Multiple unilateral craniopathies (Guillain-Alajouanine-Garcin syndrome) resulting from a malignancy of the external auditory canal: a case report

Garcin syndrome (or Gullain-Alajouanine-Garcin syndrome) was first described in the year 1926, as a paralytic unilateral cranial syndrome consisting of multiple cranial nerve palsies, without findings pointing towards increased intracranial pressure or long tract involvement. A 54-year-old female presented to us initially with ptosis and lateral rectus palsy of the right eye, later on, followed by progressive hearing loss on the right ear, right facial hemianesthesia, and progressive ipsilateral facial paralysis. A mass at the external auditory canal was seen during otoscopy, and a biopsy revealed squamous cell carcinoma. Contrast-enhanced neuroimaging also showed an enhancing mass lesion at the right skull base

The work environment disability-adjusted life year for use with life cycle assessment: a methodological approach

BACKGROUND: Life cycle assessment (LCA) is a systems-based method used to determine potential impacts to the environment associated with a product throughout its life cycle. Conclusions from LCA studies can be applied to support decisions regarding product design or public policy, therefore, all relevant inputs (e.g., raw materials, energy) and outputs (e.g., emissions, waste) to the product system should be evaluated to estimate impacts. Currently, work-related impacts are not routinely considered in LCA. The objectives of this paper are: 1) introduce the work environment disability-adjusted life year (WE-DALY), one portion of a characterization factor used to express the magnitude of impacts to human health attributable to work-related exposures to workplace hazards; 2) outline the methods for calculating the WE-DALY; 3) demonstrate the calculation; and 4) highlight strengths and weaknesses of the methodological approach. METHODS: The concept of the WE-DALY and the methodological approach to its calculation is grounded in the World Health Organization’s disability-adjusted life year (DALY). Like the DALY, the WE-DALY equation considers the years of life lost due to premature mortality and the years of life lived with disability outcomes to estimate the total number of years of healthy life lost in a population. The equation requires input in the form of the number of fatal and nonfatal injuries and illnesses that occur in the industries relevant to the product system evaluated in the LCA study, the age of the worker at the time of the fatal or nonfatal injury or illness, the severity of the injury or illness, and the duration of time lived with the outcomes of the injury or illness. RESULTS: The methodological approach for the WE-DALY requires data from various sources, multi-step instructions to determine each variable used in the WE-DALY equation, and assumptions based on professional opinion. CONCLUSIONS: Results support the use of the WE-DALY in a characterization factor in LCA. Integrating occupational health into LCA studies will provide opportunities to prevent shifting of impacts between the work environment and the environment external to the workplace and co-optimize human health, to include worker health, and environmental health

Electron Hopping Conductivity and Vapor Sensing Properties of Flexible Network Polymer Films of Metal Nanoparticles

Films of monolayer protected Au clusters (MPCs) with mixed alkanethiolate and ω-carboxylate alkanethiolate monolayers, linked together in a network polymer by carboxylate-Cu2+-carboxylate bridges, exhibit electronic conductivities (σEL) that vary with both the numbers of methylene segments in the ligands and the bathing medium (N2, liquid or vapor). A chainlength-dependent swelling/contraction of the film\u27s internal structure is shown to account for changes in σEL. The linker chains appear to have sufficient flexibility to collapse and fold with varied degrees of film swelling or dryness. Conductivity is most influenced (exponentially dependent) by the chainlength of the nonlinker (alkanethiolate) ligands, a result consistent with electron tunneling through the alkanethiolate chains and nonbonded contacts between those chains on individual, adjacent MPCs. The σEL results concur with the behavior of UV−vis surface plasmon adsorption bands, which are enhanced for short nonlinker ligands and when the films are dry. The film conductivities respond to exposure to organic vapors, decreasing in electronic conductivity and increasing in mass (quartz crystal microgravimetry, QCM). In the presence of organic vapor, the flexible network of linked nanoparticles allows for a swelling-induced alteration in either length or chemical nature of electron tunneling pathways or both

The copper-transporting capacity of ATP7A mutants associated with Menkes disease is ameliorated by COMMD1 as a result of improved protein expression

Menkes disease (MD) is an X-linked recessive disorder characterized by copper deficiency resulting in a diminished function of copper-dependent enzymes. Most MD patients die in early childhood, although mild forms of MD have also been described. A diversity of mutations in the gene encoding of the Golgi-resident copper-transporting P1B-type ATPase ATP7A underlies MD. To elucidate the molecular consequences of the ATP7A mutations, various mutations in ATP7A associated with distinct phenotypes of MD (L873R, C1000R, N1304S, and A1362D) were analyzed in detail. All mutants studied displayed changes in protein expression and intracellular localization parallel to a dramatic decline in their copper-transporting capacity compared to ATP7A the wild-type. We restored these observed defects in ATP7A mutant proteins by culturing the cells at 30°C, which improves the quality of protein folding, similar to that which as has recently has been demonstrated for misfolded ATP7B, a copper transporter homologous to ATP7A. Further, the effect of the canine copper toxicosis protein COMMD1 on ATP7A function was examined as COMMD1 has been shown to regulate the proteolysis of ATP7B proteins. Interestingly, in addition to adjusted growth temperature, binding of COMMD1 partially restored the expression, subcellular localization, and copper-exporting activities of the ATP7A mutants. However, no effect of pharmacological chaperones was observed. Together, the presented data might provide a new direction for developing therapies to improve the residual exporting activity of unstable ATP7A mutant proteins, and suggests a potential role for COMMD1 in this process

