Systemic lupus erythematosus: state of the art on clinical practice guidelines.

Systemic lupus erythematosus (SLE) is the paradigm of systemic autoimmune diseases characterised by a wide spectrum of clinical manifestations with an unpredictable relapsing-remitting course. The aim of the present work was to identify current available clinical practice guidelines (CPGs) for SLE, to provide their review and to identify physicians' and patients' unmet needs. Twenty-three original guidelines published between 2004 and 2017 were identified.

An unusual presentation of a patient with severe hypogammaglobulinemia.

We present a patient who was diagnosed with severe hypogammaglobulinemia after her newborn child presented with two episodes of meningitis. The patient had no history or symptoms suggestive of immunodeficiency. Thus far, a cause for the immunodeficiency has not been found, even after extensive immunological evaluation.

Design and rationale of the Edoxaban Treatment in routiNe clinical prActice for patients with Atrial Fibrillation in Europe (ETNA-AF-Europe) study.

Aim: Edoxaban, a nonvitamin K antagonist oral anticoagulant, is an oral factor Xa inhibitor approved for the prevention of stroke and systemic embolism in adult patients with atrial fibrillation and for the treatment and secondary prevention in adult patients with venous thromboembolism (VTE). This study details the design of the Edoxaban Treatment in routiNe clinical prActice for patients with Atrial Fibrillation in Europe (ETNA-AF-Europe) study - a postauthorization observational study, which is part of the postapproval plan for edoxaban agreed with the European Medicines Agency.

Methods: The ETNA-AF-Europe study (Clinicaltrials.

Histologic evidence that mast cells contribute to local tissue inflammation in peripheral spondyloarthritis by regulating interleukin-17A content.

Objectives: Synovial mast cells contain IL-17A, a key driver of tissue inflammation in SpA. A recent in vitro study showed that tissue-derived mast cells can capture and release exogenous IL-17A. The present study aimed to investigate if this mechanism could contribute to tissue inflammation in SpA.

Standardisation of synovial biopsy analyses in rheumatic diseases: a consensus of the EULAR Synovitis and OMERACT Synovial Tissue Biopsy Groups.

Background: The aim of this global collaboration was to develop a consensual set of items for the analysis of synovial biopsies in clinical practice and translational research through the EULAR Synovitis Study Group (ESSG) and OMERACT Synovial Tissue Biopsy Group.

Methods: Participants were consulted through a modified Delphi method. Three sequential rounds occurred over 12 months.

Correction to: Diffusion-weighted imaging for assessment of synovial inflammation in juvenile idiopathic arthritis: a promising imaging biomarker as an alternative to gadolinium-based contrast agents.

The original version of this article, published on 12 June 2017, unfortunately contained a mistake.

Forward-viewing echoendoscope versus standard echoendoscope for endoscopic ultrasound-guided tissue acquisition of solid lesions: a randomized, multicenter study.

Background: A forward-viewing linear (FVL) echoendoscope has been developed with the aim of overcoming some of the limitations of standard curved linear-array (CLA) echoendoscopes. There are no existing studies comparing the performance of the two echoendoscopes for endoscopic ultrasound-guided tissue acquisition (EUS-TA) of solid lesions other than subepithelial lesions.

Methods: This was a prospective, multicenter, randomized trial with a noninferiority design comparing FVL vs.

Prevalence of junctional ST-depression with tall symmetrical T-waves in a pre-hospital field triage system for STEMI patients.

Introduction: The prevalence of the junctional ST-depression with tall symmetrical T-waves in a field triage system for ST-Elevation Myocardial Infarction (STEMI) is unknown.

Material And Methods: We prospectively collected all transmitted 12-lead electrocardiograms (ECGs) from the STEMI field triage system in Amsterdam from 2011 to 2013. Electrocardiograms with junctional ST-depression with tall symmetrical T-waves were recognized and angiographic documentation and clinical follow up were collected.

Resuscitation for out-of-hospital cardiac arrest in adults with congenital heart disease.

Aims: Adult congenital heart disease (ACHD) patients are at increased risk of sudden cardiac death and out-of-hospital cardiac arrest (OHCA). Currently, insufficient data exist on outcome, causes and circumstances of OHCA of ACHD patients resuscitated for OHCA. We investigate these parameters in ACHD patients in comparison to OHCA in the general population.

Mixed connective tissue disease: state of the art on clinical practice guidelines.

Mixed connective tissue disease (MCTD) is a complex overlap disease with features of different autoimmune connective tissue diseases (CTDs) namely systemic sclerosis, poly/dermatomyositis and systemic lupus erythematous in patients with antibodies targeting the U1 small nuclear ribonucleoprotein particle. In this narrative review, we summarise the results of a systematic literature research which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. Since no specific CPGs on MCTD were found, other CPGs developed for other CTDs were taken into consideration in order to discuss what can be applied to MCTD even if designed for other diseases.

Systemic sclerosis: state of the art on clinical practice guidelines.

Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality. In this narrative review we summarise the results of a systematic literature research, which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines or recommendations.

First-degree relatives of axial spondyloarthritis patients of the pre-SpA cohort would consider using medication in a preventive setting.

To study the willingness of first-degree relatives of axial spondyloarthritis (axSpA) patients to use preventive medication. First-degree relatives of HLA-B27-positive axSpA patients (pre-SpA cohort) (n = 106) completed a survey including scenarios varying in disease risk, side effects, and treatment effect of hypothetical preventive medication and questions about their perceived risk of developing SpA and assessment of the severity of SpA. The willingness to use preventive medication was 63.

