Violence Defied? A Review of Prevention of Violence in the Public and Semi-public Domain

This report provides a synthesis of 48 studies of the effects of the prevention of violence in the public and semi-public domain

Aggression and violence, posttraumatic stress, and absenteeism among employees in penitentiaries

On the basis of the Labour Conditions Act of 18 March 1999, employers are obliged to take care of their employees’ safety and health, and to pursue a policy aimed at creating the best possible labour conditions. The prevention of aggression towards employees falls under this obligation. In November 2005, the Scientific Research and Documentation Centre (WODC) carried out a large-scale study into the prevalence of aggressive behaviour targeting employees of penitentiaries during their work, which was commissioned by the Judicial Penitentiary Service (DJI) (Bogaerts & Den Hartogh, 2006). One of the remarkable findings of this study was that ‘aggression and violence among employees’ is a frequently occurring phenomenon within the prison system; of the 5,750 responding employees, no less than 641 reported to have fallen victim to one or more forms of aggression and violence among employees in the course of the previous twelve months. In this context, the term ‘aggression and violence among employees’ includes experienced unwanted sexual attention, intimidation, and physical violence. Aggression and violence among employees consists either of incidents between staff members, or of incidents between executive staff members and ordinary staff members. A substantial part of prison personnel is comprised of penitentiary workers (in Dutch, so-called ‘PIW-ers’). In order to improve the safety of penitentiary workers, the Ministry of Social Affairs and Employment, the DJI, and the unions have reached an agreement about a reduction of aggression and violence among employees, laid down in the ‘Arboplus Covenant Judicial Penitentiary Service on the Policy on Absenteeism, Integral Personal Safety, and the Career Perspective of Executive Personnel’2. The goal was for this reduction to be brought about halfway through 20073. To enable itself to successfully act upon the agreement, in addition to many actions and measures, the Sector Directorate of the Prison System asked the WODC to conduct an in-depth study on aggression and violence among employees within the penitentiaries. It was decided to examine only personal factors in this study. Organisational and economic factors and the institutional features of organisations that, without any doubt, play an important role in both the prevention and the occurrence of aggression and violence among employees were not studied. Framing mechanisms, for instance, which occur in every organisation, were not included in the study, the importance of this and other concepts notwithstanding. In his book ’Frame analysis: An essay on the organization of experience’, Goffman writes: “The concept of framing is taken to label schemata of interpretation that allows individuals or groups to locate, perceive, identify, and label events and occurrences, thus rendering meaning, organizing experiences, and guiding actions.” (Goffman, 1974, p. 21) The study ‘Benchmark Penitentiaries’, which will start in the autumn of 2007, will include institutional, organisational, and economic characteristics as well, besides the personal indicators, in order to assess the quality of the penitentiaries. In this way, aggression and violence among employees will not only be linked to personal factors, but will also be related to institutional factors and characteristics specific to the organisation. Another correlation not included in the present study is that between domestic violence (partner violence), violence at work, absenteeism, and the economic costs, even though this correlation is regularly established in the literature (e.g. Reeves & O’Leary-Kelly, 2007; Swanberg, Macke, & Logan, 2007). With this study, the aim of the Judicial Penitentiary Service is to gain insight into the possible effects of aggression and violence among employees and in the factors which are at the roots of it. The DJI is especially interested in absenteeism as a possible effect of aggression and violence among employees, and in the psychosocial factors that play a role. In the present report, we will present the findings of this study. It is set up as follows. In chapter 2, we will examine the potential effects of aggression and violence among employees and the factors at their source, such as can be assumed to exist when we base ourselves on the literature. Next, we will take these findings as the basis for our hypothetical model presented in chapter 3, which will be the starting point for the empirical part of the study. We will also discuss how this model was tested. In chapter 4, we will present de results of this test and we will examine whether it is necessary to break down the model into sub-models. In chapter 5, we will subsequently formulate twelve specific research questions, which deserve further exploration in the researchers’ view, both on the basis of the literature study and of the hypothetical model derived from it. All these questions are in logical keeping with the formulated hypothetical model. Again, we will indicate how these questions were tested. Finally, chapter 6 will provide a summary of the study. In this chapter we will present some conclusions as well

Evaluation of the endoplasmic reticulum-stress response in eIF2B-mutated lymphocytes and lymphoblasts from CACH/VWM patients

