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Objectives: Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumours with malignant potential and a hereditary basis in almost 40% of patients. Germline genetic testing has transformed the management of PPGL enabling stratification of surveillance approaches, earlier diagnosis and predictive testing of at‐risk family members. Recent studies have identified somatic mutations in a further subset of patients, indicating that molecular drivers at either a germline or tumour level can be identified in up to 80% of PPGL cases. The aim of this study was to investigate the clinical utility of somatic sequencing in a large cohort of patients with PPGL in the United Kingdom. Design and Patients: Prospectively collected matched germline and tumour samples (development cohort) and retrospectively collected tumour samples (validation cohort) of patients with PPGL were investigated. Measurements: Clinical characteristics of patients were assessed and tumour and germline DNA was analysed using a next‐generation sequencing strategy. A screen for variants within 'mutation hotspots' in 68 human cancer genes was performed. Results: Of 141 included patients, 45 (32%) had a germline mutation. In 37 (26%) patients one or more driver somatic variants were identified including 26 likely pathogenic or pathogenic variants and 19 variants of uncertain significance. Pathogenic somatic variants, observed in 25 (18%) patients, were most commonly identified in the VHL, NF1, HRAS and RET genes. Pathogenic somatic variants were almost exclusively identified in patients without a germline mutation (all but one), suggesting that somatic sequencing is likely to be most informative for those patients with negative germline genetic test results. Conclusions: Somatic sequencing may further stratify surveillance approaches for patients without a germline genetic driver and may also inform targeted therapeutic strategies for patients with metastatic disease. [ABSTRACT FROM AUTHOR]

Introduction: New personal hemodialysis systems, such as the quanta SC+, are being developed; these systems are smaller and simpler to use while providing the clearances of conventional systems. Increasing the uptake of lower‐intensity assistance and full self‐care dialysis may provide economic benefits to the public health payer. In the United Kingdom, most hemodialysis patients currently receive facility‐based dialysis costing more than £36,350 per year including patient transport. As such, we aimed to describe the annual costs of using the SC+ hemodialysis system in the United Kingdom for 3×‐weekly and 3.5×‐weekly dialysis regimens, for self‐care hemodialysis provided both in‐center and at home. Methods: We applied a cost minimization approach. Costs for human resources, equipment, and consumables were sourced from the dialysis machine developer (Quanta Dialysis Technologies) based upon discussions with dialysis providers. Facility overhead expenses and transport costs were taken from a review of the literature. Findings Annual costs associated with the use of the SC+ hemodialysis system were estimated to be £26,642 for hemodialysis provided 3× weekly as home self‐care; £30,235 for hemodialysis provided 3× weekly as self‐care in‐center; £29,866 for hemodialysis provided 3.5× weekly as home self‐care; and £36,185 for hemodialysis provided 3.5× weekly as self‐care in‐center. Discussion We found that the SC+ hemodialysis system offers improved cost‐effectiveness for both 3×‐weekly and 3.5×‐weekly self‐care dialysis performed at home or as self‐care in‐center versus fully assisted dialysis provided 3× weekly with conventional machines in facilities. [ABSTRACT FROM AUTHOR]

