bluuebunny/crossref_metadata_2025 huggingface dataset:
Declining Use of the Hallervorden–Spatz Disease Eponym in the Last Two Decades
Authors: Zeidman Lawrence A. , Pandey Dilip K.
Published: July 2012

There has been a movement to rename Hallervorden–Spatz disease to pantothenate kinase-associated neurodegeneration given Hallervorden and Spatz’s complicity in murderous Nazi programs. Similar controversy surrounds Reiter syndrome, and 2 studies demonstrated decreased unqualified use of that eponym in the literature, but not in textbooks. There have been no similar studies regarding Hallervorden–Spatz disease. The authors performed a MEDLINE search (1990-2010) looking for unqualified use of Hallervorden–Spatz disease and performed statistical analysis. They defined “unqualified” as having no reference to the eponym’s disfavored use. They then looked in 6 neurology textbooks. The authors identified 156 of 278 articles (56.1%) containing unqualified use of Hallervorden–Spatz disease. But there was a declining trend ( P = .000), with 70/80 (87.5%) of articles from 1990 to 1999 and 86/198 (43.4%) from 2000 to 2010. There was also decreased unqualified use of the eponyms in textbooks, with all recent editions using pantothenate kinase-associated neurodegeneration instead. The significant decrease in unqualified use of Hallervorden–Spatz disease is reassuring.
Autoimmune Basal Ganglia Disorders
Authors: Dale Russell C. , Brilot Fabienne
Published: July 2012

The basal ganglia are deep nuclei in the brain that include the caudate, putamen, globus pallidus, and substantia nigra. Pathological processes involving the basal ganglia often result in disorders of movement and behavior. A number of different autoimmune disorders predominantly involve the basal ganglia and can result in movement and psychiatric disorders. The classic basal ganglia autoimmune disorder is Sydenham chorea, a poststreptococcal neuropsychiatric disorder. Resurgence in the interest in Sydenham chorea is the result of the descriptions of other poststreptococcal neuropsychiatric disorders including tics and obsessive-compulsive disorder, broadly termed pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection. Encephalitic processes affecting the basal ganglia are also described including the syndromes basal ganglia encephalitis, encephalitis lethargica, and bilateral striatal necrosis. Last, systemic autoimmune disorders such as systemic lupus erythematosus and antiphospholipid syndrome can result in chorea or parkinsonism. Using paradigms learned from other autoantibody associated disorders, the authors discuss the autoantibody hypothesis and the role of systemic inflammation in autoimmune basal ganglia disorders. Identification of these entities is important as the clinician has an increasing therapeutic repertoire to modulate or suppress the aberrant immune system.
CDKL5 Mutations as a Cause of Severe Epilepsy in Infancy
Authors: Jähn Johanna , Caliebe Almuth , von Spiczak Sarah , Boor Rainer , Stefanova Irina , Stephani Ulrich , Helbig Ingo , Muhle Hiltrud
Published: July 2012

CDKL5 mutations cause severe epilepsy in infancy with subsequent epileptic encephalopathy. As yet, few studies report on long-term observations in patients with CDKL5-related epileptic encephalopathy. In this study, we describe the evolution of the epilepsy phenotype and the electroencephalographic (EEG) features in 4 patients during a maximum observation period of 22 years. All 4 patients had epilepsy starting with focal seizures in the first 3 months of life, evolving to epileptic spasms between the ages of 2 and 6 years and later on to tonic seizures. In 3 patients, epilepsy was resistant to antiepileptic therapy. Although there was no common EEG pattern in all patients, late hypsarrhythmia until the age of 9 years was observed in 2 patients. CDKL5-related epileptic encephalopathies are a group of refractory seizure disorders starting in early infancy. The phenomenon of late hypsarrhythmia may help define a subgroup of patients with severe and adverse outcomes.
Unanswered Questions in Friedreich Ataxia
Authors: Lynch David R. , Deutsch Eric C. , Wilson Robert B. , Tennekoon Gihan
Published: July 2012

During the past 15 years, the pace of research advancement in Friedreich ataxia has been rapid. The abnormal gene has been discovered and its gene product characterized, leading to the development of new evidence-based therapies. Still, various unsettled issues remain that affect clinical trials. These include the level of frataxin deficiency needed to cause disease, the mechanism by which frataxin-deficient mitochondrial dysfunction leads to symptomatology, and the reason selected cells are most affected in Friedreich ataxia. In this review, we summarize these questions and propose testable hypotheses for their resolution.
From Zero Tolerance to Student Success in Ontario, Canada
Authors: Winton Sue
Published: July 2012

Since 2003, Ontario, Canada’s high school graduation rates have increased 13% while suspensions and expulsion rates have simultaneously decreased. This article examines relationships between the province’s safe school policy and Student Success/Learning to 18 (SS/L18), a policy designed to increase graduation rates. Analyses of teachers’ perceptions, policy texts, provincial data, and an external evaluation of SS/L18 suggest that efforts to increase graduation rates through SS/L18 may also be helping to reduce suspensions and expulsions—perhaps to a greater extent than recent changes to Ontario’s safe schools policy, including the elimination of zero tolerance and adoption of a progressive discipline approach.
“Very Powerful Voices”
Authors: Conner Jerusha , Zaino Karen , Scarola Emily
Published: July 2012

