snRNA-seq analysis in multinucleated myogenic FSHD cells identifies heterogeneous FSHD transcriptome signatures associated with embryonic-like program activation and oxidative stress-induced apoptosis.

The sporadic nature of DUX4 expression in FSHD muscle challenges comparative transcriptome analyses between FSHD and control samples. A variety of DUX4 and FSHD-associated transcriptional changes have been identified, but bulk RNA-seq strategies prohibit comprehensive analysis of their spatiotemporal relation, interdependence and role in the disease process. In this study, we used single-nucleus RNA-sequencing of nuclei isolated from patient- and control-derived multinucleated primary myotubes to investigate the cellular heterogeneity in FSHD.

SMCHD1 and LRIF1 converge at the FSHD-associated D4Z4 repeat and LRIF1 promoter yet display different modes of action.

Facioscapulohumeral muscular dystrophy (FSHD) is caused by the epigenetic derepression of the 4q-linked D4Z4 macrosatellite repeat resulting in inappropriate expression of the D4Z4 repeat-encoded DUX4 gene in skeletal muscle. In 5% of FSHD cases, D4Z4 chromatin relaxation is due to germline mutations in one of the chromatin modifiers SMCHD1, DNMT3B or LRIF1. The mechanism of SMCHD1- and LRIF1-mediated D4Z4 repression is not clear.

Practice testing of generic quality indicators for responsible antibiotic use in nine hospitals in the Dutch-Belgian border area.

Background: Inpatient quality indicators (IQIs) were previously developed to assess responsible antibiotic use.

Aim: Practice testing of these QIs in the hospital setting.

Method: This study was performed within a Dutch-Belgian border network of hospitals implementing the Infection Risk Scan (IRIS) point prevalence survey (PPS) as part of the i-4-1-Health project.

A proteomics study identifying interactors of the FSHD2 gene product SMCHD1 reveals RUVBL1-dependent DUX4 repression.

Structural Maintenance of Chromosomes Hinge Domain Containing 1 (SMCHD1) is a chromatin repressor, which is mutated in > 95% of Facioscapulohumeral dystrophy (FSHD) type 2 cases. In FSHD2, SMCHD1 mutations ultimately result in the presence of the cleavage stage transcription factor DUX4 in muscle cells due to a failure in epigenetic repression of the D4Z4 macrosatellite repeat on chromosome 4q, which contains the DUX4 locus. While binding of SMCHD1 to D4Z4 and its necessity to maintain a repressive D4Z4 chromatin structure in somatic cells are well documented, it is unclear how SMCHD1 is recruited to D4Z4, and how it exerts its repressive properties on chromatin.

Feedback of ATP measurement as a tool for reducing environmental contamination in hospitals in the Dutch/Belgian border area.

Design: A two-phase prospective intervention study.

Objective: The objective of this study was to determine if feedback of adenosine triphosphate (ATP) measurements decreases environmental contamination within hospitals in the Dutch/Belgian border area.

Methods: Standardized ATP measurements were conducted in nine hospitals on pre-defined fomites.

The correlation between ATP measurement and microbial contamination of inanimate surfaces.

Background: The objective of this study was to determine the correlation between adenosine triphosphate (ATP) measurements and microbial contamination using a standardized method. Secondarily, analyzing reproducibility of ATP measurements and aerobic colony counts (ACC's) on the same surface.

Methods: ATP measurements and ACC's were conducted on 10 pre-defined fomites in a hospital and nursing home setting.

ATP measurement as an objective method to measure environmental contamination in 9 hospitals in the Dutch/Belgian border area.

Background: The objective of this study was to determine the level of environmental contamination in hospitals in the Dutch/Belgian border area, using ATP measurements.

Design: A cross-sectional observational survey.

Methods: Standardized ATP measurements were conducted in 9 hospitals on 32 hospital wards.

Decreasing prevalence of contamination with extended-spectrum beta-lactamase-producing Enterobacteriaceae (ESBL-E) in retail chicken meat in the Netherlands.

Retail chicken meat is a potential source of extended-spectrum beta-lactamase-producing Enterobacteriaceae (ESBL-E). In the past decade, vast national efforts were undertaken to decrease the antibiotic use in the veterinary sector, resulting in a 58% decrease in antibiotic sales in the sector between 2009 and 2014. This decrease in antibiotic use was followed by a decrease in ESBL-E prevalence in broilers.

Comparison of infection control practices in a Dutch and US hospital using the infection risk scan (IRIS) method.

Background: The infection risk scan (IRIS) is a tool to measure the quality of infection control (IC) and antimicrobial use in a standardized way. We describe the feasilibility of the IRIS in a Dutch hospital (the Netherlands, NL) and a hospital in the United States (US).

Methods: Cross-sectional measurements were performed.

Intronic variants in FSHD: testing the potential for CRISPR-Cas9 genome editing.

Background: Facioscapulohumeral dystrophy (FSHD) is associated with partial chromatin relaxation of the retrogene containing D4Z4 macrosatellite repeats on chromosome 4, and transcriptional de-repression of in skeletal muscle. The common form of FSHD, FSHD1, is caused by a D4Z4 repeat array contraction. The less common form, FSHD2, is generally caused by heterozygous variants in .

