Search

Background: Clinical research has offered many definitions and fragmented perspectives of joint morbidity in haemophilia. As joint damage, pain and mobility impairment can be present without clinical record of persistent bleeding, a person-centric joint morbidity characterisation remained a priority for the haemophilia community, giving rise to the 'problem joint' concept. As diagnosing and managing joint morbidity is critical, the aim of this study was to analyse the holistic burden of problem joints in people with moderate or severe haemophilia A (HA). Data from the 'Cost of Haemophilia in Europe: a Socioeconomic Survey' (CHESS) cross-sectional studies were used. CHESS-Paediatrics included male paediatric patients (≤ 17 years) with congenital moderate or severe haemophilia, while CHESS II included adult males (≥ 18 years) of any severity. Both studies sought to collect detailed information on the clinical, economic and humanistic burden of haemophilia. Demographics, clinical outcomes, treatment regimen, adherence, physical activity, healthcare resource use and number of problem joints were evaluated and described by HA severity and number of problem joints (none, 1, ≥ 2). Results: In total, 1171 people with non-inhibitor moderate or severe HA from CHESS-Paediatrics (n = 703) and CHESS II (n = 468) were included in this analysis. Presence of problem joints was more prevalent among CHESS II participants (44%) than in CHESS-Paediatrics (14%). Around two-thirds (67%) of CHESS-Paediatrics and 39% of CHESS II participants received prophylactic factor VIII replacement therapy. The presence of chronic pain was greater in severe HA with ' ≥ 2' problem joints in both cohorts. Clinical symptoms and bleed-related hospitalizations were more prevalent in the presence of problem joints regardless of HA severity in both cohorts. Conclusions: This analysis of the CHESS population studies has expanded on previous work by examining the relevance of the problem joint measure of haemophilic morbidity and its associated burden. Adverse clinical symptoms and increased bleed-related hospitalizations were observed in the presence of problem joints in both children/adolescents and adults across HA severities. Use of person-centric characterizations of joint morbidity may improve analysis of long-term outcomes and lead to improvements in future haemophilia care. [ABSTRACT FROM AUTHOR]

Background: Chronic pain is reported by between 30 and 71% of people with haemophilia (PWH). Exercise is shown to be effective for pain management in other arthritides, but it remains unclear if such an approach is effective or acceptable to PWH. The aim of this study was to evaluate the feasibility and acceptability of a telerehabilitation exercise intervention for PWH living with chronic pain. Methods: This was a multisite, non-randomised, pre-post feasibility design, with a nested qualitative study. People with severe haemophilia > 18 years, living with chronic pain, were recruited. The intervention comprised 12 low-impact/moderate intensity, individualised exercise sessions and 3 knowledge-sharing and discussion sessions. Primary objectives assessed according to predefined progression criteria were as follows: (a) recruitment rate (5 participants enrolled per site over 8 weeks), (b) adherence (≥ 75% participants would adhere to ≥ 75% of sessions), (c) follow-up rate (≥ 75% completion of self-reported measures), (d) fidelity (intervention delivered as described in protocol) and (e) safety (≤ 30% participants would report adverse events). Acceptability was evaluated from thematic analysis of post-intervention participant interviews. Preliminary evaluation of self-reported pain, function and quality of life (QoL) was a secondary objective. Results were reported using descriptive statistics integrated with qualitative findings. Results: Ten PWH were recruited and completed the intervention. Nine agreed to be interviewed post intervention. Attendance at individual sessions was 84.5% compared to 52.1% for the group sessions. Outcome measures were successfully completed for 100% at baseline, 70% at intervention end and 60% at 3-month follow-up. No serious adverse events were recorded. Group median values in outcome measures (pain, function, QoL) showed minimal change post intervention. Participant interviews highlighted high levels of enjoyment, confidence in continuing exercises independently and positive views of virtual delivery and condition-specific exercise. Conclusions: Recruitment rate and safety met the predefined progression criteria. Fidelity partially met the progression criteria, but the follow-up rate for self-reported measures did not. The study was acceptable to both participants and physiotherapists. Further intervention development is needed to review approaches to outcome measure collection and refine the usefulness of the knowledge-sharing sessions. Trial registration : The study was prospectively registered on 9 July 2021: International Standard Randomised Controlled Trial Number ISRCTN 17454597. [ABSTRACT FROM AUTHOR]

Accurately predicting species' distributions is critical for the management and conservation of fish and wildlife populations. Joint species distribution models (JSDMs) account for dependencies between species often ignored by traditional species distribution models. We evaluated how a JSDM approach could improve predictive strength for stream fish communities within large watersheds (the Chesapeake Bay Watershed, USA), using a cross-validation study of JSDMs fit to data from over 50 species. Our results suggest that conditional predictions from JSDMs have the potential to make large improvements in predictive accuracy for many species, particularly for more generalist species where single species models may not perform well. For some species there was no added explanatory effect from conditional information, most of which already exhibited strong marginal predictive ability. For several rare species there were significant improvements in occurrence predictions, while the results for two invasive species considered did not show the same improvements. Overall, the optimal number of species to condition upon, as well as the effects of conditioning upon an increasing number of species, varied widely among species. [ABSTRACT FROM AUTHOR]

