Incidence of adult Huntington's disease in the UK: a UK-based primary care study and a systematic review.

OBJECTIVES: The prevalence of Huntington's disease (HD) recorded in the UK primary care records has increased twofold between 1990 and 2010. This investigation was undertaken to assess whether this might be due to an increased incidence. We have also undertaken a systematic review of published estimates of the incidence of HD. SETTING: Incident patients with a new diagnosis of HD were identified from the primary care records of the Clinical Practice Research Datalink (CPRD). The systematic review included all published estimates of the incidence of HD in defined populations. PARTICIPANTS: A total of 393 incident cases of HD were identified from the CPRD database between 1990 and 2010 from a total population of 9,282,126 persons. PRIMARY AND SECONDARY OUTCOME MEASURES: The incidence of HD per million person-years was estimated. From the systematic review, the extent of heterogeneity of published estimates of the incidence of HD was examined using the I(2) statistic. RESULTS: The data showed that the incidence of HD has remained constant between 1990 and 2010 with an overall rate of 7.2 (95% CI 6.5 to 7.9) per million person-years. The systematic review identified 14 independent estimates of incidence with substantial heterogeneity and consistently lower rates reported in studies from East Asia compared with those from Australia, North America and some--though not all--those from Europe. Differences in incidence estimates did not appear to be explained solely by differences in case ascertainment or diagnostic methods. CONCLUSIONS: The rise in the prevalence of diagnosed HD in the UK, between 1990 and 2010, cannot be attributed to an increase in incidence. Globally, estimates of the incidence of HD show evidence of substantial heterogeneity with consistently lower rates in East Asia and parts of Europe. Modifiers may play an important role in determining the vulnerability of different populations to expansions of the HD allele

A portable extensional rheometer for measuring the viscoelasticity of pitcher plant and other sticky liquids in the field.

BACKGROUND: Biological fluids often have interesting and unusual physical properties to adapt them for their specific purpose. Laboratory-based rheometers can be used to characterise the viscoelastic properties of such fluids. This, however, can be challenging as samples often do not retain their natural properties in storage while conventional rheometers are fragile and expensive devices ill-suited for field measurements. We present a portable, low-cost extensional rheometer designed specifically to enable in situ studies of biological fluids in the field. The design of the device (named Seymour) is based on a conventional capillary break-up extensional rheometer (the Cambridge Trimaster). It works by rapidly stretching a small fluid sample between two metal pistons. A battery-operated solenoid switch triggers the pistons to move apart rapidly and a compact, robust and inexpensive, USB 3 high speed camera is used to record the thinning and break-up of the fluid filament that forms between the pistons. The complete setup runs independently of mains electricity supply and weighs approximately 1 kg. Post-processing and analysis of the recorded images to extract rheological parameters is performed using open source software. RESULTS: The device was tested both in the laboratory and in the field, in Brunei Darussalam, using calibration fluids (silicone oil and carboxymethyl cellulose solutions) as well as Nepenthes pitcher plant trapping fluids as an example of a viscoelastic biological fluid. The fluid relaxation times ranged from 1 ms to over 1 s. The device gave comparable performance to the Cambridge Trimaster. Differences in fluid viscoelasticity between three species were quantified, as well as the change in viscoelasticity with storage time. This, together with marked differences between N. rafflesiana fluids taken from greenhouse and wild plants, confirms the need for a portable device. CONCLUSIONS: Proof of concept of the portable rheometer was demonstrated. Quantitative measurements of pitcher plant fluid viscoelasticity were made in the natural habitat for the first time. The device opens up opportunities for studying a wide range of plant fluids and secretions, under varying experimental conditions, or with changing temperatures and weather conditions.The following financial support is gratefully acknowledged: a Henslow Research Fellowship from the Cambridge Philosophical Society and a Leverhulme Early Career Fellowship for UB; a visiting research fellowship (POS-A/2012/116) for MDT from Xunta de Galicia’s Consellería de Cultura, Educación e Ordenación Universitaria of Spain and the European Union’s European Social Fund; and a summer project grant for CC from Sidney Sussex College, Cambridge.This is the final version of the article. It first appeared at http://www.plantmethods.com/content/11/1/16