Nationally, youth organizing groups have been gaining traction in their push for education reform; however, little research has considered how policymakers view their efforts. This study examines how 30 civic leaders in one under-resourced urban school district perceive the influence of a youth organizing group on educational policy decision making over a 15 year period. Results indicate that the group is widely recognized for having accomplished significant policy changes at school and district levels, including influencing the policy process in four key ways: insisting on accountability, elevating the role of student voice, shaping the agenda, and asserting themselves as powerful political actors.
Ethnic Giving Versus Mainstream Giving by Foreign-Born Korean Immigrants in California
Authors: Moon Seong-gin , Choi Sang Ok
Published: July 2012

Based on a large-scale philanthropic survey conducted in California, this paper empirically examines the determinants of ethnic giving versus mainstream giving practices and the interactive relationships between the two observed among foreign-born Korean immigrants. The results of our bivariate probit regression analysis suggest several interesting findings. First, acculturation serves significant functions in immigrants’ decisions to give to mainstream organizations, but does not appear to affect ethnic giving decisions as profoundly. Second, U.S. educational experiences and gender are strong predictors of mainstream giving practices, whereas household incomes and religiosity figure significantly in terms of explaining ethnic giving practices. Additionally, citizenship is related weakly to ethnic giving practices. Finally, two distinctive giving practices are negatively interrelated, meaning that immigrants who donate to ethnic organizations are less likely to give to mainstream organizations, and vice versa.
Putting Charity to the Test
Authors: Mason Dyana P.
Published: July 2012

Most nonprofit organizations rely on gifts of time and money to support their operations. However, research by nonprofit scholars on the behavioral mechanisms of charity remains underdeveloped. One methodological tool, the randomized and controlled field experiment, has not been fully embraced by nonprofit scholars publishing in nonprofit journals. This is a missed opportunity. This essay argues that the leading theoretical models on charity can and should be tested by nonprofit scholars using field experiments in partnership with nonprofit organizations. The resultant findings may contribute to the literatures on giving, altruism and collective action. Importantly, field experiments can also provide tangible knowledge for nonprofit managers seeking to improve their operations, a common interest of both scholars and practitioners.
The Influence of Family and Household Members on Individual Volunteer Choices
Authors: Nesbit Rebecca
Published: July 2012

This paper investigates the how the volunteering behaviors of family and household members influence an individual’s decision to volunteer. Using data from the 2005 Current Population Survey’s Volunteering Supplement, I test how living with volunteers and living with people who have never volunteered affect volunteering. I find that living with volunteers dramatically increases the likelihood of volunteering, especially for religious volunteering. The more volunteers the person lives with, the higher the person’s probability of volunteering. People who live with others who say that they have never volunteered in their lives are much less likely to volunteer and volunteer fewer hours. Living with volunteers also changes the methods by which people become involved in volunteering.
Administrative Coordination in Nonprofit Human Service Delivery Networks
Authors: Bunger Alicia C.
Published: July 2012

Nonprofit human service organizations operating within the same regional network are often faced with dual pressure to compete as well as coordinate administrative operations (by sharing funding, staff, or space) to enhance efficiency. Emerging evidence has demonstrated that competing organizations coordinate, despite the risks. Trust, or perceived trustworthiness between two organizations may mitigate the negative influence of competition on coordination, however there have been few explicit tests of this hypothesis among nonprofit organizations. Drawing on quantitative data collected from a network of 36 nonprofit children’s behavioral health organizations, this article empirically tests how competition and perceived trustworthiness interact to influence administrative coordination. Results support the hypothesis that trustworthiness moderates the influence of competition on administrative coordination. Findings suggest that as competing nonprofit leaders build trust, the more their agencies coordinate their administrative functions. This study highlights the importance of leaders’ perceptions for organizational strategy.
Input–output linearisation applied to the brake torque control of the self-energising electro-hydraulic brake
Authors: Kühnlein Michael , Liermann Matthias , Ewald Julian , Murrenhoff Hubertus
Published: July 2012

This paper presents the controller for a self-energising electro-hydraulic brake. A non-linear controller is chosen for the brake torque control of the railway brake, which has the major benefit of achieving predictable dynamics of the brake torque build-up throughout the working range of the self-energising electro-hydraulic brake without controller adjustment. While being more complex in implementation, the non-linear controller is more straightforward in design compared to a proportional controller, which needs to be tuned empirically. This paper focuses on design of the controller, including the required simplification of the mathematical model to the third order. Simulation results show that the desired dynamics can be achieved. Test rig measurements confirm the applicability of the non-linear brake torque controller.
Neotropical refugia
Authors: Bennett KD , Bhagwat SA , Willis KJ
Published: July 2012