Introduction: As a result of centralisation of haemophilia care to a limited number of intramural settings, many persons with haemophilia have to travel long distances to attend their haemophilia specialised treatment centre. However, regular physiotherapy treatment can be provided by primary care physiotherapists in the personʼs own region. Due to the rarity of the disease most primary care physiotherapists have limited experience with this population. This study aims to provide a clinical practice guideline for primary care physiotherapists working with persons with bleeding disorders. Method: A list of the most urgent key‐questions was derived from a previous study. Literature was summarised using the grading of recommendations assessment, development, and evaluation (GRADE) evidence‐to‐decision framework. Recommendations were drafted based on four 90 min consensus meetings with expert physiotherapists. Recommendations were finalised after feedback and >80% consensus of all stakeholders (including PWH, physiotherapists, haematologists and the corresponding societies). Results: A list of 82 recommendations was formulated to support primary care physiotherapists when treating a person with a bleeding disorder. These recommendations could be divided into 13 categories: two including recommendations on organisation of care, six on therapy for adult patients with bleeding disorders and five on therapy adaptations for paediatric care. Therapy recommendations included treatment after a joint‐ or muscle bleed, haemophilic arthropathy, chronic synovitis, non‐haemophilia related conditions and orthopaedic surgery. Conclusion: An evidence‐based practice guideline, based on current evidence from literature and clinical expertise, has been developed for primary care physiotherapists treating a person with haemophilia. To improve care, the recommendations should be implemented in daily practice. [ABSTRACT FROM AUTHOR]

Municipal and industrial wastewater effluent is an important source of water for lotic systems, especially during periods of low flow. The accumulated wastewater effluent flows—expressed as a percentage of total streamflow (ACCWW%)—contain chemical mixtures that pose a risk to aquatic life; fish may be particularly vulnerable when chronically exposed. Although there has been considerable focus on individual‐level effects of exposure to chemical mixtures found in wastewater effluent, scaling up to population‐level effects remains a challenging component needed to better understand the potential consequences of exposure in wild populations. This may be particularly important under a changing climate in which wastewater reuse could be essential to maintain river flows. We evaluated the effects of chronic exposure to wastewater effluent, as measured by ACCWW%, on the relative abundance of young‐of‐year (YOY), juvenile, and adult smallmouth bass (Micropterus dolomieu) populations in the Shenandoah River Watershed (USA). We found that increases in ACCWW% in the previous year and during the prespawn period were negatively correlated with the relative abundance of YOY, resulting in an average 41% predicted decrease in abundance (range = 0.5%–94% predicted decrease in abundance). This lagged effect suggests that adult fish reproductive performance may be compromised by chemical exposure during periods of high ACCWW%. No relationships between ACCWW% and juvenile or adult relative abundance were found, suggesting that negative effects of ACCWW% on YOY abundance may be offset due to compensatory mechanisms following higher ACCWW% exposure. Understanding the effects of wastewater effluent exposure at multiple levels of biological organization will help in the development of management strategies aimed at protecting aquatic life. Environ Toxicol Chem 2024;43:1138–1148. © 2024 The Authors. Environmental Toxicology and Chemistry published by Wiley Periodicals LLC on behalf of SETAC. This article has been contributed to by U.S. Government employees and their work is in the public domain in the USA. [ABSTRACT FROM AUTHOR]

Aim: For people with haemophilia A (PwHA), bleeding in the joints leads to joint damage and haemophilia‐related arthropathy, impacting range of motion and life expectancy. Existing guidelines for managing haemophilia A support healthcare professionals (HCPs) and PwHA in their efforts to preserve joint health. However, such guidance should be reviewed, considering emerging evidence and consensus as presented in this manuscript. Methods: Fifteen HCPs experienced in the management of PwHA in the UK participated in a three‐round Delphi panel. Consensus was defined at ≥70% of panellists agreeing or disagreeing for Likert‐scale questions, and ≥70% selecting the same option for multiple‐ or single‐choice questions. Questions not reaching consensus were revised for the next round. Results: 26.8% (11/41), 44.8% (13/29) and 93.3% (14/15) of statements reached consensus in Rounds 1, 2 and 3, respectively. HCPs agreed that prophylaxis should be offered to patients with a baseline factor VIII (FVIII) level of ≤5 IU/dL and that, where there is no treatment burden, the aim of prophylaxis should be to achieve a trough FVIII level ≥15 IU/dL and maintain a longer period with FVIII levels of ≥20–30 IU/dL to provide better bleed protection. The aspirational goal for PwHA is to prevent all joint bleeds, which may be achieved by maintaining normalised (50–150 IU/dL) FVIII levels. Conclusion: The panel of experts were largely aligned on approaches to preserving joint health in PwHA, and this consensus may help guide HCPs. [ABSTRACT FROM AUTHOR]