Summary: Background: The skin of patients with atopic dermatitis is characterized by abnormal stratum corneum lipid levels. Consequently, the lamellar matrices are disrupted and skin barrier function is diminished, increasing skin sensitivity to irritants and allergens. Objectives: To determine whether a cream containing ceramides, triglycerides and cholesterol in a multivesicular emulsion can reinforce the skin barrier and protect against skin irritation. Methods: A randomized observer‐blind intrapatient‐controlled study in 34 adults with dry, eczema‐prone skin was conducted. Each participant underwent 4 weeks of treatment with the test cream on one forearm and lower leg and a reference emollient cream on the other. Skin properties were determined before and after treatment. Lipid structure was assessed by Fourier‐transform infrared spectroscopy using a novel interface. Results: Skin barrier integrity was greater at sites treated with the test cream [effect size for area under the transepidermal water loss curve −162, 95% confidence interval (CI) −206 to −118]. Skin sensitivity to sodium lauryl sulfate was reduced (−0·5 points visual redness, 97·57% CI −1·00 to −0·25), as was transepidermal water loss (−15·3 g m−2 h−1, 95% CI −20·3 to −10·4) compared with the reference. Sites treated with the test cream displayed enhanced lipid chain ordering, which was significantly associated with skin barrier integrity (r = 0·61). Compared with the reference, treatment with the test cream increased hydration (8·61 capacitance units, 95% CI 6·61–10·6) and decreased signs of dryness. Conclusions: The test cream facilitates skin barrier restoration and protects the skin from dryness and irritation. Compared with a commonly prescribed emollient in the UK, the test cream is highly suited to the management of dry, sensitive skin. [ABSTRACT FROM AUTHOR]

Objective: Phaeochromocytomas and paragangliomas (PPGL) are rare, but strongly heritable tumours. Variants in succinate dehydrogenase (SDH) subunits are identified in approximately 25% of cases. However, clinical and genetic information of patients with SDHC variants are underreported. Design: This retrospective case series collated data from 18 UK Genetics and Endocrinology departments. Patients: Both asymptomatic and disease‐affected patients with confirmed SDHC germline variants are included. Measurements: Clinical data including tumour type and location, surveillance outcomes and interventions, SDHC genetic variant assessment, interpretation, and tumour risk calculation. Results: We report 91 SDHC cases, 46 probands and 45 non‐probands. Fifty‐one cases were disease‐affected. Median age at genetic diagnosis was 43 years (range: 11–79). Twenty‐four SDHC germline variants were identified including six novel variants. Head and neck paraganglioma (HNPGL, n = 30, 65.2%), extra‐adrenal paraganglioma (EAPGL, n = 13, 28.2%) and phaeochromocytomas (PCC) (n = 3, 6.5%) were present. One case had multiple PPGLs. Malignant disease was reported in 19.6% (9/46). Eight cases had non‐PPGL SDHC‐associated tumours, six gastrointestinal stromal tumours (GIST) and two renal cell cancers (RCC). Cumulative tumour risk (95% CI) at age 60 years was 0.94 (CI: 0.79–0.99) in probands, and 0.16 (CI: 0–0.31) in non‐probands, respectively. Conclusions: This study describes the largest cohort of 91 SDHC patients worldwide. We confirm disease‐affected SDHC variant cases develop isolated HNPGL disease in nearly 2/3 of patients, EAPGL and PCC in 1/3, with an increased risk of GIST and RCC. One fifth developed malignant disease, requiring comprehensive lifelong tumour screening and surveillance. [ABSTRACT FROM AUTHOR]

With the recent increase in research into ferroelectric, anti‐ferroelectric and piezoelectric materials, studying the solid‐state properties in situ under applied electric fields is vital in understanding the underlying processes. Where this behaviour is the result of atomic displacements, crystallographic insight has an important role. This work presents a sample environment designed to apply an electric field to single‐crystal samples in situ on the small‐molecule single‐crystal diffraction beamline I19, Diamond Light Source (UK). The configuration and operation of the cell is described as well as its application to studies of a proton‐transfer colour‐change material. [ABSTRACT FROM AUTHOR]

Can a union be both democratic and administratively efficient, or are these goals always at odds? Building on the Webbs' focus on this critical question, this article analyses and compares the changing administrative policies and practices of US, UK and Australian trade unions over a 25‐year period. We conducted surveys of unions in all three countries to gather information on union policies and practices involving the unions' human resources, hiring, budgeting and strategic planning. Using these novel longitudinal data, we contribute to industrial relations scholarship by showing that unions have increasingly adopted formal, systematic practices in these areas. The article is grounded in theory and also has practical relevance given the important implications that our findings may have for the revitalization of unions in the three countries and beyond. [ABSTRACT FROM AUTHOR]