Non-specific psychological distress, smoking status and smoking cessation: United States National Health Interview Survey 2005

<p>Abstract</p> <p>Background</p> <p>It is well established that smoking rates in people with common mental disorders such as anxiety or depressive disorders are much higher than in people without mental disorders. It is less clear whether people with these mental disorders want to quit smoking, attempt to quit smoking or successfully quit smoking at the same rate as people without such disorders.</p> <p>Methods</p> <p>We used data from the 2005 Cancer Control Supplement to the United States National Health Interview Survey to explore the relationship between psychological distress as measured using the K6 scale and smoking cessation, by comparing current smokers who had tried unsuccessfully to quit in the previous 12 months to people able to quit for at least 7 to 24 months prior to the survey. We also used data from the 2007 Australian National Survey of Mental Health and Wellbeing to examine the relationship between psychological distress (K6) scores and duration of mental illness.</p> <p>Results</p> <p>The majority of people with high K6 psychological distress scores also meet diagnostic criteria for mental disorders, and over 90% of these people had first onset of mental disorder more than 2 years prior to the survey. We found that people with high levels of non-specific psychological distress were more likely to be current smokers. They were as likely as people with low levels of psychological distress to report wanting to quit smoking, trying to quit smoking, and to have used smoking cessation aids. However, they were significantly less likely to have quit smoking.</p> <p>Conclusions</p> <p>The strong association between K6 psychological distress scores and mental disorders of long duration suggests that the K6 measure is a useful proxy for ongoing mental health problems. As people with anxiety and depressive disorders make up a large proportion of adult smokers in the US, attention to the role of these disorders in smoking behaviours may be a useful area of further investigation for tobacco control.</p

Simulation-based analysis of reconfigurable system of system network topologies for resilience using Bayesian networks

An emerging understanding of resilient systems is as a management principle or framework allowing for reconfiguration or adaptation in the face of threats or shocks. This is a new approach engineered system resilience: the more traditional approach is that systems may focus on resistance to threats, and speedy recovery if vulnerabilities are breached. This notion can potentially integrate interdisciplinary research currently pursued in systems engineering, design theory, infrastructure risk analysis, and statistical learning to create an approach that permits both evaluation of system resilience and also the value of system evolvability in the face of operational hazards. In prior work, a vision for reconfigurable systems based on Bayesian Networks was articulated but not tested or demonstrated. In this paper, we demonstrate a Bayesian Network inspired approach to measuring the value of re-configurability in systems of systems that can be represented in directed acyclic graphs using a simulation-based approach. For the purpose of our investigation, re-configurability means that a system can adapt its structure to structural failures in either system components or links between components. The latter is called structural flexibility, whereas the former is called functional flexibility. Undirected Bayesian Networks are used to structure the relationships between the subsystem components, and the graphical model is then used in conjunction with concepts from functional dependency network theory to evaluate the response the system under updated configurations.Non UBCUnreviewedThis collection contains the proceedings of ICASP12, the 12th International Conference on Applications of Statistics and Probability in Civil Engineering held in Vancouver, Canada on July 12-15, 2015. Abstracts were peer-reviewed and authors of accepted abstracts were invited to submit full papers. Also full papers were peer reviewed. The editor for this collection is Professor Terje Haukaas, Department of Civil Engineering, UBC Vancouver.Facult

Integrated spatial community resilience decision tool unifying social vulnerability indices and relative sea-level rise predictions

Infrastructure resilience and social resilience have been independently investigated, but have not yet been integrated in the evaluation of preparedness and natural hazard response investments. In this paper, we present an integrated spatial evaluation of vulnerability to relative sea level rise in the US states of Delaware, Maryland, and Virginia. This paper is motivated by the notion that there may be an interaction between community resilience measured by social indicators and community resilience measured by technical vulnerability indicators. We evaluate this hypothesis by creating a decision support tool integrating relative sea level rise (RSLR) and social vulnerability index (SoVI) for those communities delineated by county. These results will be useful to policy makers by explicitly communicating the tradeoffs between SoVI and technical vulnerability, while also illustrating the potential for prioritizing regions based on the interaction between social resilience and infrastructure resilience.Non UBCUnreviewedThis collection contains the proceedings of ICASP12, the 12th International Conference on Applications of Statistics and Probability in Civil Engineering held in Vancouver, Canada on July 12-15, 2015. Abstracts were peer-reviewed and authors of accepted abstracts were invited to submit full papers. Also full papers were peer reviewed. The editor for this collection is Professor Terje Haukaas, Department of Civil Engineering, UBC Vancouver.FacultyOthe

Cerebral cholesterol granuloma in homozygous familial hypercholesterolemia

Familial hypercholesterolemia (FH) is characterized by the accumulation of excess cholesterol in tissues including the artery wall and tendons. We describe a patient with homozygous FH who presented with asymptomatic cholesterol granuloma of the brain. The patient's plasma low-density lipoprotein cholesterol level was remarkably responsive to combination hypolipidemic therapy with statin plus ezetimibe. This case illustrates another potential complication of whole-body cholesterol excess and underscores the differences in phenotype and in response to therapy among patients with FH