A family of parsimonious contaminated shifted asymmetric Laplace mixtures is developed for unsupervised classification of asymmetric clusters in the presence of outliers and noise. A series of constraints are applied to a modified factor analyzer structure of the component scale matrices, yielding a family of twelve models. Application of the modified factor analyzer structure and these parsimonious constraints makes these models effective for the analysis of high-dimensional data by reducing the number of free parameters that need to be estimated. A variant of the expectation-maximization algorithm is developed for parameter estimation with convergence issues being discussed and addressed. Popular model selection criteria like the Bayesian information criterion and the integrated complete likelihood (ICL) are utilized, and a novel modification to the ICL is also considered. Through a series of simulation studies and real data analyses, that includes comparisons to well-established methods, we demonstrate the improvements in classification performance found using the proposed family of models. [ABSTRACT FROM AUTHOR]

Estimating relative abundance is critical for informing conservation and management efforts and for making inferences about the effects of environmental change on populations. Freshwater fisheries span large geographic regions, occupy diverse habitats and consist of varying species assemblages. Monitoring schemes used to sample these diverse populations often result in populations being sampled at different times and under different environmental conditions. Varying sampling conditions can bias estimates of abundance when compared across time, location and species, and properly accounting for these biases is critical for making inferences.We develop a joint species distribution model (JSDM) that accounts for varying sampling conditions due to the environment and time of sampling when estimating relative abundance. The novelty of our JSDM is that we explicitly model sampling effort as the product of known quantities based on time and gear type and an unknown functional relationship to capture seasonal variation in species life history.We use the model to study relative abundance of six freshwater fish species across the state of Minnesota, USA. Our model enables estimates of relative abundance to be compared both within and across species and lakes, and captures the inconsistent sampling present in the data. We discuss how gear type, water temperature and day of the year impact catchability for each species at the lake level and throughout a year. We compare our estimates of relative abundance to those obtained from a model that assumes constant catchability to highlight important differences within and across lakes and species.Synthesis and applications: Our method illustrates that assumptions relating indices of abundance to observed catch data can greatly impact model inferences derived from JSDMs. Specifically, not accounting for varying sampling conditions can bias inference of relative abundance, restricting our ability to detect responses to management interventions and environmental change. While our focus is on freshwater fisheries, this model architecture can be adopted to other systems where catchability may vary as a function of space, time and species. [ABSTRACT FROM AUTHOR]

Background: Improved approaches for chronic pain management are a clinical and research priority for people with haemophilia (PWH). Involving people with lived experience in the design of a complex rehabilitation intervention strengthens the credibility and plausibility of the intervention, particularly in relation to rare disorders. Here we describe using a 'Theory of Change' (ToC) dialogue-based stakeholder process to create a programme theory for a telerehabilitation intervention. Methods: An online workshop was convened and stakeholders received a briefing document in advance. Five stakeholders took part (3 PWH and 2 physiotherapists). At the workshop the group first agreed the overall aim of the intervention. Discussions then identified the resources, activities, barriers and enablers needed to achieve this outcome. All discussions were recorded and annotated by the workshop moderator. Behaviour change techniques were mapped for inclusion in the theory. Results: A programme theory and narrative report were produced. All stakeholders reviewed these for clarity and to ensure a true reflection of the workshop discussions. Agreement was based on how meaningful, well-defined, do-able, plausible, credible, and testable each component was. Stakeholders highlighted the importance of issues unique to PWH. Key components included the need for physiotherapists to be knowledgeable of the condition, a range of exercises that were inclusive of all abilities, and the need for people to feel safe and supported whilst taking part. Conclusions: Co-developed theory based approaches to intervention design offer an inclusive and transparent way to develop novel and meaningful interventions for people with complex health conditions. The ToC is wholly transparent in its design and content. Together with the identified behaviour change techniques, the theory informs the protocol for a feasibility study evaluating a telerehabilitation intervention. Importantly, it allows the opportunity to revise, adapt and improve the programme theory for further implementation and evaluation. [ABSTRACT FROM AUTHOR]

Introduction: Joint bleeds in haemophilia cause destruction of articular structures, impaired function and pain. Up to 70% of people with haemophilia (PWH) report chronic pain. Little is known about the pain experiences in PWH in the UK. Aim: To identify prevalence and perceptions of pain among PWH living in the UK. Methods: A cross‐sectional, non‐interventional survey study conducted among PWH (all severities). The survey incorporated elements from validated tools (EQ‐5D; EQ‐VAS) and was distributed via participating treatment centres. Results: Five hundred and ninety‐nine PWH responded, 91% aged > 18. 81% used factor prophylactically or on demand. More pain was reported by those treated on demand versus prophylaxis particularly in those who reported daily pain. 65% reported 'problem joints' based on individual impact rather than medically defined 'target joints', 2/3 reported multiple joint issues. The ankle was most commonly affected. 59% reported frequent pain, with 56% aware of pain constantly or most of the time and were more likely to report less favourable EQ‐5D or EQ‐VAS scores (p <.001). Pain frequency/awareness was consistent across all severities. Most discussed pain with care teams, 31% only when asked; 25% did not discuss it. Pain discussions resulted in physiotherapy referral (63%) analgesia prescription (48%), and a minority specialist pain referral (9%). Most felt well supported with regard to their pain, but 70% reported learning to live with it. Conclusion: Pain affects PWH of all ages and severities even in a well‐resourced country significantly impacting quality of life. Clinicians must be more aware of chronic pain in PWH. Biopsychosocial approaches to pain assessment and management are recommended. [ABSTRACT FROM AUTHOR]

