C1 inhibitor deficiency: 2014 United Kingdom consensus document

C1 inhibitor deficiency is a rare disorder manifesting with recurrent attacks of disabling and potentially life-threatening angioedema. Here we present an updated 2014 United Kingdom consensus document for the management of C1 inhibitor-deficient patients, representing a joint venture between the United Kingdom Primary Immunodeficiency Network and Hereditary Angioedema UK. To develop the consensus, we assembled a multi-disciplinary steering group of clinicians, nurses and a patient representative. This steering group first met in 2012, developing a total of 48 recommendations across 11 themes. The statements were distributed to relevant clinicians and a representative group of patients to be scored for agreement on a Likert scale. All 48 statements achieved a high degree of consensus, indicating strong alignment of opinion. The recommendations have evolved significantly since the 2005 document, with particularly notable developments including an improved evidence base to guide dosing and indications for acute treatment, greater emphasis on home therapy for acute attacks and a strong focus on service organisation. This article is protected by copyright. All rights reserved

On the Cooling of the Neutron Star in Cassiopeia A

We demonstrate that the high-quality cooling data observed for the young neutron star in the supernova remnant Cassiopeia A over the past 10 years--as well as all other reliably known temperature data of neutron stars--can be comfortably explained within the "nuclear medium cooling" scenario. The cooling rates of this scenario account for medium-modified one-pion exchange in dense matter and polarization effects in the pair-breaking formations of superfluid neutrons and protons. Crucial for the successful description of the observed data is a substantial reduction of the thermal conductivity, resulting from a suppression of both the electron and nucleon contributions to it by medium effects. We also find that possibly in as little as about ten years of continued observation, the data may tell whether or not fast cooling processes are active in this neutron star.Comment: 4 pages, 3 figure

Incidence of community-acquired lower respiratory tract infections and pneumonia among older adults in the United Kingdom: a population-based study.

Community-acquired lower respiratory tract infections (LRTI) and pneumonia (CAP) are common causes of morbidity and mortality among those aged ≥65 years; a growing population in many countries. Detailed incidence estimates for these infections among older adults in the United Kingdom (UK) are lacking. We used electronic general practice records from the Clinical Practice Research Data link, linked to Hospital Episode Statistics inpatient data, to estimate incidence of community-acquired LRTI and CAP among UK older adults between April 1997-March 2011, by age, sex, region and deprivation quintile. Levels of antibiotic prescribing were also assessed. LRTI incidence increased with fluctuations over time, was higher in men than women aged ≥70 and increased with age from 92.21 episodes/1000 person-years (65-69 years) to 187.91/1000 (85-89 years). CAP incidence increased more markedly with age, from 2.81 to 21.81 episodes/1000 person-years respectively, and was higher among men. For both infection groups, increases over time were attenuated after age-standardisation, indicating that these rises were largely due to population aging. Rates among those in the most deprived quintile were around 70% higher than the least deprived and were generally higher in the North of England. GP antibiotic prescribing rates were high for LRTI but lower for CAP (mostly due to immediate hospitalisation). This is the first study to provide long-term detailed incidence estimates of community-acquired LRTI and CAP in UK older individuals, taking person-time at risk into account. The summary incidence commonly presented for the ≥65 age group considerably underestimates LRTI/CAP rates, particularly among older individuals within this group. Our methodology and findings are likely to be highly relevant to health planners and researchers in other countries with aging populations

Orthogonal on-rotor sensing vibrations for condition monitoring of rotating machines

Thanks to the fast development of micro-electro-mechanical systems (MEMS) technologies, MEMS accelerometers show great potentialities for machine condition monitoring. To overcome the problems of a poor signal to noise ratio (SNR), complicated modulation, and high costs of vibration measurement and computation using conventional integrated electronics piezoelectric accelerometers, a triaxial MEMS accelerometer-based on-rotor sensing (ORS) technology was developed in this study. With wireless data transmission capability, the ORS unit can be mounted on a rotating rotor to obtain both rotational and transverse dynamics of the rotor with a high SNR. The orthogonal outputs lead to a construction method of analytic signals in the time domain, which is versatile in fault detection and diagnosis of rotating machines. Two case studies based on an induction motor were carried out, which demonstrated that incipient bearing defect and half-broken rotor bar can be effectively diagnosed by the proposed measurement and analysis methods. Comparatively, vibration signals from translational on-casing accelerometers are less capable of detecting such faults. This demonstrates the superiority of the ORS vibrations in fault detection of rotating machines