<p>Abstract</p> <p>Background</p> <p>Eukaryotic translation initiation factor 2B (eIF2B), a guanine nucleotide exchange factor (GEF) and a key regulator of translation initiation under normal and stress conditions, causes an autosomal recessive leukodystrophy of a wide clinical spectrum. EBV-immortalised lymphocytes (EIL) from eIF2B-mutated patients exhibit a decrease in eIF2B GEF activity. eIF2B-mutated primary fibroblasts have a hyper-induction of activating transcription factor 4 (ATF4) which is involved in the protective unfolded protein response (UPR), also known as the ER-stress response. We tested the hypothesis that EIL from eIF2B-mutated patients also exhibit a heightened ER-stress response.</p> <p>Methods</p> <p>We used thapsigargin as an ER-stress agent and looked at polysomal profiles, rate of protein synthesis, translational activation of <it>ATF4</it>, and transcriptional induction of stress-specific mRNAs (<it>ATF4, CHOP, ASNS, GRP78</it>) in normal and eIF2B-mutated EIL. We also compared the level of stress-specific mRNAs between EIL and primary lymphocytes (PL).</p> <p>Results</p> <p>Despite the low eIF2B GEF activity in the 12 eIF2B-mutated EIL cell lines tested (range 40-70% of normal), these cell lines did not differ from normal EIL in their ATF4-mediated ER-stress response. The absence of hyper-induction of ATF4-mediated ER-stress response in eIF2B-mutated EIL in contrast to primary fibroblasts is not related to their transformation by EBV. Indeed, PL exhibited a higher induction of the stress-specific mRNAs in comparison to EIL, but no hyper-induction of the UPR was noticed in the eIF2B-mutated cell lines in comparison to controls.</p> <p>Conclusions</p> <p>Taken together with work of others, our results demonstrate the absence of a major difference in ER-stress response between controls and eIF2B-mutated cells. Therefore, components of the ER-stress response cannot be used as discriminantory markers in eIF2B-related disorders.</p

Genomic Evidence for Complex Domestication History of the Cultivated Tomato in Latin America

The process of plant domestication is often protracted, involving underexplored intermediate stages with important implications for the evolutionary trajectories of domestication traits. Previously, tomato domestication history has been thought to involve two major transitions: one from wild Solanum pimpinellifolium L. to a semidomesticated intermediate, S. lycopersicum L. var. cerasiforme (SLC) in South America, and a second transition from SLC to fully domesticated S. lycopersicum L. var. lycopersicum in Mesoamerica. In this study, we employ population genomic methods to reconstruct tomato domestication history, focusing on the evolutionary changes occurring in the intermediate stages. Our results suggest that the origin of SLC may predate domestication, and that many traits considered typical of cultivated tomatoes arose in South American SLC, but were lost or diminished once these partially domesticated forms spread northward. These traits were then likely reselected in a convergent fashion in the common cultivated tomato, prior to its expansion around the world. Based on these findings, we reveal complexities in the intermediate stage of tomato domestication and provide insight on trajectories of genes and phenotypes involved in tomato domestication syndrome. Our results also allow us to identify underexplored germplasm that harbors useful alleles for crop improvement

Adult-onset Alexander disease with typical "tadpole" brainstem atrophy and unusual bilateral basal ganglia involvement: a case report and review of the literature

<p>Abstract</p> <p>Background</p> <p>Alexander disease (ALX) is a rare neurological disorder characterized by white matter degeneration and cytoplasmic inclusions in astrocytes called Rosenthal fibers, labeled by antibodies against glial fibrillary acidic protein (GFAP). Three subtypes are distinguished according to age at onset: infantile (under age 2), juvenile (age 2 to 12) and adult (over age 12). Following the identification of heterozygous mutations in <it>GFAP </it>that cause this disease, cases of adult-onset ALX have been increasingly reported.</p> <p>Case Presentation</p> <p>We present a 60-year-old Japanese man with an unremarkable past and no family history of ALX. After head trauma in a traffic accident at the age of 46, his character changed, and dementia and dysarthria developed, but he remained independent. Spastic paresis and dysphagia were observed at age 57 and 59, respectively, and worsened progressively. Neurological examination at the age of 60 revealed dementia, pseudobulbar palsy, left-side predominant spastic tetraparesis, axial rigidity, bradykinesia and gaze-evoked nystagmus. Brain MRI showed tadpole-like atrophy of the brainstem, caused by marked atrophy of the medulla oblongata, cervical spinal cord and midbrain tegmentum, with an intact pontine base. Analysis of the <it>GFAP </it>gene revealed a heterozygous missense mutation, c.827G>T, p.R276L, which was already shown to be pathogenic in a case of pathologically proven hereditary adult-onset ALX.</p> <p>Conclusion</p> <p>The typical tadpole-like appearance of the brainstem is strongly suggestive of adult-onset ALX, and should lead to a genetic investigation of the <it>GFAP </it>gene. The unusual feature of this patient is the symmetrical involvement of the basal ganglia, which is rarely observed in the adult form of the disease. More patients must be examined to confirm, clinically and neuroradiologically, extrapyramidal involvement of the basal ganglia in adult-onset ALX.</p