This article discusses a case of a person with severe Haemophilia A who experienced chronic knee pain. The pain was initially thought to be associated with haemophilic arthropathy, but further investigation revealed that it was caused by bone marrow oedema syndrome. The patient was treated with zoledronic acid, which resulted in gradual improvement in the knee pain. This case highlights the importance of considering other potential causes of chronic joint pain in individuals with haemophilia. [Extracted from the article]

Introduction: The "problem joint" (PJ) concept was developed to address patient‐centric needs for a more holistic assessment of joint morbidity for people with haemophilia (PwH). Aim: To quantify the humanistic burden of PJs in PwH to further support validation of the PJ outcome measure. Methods: Multivariable regression models evaluated the relationship between PJs and health‐related quality of life (HRQoL, EQ‐5D‐5L) and overall work productivity loss (WPL) using data from the 'Cost of HaEmophilia: a Socioeconomic Survey' population studies (adults: CHESS II, CHESS US+; children/adolescents: CHESS‐Paeds). Covariates included were haemophilia severity, age, comorbidities and education. Results: The CHESS II sample included 292 and 134 PwH for HRQoL and WPL analyses, mean age 38.6 years (39% ≥1 PJ, 61% none). CHESS US+ included 345 and 239 PwH for HRQoL and WPL, mean age 35 years (43% ≥1 PJ, 57% none). CHESS‐Paeds included 198 PwH aged 4–17 (HRQoL only), mean age 11.5 years (19% ≥1 PJ, 81% none). In CHESS II and CHESS US+, presence of PJs was associated with worse HRQoL (Both p <.001). Few CHESS‐Paeds participants had PJs, with no significant correlation with HRQoL. In CHESS II, upper body PJs were significantly correlated to WPL (p <.05). In CHESS US+, having ≥1 PJ or upper and lower body PJs were significantly correlated to WPL (vs. none; both p <.05). Conclusion: This study has shown a meaningful burden of PJs on PwH, which should be considered in clinical and health policy assessments of joint health. [ABSTRACT FROM AUTHOR]

The philosophical underpinnings of Durkheim's failed Darwinian encounter have been neglected by environmental and mainstream sociologists. Although he claimed to employ Darwinian insights, Durkheim wrote during the eclipse of population thinking by an essentialist revival in biology. His inability to grasp the former and embrace of a specific variety of the latter explain the limitations and contradictions in his incipient environmental sociology and challenge the broader disciplinary myth that Durkheim discovered a new approach to theorizing society. Even his repudiation of Lamarckian analogies relied upon and reinforced his more fundamental commitment to essentialism. That commitment has contributed to the persistence of developmentalism within sociology and delayed a second Darwinian revolution. Seizing the opportunity that Durkheim missed by confronting the deeper lessons of the first Darwinian revolution offers the best hope for constructing a post-exemptionalist theory of societal-environmental interactions and addressing enduring disciplinary concerns with structural diversity and human agency. [ABSTRACT FROM AUTHOR]

The article discusses the different types of restrictive interventions and describes some strategies that can support reduction of their use in mental health settings. Topics discussed include different types of restrictive interventions such as enhanced observations, seclusion and restraint; ethical issues and legal framework around restrictive interventions; and strategies to prevent behaviour that challenges and reduce the need for restrictive interventions.

Haemophilia treatment has seen great advances in recent years with an accompanied reduced risk of physical activity (PA) related bleeds. Based on its known health benefits, people with haemophilia (PWH) are currently encouraged to regularly engage in PA. However, this may not always translate to increased levels of PA. In this narrative review we aim to provide a brief overview of what is currently understood regarding PA levels and influences for the three broad age groups of children and adolescents, adults and older adults. We also provide recommendations for members of the haemophilia team on important aspects related to promotion of PA in their clinical practice. We highlight that PA behaviour is multifactorial and that many PWH still have limited access to adequate care. Whilst some still face unique challenges to being more physically active, overall, the barriers and facilitators to activity are very similar to that of the general population. [ABSTRACT FROM AUTHOR]

Study models are frequently used in orthodontics and are a key part of both initial assessment and treatment planning. They are an important part of comprehensive orthodontic records; furthermore, study models are an essential diagnostic aid that can be studied in detail without the patient being present. We present a case of a 14-year-old boy with an abnormal palatal swelling detected by his specialist orthodontist after review of study models taken at the initial appointment. At the assessment alone, the significance of the abnormality was not recognised and without the help of study models, diagnosis and treatment of his fibrous dysplasia may have been significantly delayed. [ABSTRACT FROM AUTHOR]

