Primary Small Bowel Liposarcoma (Atypical Lipomatous Tumour) with Myogenic Differentiation

Primary small intestinal liposarcomas originating in the small bowel are uncommon with a generally poor prognosis due to the advanced stage at the time of diagnosis. We describe a case of primary small bowel dedifferentiated liposarcoma presenting as a solid mass in the right iliac fossa. The current case is unusual as the tumour seemingly originated from the bowel and the well-differentiated component was seen extensively infiltrating the bowel wall including the small bowel submucosa

Morphological variation in Leptodactylus lutzi (Anura, Leptodactylidae) with description of its advertisement call and notes on its courtship behavior

Prior to this study, Leptodactylus lutzi was known only from three localities in west-central Guyana. We refer here to a series of 60 additional specimens of L. lutzi collected in Kaieteur National Park, Mount Ayanganna, Mount Wokomung and the Merume Mountains in the Pakaraima Mountains region, substantially extending the known distribution of the species. We provide a revised diagnosis and an expanded description of L. lutzi discussing the extent of color-pattern variation in the species. Additionally, we describe its advertisement call and provide data on its reproductive biology

A new species of Colostethus (Anura, Dendrobatidae) from French Guiana with a redescription of Colostethus beebei (Noble, 1923) from its type locality

A new species of Colostethus, long mistaken for Colostethus beebei, is described from French Guiana. The new species can be distinguished from congeners by absence of median lingual process, first finger longer than second, third finger not distinctly swollen in males, differences in tadpole morphology, coloration and pattern (e.g. absence of dorsolateral stripe), bioacoustics, and reproductive behavior. A complete redescription of Colostethus beebei plus description of its tadpole and call is provided on the basis of recently collected topotypic specimens. The range of C. beebei is restricted to the Kaieteur plateau, Pakaraima Mountains, Guyana

Quality of Life with Late-Onset Pompe Disease: Qualitative Interviews and General Public Utility Estimation in the United Kingdom

Background: Late-onset Pompe disease (LOPD) is a rare, progressive neuromuscular condition typically characterized by weakness of skeletal muscles, including those involved in respiration and diaphragmatic dysfunction. Individuals with LOPD typically eventually require mobility and/or ventilatory support. / Objectives: This study aimed to develop health state vignettes and estimate health state utility values for LOPD in the United Kingdom. / Methods: Vignettes were developed for 7 health states of LOPD with states defined in terms of mobility and/or ventilatory support. Vignettes were drafted based on patient-reported outcome data from the Phase 3 PROPEL trial (NCT03729362) and supplemented by a literature review. Qualitative interviews with individuals living with LOPD and clinical experts were conducted to explore the health-related quality-of-life (HRQoL) impact of LOPD and to review the draft vignettes. Vignettes were finalized following a second round of interviews with individuals living with LOPD and used in health state valuation exercises with people of the UK population. Participants rated the health states using the EQ-5D-5L, visual analog scale, and time trade-off interviews. / Results: Twelve individuals living with LOPD and 2 clinical experts were interviewed. Following the interviews, 4 new statements were added regarding dependence on others, bladder control problems, balance issues/fear of falling, and frustration. One hundred interviews with a representative UK population sample were completed. Mean time trade-off utilities ranged from 0.754 (SD = 0.31) (no support) to 0.132 (SD = 0.50) (invasive ventilatory and mobility support-dependent). Similarly, EQ- 5D-5L utilities ranged from 0.608 (SD = 0.12) to -0.078 (SD = 0.22). / Discussion: The utilities obtained in the study are consistent with utilities reported in the literature (0.670-0.853 for nonsupport state). The vignette content was based on robust quantitative and qualitative evidence and captured the main HRQoL impacts of LOPD. The general public rated the health states consistently lower with increasing disease progression. There was greater uncertainty around utility estimates for the severe states, suggesting that participants found it harder to rate them. / Conclusion: This study provides utility estimates for LOPD that can be used in economic modeling of treatments for LOPD. Our findings highlight the high disease burden of LOPD and reinforce the societal value of slowing disease progression

Asynchronous simulation of Boolean networks by monotone Boolean networks

International audienceWe prove that the fully asynchronous dynamics of a Boolean network f : {0, 1}^n → {0, 1}^n without negative loop can be simulated, in a very specific way, by a monotone Boolean network with 2n components. We then use this result to prove that, for every even n, there exists a monotone Boolean network f : {0, 1}^n → {0, 1}^n , an initial configuration x and a fixed point y of f such that: (i) y can be reached from x with a fully asynchronous updating strategy, and (ii) all such strategies contains at least 2^{n/2} updates. This contrasts with the following known property: if f : {0, 1}^n → {0, 1}^n is monotone, then, for every initial configuration x, there exists a fixed point y such that y can be reached from x with a fully asynchronous strategy that contains at most n updates