Applying GNSS Reflectometry-Based Stare Processing to Modeling and Remote Sensing of Wind-Driven Ocean Surface Roughness

Measuring ocean surface winds is important for weather forecasting, ship routing, understanding our changing climate, and more. Satellite measurements of ocean winds are enabled by scatterometers, radiometers, altimeters, and synthetic aperture radar, as well as Global navigation satellite systems (GNSS) reflectometry (GNSS-R). GNSS-R is a method of remote sensing where passive receivers utilize existing transmissions from GNSS satellites reflected from the surface of Earth. A prominent GNSS-R mission is Cyclone GNSS (CYGNSS), a constellation of eight small satellites that estimate surface wind speed. The GPS receiver on board CYGNSS produces delay-Doppler maps (DDMs), which contain information about the signal scattered from the wind-driven ocean surface roughness. Neither CYGNSS nor any other spaceborne GNSS-R mission is currently capable of estimating surface wind direction. The focus of this work is a technique called stare processing and its application to modeled DDMs and real CYGNSS data for remote sensing of ocean surface winds. Stare processing extracts information from a sequence of DDMs, ordinarily processed independently, without significantly degrading spatial resolution. We have developed and rigorously described a generalized framework for stare processing that can be easily adapted to any DDM-based data. The main output is termed the stare profile and is designed to represent the wind-dependent ocean surface slope statistics, as modeled by a probability density function of surface slopes (slope PDF). The stare profiles contain a greater diversity of slope PDF information that a typical DDM-based observable of comparable spatial resolution. In modeled DDMs, the stare profiles are sensitive to wind speed and direction while being nearly independent of satellite geometry. The stare profiles extracted from the CYGNSS data, however, are different, highlighting shortcomings in the assumptions used to model the DDMs as well as significant remaining calibration errors in the CYGNSS data. In particular, a sensitivity to wind direction is not observed in the CYGNSS data. We also introduce a novel method for empirically reconstructing the slope PDF from CYGNSS data.</p

Design and Characterization of a New Pulsed Molecular-Beam Valve

A common technique for producing samples of cold molecules that can be trapped and studied is electromagnetic deceleration of a fast moving, but internally cold, supersonic beam. The quality of these samples, which are ideally as cold and dense as possible, is fundamentally limited by the quality of the beam coupled to the decelerator. This thesis outlines the design, construction, and characterization of a new pulsed valve that produces samples of molecules as short as fifty microseconds.</p

Endoplasmic reticulum stress stimulates the release of extracellular vesicles carrying danger-associated molecular pattern (DAMP) molecules.

Disturbances in endoplasmic reticulum (ER) function lead to ER stress which, when severe or prolonged, may result in apoptosis. Severe ER stress has been implicated in several pathological conditions including pre-eclampsia, a multisystem disorder of pregnancy associated with the release of pro-inflammatory factors from the placenta into the maternal circulation. Here, we show that severe ER stress induced by two distinct mechanisms in BeWo choriocarcinoma cells leads to the release of extracellular vesicles (EVs) carrying pro-inflammatory damage-associated molecular pattern (DAMP) molecules. Co-treatment with the antioxidant pyrrolidine dithiocarbamate results in a reduction in ER stress-induced EV-associated DAMP release. We further demonstrate that severe ER stress is associated with changes in the expression of several stress-related proteins, notably Cited-2 and phosphorylated JNK. Together, these data indicate that severe ER stress-mediated release of EV-associated DAMPs may contribute to the heightened systemic maternal inflammatory response characteristic of pre-eclampsia and may also be relevant to other chronic inflammatory diseases which display elevated ER stress