Over the past two decades there have been many advancements in modeling capture–recapture (CR) data to account for emerging data collection technology and techniques. Spatial capture–recapture (SCR) models have been introduced to estimate population size and numerous other demographic parameters from spatially explicit CR data. Recently SCR models have also begun incorporating realistic animal movement to account for individual dispersion and attraction to resources. While some species of animals are known to exhibit attractive behavior, nearly all SCR models assume complete independence among individual's movement and capture probability. In this article, we introduce an SCR model which allows for attractions between individuals via their daily location. We demonstrate via a simulation study that accounting for the attractions specified by our model, when present, can improve population size estimation. In addition, we apply our model to an iconic SCR dataset to estimate the population size and attraction parameters of a Bengal tiger (Panthera tigris tigris) population. [ABSTRACT FROM AUTHOR]

Purpose: Approximately 35%‐50% of people with haemophilia (PWH) report living with chronic musculoskeletal pain. Although exercise based rehabilitation is effective for pain in other arthritises, there are no published guidelines for management of chronic pain in PWH. This review aims to evaluate and appraise the current evidence of effectiveness of physiotherapy interventions on (a) pain intensity, (b) quality of life (QoL) and (c) function in PWH. Methods: A systematic review of five databases AMED and CINAHL, EMBASE and MEDLINE and PEDro, as well as trial registries, grey literature and hand searching key journals was completed. Included studies were critically appraised and evaluated for risk of bias. The GRADE approach was used to rate the quality of the evidence. Results: Nine trials consisting of 235 participants met the inclusion criteria. All studies had an overall risk of bias with low methodological quality. Meta‐analysis was not possible due to heterogeneity across trials. Studies comparing a range of physiotherapy interventions against no intervention showed no clear beneficial effect on pain intensity or QoL. Only one study, investigating hydrotherapy or land‐based exercise against control, showed positive effect for pain intensity, but rated very low on GRADE assessment. Studies comparing one physiotherapy intervention against another showed no clear benefit on pain intensity, QoL or function. LASER with exercise and hydrotherapy were shown to have some positive effects on pain intensity, but no clear benefit on function. Conclusions: At present, there is limited evidence for the use of physiotherapy interventions in addressing the issue of pain in PWH. Better designed trials with higher quality and explicit methodology along with user involvement are needed to assess the efficacy of any proposed intervention. [ABSTRACT FROM AUTHOR]

This article recommends that law libraries and their librarians use the upcoming U.S. News & World Report's academic rankings for law schools as an opportunity to enhance academic law libraries' standing in the legal profession and to elevate law librarians' statures within law schools. [ABSTRACT FROM AUTHOR]

Background Postoperative delirium is a serious neuropsychiatric condition that occurs in up to 65% of hip fracture patients(1). However, it remains poorly recognised as a postoperative surgical complication despite its association with increased mortality, longer length of stay (LOS), cognitive decline and increased risk of discharge institutionalisation. Methods As part of the delirium audit, all hip fracture patient notes were reviewed from Oct-Nov 2017 pre-implementation of an Orthogeriatric Service and Oct-Nov 2018 post-implementation. Documentation of delirium or use of the terms 'new confusion, altered attention, change in behaviour' in the orthopaedic, orthogeriatric and allied health professional notes were reviewed. The 4AT was used to routinely screen for delirium in the pre-and post-operative period by the Orthogeriatrics team(2). Results The number of patients in the 2017 group was 24, mean age 78, female 76% compared with 25 in the 2018 group, mean age 75.9, female 81%. In 2017, delirium was diagnosed in 4% of patients by the Orthopaedic surgeons and 37.5% by Allied Health Professionals. In 2018 delirium was diagnosed in 4% by the Orthopaedic surgeons, 40% by Allied Health Professionals and 44% by the Orthogeriatrics team in 2018. Pre-operative delirium occurred in 16% of patients and postoperative delirium occurred in 44% of patients in 2018. A clear delirium management plan was documented in 0% of charts in 2017 versus 100% in 2018. LOS in 2017 was 12.7 days vs 12.4 in 2018. However, discharge location varied greatly with 33.3% discharged to convalescence in 2017 versus 19% in 2018, 12.5% to Longterm care in 2017 versus 4% in 2018, 20.8% to rehabilitation in 2017 vs 52% in 2018 and 16.7% home in 2017 vs 24% in 2018. Conclusion Prompt diagnosis and management of delirium is essential in optimising postoperative cognitive function and preserving independence. Our study highlights the need for routine delirium screening and improved awareness of delirium amongst all healthcare professionals. [ABSTRACT FROM AUTHOR]

Introduction: European guidelines on the care of haemophilia recommend ready access to a range of services provided by a multidisciplinary team of specialists including physiotherapy. However, the scope of physiotherapy provided is unknown. Methods: The Physiotherapists Committee of the European Association for Haemophilia and Allied Disorders (EAHAD) conducted a web‐based survey to quantify the role and scope of practice of physiotherapists involved in haemophilia care. The survey was sent to more than 200 physiotherapists registered on the EAHAD database. Questions concerned their work practices including assessment and treatment activities and level of autonomy. Results: Eighty physiotherapists from twenty‐four European countries responded. Considerable heterogeneity exists in roles, responsibilities, and clinical practice of physiotherapists, particularly in access to and type of physiotherapy treatment provided, as well as the skill set and autonomy of physiotherapists to make independent assessment and treatment decisions. Discussion: This pan‐European survey establishes a context to support physiotherapy role development and professional identity. Key recommendations include the following: (a) establishing a pan‐European network to support collaboration and education for physiotherapists working in haemophilia, (b) developing a core skills and capability framework to ensure person‐centred approaches are central as well as working in partnership with those with the condition to maximize early recovery, support self‐management and enablement in remaining active and independent, (c) regular training, standardized validation and maintenance of competency for assessment tools, (d) well‐designed randomized clinical studies with larger numbers of participants from multiple sites should be the focus of future research. [ABSTRACT FROM AUTHOR]