The Archaeology of the Siege of Fort William, 1746

In August and September 2007, the Centre for Battlefield Archaeology and Glasgow University Archaeological Research Division (GUARD) conducted a programme of archaeological investigation of the remains of the old fort at Fort William and part of The Parade in the town of Fort William on the west coast of Scotland. The fieldwork involved geophysical survey at the fort and The Parade, followed by trial excavation of anomalies. Trial trenches at The Parade exposed several rich midden deposits and material providing evidence for the burning of the town of Maryburgh, as suggested in contemporary accounts in 1746. The results at the fort were not so positive, as most traces of the garrison were removed in the 19th and 20th centuries through its use as a railway yard; however, a trench outside the fort suggests survival of midden deposits pre-dating this period of destruction. This part-Heritage Lottery assisted project was a Highland 2007 initiative supported by Lochaber Community Fund and Highland Council, and included active participation on the part of the local community, including school groups and metal detectorists

Development of an online information and support resource for adolescent idiopathic scoliosis patients considering surgery: perspectives of health care providers

<p>Abstract</p> <p>Background</p> <p>Adolescents with idiopathic scoliosis who are considering spinal surgery face a major decision that requires access to in-depth information and support. Unfortunately, most online resources provide incomplete and inconsistent information and minimal social support. The aim of this study was to develop an online information and support resource for adolescent idiopathic scoliosis (AIS) patients considering spinal surgery. Prior to website development, a user-based needs assessment was conducted. The needs assessment involved a total of six focus groups with three stakeholder groups: (1) post-operative AIS patients or surgical candidates (10-18 years) (n = 11), (2) their parents (n = 6) and (3) health care providers (n = 11). This paper reports on the findings from focus groups with health care providers.</p> <p>Methods</p> <p>Focus group methodology was used to invite a range of perspectives and stimulate discussion. During audio-recorded focus groups, an emergent table of website content was presented to participants for assessment of relevance, viability and comprehensiveness in targeting global domains of need. Specifically, effective presentation of content, desired aspects of information and support, and discussions about the value of peer support and the role of health professionals were addressed. Focus group transcripts were then subject to content analysis through a constant comparative review and analysis.</p> <p>Results</p> <p>Two focus groups were held with health care providers, consisting of 5 and 6 members respectively. Clinicians provided their perceptions of the information and support needs of surgical patients and their families and how this information and support should be delivered using internet technology. Health care providers proposed four key suggestions to consider in the development of this online resource: (1) create the website with the target audience in mind; (2) clearly state the purpose of the website and organize website content to support the user; (3) offer a professionally-moderated interactive support component; and (4) ensure accessibility of website information and support by considering the age, gender, reading level and geographic location of potential users.</p> <p>Conclusions</p> <p>Health care providers collectively identified the need for the development of an online information and support resource for adolescents considering surgery for AIS and their families and described the proposed website as a positive and needed adjunct to current clinical care.</p

(In)formalization and the civilizing process : applying the work of Norbert Elias to housing-based anti-social behaviour interventions in the UK

This paper uses Norbert Elias's theory of the civilizing process to examine trends in social conduct in the UK and to identify how problematic “anti‐social” behaviour is conceptualized and governed through housing‐based mechanisms of intervention. The paper describes how Elias's concepts of the formalization and informalization of conduct and the construction of established and outsider groups provide an analytical framework for understanding social relations. It continues by discussing how de‐civilizing processes are also evident in contemporary society, and are applied to current policy discourse around Respect and anti‐social behaviour. The paper uses the governance of “anti‐social” conduct through housing mechanisms in the UK to critique the work of Elias and concludes by arguing that a revised concept of the civilizing process provides a useful analytical framework for future studies

Towards an understanding of the information and support needs of surgical adolescent idiopathic scoliosis patients: a qualitative analysis

<p>Abstract</p> <p>Background</p> <p>Informed decision making for adolescents and families considering surgery for scoliosis requires essential information, including expected outcomes with or without treatment and the associated risks and benefits of treatment. Ideally families should also receive support in response to their individual concerns. The aim of this study was to identify health-specific needs for online information and support for patients with adolescent idiopathic scoliosis who have had or anticipate having spinal surgery.</p> <p>Methods</p> <p>Focus group methodology was chosen as the primary method of data collection to encourage shared understandings, as well as permit expression of specific, individual views. Participants were considered eligible to participate if they had either experienced or were anticipating surgery for adolescent idiopathic scoliosis within 12 months, were between the ages of 10 and 18 years of age, and were English-speaking.</p> <p>Results</p> <p>Two focus groups consisting of 8 adolescents (1 male, 7 female) and subsequent individual interviews with 3 adolescents (1 male, 2 female) yielded a range of participant concerns, in order of prominence: (1) recovery at home; (2) recovery in hospital; (3) post-surgical appearance; (4) emotional impact of surgery and coping; (5) intrusion of surgery and recovery of daily activities; (6) impact of surgery on school, peer relationships and other social interactions; (7) decision-making about surgery; (8) being in the operating room and; (9) future worries.</p> <p>Conclusion</p> <p>In conclusion, adolescents welcomed the possibility of an accessible, youth-focused website with comprehensive and accurate information that would include the opportunity for health professional-moderated, online peer support.</p