Patterns of endemism in the Neotropics have been explained by restriction of forest to ‘refugia’ in arid cold-stages of the Quaternary (Haffer J (1969) Speciation in Amazonian forest birds. Science 165: 131–137). The palaeoecological record, however, shows no such forest contraction. We review palaeoecological and phylogenetic data on the response of Neotropical taxa and communities to climatic changes of the Cenozoic. Solar insolation varies over this period with latitude and geography, including shifts in opposite directions between high and low latitudes. In the Neotropics, distribution and abundance patterns originate on a wide range of timescales through the Cenozoic, down to the currently dominant precession forcing (20 kyr). In contrast, distributions and abundances at higher latitudes are controlled by obliquity forcing (40 kyr). The patterns observed by Haffer (1969) are likely derived from pre-Quaternary radiations and are not inconsistent with palaeoecological findings of continuous forest cover in major areas of the Neotropics during the Quaternary. The relative proportions of speciation processes have changed through time between predominantly sympatric to predominantly allopatric depending on the prevailing characteristics of orbitally forced climatic changes. Behaviour of Neotropical organisms and ecosystems on long timescales may be inﬂuenced much more by precessional forcing than by the obliquity forcing that controls high-latitude climate change and glaciations.
The prevalence of oral mucosal lesions and related factors in 188 patients with systemic lupus erythematosus
Authors: Khatibi M , Shakoorpour AH , Jahromi Z Moosavian , Ahmadzadeh A
Published: July 2012

Background: One of the most important concerns about systemic lupus erythematosus (SLE) is the presence of oral lesions, which in turn is one of the diagnostic criteria for SLE. Regarding the wide range of oral lesions and the absence of comprehensive data in Iran, this study aimed to evaluate the prevalence of oral mucosal lesions and related factors in patients with SLE at three rheumatology clinics in Tehran. Methods: This descriptive study was performed on 188 patients by observation, clinical examination, completing questionnaire and also evaluating patients’ medical records. The lesions explored were ulcer, erythema, erythematous center with white striae or spots, and white plaques. Related factors taken into consideration were age, sex, smoking, frequency of pregnancy, oral health status, duration of disease, medications taken daily dosage of corticosteroid drugs, and the state of disease control. Sampling was done by the continuous method and data obtained were analyzed by SPSS version 16.0 and ordinal regression tests. Findings: Out of 188 patients, 102 (54.3%) patients had oral mucosal lesions. The most prevalent lesion was ulcer (28.1%) and the most common region involved was the buccal and labial mucosa. Conclusion: With regard to the high prevalence of oral mucosal lesions in patients with SLE, it is of paramount importance to emphasize early detection of these lesions as a mean of diagnosis of disease and faster initiation of treatment.
Acute onset of systemic lupus erythematosus with extensive gastrointestinal and genitourinary involvement
Authors: Tan T-C , Wansaicheong GK-L , Thong BY-H
Published: July 2012

Lupus enteritis may occur as an acute presentation of systemic lupus erythematosus (SLE), and is not uncommonly associated with acute uretero-cystitis and/or hydronephrosis. Extensive involvement of the gastrointestinal tract from the stomach to the rectum in acute SLE is uncommon. We describe a 22-year-old Chinese female who presented with acute extensive gastrointestinal and genitourinary involvement who recovered following pulse intravenous methylprednisolone and cyclophosphamide. A high index of clinical suspicion is required to arrive at the diagnosis of SLE where only acute gastrointestinal and genitourinary manifestations are predominant, and other clinical, haematological and biochemical features of SLE are absent.
Embracing New Directions in Workplace Bullying Research
Authors: Samnani Al-Karim
Published: July 2012

This article examines the workplace bullying literature through different paradigmatic lenses. To date, the workplace bullying literature has been dominated by the functionalist perspective, which currently represents the pervading paradigmatic approach in organizational research. The author destabilizes the functionalist approach by examining the workplace bullying literature through three alternative paradigms, namely, interpretivism, critical management theory, and postmodernism. This provides an illustration of the different ways in which workplace bullying can be perceived, understood, and researched. Moreover, because alternative paradigmatic lenses draw upon varying theoretical and methodological approaches, paradigmatic analysis can offer a more complete and comprehensive understanding of the phenomenon. The author uses these lenses to ground workplace bullying in paradigmatically driven theoretical frameworks, while using these theoretical frameworks to propose research questions that can direct us toward gaining a more composite body of scholarship.
Crypt Apoptotic Count Reproducibility in Small Bowel Allograft Biopsies
Authors: Talmon Geoffrey , El Behery Radwa , Radio Stanley , Fischer Ryan , Shostrom Valerie , Wisecarver James
Published: July 2012

Enumeration of crypt apoptotic bodies (AB) is used in the diagnosis of acute cellular rejection (ACR) in small bowel allografts. Due to differing definitions, there is a potential for variation in AB numbers between pathologists that may affect diagnosis. Thirty biopsies from allografts were obtained: 10 negative for ACR, 10 indeterminate, and 10 with mild ACR. Three pathologists blindly reviewed each case twice and counted the maximal AB number per 10 crypts. Intraobserver kappa ranged from 0.423 to 0.913 and interobserver kappa from 0.478 to 0.686 depending on AB definition. Intraobserver diagnosis agreement based on classical AB number occurred in 81 of 90 instances (90%) and in 77 of 90 using the liberal definition (85%), P < .01). There was substantial intraobserver and moderate to substantial interobserver agreement in crypt AB counts. However, both intraobserver and interobserver variability would have led to diagnostic discrepancies in a small subset of cases.
Epiphyseal Ewing Sarcoma
Authors: Pérez-González Yosmar , García-Esparza Elena , Conde Esther , Azorín Daniel
Published: July 2012