The United Kingdom National Health Service (NHS) response to the coronavirus pandemic (COVID-19) involved far-reaching service reconfigurations to release clinical capacity, including staff redeployment and whole department relocations. 4 Kulkarni R. Use of telehealth in the delivery of comprehensive care for patients with haemophilia and other inherited bleeding disorders. A virtual meeting of UK NHS haemophilia physiotherapists took place on 3rd June 2020 to share how services had been affected during the pandemic and review potential implications for physiotherapy in the future. [Extracted from the article]

We encourage therapists to critically examine their own practice, as well as asking centre directors and therapy managers to see the value of a well-defined physiotherapy service within haemophilia. GLO:EVW/01mar21:hae14150-fig-0001.jpg PHOTO (COLOR): 1 Recommendation for reviewing provision of a local haemophilia physiotherapy service gl It remains unclear if new treatments (EHL's, non-substitutive therapies) will considerably reduce the frequency of bleeding in patients treated. Acute pain significantly impacts quality of life and is greater when present on a backdrop of ongoing persistent pain.6 An increase in age is also associated with a decrease in quality of life predominantly related to haemophilia-specific MSK complications, that is, joint disease. [Extracted from the article]

Introduction: Comprehensive musculoskeletal assessment for monitoring joint health in haemophilia includes both physical assessment with Haemophilia Joint Health Score (HJHS) and assessment of self‐reported function by Haemophilia Activities List (HAL). Methods: Correlation between physical assessment and joint function was undertaken between HJHS and HAL in patients with SHA and SHB who had both assessments at the same visit over a one‐year period. Results: Data from 120 patients (96‐SHA/24 = SHB) with a median age 33 years (range 19‐73) were included. Median total HJHS was 19, increasing with age: 18‐30 years—7, 31‐50 years—25 and 51‐73 years—44. Similarly, median total HAL score was 80 with decreased function associated with increasing age: 18‐30 years—90.4, 31‐59 years—71.7, 51‐73 years—49.5. Median Total HJHS and HAL demonstrated strong correlation (rs = 0.66, P < 0.01). Moderate‐to‐strong correlations were seen across the entire age group between the HJHS LL and UL subtotals and corresponding limb HAL domains. Within age groups, correlations were less significant particularly for the upper UL domains in HAL and the UL HJHS score. The wide range of ROM in joints categorized as markedly affected (ie, ROM loss score = 3) highlights the potential ceiling effect of this domain score and its use in chronically damaged joints. Conclusion: HJHS and HAL showed moderate‐to‐strong correlation with discrepancy in some individual patients. Prospective studies are required to better understand the clinical utility of both especially in severe joint disease where HAL may have a potential advantage. [ABSTRACT FROM AUTHOR]

Psychological research on the topic of wisdom is limited in its incorporation of religion and spirituality. This gap in psychological literature may serve to limit a thorough understanding of wisdom. Positive psychology may allow for some rapprochement in wisdom and spirituality. In collaboration with leaders of a local Friends (Quaker) congregation, two studies investigated the effects of a spiritually informed wisdom intervention delivered in the context of a faith community. Participants for Study 1 consisted of 27 young adults (24 completed both the pre and post questionnaire) and a comparison group consisting of 32 young adults. The intervention was designed to increase participants’ abilities in cognitive, affective and moral domains. Significant group by time interaction effects were found among measurements of practical wisdom, post-formal thinking and subjective well-being, with those in the experimental group showing changes in the expected direction. Study 2 involved qualitative analyses of interviews with 15 of the wisdom participants regarding their changing views of wisdom. Themes emerging from the interviews included a multiple-perspectives view of wisdom, the relational nature of learning wisdom, the importance of taking a reflective perspective, and congruence between what one knows and how one lives. Implications for studying wisdom alongside religion and spirituality are considered. [ABSTRACT FROM AUTHOR]

The phloem sucrose transporter, AtSUC2, is promiscuous with respect to substrate recognition, transporting a range of glucosides in addition to sucrose, including naturally occurring coumarin glucosides. We used the inherent fluorescence of coumarin glucosides to probe the specificity of AtSUC2 for its substrates, and determined the structure-activity relationships that confer phloem transport in vivo using Arabidopsis seedlings. In addition to natural coumarin glucosides, we synthesized new compounds to identify key structural features that specify recognition by AtSUC2. Our analysis of the structure-activity relationship revealed that the presence of a free hydroxyl group on the coumarin moiety is essential for binding by AtSUC2 and subsequent phloem mobility. Structural modeling of the AtSUC2 substrate-binding pocket explains some important structural requirements for the interaction of coumarin glucosides with the AtSUC2 transporter. [ABSTRACT FROM AUTHOR]

The article focuses on the overview of the government's policing powers related to medical care in the U.S. Topics discussed include influence of health officials over the individual's health care choices; enactment of Food and Drug Act and Maternity and Infancy Act for addressing the controversies surrounding medical treatment; and Hironimus v. Nebus court case on the same.

