1,099 research outputs found

    Hakka Indenture Museum

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    https://openscholarship.wustl.edu/bcs/1362/thumbnail.jp

    Countering the Australian 'ndrangheta: The criminalisation of mafia behaviour in Australia between national and comparative criminal law

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    Mafia-type criminal groups belonging to, or originated from, the Calabrian ‘ndrangheta from Southern Italy, have been object of recent academic research and media attention in Australia. The Australian ‘ndrangheta, as qualified form of organised crime, poses new challenges for law enforcement in the country. This paper briefly looks at the strategies to fight organised crime in Australia, with specific focus on anti-association laws. By using a comparative approach, the paper will look at the criminalisation of mafias as qualified forms of organised crime in other two jurisdictions, Italy and the USA, to advocate for an effective mafia criminalisation in Australia. In conclusion, this paper will argue that, in order to also fight mafia phenomena, criminal law in Australia should focus on behaviours of organised crime groups rather than only on the criminalisation of proscribed associations and their illegal activities

    Prevalence of CADASIL and Fabry Disease in a Cohort of MRI Defined Younger Onset Lacunar Stroke.

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    BACKGROUND AND PURPOSE: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), caused by mutations in the NOTCH3 gene, is the most common monogenic disorder causing lacunar stroke and cerebral small vessel disease (SVD). Fabry disease (FD) due to mutations in the GLA gene has been suggested as an underdiagnosed cause of stroke, and one feature is SVD. Previous studies reported varying prevalence of CADASIL and FD in stroke, likely due to varying subtypes studied; no studies have looked at a large cohort of younger onset SVD. We determined the prevalence in a well-defined, MRI-verified cohort of apparently sporadic patients with lacunar infarct. METHODS: Caucasian patients with lacunar infarction, aged ≤70 years (mean age 56.7 (SD8.6)), were recruited from 72 specialist stroke centres throughout the UK as part of the Young Lacunar Stroke DNA Resource. Patients with a previously confirmed monogenic cause of stroke were excluded. All MRI's and clinical histories were reviewed centrally. Screening was performed for NOTCH3 and GLA mutations. RESULTS: Of 994 subjects five had pathogenic NOTCH3 mutations (R169C, R207C, R587C, C1222G and C323S) all resulting in loss or gain of a cysteine in the NOTCH3 protein. All five patients had confluent leukoaraiosis (Fazekas grade ≥2). CADASIL prevalence overall was 0.5% (95% CI 0.2%-1.1%) and among cases with confluent leukoaraiosis 1.5% (95% CI 0.6%-3.3%). No classic pathogenic FD mutations were found; one patient had a missense mutation (R118C), associated with late-onset FD. CONCLUSION: CADASIL cases are rare and only detected in SVD patients with confluent leukoaraiosis. No definite FD cases were detected.The UK Young Lacunar Stroke DNA Study was funded by a grants from the Wellcome Trust (WT072952, www.wellcome.ac.uk) and the Stroke Association (TSA 2010/01& TSA 2013/02, www.stroke.org.uk). Fabry disease screening was supported by an unrestricted scientific grant from Shire Human Genetic Therapies (www.shire.com). The sponsors of the study had no role in the study design, data collection, data analysis, interpretation, writing of the manuscript, or the decision to submit the manuscript for publication. L R-J’s salary is funded by a Stroke Association/ British Heart Foundation grant. (TSA/BHF 2010/01). HM is supported by an National Institute for Health Research Senior Investigator award (www.nihr.ac.uk). HM and SB are supported by the Cambridge University Trust National Institute for Health Research Comprehensive Research Centre (www.cambridge-brc.org.uk).This is the final version of the article. It first appeared from PLoS via http://dx.doi.org/10.1371/journal.pone.013635

    Association of Systemic Lupus Erythematosus Clinical Features with European Population Genetic Substructure

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    Systemic Lupus Erythematosus (SLE) is an autoimmune disease with a very varied spectrum of clinical manifestations that could be partly determined by genetic factors. We aimed to determine the relationship between prevalence of 11 clinical features and age of disease onset with European population genetic substructure. Data from 1413 patients of European ancestry recruited in nine countries was tested for association with genotypes of top ancestry informative markers. This analysis was done with logistic regression between phenotypes and genotypes or principal components extracted from them. We used a genetic additive model and adjusted for gender and disease duration. Three clinical features showed association with ancestry informative markers: autoantibody production defined as immunologic disorder (P = 6.8×10(-4)), oral ulcers (P = 6.9×10(-4)) and photosensitivity (P = 0.002). Immunologic disorder was associated with genotypes more common in Southern European ancestries, whereas the opposite trend was observed for photosensitivity. Oral ulcers were specifically more common in patients of Spanish and Portuguese self-reported ancestry. These results should be taken into account in future research and suggest new hypotheses and possible underlying mechanisms to be investigated. A first hypothesis linking photosensitivity with variation in skin pigmentation is suggested

    A qualitative reading of the ecological (dis)organisation of criminal associations. The case of the ?Famiglia Basilischi? in Italy

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    This paper combines the theoretical foundations of organisational ecology - one of the most important approaches in economic sociology - with classic criminological theories to interpret the birth, evolution and death of criminal associations. This mixed approach will support the interpretation of organised crime groups as phenomena strictly linked to the environment as well as to other competitors in criminal markets. This paper analyses the birth, evolution and death of a criminal association in Basilicata, Southern Italy, known as the ?Famiglia Basilischi?. The case is exemplary of how ecological conditions affect the success or failure of a newly formed criminal association. These conditions can therefore be indicators to interpret organised criminal activities in similar environments

    Investigation of the crystallisation inhibitory properties of albumin isolated from the urine of Black and White South Africans

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    Bibliography: leaves 81-94.Stone incidence in South African blacks is extremely rare. The hypothesis to be tested is whether this apparent immunity can be attributed to the presence of more potent inhibitors of calcium oxalate (CaOx) crystallisation in urine from blacks compared to those occurring in the urine of white subjects. Urinary proteins have been shown to be inhibitors. This study investigated the crystallisation inhibitory properties of albumin isolated from the urine of healthy black (BA) and healthy white (WA) South Africans as well as those of human serum albumin (HSA). Albumin was selected because previous studies on other population groups had revealed that the protein inhibits CaOx aggregation. This observation is significant because crystal aggregation is considered to be more important for stone formation than crystal nucleation or growth

    Widening the Antimafia Net. Mafia Behaviour, Cultural Transmission and Children Protection in Calabrian mafia families

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    This article explores proceedings by the Youth Tribunal of Reggio Calabria, Southern Italy, aimed at the protection of children in families where one or both parents are investigated for mafia offences. The findings show that preventing the transmission of mafia (‘ndrangheta) culture in the local context has become an essential part of child protection measures. This article will argue that when discussing child protection in criminal families, it is necessary (a) to question the nature of the bonds of these families with the socio-cultural context, and (b) to concretely assess the way this context wishes to affect the family’s criminality
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