOS with onset < 18 years (including 22 VEOS < 13 years).

Results: The importance of better diagnosing EOS group, and in particularly VEOS, appeared in a longer DUP Duration of Untreated Psychosis (respectively, 2.

Confirmation of a Two-Factor Solution to the Questionnaire of Cognitive and Affective Empathy in a French Population of Patients With Schizophrenia Spectrum Disorders.

The Questionnaire of Cognitive and Affective Empathy (QCAE) is a tool for self-assessing the cognitive and emotional components of empathy. A study showed that a two-factor model fits the data of patients with schizophrenia, whereas other reports on healthy subjects have suggested a five-factor decomposition. We aimed to replicate the model of et al.

Targeted Temperature Management for Cardiac Arrest with Nonshockable Rhythm.

Background: Moderate therapeutic hypothermia is currently recommended to improve neurologic outcomes in adults with persistent coma after resuscitated out-of-hospital cardiac arrest. However, the effectiveness of moderate therapeutic hypothermia in patients with nonshockable rhythms (asystole or pulseless electrical activity) is debated.

Methods: We performed an open-label, randomized, controlled trial comparing moderate therapeutic hypothermia (33°C during the first 24 hours) with targeted normothermia (37°C) in patients with coma who had been admitted to the intensive care unit (ICU) after resuscitation from cardiac arrest with nonshockable rhythm.

Human Autoantibodies Against N-Methyl-D-Aspartate Receptor Modestly Alter Dopamine D1 Receptor Surface Dynamics.

Circulating autoantibodies directed against extracellular domains of the glutamatergic N-methyl-D-aspartate receptors (NMDAR-Ab) elicit psychotic symptoms in humans and behavioral deficits in animal models. Recent advances suggest that NMDAR-Ab exert their pathogenic action by altering the trafficking of NMDAR, which results in a synaptic NMDAR hypofunction consistent with the consensual glutamatergic hypothesis of psychotic disorders. Yet, dysfunction in the dopaminergic signaling is also proposed to be at the core of psychotic disorders.

Longitudinal relationships between cognition and functioning over 2 years in euthymic patients with bipolar disorder: a cross-lagged panel model approach with the FACE-BD cohort.

Background: Longitudinal studies of the relationship between cognition and functioning in bipolar disorder are scarce, although cognition is thought to be a key determinant of functioning. The causal structure between cognition and psychosocial functioning in bipolar disorder is unknown.

Aims: We sought to examine the direction of causality between cognitive performance and functional outcome over 2 years in a large cohort of euthymic patients with bipolar disorder.

Predictors of clinically urgent intracranial pathology at neuroimaging in children with complex febrile seizures: a retrospective cross-sectional study.

Aim: To assess the prevalence of clinically urgent intracranial pathology (CUIP) in children visiting the emergency department with a complex febrile seizure (CFS).

Methods: Retrospective cohort review. We analysed the visits of patients for a CFS from January 2007 to December 2011 in seven paediatric emergency departments.

Angiography and Percutaneous Coronary Intervention for Chronic Total Coronary Occlusion in Daily Practice (from a Large French Registry [CARDIO-ARSIF]).

The aim of this study was to provide contemporary data on chronic total occlusion (CTO) prevalence and management in a large unselected population representing the daily activity of cathlabs, in the greater Paris area, and to compare percutaneous coronary intervention (PCI) features in patients with and without CTO. Procedures were collected from the CARDIO-ARSIF (Agence Régionale de Santé Ile de France) registry from 2012 to 2015. Patients with acute coronary syndrome or previous coronary artery bypass grafting were excluded.

Air pollution and humidity as triggering factors for stroke. Results of a 12-year analysis in the West Paris area.

Background And Purpose: Previous studies have suggested an association between stroke and meteorological factors, air pollution and acute respiratory infections as triggering factors. Often, these factors have been evaluated separately. We evaluated the association between all these environmental triggering factors and calls for suspected stroke in a suburb in west Paris from 2004 to 2015.

Classification criteria for autoinflammatory recurrent fevers.

Background: Different diagnostic and classification criteria are available for hereditary recurrent fevers (HRF)-familial Mediterranean fever (FMF), tumour necrosis factor receptor-associated periodic fever syndrome (TRAPS), mevalonate kinase deficiency (MKD) and cryopyrin-associated periodic syndromes (CAPS)-and for the non-hereditary, periodic fever, aphthosis, pharyngitis and adenitis (PFAPA). We aimed to develop and validate new evidence-based classification criteria for HRF/PFAPA.

Methods: Step 1: selection of clinical, laboratory and genetic candidate variables; step 2: classification of 360 random patients from the Eurofever Registry by a panel of 25 clinicians and 8 geneticists blinded to patients' diagnosis (consensus ≥80%); step 3: statistical analysis for the selection of the best candidate classification criteria; step 4: nominal group technique consensus conference with 33 panellists for the discussion and selection of the final classification criteria; step 5: cross-sectional validation of the novel criteria.

Early blood transcriptomic signature predicts patients' outcome after out-of-hospital cardiac arrest.

Background: Early prognostication is a major challenge after out-of-hospital cardiac arrest (OHCA).

Aims: We hypothesized that a genome-wide analysis of blood gene expression could offer new prognostic tools and lines of research.

Methods: Sixty-nine patients were enrolled from an ancillary study of the clinical trial NCT00999583 that tested the effect of erythropoietin (EPO) after OHCA.

Prevalence of and Risk Factors for Extrapyramidal Side Effects of Antipsychotics: Results From the National FACE-SZ Cohort.

Background: Extrapyramidal side effects (EPS) have been identified as a complication of antipsychotic treatment. Previous meta-analyses have investigated EPS prevalence and risk factors in randomized clinical trials with highly selected patients, but studies in real-world schizophrenia are missing.

Objective: To examine the prevalence and clinical correlates associated with EPS in a nonselected national multicenter sample of stabilized patients with schizophrenia.

The European network for care of children with paediatric rheumatic diseases: care across borders.

Objectives: To provide an overview of the paediatric rheumatology (PR) services in Europe, describe current delivery of care and training, set standards for care, identify unmet needs and inform future specialist service provision.

Methods: An online survey was developed and presented to national coordinating centres of the Paediatric Rheumatology International Trials Organisation (PRINTO) (country survey) and to individual PR centres (centre and disease surveys) as a part of the European Union (EU) Single Hub and Access point for paediatric Rheumatology in Europe project. The survey contained components covering the organization of PR care, composition of teams, education, health care and research facilities and assessment of needs.

Decision to deploy coronary reperfusion is not affected by the volume of ST-segment elevation myocardial infarction patients managed by prehospital emergency medical teams.

Objective: Mortality in patients with ST-segment elevation myocardial infarction (STEMI) has been associated with the volume of activity of percutaneous coronary intervention (PCI) facilities. This observational study investigated whether the coronary reperfusion-decision rate is associated with the volume of activity in a prehospital emergency setting.

Methods: Prospectively collected data for the period 2003-2013 were extracted from a regional registry of all STEMI patients handled by eight dispatch centers (SAMUs) in and around Paris [41 mobile ICU (MICUs)].

Incidence, Mortality, and Outcome-Predictors of Sudden Cardiac Arrest Complicating Myocardial Infarction Prior to Hospital Admission.

Background: Mortality of ST-segment-elevation myocardial infarction (STEMI) decreased drastically, mainly through reduction in inhospital mortality. Prehospital sudden cardiac arrest (SCA) became one of the most feared complications. We assessed the incidence, outcome, and prognosis' predictors of prehospital SCA occurring after emergency medical services (EMS) arrival.