Wilson's disease (hepatolenticular degeneration)

This issue of eMedRef provides information to clinicians on the pathophysiology, diagnosis, and therapeutics of Wilson's disease

The effect of social media communication on consumer perceptions of brands

Researchers and brand managers have limited understanding of the effects social media communication has on how consumers perceive brands. We investigated 504 Facebook users in order to observe the impact of firm-created and user-generated social media communication on brand equity, brand attitude and purchase intention by using a standardized online survey throughout Poland. To test the conceptual model, we analyzed 60 brands across three different industries: non-alcoholic beverages, clothing and mobile network operators. When analyzing the data, we applied the structural equation modeling technique to both investigate the interplay of firm-created and user-generated social media communication and examine industry-specific differences. The results of the empirical studies showed that user-generated social media communication had a positive influence on both brand equity and brand attitude, whereas firm-created social media communication affected only brand attitude. Both brand equity and brand attitude were shown to have a positive influence on purchase intention. In addition, we assessed measurement invariance using a multi-group structural modeling equation. The findings revealed that the proposed measurement model was invariant across the researched industries. However, structural path differences were detected across the models

Demonstration of integrated microscale optics in surface-electrode ion traps

In ion trap quantum information processing, efficient fluorescence collection is critical for fast, high-fidelity qubit detection and ion-photon entanglement. The expected size of future many-ion processors require scalable light collection systems. We report on the development and testing of a microfabricated surface-electrode ion trap with an integrated high numerical aperture (NA) micromirror for fluorescence collection. When coupled to a low NA lens, the optical system is inherently scalable to large arrays of mirrors in a single device. We demonstrate stable trapping and transport of 40Ca+ ions over a 0.63 NA micromirror and observe a factor of 1.9 enhancement in photon collection compared to the planar region of the trap.Comment: 15 pages, 8 figure

A Conversation on Labour & Practice

Automated approaches to design, fabrication, and construction present disruptive and potentially transformative challenges to the conventional practice of architecture, as computational workflows recalibrate traditional roles and responsibilities in the production of buildings. How does computational design change how labor is defined and enacted in architectural and construction practice? What are the ethical implications and questions that arise in this context, particularly as we consider the implications of uncompensated or under-compensated labor of those doing computational work? This keynote event brings together three architects and thinkers to critically explore the intersections between computation, labor, and practice. Peggy Deamer is Professor Emerita of Yale University’s School of Architecture, principal in the firm of Deamer, Studio, and a founding member of the Architecture Lobby, a group advocating for the value of architectural design and labor. Billie Faircloth is a Partner at KieranTimberlake, where she leads a transdisciplinary group leveraging research, design, and problem-solving processes across fields including environmental management, chemical physics, materials science, and architecture. Mollie Claypool is an architecture theorist and activist at AUAR and UCL Bartlett. Her work broadly focuses on issues of social justice highlighted by increasing automation in architecture and design production, such as the future of work, housing, platforms, localised manufacturing, and circular economies

Use of sonic tomography to detect and quantify wood decay in living trees.

Premise of the studyField methodology and image analysis protocols using acoustic tomography were developed and evaluated as a tool to estimate the amount of internal decay and damage of living trees, with special attention to tropical rainforest trees with irregular trunk shapes.Methods and resultsLiving trunks of a diversity of tree species in tropical rainforests in the Republic of Panama were scanned using an Argus Electronic PiCUS 3 Sonic Tomograph and evaluated for the amount and patterns of internal decay. A protocol using ImageJ analysis software was used to quantify the proportions of intact and compromised wood. The protocols provide replicable estimates of internal decay and cavities for trees of varying shapes, wood density, and bark thickness.ConclusionsSonic tomography, coupled with image analysis, provides an efficient, noninvasive approach to evaluate decay patterns and structural integrity of even irregularly shaped living trees

A Reference Genome For The Nectar-Robbing Black-Throated Flowerpiercer (Diglossa Brunneiventris)

