699 research outputs found
Modeling and Rescue of RP2 Retinitis Pigmentosa Using iPSC-Derived Retinal Organoids
No full textCheetham and colleagues show that 3D retinal organoids lacking the RP2 protein develop rod photoreceptor degeneration that can be prevented with AAV gene augmentation for RP2
A tale of two siblings: two cases of AML arising from a single pre-leukemic DNMT3A mutant clone
No full textLetters to the EditorC N Hahn, D M Ross, J Feng, A Beligaswatte, D K Hiwase, W T Parker, M Ho, M Zawitkowski, K L Ambler, G D Cheetham, Y K Lee, M Babic, C M Butcher, G A Engler, A L Brown, R J D'Andrea, I D Lewis, A W Schreiber, L B To and H S Scot
Hypertonic saline test for the investigation of posterior pituitary function
No full textThe hypertonic saline test is a useful technique for distinguishing partial diabetes insipidus from psychogenic polydipsia, and for the diagnosis of complex disorders of osmoreceptor and posterior pituitary function. However, there is little information concerning its use in childhood. The experience of using this test in five children (11 months to 18 years) who presented diagnostic problems is reported. In two patients, in whom water deprivation tests were equivocal or impractical, an inappropriately low antidiuretic hormone (ADH) concentration ( 295 mosmol/kg). In two children--one presenting with adipsic hypernatraemia and the other with hyponatraemia complicating desmopressin treatment of partial diabetes insipidus--defects of osmoreceptor function were identified. Confirming a diagnosis of idiopathic syndrome of inappropriate ADH secretion (SIADH) was possible in a patient with no other evidence of pituitary dysfunction. The hypertonic saline test was well tolerated, easy to perform, and diagnostic in all cases
The acquisition of professional competence.
No full textThe research began by examining professions, both historically and as they are today. It went on to investigate the nature of professional practice and professional competence. In particular, it attempted to throw fresh light on how professional competence is acquired and maintained. The research consisted of an extensive literature study and empirical work which included in- depth interviews with practitioners from 20 different professions. This was followed by a series of larger-scale postal surveys across six of these. The chosen professions ranged from traditional ones, including the Church, the Bar and Medicine, to newer professional-type occupations, such as Training, Personnel Management and Information Technology Consultancy. A total of 80 people were interviewed and a further 372 completed survey questionnaires. Following a review of existing models of competence, a new, 'provisional model of professional competence' was developed. This drew on a number of earlier models and related concepts, and was influenced by both reflective practitioner and competence based approaches to professional development. The new model was exposed to expert and academic criticism through a series of conference and journal papers, comments being received from a total of 70 people. The model was also tested empirically, after which a revised version was developed. The empirical stage of the research had a number of strands, in addition to being used for testing the model. It examined the nature of modem professions and tested the validity of certain characteristics traditionally associated with professions - altruism, autonomy, self- regulation, etc. It also examined the nature of professional practice and tested the validity of two competing epistemologies - 'technical-rationality' and 'knowing-in-action' (reflective practice). It investigated how professional competence is acquired, examining the contribution made by both formal development programmes and various kinds of informal experience to which practitioners are exposed. It sought to identify the conditions and environments that are important to professional learning, especially in early practice. Finally, it looked at how professionals maintain their competence through personal learning and continuing professional development (CPD). Both qualitative and quantitative methods were employed throughout. Analysis of results led to the identification of 12 general learning processes or 'learning mechanisms'. These formed the basis of a taxonomy which was used to classify the range of informal learning experiences and events reported by respondents. Outputs from the research included: "a new model of professional competence; "a taxonomy of informal professional learning methods; "a proposed new (or modified) epistemology of professional practice; "a proposed new paradigm of professional development, together with a linked self- development paradigm; an exemplar professional development model; and "a number of practical suggestions for improving professional development programmes. The research has contributed to knowledge by throwing new light on the nature of both professional practice and professional competence, and providing insights into the ways in which people become effective in their professional role
"Test me and treat me" - attitudes to vitamin D deficiency and supplementation: a qualitative study
No full text© 2015 BMJ Open, "Test me and treat me"-attitudes to vitamin D deficiency and supplementation: a qualitative study. This manuscript version is made available under the Creative Commons Attribution Licens
Producing & Consuming Public Space: A ‘Rhythmanalysis’ of the Urban Park
No full textResearch suggests an opportunity to offer a more comprehensive analysis of temporal consumption experiences encountered by park users, and the subsequent contribution to a perceived ‘sense of place’. Using visual ethnography and rhythmanalysis, our study distances our analysis from textual accounts of park usage as well as provide policy recommendations
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Proceedings of the Rank Forum on Vitamin D
No full textThe Rank Forum on Vitamin D was held on 2nd and 3rd July 2009 at the University of Surrey, Guildford, UK. The workshop consisted of a series of scene-setting presentations to address the current issues and challenges concerning vitamin D and health, and included an open discussion focusing on the identification of the concentrations of serum 25-hydroxyvitamin D (25(OH)D) (a marker of vitamin D status) that may be regarded as optimal, and the implications this process may have in the setting of future dietary reference values for vitamin D in the UK. The Forum was in agreement with the fact that it is desirable for all of the population to have a serum 25(OH)D concentration above 25 nmol/l, but it discussed some uncertainty about the strength of evidence for the need to aim for substantially higher concentrations (25(OH)D concentrations . 75 nmol/l). Any discussion of ‘optimal’ concentration of serum 25(OH)D needs to define ‘optimal’ with care since it is important to consider the normal distribution of requirements and the vitamin D needs for a wide range of outcomes. Current UK reference values concentrate on the requirements of particular subgroups of the population; this differs from the approaches used in other European countries where a wider range of age groups tend to be covered. With the re-emergence of rickets and the public health burden of low vitamin D status being already apparent, there is a need for urgent action from policy makers and risk managers. The Forum highlighted
concerns regarding the failure of implementation of existing strategies in the UK for achieving current vitamin D recommendations
Double-Masked, Randomized, Phase 2 Evaluation of Abicipar Pegol (an Anti-VEGF DARPin Therapeutic) in Neovascular Age-Related Macular Degeneration.