Ewing sarcoma is the second most common pediatric malignant bone neoplasm after osteosarcoma. Ewing sarcoma comprises “small, round, blue-cell” tumors thought to arise from neural crest cells. The authors report the case of a 14-year-old boy that presented with a nonpainful circumscribed lesion. The radiographs showed a lytic lesion at the tibial epiphysis with a large soft tissue mass, best depicted in the magnetic resonance imaging scan that suggested an aggressive lesion. A needle biopsy of the lesion was performed. The diagnosis of Ewing sarcoma was made based on microscopic, immunohistochemical, polymerase chain reaction, and fluorescence in situ hybridization. This is the third case report about a primary epiphyseal Ewing sarcoma and the fist one with molecular confirmation.
Cellular Metanephric Stromal Tumor in a Postmenopausal Woman
Authors: Chaudhri Ali Aftab , Lee Lili , Das Kasturi
Published: July 2012

Metanephric stromal tumor (MST) of the kidney is a rare pediatric neoplasm which has rarely been reported in adults. The authors present a case of MST, cellular type, an unreported variant, in a 56-year-old postmenopausal woman. The 9.0-cm tumor was solid, unencapsulated, and well circumscribed, with a firm homogeneous cut surface. The tumor was purely stromal with dense spindle-cell proliferations displaying prominent schwannoma-like architecture, intratumoral angiodysplasia, and entrapped native renal tubules with “onion-skin” tumor collarettes. The tumor stroma expressed CD34, estrogen, and progesterone and did not express desmin, smooth muscle actin, S-100, or TLE-1. Considering the characteristic morphology and immunophenotype, a diagnosis of “metanephric stromal tumor, cellular type,” was made. This is an exceptionally rare disease manifestation in an adult patient and the first description of MST with such high stromal cellularity, making this case unique in both clinical and pathological presentation.
Hybrid Schwannoma/Perineurioma
Authors: Yang Xiaoqun , Zeng Yu , Wang Jian
Published: July 2012

Hybrid schwannoma/perineurioma is a recently described benign nerve sheath tumor that typically manifests as a dermal tumor on the extremities and trunk. Occurrence outside the skin is uncommon. This article describes 10 cases of hybrid schwannoma/perineurioma arising in diverse anatomic locations. They all occurred in adult patients (age range 27-81 years, median 35 years) with a marked female predominance (2 males and 8 females). Of the 10 tumors, 7 were situated in the subcutis of trunk (n = 3), extremities (n = 2), neck (n = 1), and labium majus (n = 1) and 3 in the submucosa of nasal cavity, sigmoid colon, and rectum, respectively. Histologically, they were composed of intimately admixed plump spindle cells and elongated slender spindle cells forming storiform, lamellar or fascicular architecture. By immunohistochemistry, the tumor showed dual differentiation of schwannian cells (plump-spindled) and perineurial cells (slender-spindled), characterized by strong S100 protein expression in the former component and variable immunoreactivity of epithelial membrane antigen, claudin-1, and CD34 in the latter. Awareness of its morphological characteristics and potential occurrence in diverse sites may aid in the recognition of this rare tumor type.
Performance of an elastic polymer foam cushion in attenuating responses of shipboard standing-men to ship vertical shock
Authors: Chen Yong , Wang Yu , Hua Hong-Xing
Published: July 2012

How to protect shipboard personnel from the shock events induced by underwater explosion is a very interesting subject for ship designers. In this study, the potential attenuation performance of an elastic polymer foam cushion inserted between standing-man and ship deck is investigated theoretically. An 8-degree-of-freedom nonlinear lumped-parameter model is applied to predict the standing-man’s biodynamic responses and injury potential. The cushion is modeled by a chain of masses separated by nonlinear springs and dampers in parallel to simulate the micro inertia, stiffness and rate-dependent effects exhibited by common polymer cellular materials. Two variables, kickoff speed ratio and deck reaction force ratio corresponding to two types of typical injury potential of standing-men, are defined as evaluation parameters. The influence of critical buckling force level, material rate dependent effect as well as some other design factors on the attenuation performance of the foam cushion is discussed in detail. Some general design rules are also presented.
Transverse vibration analysis of generally laminated two-segment composite shafts with a lumped mass using generalized differential quadrature
Authors: Hajianmaleki Mehdi , Qatu Mohamad S
Published: July 2012

The use of composite materials for weight reduction of shaft drivelines has positive impacts in different applications. The driveshafts design is mostly dictated by its natural frequencies. In order to maximize the natural frequencies, shafts are built in one, two or more segments. Analysis of such components has complexities in dealing with two-segments, shear deformation, and material coupling effects. This paper presents a theory that includes shear deformation and rotary inertia along with coupling effects to analyze transverse vibration behavior of generally laminated shafts with intermediate joints. Equivalent modulus of elasticity is used instead of traditional stiffness terms to account for material coupling. The equations of motion are solved using generalized differential quadrature. Results were compared with those published in the literature and finite element simulations, and it has been shown that the present method can accurately predict transverse vibration behaviors of generally laminated two-segment shafts with a lumped mass.
The Long-Term Influence of Service Employee Attrition on Customer Outcomes and Profits
Authors: Subramony Mahesh , Holtom Brooks C.
Published: July 2012