Decline in AmpC β-lactamase-producing Escherichia coli in a Dutch teaching hospital (2013-2016).

Objective: The objective of this study is to determine the prevalence of rectal carriage of plasmid- and chromosome-encoded AmpC β-lactamase-producing Escherichia coli and Klebsiella spp. in patients in a Dutch teaching hospital between 2013 and 2016.

Methods: Between 2013 and 2016, hospital-wide yearly prevalence surveys were performed to determine the prevalence of AmpC β-lactamase-producing E.

Extended-spectrum beta-lactamase producing Enterobacteriaceae (ESBL-E) isolated from bean sprouts in the Netherlands.

Community-acquired carriage and infections due to extended-spectrum beta-lactamase producing Enterobacteriaceae (ESBL-E) are increasing worldwide, resulting in increased morbidity, mortality and healthcare costs. The origins of community-acquired ESBL-E carriage and infections remain unclear. Bean sprouts are a potential source of Enterobacteriaceae for the community, as illustrated by outbreaks of pathogenic Enterobacteriaceae in the past.

The infection risk scan (IRIS): standardization and transparency in infection control and antimicrobial use.

Background: Infection control needs user-friendly standardized instruments to measure the compliance to guidelines and to implement targeted improvement actions. This abstract describes a tool to measure the quality of infection control and antimicrobial use, the Infection Risk Scan (IRIS). It has been applied in a hospital, several nursing homes and a rehabilitation clinic in the Netherlands.

[The Infection RIsk Scan in clinical practice: improving infection prevention and antibiotic use through transparency].

Objective: The increase in Highly Resistant Micro-Organisms (HRMO) in hospitals and nursing homes requires an intensification of infection prevention measures. This paper describes a new, standardised approach to infection prevention and monitoring of antibiotic use.

Design: Cross-sectional, observational study.

Trends in Extended Spectrum Beta-Lactamase (ESBL) Producing Enterobacteriaceae and ESBL Genes in a Dutch Teaching Hospital, Measured in 5 Yearly Point Prevalence Surveys (2010-2014).

This paper describes the trends in prevalence of ESBL producing Enterobacteriaceae (ESBL-E) and ESBL genes, measured in five consecutive yearly Point Prevalence Surveys (PPS). All patients present in the hospital and in a day-care clinic (including patients on dialysis) on the day of the survey, were screened for perianal ESBL-E carriage. Perianal swabs were taken and cultured using an enrichment broth and a selective agar plate.

Retrospective identification of a previously undetected clinical case of OXA-48-producing K. pneumoniae and E. coli: the importance of adequate detection guidelines.

Introduction: The laboratory detection of OXA-48-carbapenemase-producing Enterobacteriaceae is difficult, as minimum inhibition concentrations for carbapenems are often below the clinical breakpoint. In 2011, the Dutch national guideline for the detection of highly resistant micro-organisms was issued, which includes recommendations on the use of carbapenem screening breakpoints for the detection of carbapenemase-producing Enterobacteriaceae.

Materials And Methods: During a validation study of the Check-MDR CT103 microarray (Check-Points, Wageningen, The Netherlands) in 2013, an OXA-48-like carbapenemase gen was identified in two isolates that were previously obtained from a patient with non-Hodgkin lymphoma in 2007.

Measuring the quality of infection control in Dutch nursing homes using a standardized method; the Infection prevention RIsk Scan (IRIS).

Background: We developed a standardised method to assess the quality of infection control in Dutch Nursing Home (NH), based on a cross-sectional survey that visualises the results. The method was called the Infection control RIsk Infection Scan (IRIS). We tested the applicability of this new tool in a multicentre surveillance executed June and July 2012.

Next-generation sequencing for typing and detection of resistance genes: performance of a new commercial method during an outbreak of extended-spectrum-beta-lactamase-producing Escherichia coli.

Next-generation sequencing (NGS) has the potential to provide typing results and detect resistance genes in a single assay, thus guiding timely treatment decisions and allowing rapid tracking of transmission of resistant clones. We evaluated the performance of a new NGS assay (Hospital Acquired Infection BioDetection System; Pathogenica) during an outbreak of sequence type 131 (ST131) Escherichia coli infections in a nursing home in The Netherlands. The assay was performed on 56 extended-spectrum-beta-lactamase (ESBL) E.

Detection and occurrence of plasmid-mediated AmpC in highly resistant gram-negative rods.

Objectives: The aim of this study was to compare the current screening methods and to evaluate confirmation tests for phenotypic plasmidal AmpC (pAmpC) detection.

Methods: For this evaluation we used 503 Enterobacteriaceae from 18 Dutch hospitals and 21 isolates previously confirmed to be pAmpC positive. All isolates were divided into three groups: isolates with 1) reduced susceptibility to ceftazidime and/or cefotaxime; 2) reduced susceptibility to cefoxitin; 3) reduced susceptibility to ceftazidime and/or cefotaxime combined with reduced susceptibility to cefoxitin.