No full textPURPOSE
To evaluate safety and efficacy of the vascular endothelial growth factor binding protein abicipar pegol (abicipar) versus ranibizumab for neovascular age-related macular degeneration.
METHODS
Phase 2, multicenter, randomized, double-masked comparison (REACH study, stage 3). Patients (n = 64) received intravitreal injections of abicipar 1 mg or 2 mg at baseline, week 4, and week 8 (3 injections) or ranibizumab 0.5 mg at baseline and monthly (5 injections).
RESULTS
In the abicipar 1 mg (n = 25), abicipar 2 mg (n = 23), and ranibizumab (n = 16) arms, respectively, least-squares mean best-corrected visual acuity (BCVA) change from baseline was +6.2, +8.3, and +5.6 letters at week 16 (primary endpoint) and +8.2, +10.0, and +5.3 letters at week 20. Least-squares mean central retinal thickness (CRT) reduction from baseline was 134, 113, and 131 μm at week 16 and 116, 103, and 138 μm at week 20. Intraocular inflammation adverse events (AEs), reported in 5/48 (10.4%) abicipar-treated patients, resolved without sustained vision loss or other sequelae.
CONCLUSIONS
Abicipar demonstrated durability of effect: BCVA and CRT improvements were similar between abicipar and ranibizumab at weeks 16 and 20 (8 and 12 weeks after the last abicipar injection and 4 weeks after the last ranibizumab injection). No serious AEs were reported
A Rosenthal fiber encephalomyelopathy resembling Alexander's Disease in 3 sheep
No full textWe report an encephalomyelopathy in three 18-month-old Merino sheep with features of adult-onset Alexander’s disease (AD), a human primary astrocytic disorder. The signature histologic finding was the presence of numerous hypereosinophilic, intra-astrocytic inclusions (Rosenthal fibers), mainly in perivascular, subpial, and subependymal sites, especially in the caudal brain stem and spinal cord. Although AD usually results from mutations in the glial fibrillary acidic protein (GFAP) gene, no such mutation was detected in these sheep. However, the annual clinical presentation of this disorder in a few sheep in the affected flock is suggestive of a familial pattern of occurrence.A.E. Kessell, J.W. Finnie, J. Manavis, G.D. Cheetham and P.C. Blumberg
Magnetic and structural studies of some mixed metal oxides
No full textPowder neutron diffraction and magnetic susceptibility measurements of the antiferromagnetic phases of MnxNi1-xO,MnxCo1-xO, (MnxFe1-x)zO and (CoxFe1-x)zO reveal that the magnetic moments of unlike ions are always effectively collinear, despite the presence of competing anisotropies. The magnetic moments of MnxNi1-xO (x = 0.24,0.48 and 0.77) at 5K are confined to (111) planes by dipole-dipole forces, and the small trigonal exchangestrictions are the products of opposed antiferromagnetic Mn2+-Mn2+ and ferromagnetic Ni2+-Mn2+ nearest neighbour interactions. In MnxCo1-xO (x = 0.05, 0.10, 0.25,0.36) at 5K, the orbital degener- acy of Co2+ is removed by both Jahn-Teller (J.T) and spin-orbit coupling (S.O) mechanisms, leading to orthorhombic or triclinic symmetries and partially quenched Co2+ moments. Monoclinic symmetry is observed for 0.512+ moments are evident. The cobalt concentration is too small to support a cooperative J.T stabilisation in Mn0.83Co0.17O, where a magnetostriction (c/a MnO»FezO≈NiO. The weak trigonal anisotropy of Fe2+ correlates with the near-cubic symmetries of (CoxFe1-x)z( (x = 0.04,0.12) and (MnxFe1-x)zO (0.11) magnetostriction of Fe2+ is observed in (CoxFe1-x)zO with x > 0.5. Measurements of vacancy-ferric interstitial ratios for (MnxFe1-x)zO and (CoxFe1-x)zO suggest that non-stoichiometry is accommodated by 6:2 or 8:3 defect clusters in the former, and by larger units in the latter. The observed magnetic moments of defective samples are normally larger than those calculated according to a previous model for FezO, and require the postulation of partial antiferromagnetic order around the clusters. The room temperature Mossbauer effect parameters of (MnxFe1-x)zO (02+ and Mn2+ ions are randomly distributed over octahedral sites, ferric ions are localised around defect clusters
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