The authors proposed and tested a model linking service-employee attrition, customer-perceived service outcomes, and financial performance utilizing time-lagged data obtained from 64 business units of a temporary help services (staffing) firm. Using the notion of relational assets, the authors predicted that employee attrition (both voluntary turnover and downsizing) would disrupt the existing stock of relationships between customer-facing employees and their customers, which would have negative effects on customer outcomes and future financial performance of business units. The authors found that (a) the relationship between voluntary turnover and customer-perceived service brand image (SBI) was fully mediated by customers’ evaluations of service delivery, (b) the relationship between downsizing and SBI was fully mediated by the customer orientation levels of the unit staff, and (c) SBI significantly predicted future unit profitability. These findings point to critical factors that leaders must address when experiencing elevated levels of turnover or considering downsizing. These include focusing on developing customer orientation levels among employees through the effective use of selection, training, performance management, and compensation, minimizing employee voluntary turnover by creating positive work environments, and factoring in the long-term costs of downsizing on the organization’s SBI and future profitability.
Double differentiation in a cross-national comparison of populist political movements and online media uses in the United States and the Netherlands
Authors: Groshek Jacob , Engelbert Jiska
Published: July 2012

In a context of highly visible and politically influential populist movements, this study considers the online self-representation of the Tea Party Patriots (TPP) in the United States and the Party for Freedom (PVV) in the Netherlands. A multi-methodological approach was adopted to compare the discursive manifestation of key populism concepts: leadership characteristics, adversary definition and mobilizing information. Analyses reconstruct and account for similarities and differences in discursive framing strategies of ‘double differentiation’ through which both movements attempt inclusion in and exclusion from the political establishment, and, in doing so, mobilize communities of support. Altogether, this study advances the understanding of what constitutes ‘unmediated’ content that is presented through user-generated media production, and how self-determined media spaces have facilitated shifts in populist media legitimation and political representation in two politically unique countries.
Hispanics
Authors: Page Jill
Published: July 2012

Hispanic students are a growing and pervasive population within higher education. This position paper examines population characteristics and educational patterns of Hispanic students that underscore failures of the higher education system in serving these students, in addition to institutional issues and cultural values that further complicate their college experience. These trends warrant attention from institutions toward policy changes and program initiatives to better serve Hispanic students.
Sexual Assault Nurse Examiners’ Perceptions of the Influence of Race and Ethnicity on Victims’ Responses to Rape
Authors: Maier Shana L.
Published: July 2012

Race/ethnicity plays a role in the perception of rape and experiences of victims. This research explores sexual assault nurse examiners’ (SANE) perceptions of the influence of victims’ race/ethnicity on their responses in hospitals to rape, and whether SANEs have different ways of addressing the needs of victims of color when treating them. Data from interviews with 40 SANEs indicate that the majority believe victims of color respond to rape differently or face different issues than White victims do, and 30% of SANEs believed race/ethnicity with other factors influence responses. Half of SANEs interviewed have different ways of responding to victims based on race/ethnicity.
The intestinal microbiota dysbiosis and Clostridium difficile infection: is there a relationship with inflammatory bowel disease?
Authors: Bien Justyna , Palagani Vindhya , Bozko Przemyslaw
Published: July 2012

Gut microbiota is a compilation of microorganisms dwelling in the entire mammalian gastrointestinal tract. They display a symbiotic relationship with the host contributing to its intestinal health and disease. Even a slight fluctuation in this equipoise may be deleterious to the host, leading to many pathological conditions like Clostridium difficile infection or inflammatory bowel disease (IBD). In this review, we focus on the role of microbial dysbiosis in initiation of C. difficile infection and IBD, and we also touch upon the role of specific pathogens, particularly C. difficile, as causative agents of IBD. We also discuss the molecular mechanisms activated by C. difficile that contribute to the development and exacerbation of gastrointestinal disorders.
Defining indoor heat thresholds for health in the UK
Authors: Anderson Mindy , Carmichael Catriona , Murray Virginia , Dengel Andy , Swainson Michael
Published: July 2012

Introduction:

It has been recognised that as outdoor ambient temperatures increase past a particular threshold, so do mortality/morbidity rates. However, similar thresholds for indoor temperatures have not yet been identified. Due to a warming climate, the non-sustainability of air conditioning as a solution, and the desire for more energy-efficient airtight homes, thresholds for indoor temperature should be defined as a public health issue.

Aims:

The aim of this paper is to outline the need for indoor heat thresholds and to establish if they can be identified. Our objectives include: describing how indoor temperature is measured; highlighting threshold measurements and indices; describing adaptation to heat; summary of the risk of susceptible groups to heat; reviewing the current evidence on the link between sleep, heat and health; exploring current heat and health warning systems and thresholds; exploring the built environment and the risk of overheating; and identifying the gaps in current knowledge and research.

