1,225 research outputs found
Safety, tolerability and pharmacokinetics of eteplirsen in young boys aged 6-48 months with Duchenne muscular dystrophy amenable to exon 51 skipping
Eteplirsen is FDA-approved for the treatment of Duchenne muscular dystrophy (DMD) in exon 51 skip-amenable patients. Previous studies in boys > 4 years of age indicate eteplirsen is well tolerated and attenuates pulmonary and ambulatory decline compared with matched natural history cohorts. Here the safety, tolerability and pharmacokinetics of eteplirsen in boys aged 6â48 months is evaluated. In this open-label, multicenter, dose-escalation study (NCT03218995), boys with a confirmed mutation of the DMD gene amenable to exon 51 skipping (Cohort 1: aged 24â48 months, n = 9; Cohort 2: aged 6 to 4 years of age. These data support the safety and tolerability of eteplirsen at the approved 30-mg/kg dose in boys as young as 6 months old
Droplet Generation by Disintegration of Oil Films at the Rim of a Rotating Disk
ABSTRACT A fundamental study has been performed to examine oil film disintegration mechanisms at the rim of a rotating disk. The configuration investigated is an abstraction of one of the droplet generation sources in an aero-engine bearing compartment. The paper aims to contribute to both the determination of directly applicable droplet characteristics and the establishment of a data-base that can be used for the development of droplet generation models. The near-term objectives of the study are (i) to identify disintegration modes relevant with respect to aeroengine bearing compartment operating conditions, (ii) to determine droplet sizes under those operating conditions, and (iii) to measure individual droplet diameter/velocity relationships. The long-term objective is to incorporate this information into advanced CFD-based design tools. The disintegration modes identified here were similar to previously reported flow regimes generated by rotary atomizer
Evidence for field enhanced electron capture by EL2 centers in semiâinsulating GaAs and the effect on GaAs radiation detectors
The performance of Schottky contact semiconductor radiation detectors fabricated from semiâinsulating GaAs is highly sensitive to charged impurities and defects in the material. The observed behavior of semiâinsulating GaAs Schottky barrier alpha particle detectors does not match well with models that treat the semiâinsulating material as either perfectly intrinsic or as material with deep donors (EL2) of constant capture cross section compensated with shallow acceptors. We propose an explanation for the discrepancy based on enhanced capture of electrons by EL2 centers at high electric fields and the resulting formation of a quasineutral region in the GaAs. Presented is a simple model including field enhanced electron capture which shows good agreement with experimental alpha particle pulse height measurements.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/71231/2/JAPIAU-75-12-7910-1.pd
The variability of song variability in zebra finch (Taeniopygia guttata) populations
Birdsong is a classic example of a learned social behaviour. Song behaviour is also influenced by genetic factors, and understanding the relative contributions of genetic and environmental influences remains a major goal. In this study, we take advantage of captive zebra finch populations to examine variation in a population-level song trait: song variability. Song variability is of particular interest in the context of individual recognition and in terms of the neuro-developmental mechanisms that generate song novelty. We find that the Australian zebra finch Taeniopygia guttata castanotis (TGC) maintains higher song diversity than the Timor zebra finch T. g. guttata (TGG) even after experimentally controlling for early life song exposure, suggesting a genetic basis to this trait. Although wild-derived TGC were intermediate in song variability between domesticated TGC populations and TGG, the difference between domesticated and wild TGC was not statistically significant. The observed variation in song behaviour among zebra finch populations represents a largely untapped opportunity for exploring the mechanisms of social behaviour
The European Hernia Society Prehabilitation Project: A Systematic Review of Intra-Operative Prevention Strategies for Surgical Site Occurrences in Ventral Hernia Surgery
Abdominal wall repair; Hernia; Surgical site infectionReparaciĂłn de la pared abdominal; Hernia; InfecciĂłn del sitio quirĂșrgicoReparaciĂł de la paret abdominal; HĂšrnia; InfecciĂł del lloc quirĂșrgicBackground: Ventral hernia repair is one of the most commonly performed surgical procedures worldwide. To reduce the risk of complications, pre- and intra-operative strategies have received increasing focus in recent years. To assess possible preventive surgical strategies, this European Hernia Society endorsed project was launched. The aim of this review was to evaluate the current literature focusing on pre- and intra-operative strategies for surgical site occurrences (SSO) and specifically surgical site infection (SSI) in ventral hernia repair.