Applicability of multiple quantitative magnetic resonance methods in genetic brain white matter disorders

Background and purpose: Magnetic resonance imaging (MRI) measures of tissue microstructure are important for monitoring brain white matter (WM) disorders like leukodystrophies and multiple sclerosis. They should be sensitive to underlying pathological changes. Three whole-brain isotropic quantitative methods were applied and compared within a cohort of controls and leukodystrophy patients: two novel myelin water imaging (MWI) techniques (multi-compartment relaxometry diffusion-informed MWI: MCR-DIMWI, and multi-echo T2 relaxation imaging with compressed sensing: METRICS) and neurite orientation dispersion and density imaging (NODDI).// Methods: For 9 patients with different leukodystrophies (age range 0.4-62.4 years) and 15 control subjects (2.3-61.3 years), T1-weighted MRI, fluid-attenuated inversion recovery, multi-echo gradient echo with variable flip angles, METRICS, and multi-shell diffusion-weighted imaging were acquired on 3 Tesla. MCR-DIMWI, METRICS, NODDI, and quality control measures were extracted to evaluate differences between patients and controls in WM and deep gray matter (GM) regions of interest (ROIs). Pearson correlations, effect size calculations, and multi-level analyses were performed.// Results: MCR-DIMWI and METRICS-derived myelin water fractions (MWFs) were lower and relaxation times were higher in patients than in controls. Effect sizes of MWF values and relaxation times were large for both techniques. Differences between patients and controls were more pronounced in WM ROIs than in deep GM. MCR-DIMWI-MWFs were more homogeneous within ROIs and more bilaterally symmetrical than METRICS-MWFs. The neurite density index was more sensitive in detecting differences between patients and controls than fractional anisotropy. Most measures obtained from MCR-DIMWI, METRICS, NODDI, and diffusion tensor imaging correlated strongly with each other.// Conclusion: This proof-of-concept study shows that MCR-DIMWI, METRICS, and NODDI are sensitive techniques to detect changes in tissue microstructure in WM disorders

Homozygous UBA5 Variant Leads to Hypomyelination with Thalamic Involvement and Axonal Neuropathy

The enzyme ubiquitin-like modifier activating enzyme 5 (UBA5) plays an important role in activating ubiquitin-fold modifier 1 (UFM1) and its associated cascade. UFM1 is widely expressed and known to facilitate the post-translational modification of proteins. Variants in UBA5 and UFM1 are involved in neurodevelopmental disorders with early-onset epileptic encephalopathy as a frequently seen disease manifestation. Using whole exome sequencing, we detected a homozygous UBA5 variant (c.895C > T p. [Pro299Ser]) in a patient with severe global developmental delay and epilepsy, the latter from the age of 4 years. Magnetic resonance imaging showed hypomyelination with atrophy and T2 hyperintensity of the thalamus. Histology of the sural nerve showed axonal neuropathy with decreased myelin. Functional analyses confirmed the effect of the Pro299Ser variant on UBA5 protein function, showing 58% residual protein activity. Our findings indicate that the epilepsy currently associated with UBA5 variants may present later in life than previously thought, and that radiological signs include hypomyelination and thalamic involvement. The data also reinforce recently reported associations between UBA5 variants and peripheral neuropathy

高信頼度高能率映像配信サービス実現のためのコンテンツハンドリング技術に関する研究

博士（工学）神戸大

A clinical approach to the diagnosis of patients with leukodystrophies and genetic leukoencephelopathies

Leukodystrophies (LD) and genetic leukoencephalopathies (gLE) are disorders that result in white matter abnormalities in the central nervous system (CNS). Magnetic resonance (MR) imaging (MRI) has dramatically improved and systematized the diagnosis of LDs and gLEs, and in combination with specific clinical features, such as Addison’s disease in Adrenoleukodystrophy or hypodontia in Pol-III related or 4H leukodystrophy, can often resolve a case with a minimum of testing. The diagnostic odyssey for the majority LD and gLE patients, however, remains extensive – many patients will wait nearly a decade for a definitive diagnosis and at least half will remain unresolved. The combination of MRI, careful clinical evaluation and next generation genetic sequencing holds promise for both expediting the diagnostic process and dramatically reducing the number of unresolved cases. Here we present a workflow detailing the Global Leukodystrophy Initiative (GLIA) consensus recommendations for an approach to clinical diagnosis, including salient clinical features suggesting a specific diagnosis, neuroimaging features and molecular genetic testing. We also discuss recommendations on the use of broad-spectrum next-generation sequencing in instances of ambiguous MRI or clinical findings. We conclude with a proposal for systematic trials of genome-wide agnostic testing as a first line diagnostic in LDs and gLEs given the increasing number of genes associated with these disorders