Familial Mediterranean fever mutations are hypermorphic mutations that specifically decrease the activation threshold of the Pyrin inflammasome.

Objectives: FMF is the most frequent autoinflammatory disease and is associated in most patients with bi-allelic MEFV mutations. MEFV encodes Pyrin, an inflammasome sensor activated following RhoGTPase inhibition. The functional consequences of MEFV mutations on the ability of Pyrin variants to act as inflammasome sensors are largely unknown.

Prevalence of work-related common psychiatric disorders in primary care: The French Héraclès study.

General practitioners (GP), on the frontline for individuals with mental health problems, often deal with work-related common psychiatric disorders. We aimed to determine the prevalence of work-related common psychiatric disorders in general practice and associated patients' and GPs' characteristics. HERACLES, a cross-sectional study among 2019 working patients of 121 GPs in the Nord - Pas-de-Calais region in France.

"It Was the Only Thing I Could Hold Onto, But…": Receiving a Letter of Condolence After Loss of a Loved One in the ICU: A Qualitative Study of Bereaved Relatives' Experience.

Objectives: Family members of patients who die in the ICU often remain with unanswered questions and suffer from lack of closure. A letter of condolence may help bereaved relatives, but little is known about their experience of receiving such a letter. The objective of the study was to understand bereaved family members' experience of receiving a letter of condolence.

Time-Course Reduction in Patient Exposure to Radiation From Coronary Interventional Procedures: The Greater Paris Area Percutaneous Coronary Intervention Registry.

Background: The frequency of complex percutaneous coronary interventions (PCIs) has increased in the last few years, with a growing concern on the radiation dose received by the patients. Multicenter data from large unselected populations on patients' radiation doses during coronary angiography (CA) and PCI and temporal trends are lacking. This study sought to evaluate the temporal trends in patients' exposure to radiation from CA and PCI.

Standardizing hysteroscopy teaching: development of a curriculum using the Delphi method.

Background: Hysteroscopy is performed often and in many indications but is challenging to learn. Hands-on training in live patients faces ethical, legal, and economic obstacles. Virtual reality simulation may hold promise as a hysteroscopy training tool.

Weekly high-dose liposomal amphotericin B (L-AmB) in critically ill septic patients with multiple Candida colonization: The AmBiDex study.

Background: To demonstrate the feasibility and safety of weekly high-dose liposomal amphotericin B (L-AmB) (as a pre-emptive antifungal treatment) for 2 weeks in patients with septic shock and Candida colonization.

Methods: Pilot, multicentre, open-label, prospective study conducted in seven French ICUs. Non-immunocompromised patients, receiving mechanical ventilation were eligible if they presented ICU-acquired severe sepsis requiring newly administered antibacterial agents and Candida colonization in at least two sites.

International and multidisciplinary expert recommendations for the use of biologics in systemic lupus erythematosus.

Background/purpose: Despite conventional immunosuppressants, active and steroid-dependent systemic lupus erythematosus (SLE) represents a therapeutic challenge. Only one biologic, belimumab, has been approved, but other biologics are sometimes used off-label. Given the lack of evidence-based data in some clinical situations encountered in real life, we developed expert recommendations for the use of biologics for SLE.

Is 5 days of oral fluoroquinolone enough for acute uncomplicated pyelonephritis? The DTP randomized trial.

The treatment duration of acute uncomplicated pyelonephritis (AUP) is still under debate. As shortening treatment duration could be a means to reduce antimicrobial resistance, we aimed to establish whether 5 days of antibiotic treatment is non-inferior to 10 days in patients with AUP. We performed an open-label prospective randomized trial comparing 5 days to 10 days of fluoroquinolone treatment for AUP.

Do All Children Who Present With a Complex Febrile Seizure Need a Lumbar Puncture?

Study Objective: We assess the prevalences of bacterial meningitis and herpes simplex virus meningoencephalitis (HSV-ME) in children with a complex febrile seizure and determine these prevalences in the subgroup of children with a clinical examination result not suggestive of meningitis or encephalitis.

Methods: This multicenter retrospective study was conducted in 7 pediatric emergency departments (EDs) in the region of Paris, France. Visits of patients aged 6 months to 5 years for a complex febrile seizure from January 2007 to December 2011 were analyzed.

Effect of a condolence letter on grief symptoms among relatives of patients who died in the ICU: a randomized clinical trial.

Purpose: Family members of patients who die in the intensive care unit (ICU) may experience symptoms of stress, anxiety, depression, posttraumatic stress disorder (PTSD), and/or prolonged grief. We evaluated whether grief symptoms were alleviated if the physician and the nurse in charge at the time of death sent the closest relative a handwritten condolence letter.

Methods: Multicenter randomized trial conducted among 242 relatives of patients who died at 22 ICUs in France between December 2014 and October 2015.

Assessment of global longitudinal strain at low-dose anthracycline-based chemotherapy, for the prediction of subsequent cardiotoxicity.

Aims: We sought to assess whether global longitudinal strain (GLS) measured early during treatment with anthracyclines (at a cumulative dose of 150 mg/m2) can predict subsequent alterations in left ventricular ejection fraction.