Methods:

A global literature search of key databases was conducted using a pre-defined set of keywords to retrieve peer-reviewed and grey literature. The paper will apply the findings to the context of the UK.

Results:

A summary of 96 articles, reports, government documents and textbooks were analysed and a gap analysis was conducted. Evidence on the effects of indoor heat on health implies that buildings are modifiers of the effect of climate on health outcomes. Personal exposure and place-based heat studies showed the most significant correlations between indoor heat and health outcomes. However, the data are sparse and inconclusive in terms of identifying evidence-based definitions for thresholds. Further research needs to be conducted in order to provide an evidence base for threshold determination.

Conclusions:

Indoor and outdoor heat are related but are different in terms of language and measurement. Future collaboration between the health and building sectors is needed to develop a common language and an index for indoor heat and health thresholds in a changing climate.
Gene therapy/bone marrow transplantation in ADA-deficient mice: roles of enzyme-replacement therapy and cytoreduction
Authors: Carbonaro Denise A. , Jin Xiangyang , Wang Xingchao , Yu Xiao-Jin , Rozengurt Nora , Kaufman Michael L. , Wang Xiaoyan , Gjertson David , Zhou Yang , Blackburn Michael R. , Kohn Donald B.
Published: July 2012

Gene therapy (GT) for adenosine deaminase–deficient severe combined immune deficiency (ADA-SCID) can provide significant long-term benefit when patients are given nonmyeloablative conditioning and ADA enzyme-replacement therapy (ERT) is withheld before autologous transplantation of γ-retroviral vector-transduced BM CD34+ cells. To determine the contributions of conditioning and discontinuation of ERT to the therapeutic effects, we analyzed these factors in Ada gene knockout mice (Ada−/−). Mice were transplanted with ADA-deficient marrow transduced with an ADA-expressing γ-retroviral vector without preconditioning or after 200 cGy or 900 cGy total-body irradiation and evaluated after 4 months. In all tissues analyzed, vector copy numbers (VCNs) were 100- to 1000-fold greater in mice receiving 900 cGy compared with 200 cGy (P < .05). In mice receiving 200 cGy, VCN was similar whether ERT was stopped or given for 1 or 4 months after GT. In unconditioned mice, there was decreased survival with and without ERT, and VCN was very low to undetectable. When recipients were conditioned with 200 cGy and received transduced lineage-depleted marrow, only recipients receiving ERT (1 or 4 months) had detectable vector sequences in thymocytes. In conclusion, cytoreduction is important for the engraftment of gene-transduced HSC, and short-term ERT after GT did not diminish the capacity of gene-corrected cells to engraft and persist.
Molecular evolution of a-kinase anchoring protein (AKAP)-7: implications in comparative PKA compartmentalization
Authors: Johnson Keven R , Nicodemus-Johnson Jessie , Carnegie Graeme K , Danziger Robert S
Published: July 2012

Background

A-Kinase Anchoring Proteins (AKAPs) are molecular scaffolding proteins mediating the assembly of multi-protein complexes containing cAMP-dependent protein kinase A (PKA), directing the kinase in discrete subcellular locations. Splice variants from the AKAP7 gene (AKAP15/18) are vital components of neuronal and cardiac phosphatase complexes, ion channels, cardiac Ca2+ handling and renal water transport.

Results

Shown in evolutionary analyses, the formation of the AKAP7-RI/RII binding domain (required for AKAP/PKA-R interaction) corresponds to vertebrate-specific gene duplication events in the PKA-RI/RII subunits. Species analyses of AKAP7 splice variants shows the ancestral AKAP7 splice variant is AKAP7α, while the ancestral long form AKAP7 splice variant is AKAP7γ. Multi-species AKAP7 gene alignments, show the recent formation of AKAP7δ occurs with the loss of native AKAP7γ in rats and basal primates. AKAP7 gene alignments and two dimensional Western analyses indicate that AKAP7γ is produced from an internal translation-start site that is present in the AKAP7δ cDNA of mice and humans but absent in rats. Immunofluorescence analysis of AKAP7 protein localization in both rat and mouse heart suggests AKAP7γ replaces AKAP7δ at the cardiac sarcoplasmic reticulum in species other than rat. DNA sequencing identified Human AKAP7δ insertion-deletions (indels) that promote the production of AKAP7γ instead of AKAP7δ.

Conclusions

This AKAP7 molecular evolution study shows that these vital scaffolding proteins developed in ancestral vertebrates and that independent mutations in the AKAP7 genes of rodents and early primates has resulted in the recent formation of AKAP7δ, a splice variant of likely lesser importance in humans than currently described.
Prevalence and socio-behavioral influence of early childhood caries, ECC, and feeding habits among 6 – 36 months old children in Uganda and Tanzania
Authors: Masumo Ray , Bardsen Asgeir , Mashoto Kijakazi , Astrom Anne Nordrehaug
Published: July 2012

Background

Early childhood caries (ECC) is a serious problem that has remained unexplored in sub-Saharan Africa.This study aimed to identify possible socio-behavioral correlates of ECC focusing 6–36 months old children and their caretakers.