Introduction: The replenishment of skin lipids depleted in the dry skin state is a desirable therapeutic target to restore skin moisturization; however, there is limited evidence demonstrating the success of this approach through the use of topical emollients. The purpose of this study was to provide evidence of the benefits of a cream and equivalent lotion containing skin lipids in a multi-vesicular emulsion for the management of dry skin. The hypothesis was that the test cream and test lotion could sustain skin moisturization for longer than traditional emollients by sustainably delivering skin lipids. Methods: A double-blind intra-subject vehicle-controlled single open-application test on the lower legs in people with dry, atopic dermatitis (atopic eczema)-prone, skin was conducted. There were six treatment sites, three per lower leg in each participant, which were treated with the test cream, the test lotion, three reference creams commonly prescribed in the UK and no treatment as a control. After baseline measurements of skin hydration, 100 μl of the test/reference creams was applied to each of the relevant treatment sites (random site allocation). Following treatment, measurements of skin hydration and scoring of visual dryness was conducted at timed intervals (3, 6, 12 and 24 h post-product application). Results: The test cream and lotion both significantly increased skin hydration and reduced skin dryness for at least 24 h following a single application compared to a no treatment control site. Compared to three reference emollient creams the test cream and test lotion were the only products capable of sustaining clinically meaningful improvements in skin moisturization for 24 h. Conclusion: The sustained moisturization imparted by the test products reduces the need for frequent emollient application, often requiring 3–4 applications per day for traditional emollients, and should reduce the high burden of managing dry skin conditions like atopic dermatitis. [ABSTRACT FROM AUTHOR]

Research into stranger sexual homicide remains limited as is our understanding of this crime, and this can hinder criminal investigations and the assessment and management of offenders. This study aims to address this. Using data gathered by various British police forces, this study presents a descriptive profile of adult male-on-female stranger sexual homicide in Great Britain in recent years. Along with demographic and occupational data on offenders and victims, the criminal histories of offenders are illustrated as are their offense behaviors. The results are discussed in light of similar research, and future directions and implications are considered. [ABSTRACT FROM AUTHOR]

This article examines postwar government policy in Britain, as reflected in annual budget speeches. Like previous research, it aims to content-analyse these speeches to derive estimates of actual, as opposed to intended, government policy stances. Unlike previous research, it also aims to capture and measure the gap between intentions (as represented in electoral manifestos) and actual policy. This gap cannot be assessed from the final output of the Wordscores content analysis programme (in either the original version or the Martin- Vanberg variation), but it can be teased out of the raw output. This teasing-out process reveals the gap to be very small: there is no evidence that British governments either moderate or amplify their left-right stances when in office. This new measurement of government position is then used to cast further light on policy representation in Britain. The findings show that policy positions respond significantly to changes in public opinion as well as to electoral turnover, but do not exhibit or even approach the ideological congruence anticipated by the 'median mandate' interpretation of representative democracy. [ABSTRACT FROM AUTHOR]

Since 1999, the Dutch trauma care has been regionalized into ten trauma systems. This study is the first to review such a trauma system. The aim was to examine the sensitivity of prehospital triage criteria [triage revised trauma score (T-RTS)] in identifying major trauma patients and to evaluate the current level of trauma care of a regionalized Dutch trauma system for major trauma patients. Major trauma patients ( n=511) (June 2001–December 2003) were selected from a regional trauma registry database. The prehospital T-RTS was computed and standardized W scores (Ws) were generated to compare observed vs expected survival based on contemporary US- and UK-norm databases. The T-RTS showed low sensitivity for the prehospital identification of major trauma patients [34.1% (T-RTS≤10)]. Nevertheless, 78.0% of all major trauma patients were directly managed by the trauma center. These patients were more severely injured than their counterparts at non-trauma-center hospitals ( p<0.001). No significant difference emerged between the mortality rates of both groups. The Ws {−0.46 calculated on the US model [95% confidence interval (CI) ranging from −1.99 to 1.07]} [0.60 calculated on the UK model (95% CI ranging from −1.25 to 2.44)] did not differ significantly from zero. The trauma center managed most of the major trauma patients in the trauma system but the triage criteria need to be reconsidered. The level of care of the regional trauma system was shown to measure up to US and UK benchmarks. [ABSTRACT FROM AUTHOR]