Methods: A systematic review was conducted and reported in line with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. Databases used were Pubmed and Web of Science. Original retrospective or prospective human adult studies describing at least one intra-operative intervention to reduce SSO after ventral hernia repair were considered eligible.
Results: From a total of 4775 results, a total of 18 papers were considered suitable after full text reading. Prehospital chlorhexidine gluconate (CHG) scrub appears to increase the risk of SSO in patients undergoing ventral hernia repair, while there is no association between any type of surgical hat worn and the incidence of postoperative wound events. Intraoperative measures as prophylactic negative pressure therapy, surgical drain placement and the use of quilt sutures seem beneficial for decreasing the incidence of SSO and/or SSI. No positive effect has been shown for antibiotic soaking of a synthetic mesh, nor for the use of fibrin sealants.
Conclusion: This review identified a limited amount of literature describing specific preventive measures and techniques during ventral hernia repair. An advantage of prophylactic negative pressure therapy in prevention of SSI was observed, but different tools to decrease SSIs and SSOs continuously further need our full attention to improve patient outcomes and to lower overall costs
The First Detections of the Extragalactic Background Light at 3000, 5500, and 8000A (II): Measurement of Foreground Zodiacal Light
We present a measurement of the absolute surface brightness of the zodiacal
light (3900-5100A) toward a fixed extragalactic target at high ecliptic
latitude based on moderate resolution (~1.3A per pixel) spectrophotometry
obtained with the du Pont 2.5m telescope at Las Campanas Observatory in Chile.
This measurement and contemporaneous Hubble Space Telescope data from WFPC2 and
FOS comprise a coordinated program to measure the mean flux of the diffuse
extragalactic background light (EBL). The zodiacal light at optical wavelengths
results from scattering by interplanetary dust, so that the zodiacal light flux
toward any extragalactic target varies seasonally with the position of the
Earth. This measurement of zodiacal light is therefore relevant to the specific
observations (date and target field) under discussion. To obtain this result,
we have developed a technique that uses the strength of the zodiacal Fraunhofer
lines to identify the absolute flux of the zodiacal light in the
multiple-component night sky spectrum. Statistical uncertainties in the result
are 0.6% (1 sigma). However, the dominant source of uncertainty is systematic
errors, which we estimate to be 1.1% (1 sigma). We discuss the contributions
included in this estimate explicitly. The systematic errors in this result
contribute 25% in quadrature to the final error in our coordinated EBL
measurement, which is presented in the first paper of this series.Comment: Accepted for publication in ApJ, 22 pages using emulateapj.sty,
version with higher resolution figures available at
http://www.astro.lsa.umich.edu/~rab/publications.html or at
http://nedwww.ipac.caltech.edu/level5/Sep01/Bernstein2/frames.htm
Protocol for a randomised controlled trial of fetal scalp blood lactate measurement to reduce caesarean sections during labour: the Flamingo trial [ACTRN12611000172909]
The end of the beginning? Taking forward local democratic renewal in the post-referendum North East.
This article draws upon the authorâs commissioned research on the nature of regional governance following the 2004 Referendum in the North East on elected regional assemblies. The article aimed to both capture these views and to assess how the âNo vote in the referendum has impacted on subsequent developments in sub-national governance. The article provides both an empirical overview of recent developments and engages with the wider conceptual debates on democratic renewal. The arguments covered in this output are aimed at both academic and practitioner audiences, and have been also disseminated at regional and national conferences
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