Methods

Cross sectional studies were conducted in a high fluoride rural area, Manyara, Tanzania and a low fluoride urban area, Kampala, Uganda. Totals of 1221 and 816 child - caretaker pairs attending health care facilities for growth monitoring were recruited in Manyara and Kampala, respectively. All caretakers completed face to face interviews at the health care facility. Children underwent oral clinical examination whereby ECC and Enamel hypoplasia were recorded using the dmft (WHO 1997) and the DDE index (FDI 1992).

Results

The prevalence of ECC was 3.7% in Manyara and 17.6% in Kampala. According to multiple logistic regression analyses, received oral health information from health worker was the strongest determinant of ECC in Manyara, adjusted OR 0.3, 95% CI 0.09 – 0.93. In Kampala, visible plaque, high sugar intake and presence of enamel hypoplasia associated with ECC, adjusted ORs 2.8 (95% CI 1.61- 4.95), 3.0 (95% CI 1.39 – 6.34) and 2.3 (95% CI 1.36 - 3.95).

Conclusion

Oral health education aimed at caretakers of 6–36 months, including health care workers’ information regarding the detrimental consequences for oral health of frequent sugar consumption and poor oral hygiene is important for prevention of ECC in Tanzania and Uganda.
The role of barrier membranes for guided bone regeneration and restoration of large bone defects: current experimental and clinical evidence
Authors: Dimitriou Rozalia , Mataliotakis George I , Calori Giorgio Maria , Giannoudis Peter V
Published: July 2012

Treatment of large bone defects represents a great challenge in orthopedic and craniomaxillofacial surgery. Although there are several methods for bone reconstruction, they all have specific indications and limitations. The concept of using barrier membranes for restoration of bone defects has been developed in an effort to simplify their treatment by offering a sinlge-staged procedure. Research on this field of bone regeneration is ongoing, with evidence being mainly attained from preclinical studies. The purpose of this review is to summarize the current experimental and clinical evidence on the use of barrier membranes for restoration of bone defects in maxillofacial and orthopedic surgery. Although there are a few promising preliminary human studies, before clinical applications can be recommended, future research should aim to establish the 'ideal' barrier membrane and delineate the need for additional bone grafting materials aiming to 'mimic' or even accelerate the normal process of bone formation. Reproducible results and long-term observations with barrier membranes in animal studies, and particularly in large animal models, are required as well as well-designed clinical studies to evaluate their safety, efficacy and cost-effectiveness.
CTGC motifs within the HIV core promoter specify Tat-responsive pre-initiation complexes
Authors: Wilhelm Emmanuelle , Doyle Marie-Christine , Nzaramba Isaac , Magdzinski Alexandre , Dumais Nancy , Bell Brendan
Published: July 2012

Background

HIV latency is an obstacle for the eradication of HIV from infected individuals. Stable post-integration latency is controlled principally at the level of transcription. The HIV trans-activating protein, Tat, plays a key function in enhancing HIV transcriptional elongation. The HIV core promoter is specifically required for Tat-mediated trans-activation of HIV transcription. In addition, the HIV core promoter has been shown to be a potential anti-HIV drug target. Despite the pivotal role of the HIV core promoter in the control of HIV gene expression, the molecular mechanisms that couple Tat function specifically to the HIV core promoter remain unknown.

Results

Using electrophoretic mobility shift assays (EMSAs), the TATA box and adjacent sequences of HIV essential for Tat trans-activation were shown to form specific complexes with nuclear extracts from peripheral blood mononuclear cells, as well as from HeLa cells. These complexes, termed pre-initiation complexes of HIV (PICH), were distinct in composition and DNA binding specificity from those of prototypical eukaryotic TATA box regions such as Adenovirus major late promoter (AdMLP) or the hsp70 promoter. PICH contained basal transcription factors including TATA-binding protein and TFIIA. A mutational analysis revealed that CTGC motifs flanking the HIV TATA box are required for Tat trans-activation in living cells and correct PICH formation in vitro. The binding of known core promoter binding proteins AP-4 and USF-1 was found to be dispensable for Tat function. TAR RNA prevented stable binding of PICH-2, a complex that contains the general transcription factor TFIIA, to the HIV core promoter. The impact of TAR on PICH-2 specifically required its bulge sequence that is also known to interact with Tat.

Conclusion

Our data reveal that CTGC DNA motifs flanking the HIV TATA box are required for correct formation of specific pre-initiation complexes in vitro and that these motifs are also required for Tat trans-activation in living cells. The impact of TAR RNA on PICH-2 stability provides a mechanistic link by which pre-initiation complex dynamics could be coupled to the formation of the nascent transcript by the elongating transcription complex. Together, these findings shed new light on the mechanisms by which the HIV core promoter specifically responds to Tat to activate HIV gene expression.
Condor-COPASI: high-throughput computing for biochemical networks
Authors: Kent Edward , Hoops Stefan , Mendes Pedro
Published: July 2012

Background

Mathematical modelling has become a standard technique to improve our understanding of complex biological systems. As models become larger and more complex, simulations and analyses require increasing amounts of computational power. Clusters of computers in a high-throughput computing environment can help to provide the resources required for computationally expensive model analysis. However, exploiting such a system can be difficult for users without the necessary expertise.