Black-throated Flowerpiercers (Diglossa brunneiventris) are one species representing a phenotypically specialized group of tanagers (Thraupidae) that have hooked bills which allow them to feed by stealing nectar from the base of flowers. Members of the genus are widely distributed in montane regions from Mexico to northern Argentina, and previous studies of Diglossa have focused on their systematics, phylogenetics, and interesting natural history. Despite numerous studies of species within the genus, no genome assembly exists to represent these nectivorous tanagers. We described the assembly of a genome sequence representing a museum-vouchered, wild, female D. brunneiventris collected in Peru. By combining Pacific Biosciences Sequel long-read technology with 10x linked-read and reference-based scaffolding, we produced a 1.08 Gbp pseudochromosomal assembly including 600 scaffolds with a scaffold N50 of 67.3 Mbp, a scaffold L50 of 6, and a BUSCO completeness score of 95%. This new assembly improves representation of the diverse species that comprise the tanagers, improves on scaffold lengths and contiguity when compared to existing genomic resources for tanagers, and provides another avenue of research into the genetic basis of adaptations common to a nectivorous lifestyle among vertebrates

Adverse effects of pegaspargase in pediatric patients receiving doses greater than 3,750 IU

BackgroundIncreased toxicities have been identified with higher doses of pegaspargase (PEG‐ASP) in adults. This has led to routine use of a dose cap of 3,750 IU for adult acute lymphoblastic leukemia (ALL) patients in most institutions. In pediatric ALL patients, PEG‐ASP is not capped. There is concern at our institution that larger doses may result in increased rates of adverse effects and that increased monitoring may be warranted in pediatric patients receiving doses greater than 3,750 IU. The objective of this study is to quantify the difference in the rates of PEG‐ASP‐associated adverse events between pediatric patients who received doses greater than 3,750 IU and less than or equal to 3,750 IU.MethodsRetrospective chart review of patients 1–21 years old with pre‐B‐cell ALL who received PEG‐ASP between 2007 and 2014 at an academic medical center.ResultsOf 183 patients included in the analysis, 24 received PEG‐ASP doses higher than 3,750 IU and 159 received doses less than or equal to 3,750 IU. The incidence of venous thromboembolism (VTE) was significantly higher for patients in the group that received more than 3,750 IU compared with those who received 3,750 IU or less (20.8 vs. 1.89%, respectively; P = 0.0011). The incidence of pancreatitis (P = 0.0306) and hyperglycemia (P = 0.0089) were also higher in the group that received more than 3,750 IU.ConclusionsPEG‐ASP doses higher than 3,750 IU are associated with higher rates of VTE, pancreatitis, and hyperglycemia in pediatric patients with pre‐B‐cell ALL. Patients receiving more than 3,750 IU should have increased monitoring, and larger, multicenter trials are needed to determine if monitoring, VTE prophylaxis, and potential dose capping recommendations should be added to clinical trial protocols.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/138220/1/pbc26555_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/138220/2/pbc26555.pd

Brave new brains: sociology, family and the politics of knowledge

This article critically explores sociological arguments for greater biosocial synthesis, centring contemporary developments in public policy to demonstrate how such a reframing of humanity tends to reinforce existing political orders and socially patterned normativities. The case for further amalgamation of the social and life sciences is examined to suggest that production of somatic markers of truth from relational encounters largely relies upon an anaemic and politically contained version of the social as acquired in early childhood. More specifically, the gendered, classed and culturally specific practice of parenting children has come to occupy a new significance in accounts of social brains and environmentally reactive genomes. This is highlighted through a discussion of ‘early intervention’ as a heavily biologised policy rationale framing opportunities for biosocial collaboration. It is argued that late capitalist objectives of personal investment and optimisation are driving this assimilation of the social and life sciences, pursuing an agenda that traces and re-scores longstanding social divisions in the name of progress

Smart Branching

Clay extrusion 3D printing with 6-axis industrial robots and ceramic firing pipelines has inspired designers’ reflection on material properties, design methodologies, automatic manufacturing, and logistics of fabrication. It brings the potential for innovative industrial bespoke production of architectural components. Plasticity and malleability are merits to the creative freedom of the form, yet they also pose technical challenges. The goals of the research are designing specifically printable geometries at a durable scale and optimizing methodologies for fabrication to ensure both quality and efficiency. Through the design and fabrication of a 1.3m-high physical prototype sampled from our facade proposal, we developed a relatively automated project pipeline. It aims to achieve the generative and evolutionary design and a non-planar clay deposition method for tubular branching components