Toward a simulation approach for alkene ring-closing metathesis : scope and limitations of a model for RCM

A published model for revealing solvent effects on the ring-closing metathesis (RCM) reaction of di-Et diallylmalonate 7 has been evaluated over a wider range of conditions, to assess its suitability for new applications. Unfortunately, the model is too flexible and the published rate consts. do not agree with exptl. studies in the literature. However, by fixing the values of important rate consts. and restricting the concn. ranges studied, useful conclusions can be drawn about the relative rates of RCM of different substrates, precatalyst concn. can be simulated accurately and the effect of precatalyst loading can be anticipated. Progress has also been made toward applying the model to precatalyst evaluation, but further modifications to the model are necessary to achieve much broader aims

Projected Images of Major Chinese Outbound Destinations

This study aimed to explore the projected images of major outbound destinations based on popular travel magazines in China. Travel articles on Hong Kong, Macau, Japan, South Korea, Vietnam and Taiwan from 2006 to 2008 were content analyzed. Japan was reported on most, and the projected images of the six destinations are dominated by leisure and recreation, and culture, history and art. Correspondence analysis was used to examine relationships between destinations and popular image attributes. The results showed that South Korea and Macau had distinct projected images, whereas Japan, Taiwan, Hong Kong and Vietnam shared many similar image attributes. Practical implications for destination marketing organizations are provided

Swimming against the tide: a study of a neighbourhood trying to rediscover its ‘reason for being’– the case of South Bank, Redcar and Cleveland.

Many of the programmes and initiatives to regenerate deprived neighbourhoods appear to have had limited lasting impact. It has been argued that one reason for this is that we still have little real understanding of the nature and scale of the problems some communities face (Bernt, 2009). This article attempts to add to our knowledge through close study of an area with multiple problems and a history of failed regeneration attempts. An in-depth case study, undertaken to explore the current situation and future prospects of South Bank, a small neighbourhood in the North East of England, highlights transferable knowledge which may be applied to other regeneration areas. The analysis considers the nature and consequences of industrial decline; entrenched deprivation; the stigmatization of communities; the value of community consultation and the potential impact of retail-led regeneration. We question whether negative stigma attached to places can be changed and we ask what the future may hold for deprived communities now that public sector funding has largely dried up, and we consider an alternative approach: the potential impacts of private sector retail-led regeneration in the absence of public sector funding

Cardiovascular disease treatment among patients with severe mental illness: a data linkage study between primary and secondary care

Background Suboptimal treatment of cardiovascular diseases (CVD) among patients with severe mental illness (SMI) may contribute to physical health disparities. Aim To identify SMI characteristics associated with meeting CVD treatment and prevention guidelines. Design and setting Population-based electronic health record database linkage between primary care and the sole provider of secondary mental health care services in south east London, UK. Method Cardiovascular disease prevalence, risk factor recording, and Quality and Outcomes Framework (QOF) clinical target achievement were compared among 4056 primary care patients with SMI whose records were linked to secondary healthcare records and 270 669 patients without SMI who were not known to secondary care psychiatric services, using multivariate logistic regression modelling. Data available from secondary care records were then used to identify SMI characteristics associated with QOF clinical target achievement. Results Patients with SMI and with coronary heart disease and heart failure experienced reduced prescribing of beta blockers and angiotensin-converting enzyme inhibitor/angiotensin receptor blockers (ACEI/ARB). A diagnosis of schizophrenia, being identified with any indicator of risk or illness severity, and being prescribed with depot injectable antipsychotic medication was associated with the lowest likelihood of prescribing. Conclusion Linking primary and secondary care data allows the identification of patients with SMI most at risk of undertreatment for physical health problems