Results

We present Condor-COPASI, a server-based software tool that integrates COPASI, a biological pathway simulation tool, with Condor, a high-throughput computing environment. Condor-COPASI provides a web-based interface, which makes it extremely easy for a user to run a number of model simulation and analysis tasks in parallel. Tasks are transparently split into smaller parts, and submitted for execution on a Condor pool. Result output is presented to the user in a number of formats, including tables and interactive graphical displays.

Conclusions

Condor-COPASI can effectively use a Condor high-throughput computing environment to provide significant gains in performance for a number of model simulation and analysis tasks. Condor-COPASI is free, open source software, released under the Artistic License 2.0, and is suitable for use by any institution with access to a Condor pool. Source code is freely available for download athttp://code.google.com/p/condor-copasi/, along with full instructions on deployment and usage.
Haloperidol augmentation of fluvoxamine in skin picking disorder: a case report
Authors: Luca Maria , Vecchio Costanza , Luca Antonina , Calandra Carmela
Published: July 2012

Introduction

Compulsive skin picking, being part of the broader category of impulse control disorders, is considered a residual diagnosis in the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision. It is characterized by excessive scratching or picking of normal skin, or skin with minor surface irregularities, and occurs in 2% of patients attending dermatology clinics. Despite the clinical relevance of this disorder, no clear guidelines are available yet; clinical management is, therefore, compromised and the day-to-day clinical practice is burdened by difficulties. Studies on selective serotonin reuptake inhibitors and anti-epileptic drugs have provided limited results. The association between anti-depressants and anti-epileptics has been found to be beneficial in some impulse control disorders, but in skin picking no previous studies have been conducted on this pharmacological approach. There are very few reports on the efficacy of anti-psychotics in skin picking.

Case presentation

The therapeutic path described in this case report produced good results for a 59-year-old Caucasian woman. The first therapeutic approach, with fluvoxamine and oxcarbazepine was partially effective; then, the suspension of oxcarbazepine and haloperidol augmentation of fluvoxamine were adopted. After 10 weeks, a significant improvement of the disease was observed: the clinical picture and the associated symptoms were nearly solved.

Conclusions

To the best of our knowledge, this is the first article reporting the association of fluvoxamine and haloperidol in skin picking disorder. It might be useful to perform further research regarding the treatment of skin picking disorder: in clinical practice, several variables might limit the choice of certain drugs. Therefore, it would be useful for the clinician to be aware of other therapeutic options.
A cross-sectional study of pandemic influenza health literacy and the effect of a public health campaign
Authors: Jhummon-Mahadnac Namrata Devi , Knott Jonathan , Marshall Caroline
Published: July 2012

Background

To ascertain the understanding of 2009 pandemic (H1N1) influenza and relevant infection control measures in an emergency department population and to assess the effectiveness of education campaigns in informing the public about the pandemic.

Methods

Questionnaires were administered to patients, visitors, non-clinical staff and volunteers. Data were collected on knowledge, preventative measures, information sources, attitudes to government and media reporting, perceived seriousness, behaviour change and intended compliance with future measures. Results were used to construct an overall knowledge score.

Results

There were 252 participants. Traditional forms of mass media (138 [55%]) remained the principal information source. Approximately 70% (176) accurately described mode of transmission and recommended precautions and 68% (175) reported behaviour change because of the pandemic. Gaps in knowledge included failure to identify certain high risk groups. Recall of government campaigns was significantly associated with a higher knowledge score. 60% (151) thought that authorities and media had exaggerated the threat; only 40% (101) would comply with recommended measures in a future pandemic.

Conclusions

The knowledge regarding pandemic influenza was high in this population and positively affected by official campaigns. Pandemic planning should address knowledge gaps and the impression that authorities had exaggerated the public-health threat.
Eukaryotic initiation factor 4E-3 is essential for meiotic chromosome segregation, cytokinesis and male fertility in Drosophila
Authors: Hernández Greco , Han Hong , Gandin Valentina , Fabian Lacramioara , Ferreira Tiago , Zuberek Joanna , Sonenberg Nahum , Brill Julie A. , Lasko Paul
Published: July 2012

Gene expression is translationally regulated during many cellular and developmental processes. Translation can be modulated by affecting the recruitment of mRNAs to the ribosome, which involves recognition of the 5′ cap structure by the cap-binding protein eIF4E. Drosophila has several genes encoding eIF4E-related proteins, but the biological role of most of them remains unknown. Here, we report that Drosophila eIF4E-3 is required specifically during spermatogenesis. Males lacking eIF4E-3 are sterile, showing defects in meiotic chromosome segregation, cytokinesis, nuclear shaping and individualization. We show that eIF4E-3 physically interacts with both eIF4G and eIF4G-2, the latter being a factor crucial for spermatocyte meiosis. In eIF4E-3 mutant testes, many proteins are present at different levels than in wild type, suggesting widespread effects on translation. Our results imply that eIF4E-3 forms specific eIF4F complexes that are essential for spermatogenesis.