Information exchange between patients with Lynch syndrome and their genetic and non-genetic health professionals: whose responsibility?

Individuals at high risk for Lynch syndrome (LS) should be offered genetic counselling, since preventive options are available. However, uptake of genetic services and follow-up care are currently suboptimal, possibly caused by inadequate exchange of information. Therefore, this qualitative study aims to gain insight in the process of information exchange between patients diagnosed with LS and their non-genetic (i.

The Role of Quantitative Aortographic Assessment of Aortic Regurgitation by Videodensitometry in the Guidance of Transcatheter Aortic Valve Implantation.

Background: Balloon post-dilatation (BPD) is often needed for optimizing transcatheter heart valve (THV) implantation, since paravalvular leak (PVL) after transcatheter aortic valve implantation is associated with poor outcome and mortality. Quantitative assessment of PVL severity before and after BPD is mandatory to properly assess PVL, thus improving implantation results and outcomes.

Objective: To investigate a quantitative angiographic assessment of aortic regurgitation (AR) by videodensitometry before and after BPD.

Cancer survivors not participating in observational patient-reported outcome studies have a lower survival compared to participants: the population-based PROFILES registry.

Purpose: The 'Patient Reported Outcomes Following Initial treatment and Long-term Evaluation of Survivorship' (PROFILES) registry collects patient-reported outcomes (PROs) from short- and long-term cancer survivors in the Netherlands, in a population-based setting. The aim of this analysis is to assess the generalizability of observational PRO research among cancer survivors by comparing socio-demographic and clinical characteristics, and survival of participants and non-participants in cancer survivors invited for questionnaire research through the PROFILES registry.

Methods: Between 2008 and 2015, cancer survivors with different cancer diagnoses (N = 14,011) were invited to participate in PROFILES registry studies, of whom 69% (N = 9684) participated.

In-hospital mortality of patients with atrial arrhythmias: insights from the German-wide Helios hospital network of 161 502 patients and 34 025 arrhythmia-related procedures.

Aims: Atrial fibrillation (AFib) and atrial flutter (AFlut) are common arrhythmias with increased use of invasive procedures. A steady re-evaluation of relevant safety endpoints is recommended and both quality management and pay-for-performance programs are evolving. Therefore, the aims of this study were (i) to investigate and report overall in-hospital mortality and mortality of invasive arrhythmia-related procedures and (ii) to identify mortality predictors in a German-wide hospital network.

Recent Developments in Clinical Terminologies - SNOMED CT, LOINC, and RxNorm.

Objective:  To discuss recent developments in clinical terminologies. SNOMED CT (Systematized Nomenclature of Medicine Clinical Terms) is the world's largest clinical terminology, developed by an international consortium. LOINC (Logical Observation Identifiers, Names, and Codes) is an international terminology widely used for clinical and laboratory observations.

Intervention Descriptions in Medical Education: What Can Be Improved? A Systematic Review and Checklist.

Purpose: Many medical education studies focus on the effectiveness of educational interventions. However, these studies often lack clear, thorough descriptions of interventions that would make the interventions replicable. This systematic review aimed to identify gaps and limitations in the descriptions of educational interventions, using a comprehensive checklist.

Fibroblasts and Osteoblasts in Inflammation and Bone Damage.

This review discusses the important role fibroblasts play in the process of inflammation and the evidence that these cells may drive the persistence of inflammation. Fibroblasts are key components of the stroma normally involved in maintenance of extracellular matrix and tissue function; however, the term 'fibroblast' is used to describe a heterogeneous population of cells that vary in phenotype both between and within anatomical sites. Fibroblasts possess Toll-like receptors allowing them to respond to pathogen and damage-related signals by producing proinflammatory mediators such as IL-6, PGE, and GM-CSF and can produce a range of chemokines such as CXCL12, CXCL13, and CXCL8 which attract B and T lymphocytes, monocytes, and neutrophils to sites of inflammation.

Early Revision Surgery for Tibial Plateau Fractures.

Objective: To describe patients' outcomes after revision of tibial plateau fracture fixation within 12 months of the initial surgical procedure. To assess differences in objective outcome scores [International Knee Documentation Committee (IKDC), short form-36, arthrosis score, physical examination] depending on whether patients were treated for malreduction, malunion, or nonunion.

Design: Retrospective case series.

Trends in cardiovascular and bleeding outcomes in acute coronary syndrome patients treated with or without proton-pump inhibitors during the introduction of novel P2Y12 inhibitors: a five-year experience from a single-centre observational registry.

Aims: Proton-pump inhibitors (PPIs) are commonly prescribed in acute coronary syndrome (ACS) patients on antiplatelet therapy. We studied PPI prescription in ACS patients in the era of novel P2Y12 inhibitors and assessed the association between PPI use and clinical outcomes.

Methods And Results: Between 2010 and 2014, we included all consecutive ACS patients admitted to a Dutch tertiary hospital.

A revised airway epithelial hierarchy includes CFTR-expressing ionocytes.

The airways of the lung are the primary sites of disease in asthma and cystic fibrosis. Here we study the cellular composition and hierarchy of the mouse tracheal epithelium by single-cell RNA-sequencing (scRNA-seq) and in vivo lineage tracing. We identify a rare cell type, the Foxi1 pulmonary ionocyte; functional variations in club cells based on their location; a distinct cell type in high turnover squamous epithelial structures that we term 'hillocks'; and disease-relevant subsets of tuft and goblet cells.