The first Team Haemophilia Education ( THE) Meeting was held on 7-8 May 2015 in Amsterdam, The Netherlands. It aimed to promote the optimal care of patients with haemophilia through education of the multidisciplinary treatment team. This was achieved by reviewing the latest developments in haemophilia management, considering how these can be implemented in the clinic to improve patient care and providing a platform for networking and debate for all haemophilia treatment team members. The second THE Meeting was held on 19-20 May in Frankfurt, Germany, and participants included doctors, nurses, physiotherapists, patient representatives and data management staff from 20 different countries. Topics covered the role of the multidisciplinary team in delivering the best haemophilia care, challenges in the management of haemophilia across Europe, available clotting factor treatments, future treatments and the use of genetics in advising carriers of haemophilia. This report is a summary of the key developments in haemophilia care presented by various investigators and healthcare professionals at THE Meeting 2016. [ABSTRACT FROM AUTHOR]

To assess the association between different types of coercive measures (forced medication, seclusion, and restraint) used during involuntary psychiatric admission and two treatment outcomes: retrospective views of patients towards their admission and length of inpatient stay. A secondary analysis was conducted of data previously gathered by the EUNOMIA study (n = 2030 involuntarily detained inpatients across 10 European countries, of whom 770 were subject to one or more coercive measures). Associations between coercive measures and outcomes were tested through multivariable regression models adjusted for patients' socio-demographic and clinical characteristics. Use of forced medication was associated with patients being significantly less likely to justify their admission when interviewed after three months. All coercive measures were associated with patients staying longer in hospital. When the influence of other variables was considered in a multi-variate analysis, seclusion remained as a significant predictor of longer inpatient stay, adding about 25 days to the average admission. Of the three coercive measures, forced medication appears to be unique in its significant impact on patient disapproval of treatment. While all coercive measures are associated with patients staying longer in hospital, only use of seclusion is associated with longer inpatient stays independently of coerced patients’ having higher symptom scores at the time of admission. [ABSTRACT FROM AUTHOR]

The article focuses on technology involved in end-to-end encryption and strong encryption makes less susceptible to hacking and cyber-attack. Topics discussed include usage of end-to-end encryption technology is for benign purposes such as to facilitate online banking and shopping, increased use of encryption for both individual privacy and potential terrorist and controversy between the U.S. Federal Bureau of Investigation (FBI) and technology firm Apple.

Aims To compare long-term outcomes among participants randomized to buprenorphine or methadone. Design, Setting and Participants Follow-up was conducted in 2011-14 of 1080 opioid-dependent participants entering seven opioid treatment programs in the United States between 2006 and 2009 and randomized (within each program) to receive open-label buprenorphine/naloxone or methadone for up to 24 weeks; 795 participants completed in-person interviews (~74% follow-up interview rate) covering on average 4.5 years. Measurements Outcomes were indicated by mortality and opioid use. Covariates included demographics, site, cocaine use and treatment experiences. Findings Mortality was not different between the two randomized conditions, with 23 (3.6%) of 630 participants randomized to buprenorphine having died versus 26 (5.8%) of 450 participants randomized to methadone. Opioid use at follow-up was higher among participants randomized to buprenorphine relative to methadone [42.8 versus 31.7% positive opioid urine specimens, P < 0.01, effect size (h) = 0.23 (0.09, 0.38); 5.8 days versus 4.4 days of past 30-day heroin use, P < 0.05, effect size (d) = 0.14 (0.00, 0.28)]. Opioid use during the follow-up period by randomization condition was also significant (F(7,39 600) = 3.16; P < 0.001) due mainly to less treatment participation among participants randomized to buprenorphine than methadone. Less opioid use was associated with both buprenorphine and methadone treatment (relative to no treatment); no difference was found between the two treatments. Individuals who are white or used cocaine at baseline responded better to methadone than to buprenorphine. Conclusions There are few differences in long-term outcomes between buprenorphine and methadone treatment for opioid dependence, and treatment with each medication is associated with a strong reduction in opioid use. [ABSTRACT FROM AUTHOR]

Scientific literature increasingly calls for studies to translate evidence-based interventions into real-world contexts balancing fidelity to the original design and fit to the new setting. The Risk Avoidance Partnership (RAP) is a health promotion intervention originally designed to train active drug users to become Peer Health Advocates. A theoretically driven approach was used to adapt RAP to fit implementation in outpatient methadone treatment clinics and pilot it with clinic patients. Ethnographic observations and process tracking documented the RAP translation and pilot experience, and clinic and community characteristics relevant to program implementation. Clinic administrators, staff, and patients were interviewed on their values, capacities, interest in RAP, perceived challenges of implementing RAP in drug treatment clinics, and experiences during the pilot. Findings indicated that RAP core components can be met when implemented in these settings and RAP can fit with the goals, interests, and other programs of the clinic. Balancing fidelity and fit requires recognition of the mutual impacts RAP and the clinic have on each other, which generate new interactions among staff and require ongoing specification of RAP to keep abreast of clinic and community changes. Collaboration of multiple stakeholders significantly benefited translation and pilot processes. [ABSTRACT FROM AUTHOR]