The article discusses the implications of the measurement of letter visual acuity among British preschool children. Accordingly, the test aims to assert the level of children's knowledge on visual recognition as well as to match or name familiar pictures using a single optotypes in a symmetrical presentation. It also offers information on the evaluation procedure as well as the scoring systems and clinical grading.

Assesses the impacts of neonatal screening for cystic fibrosis using immunoreactive trypsinogen and direct gene analysis in Australia. Measurement of the immunoreactive trypsinogen of the first tier neonatal screening; Direct gene analysis of the cystic fibrosis mutations; Examination of the blood spot specimen.

In July and August 1990, symptoms of verticilliosis were common in maturing crops of linseed sampled in Hertfordshire, UK and throughout Germany. Grey chlorotic or brown stripes were observed on green stems: on maturing, light brown stems the stripes were dark brown. Symptoms frequently extended along the whole length of the stems and branches up to the capsules, and often spread completely round the stems. Black microscierotia developed abundantly in stems with these symptoms and Verticillium dahliae was consistently isolated from them. The incidence of verticilliosis increased from 50 to 100% in untreated plots (cv. Antares) between 8 and 22 August at Rothamsted, UK and was not decreased greatly by fungicide treatments. The incidence ranged from 49 to 98% on cultivars and breeding lines in trials at Thüle, Germany on 9 September. V. dahliae was isolated from seed from Rothamsted plots at an incidence of <10%. [ABSTRACT FROM AUTHOR]

Three collections of crown rust (Puccinia coronata), from Czechoslovakia, West Germany and the U.K., were tested for their pathogenicity on sixteen cultivars of perennial ryegrass. The Czechoslovakian collection had the highest level of infection on twelve of the cultivars, although with most cultivars there were no significant differences in infection levels. Three cultivars, Kerem, Loretta and Tando, contained qualitative resistance to crown rust which was overcome by the Czechoslovakian collection, although they retained a moderately high level of background resistance to this collection. [ABSTRACT FROM AUTHOR]

The article details the development of a telehealth system in Great Britain. The Yorkshire and Humber Regional Prison Health Development Team together with the National Health Service (NHS) and a telehealth group explore the feasibility of developing a telehealth link between English prisons and primary care trusts. The telehealth group found a willing pilot partner in Airedale NHS Hospital Trust, but its participation is limited to the piloting phase.

Presents a letter to the editor in regards to an article published in a previous issue which discussed sickness certification system in the United Kingdom.

Abstract: To determine the longevity of protective immunity following a single administration of emergency vaccine, and establish whether the immune response could be enhanced by increasing the antigen payload even further, cattle were vaccinated with an A22 Iraq vaccine containing either 1× antigen payload (field dose) or 5× antigen payload. Six months post-immunisation all cattle received a homologous virus challenge. The magnitude of the virus neutralising antibody response elicited was consistent with the response to similarly formulated A serotype vaccines with a PD50 greater than 32. All the vaccinated cattle, regardless of antigen payload, were protected from clinical disease following challenge although some cattle in both groups became sub-clinically infected. We conclude that immunisation with a single inoculation of vaccine from the UK emergency reserve can protect cattle from clinical disease for at least 6 months post-vaccination and that a boost may be unnecessary in an outbreak situation. Some animals may become sub-clinically infected but this is likely to be dependent on the severity of challenge. The study confirmed that a booster at 21 days post-vaccination was not necessary to maintain a cell-mediated response in cattle for 6 months. No increased benefits were recognised by increasing the antigen payload of this vaccine 5×. [Copyright &y& Elsevier]