Bone conduction hearing implants (BCHIs); life-changing surgery. A quality of life (QoL) study reporting the impact of BCHI surgery on 163 patients’ well-being

Objective: This study aimed to review the impact of bone conduction hearing implants (BCHIs) surgery on patients’ quality of life (QoL) across general, physical, social and overall well-being domains. Design: A prospective correlational study. Setting: Single tertiary referral center in the UK. Participants: All adult patients undergoing their first BCHI over a 10-year and 6-month period (between June 1, 2012, and December 31, 2022). A total of 163 were included out of 195 potentially eligible participants. Main outcome measure: The Glasgow Health Status Inventory (GHSI) was used to assess patients’ QoL pre and post BCHI surgery. Results: BCHI surgery showed significant improvements across all GHSI QoL domains; total (p<0.0001), general (p<0.0001), social (p<0.0001) and physical (p<0.0001). Conclusion: The largest of its kind, this study recognizes the wide-reaching impact of BCHIs on patients’ QoL, evidencing them as a highly effective and life-improving surgical intervention

Digital home monitoring for capturing daily fluctuation of symptoms; a longitudinal repeated measures study: Long Covid Multi-disciplinary Consortium to Optimise Treatments and Services across the NHS (a LOCOMOTION study)

Introduction A substantial proportion of COVID-19 survivors continue to have symptoms more than 3 months after infection, especially of those who required medical intervention. Lasting symptoms are wide-ranging, and presentation varies between individuals and fluctuates within an individual. Improved understanding of undulation in symptoms and triggers may improve efficacy of healthcare providers and enable individuals to better self-manage their Long Covid. We present a protocol where we aim to develop and examine the feasibility and usability of digital home monitoring for capturing daily fluctuation of symptoms in individuals with Long Covid and provide data to facilitate a personalised approach to the classification and management of Long Covid symptoms. Methods and analysis This study is a longitudinal prospective cohort study of adults with Long Covid accessing 10 National Health Service (NHS) rehabilitation services in the UK. We aim to recruit 400 people from participating NHS sites. At referral to study, 6 weeks and 12 weeks, participants will complete demographic data (referral to study) and clinical outcome measures, including ecological momentary assessment (EMA) using personal mobile devices. EMA items are adapted from the COVID-19 Yorkshire Rehabilitation Scale items and include self-reported activities, symptoms and psychological factors. Passive activity data will be collected through wrist-worn sensors. We will use latent class growth models to identify trajectories of experience, potential phenotypes defined by co-occurrence of symptoms and inter-relationships between stressors, symptoms and participation in daily activities. We anticipate that n=300 participants provide 80% power to detect a 20% improvement in fatigue over 12 weeks in one class of patients relative to another. Ethics and dissemination The study was approved by the Yorkshire & The Humber—Bradford Leeds Research Ethics Committee (ref: 21/YH/0276). Findings will be disseminated in peer-reviewed publications and presented at conferences. Trial registration number ISRCTN15022307

Higher Education in the melting pot

The idea of this book emerged from the Education Deans Forum (EDF) meeting held in Johannesburg in 2018. The forum discussed the twin issues of the 4IR and Decolonisation and how these were likely to impact the future development of Higher Education in South Africa. Essentially, this book provides scholarly analyses of a range of possible impacts of the two discourses. On one hand, the discourses are discussed as representing convergences and divergences in relation to their epistemological, ontological, axiological and methodological assumptions. On the other, they are portrayed as competing for dominance in the contemporary and future discourses in Higher Education. As a scholarly compilation of high-end research, the book is a must-read resource for academics generally and those in teacher education disciplines particularly. Issues of the automation of academic workspaces, impact of digital divides, the opportunities and constraints of the technologisation of curricula, pedagogies, teaching and learning and the intractable challenges of remote modalities of university instruction are dealt with by some of the leading thinkers in the South African academies