The role of the law librarian has changed from managing the contents of a library’s collection of books to knowing how to find information sources located around the world contained in a variety of formats, taking part in instruction, and participating in networking activities. Law librarians are constrained by legal and professional codes. If they are cautious, law librarians can assist, instruct, and reach out to public patrons and students while operating within the professional guidelines that govern them. [ABSTRACT FROM PUBLISHER]

Summary The ADAR RNA-editing enzymes deaminate adenosine bases to inosines in cellular RNAs. Aberrant interferon expression occurs in patients in whom ADAR1 mutations cause Aicardi-Goutières syndrome (AGS) or dystonia arising from striatal neurodegeneration. Adar1 mutant mouse embryos show aberrant interferon induction and die by embryonic day E12.5. We demonstrate that Adar1 embryonic lethality is rescued to live birth in Adar1 ; Mavs double mutants in which the antiviral interferon induction response to cytoplasmic double-stranded RNA (dsRNA) is prevented. Aberrant immune responses in Adar1 mutant mouse embryo fibroblasts are dramatically reduced by restoring the expression of editing-active cytoplasmic ADARs. We propose that inosine in cellular RNA inhibits antiviral inflammatory and interferon responses by altering RLR interactions. Transfecting dsRNA oligonucleotides containing inosine-uracil base pairs into Adar1 mutant mouse embryo fibroblasts reduces the aberrant innate immune response. ADAR1 mutations causing AGS affect the activity of the interferon-inducible cytoplasmic isoform more severely than the nuclear isoform. [ABSTRACT FROM AUTHOR]

This study examines the barriers and facilitators of retention among patients receiving buprenorphine/naloxone at eight community-based opioid treatment programs across the United States. Participants (n = 105) were recruited up to three and a half years after having participated in a randomized clinical trial comparing the effect of buprenorphine/naloxone and methadone on liver function. Semi-structured interviews were conducted with 67 patients provided with buprenorphine/naloxone who had terminated early and 38 patients who had completed at least 24 weeks of the trial. Qualitative data were analyzed using the constant comparison method. Barriers to buprenorphine/naloxone retention that emerged included factors associated with: (1) the design of the clinical trial; (2) negative medication or treatment experience; and (3) personal circumstances. The facilitators comprised: (1) positive experience with the medication; (2) personal determination and commitment to complete; and (3) staff encouragement and support. The themes drawn from interviews highlight the importance of considering patients’ prior experience with buprenorphine/naloxone and methadone, medication preference, personal circumstances, and motivation to abstain from illicit use or misuse of opioids, as these may influence retention. Ongoing education of patients and staff regarding buprenorphine/naloxone, especially in comparison to methadone, and support from staff and peers are essential. [ABSTRACT FROM AUTHOR]

Nearly 150 different enzymatically modified forms of the four canonical residues in RNA have been identified. For instance, enzymes of the ADAR (adenosine deaminase acting on RNA) family convert adenosine residues into inosine in cellular dsRNAs. Recent findings show that DNA endonuclease V enzymes have undergone an evolutionary transition from cleaving 3 to deoxyinosine in DNA and ssDNA to cleaving 3 to inosine in dsRNA and ssRNA in humans. Recent work on dsRNA-binding domains of ADARs and other proteins also shows that a degree of sequence specificity is achieved by direct readout in the minor groove. However, the level of sequence specificity observed is much less than that of DNA major groove-binding helix-turn- helix proteins. We suggest that the evolution of DNA-binding proteins following the RNA to DNA genome transition represents the major advantage that DNA genomes have over RNA genomes. We propose that a hypothetical RNA modification, a RRAR (ribose reductase acting on genomic dsRNA) produced the first stretches of DNA in RNA genomes. We discuss why this is the most satisfactory explanation for the origin of DNA. The evolution of this RNA modification and later steps to DNA genomes are likely to have been driven by cellular genome co-evolution with viruses and intragenomic parasites. RNA modifications continue to be involved in host-virus conflicts; in vertebrates, edited cellular dsRNAs with inosine-uracil base pairs appear to be recognized as self RNA and to suppress activation of innate immune sensors that detect viral dsRNA. [ABSTRACT FROM AUTHOR]

A charge transport model has been applied to predict dielectric breakdown in copper interconnects. The model very accurately predicts the lifetimes of dense and porous dielectrics over long periods. The predictions of the model significantly vary from currently available methodologies, which fail to adequately account for copper charge transport. We apply this charge transport model to simulate the TDDB lifetime activation energy ( Ea, eV) in copper interconnects. The model again very accurately predicts the available activation energy data, in actual interconnect test structures, over a wide range of electric fields. The model can be applied to determine the reliable operating voltage for a given operating temperature. [ABSTRACT FROM PUBLISHER]