Methods And Results: Eighty-six patients with Hodgkin's disease, non-Hodgkin's lymphoma, or acute leukaemia and receiving anthracyclines were prospectively included. Patients underwent complete echocardiography on four occasions: baseline (V1); after reaching a cumulative dose of 150 mg/m2 (V2); end of treatment (V3); and 1 year follow-up (V4).

Impact of transfusion on patients with sepsis admitted in intensive care unit: a systematic review and meta-analysis.

Red blood cell transfusion (RBCT) threshold in patients with sepsis remains a matter of controversy. A threshold of 7 g/dL for stabilized patients with sepsis is commonly proposed, although debated. The aim of the study was to compare the benefit and harm of restrictive versus liberal RBCT strategies in order to guide physicians on RBCT strategies in patients with severe sepsis or septic shock.

Type 0 Spinal Muscular Atrophy: Further Delineation of Prenatal and Postnatal Features in 16 Patients.

Background: Spinal muscular atrophy (SMA) is caused by homozygous inactivation of the SMN1 gene. The SMN2 copy number modulates the severity of SMA. The 0SMN1/1SMN2 genotype, the most severe genotype compatible with life, is expected to be associated with the most severe form of the disease, called type 0 SMA, defined by prenatal onset.

The real-life effectiveness of psychosocial therapies on social autonomy in schizophrenia patients: Results from a nationwide cohort study in France.

The objectives of the present study were to describe the prescribing patterns for psychosocial therapies in routine clinical practice and to assess the impact of psychoeducation on symptoms and social autonomy of patients with schizophrenia. We used data from the nationwide French ESPASS observational cohort study including 5967 patients with schizophrenia, which provided data on exposure to psychosocial therapies from 4961 (83%) participants. Patients who initiated psychosocial therapy within the first 3 months of study onset (n=143) were compared to patients not subject to psychosocial therapy throughout follow up (n=4268), using parametric tests.

Development of the autoinflammatory disease damage index (ADDI).

Objectives: Autoinflammatory diseases cause systemic inflammation that can result in damage to multiple organs. A validated instrument is essential to quantify damage in individual patients and to compare disease outcomes in clinical studies. Currently, there is no such tool.

Identifying Patients at Risk for Prehospital Sudden Cardiac Arrest at the Early Phase of Myocardial Infarction: The e-MUST Study (Evaluation en Médecine d'Urgence des Stratégies Thérapeutiques des infarctus du myocarde).

Background: In-hospital mortality of ST-segment-elevation myocardial infarction (STEMI) has decreased drastically. In contrast, prehospital mortality from sudden cardiac arrest (SCA) remains high and difficult to reduce. Identification of the patients with STEMI at higher risk for prehospital SCA could facilitate rapid triage and intervention in the field.

Abnormal rate of intraoperative and postoperative implant positioning outliers using "MRI-based patient-specific" compared to "computer assisted" instrumentation in total knee replacement.

Purpose: The aim of this study was to analyze first intraoperative alignment and reason to abandon the use of patient-specific instrumentation using intraoperative CAS measurement, secondly assess by postoperative CT analysis if CI, based on preoperative 3D-MRI data, improved postoperative component positioning (including femoral rotation) and lower limb alignment as compared with results obtained with CAS.

Methods: In this randomized controlled trial, 80 consecutive patients scheduled to undergo TKA were enrolled. Eligible knees were randomized to the group of PSI-TKAs (n = 40) or to the group of CAS-TKAs (n = 40).

Determination of Adherence Profiles in Schizophrenia Using Self-Reported Adherence: Results From the FACE-SZ Dataset.

Objective: Medication nonadherence is one of the most important, and potentially modifiable, prognostic factors in the outcome of patients with schizophrenia. The aim of this article is to propose a new classification of adherence profiles according to the Medication Adherence Rating Scale (MARS) in a large community-dwelling sample of French patients with schizophrenia to provide a new tool to help clinicians in daily practice.

Methods: 319 community-dwelling patients from a national network of 10 Schizophrenia Expert Centers were interviewed between January 2009 and January 2014.

International Retrospective Chart Review of Treatment Patterns in Severe Familial Mediterranean Fever, Tumor Necrosis Factor Receptor-Associated Periodic Syndrome, and Mevalonate Kinase Deficiency/Hyperimmunoglobulinemia D Syndrome.

Objective: Periodic fever syndrome (PFS) conditions are characterized by recurrent attacks of fever and localized inflammation. This study examined the diagnostic pathway and treatments at tertiary centers for familial Mediterranean fever (FMF), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), and mevalonate kinase deficiency (MKD)/hyperimmunoglobulinemia D syndrome (HIDS).

Methods: PFS specialists at medical centers in the US, the European Union, and the eastern Mediterranean participated in a retrospective chart review, providing de-identified data in an electronic case report form.

Facial Nerve Monitoring During Parotidectomy:A Two-Center Retrospective Study.

Introduction: We present a retrospective two-center study series and discussion of the current literature to assess the benefits of facial nerve monitoring during parotidectomy.

Materials And Methods: From 2007 to 2012, 128 parotidectomies were performed in 125 patients. Of these, 47 procedures were performed without facial nerve monitoring (group 1) and 81 with facial nerve monitoring (group 2).