5 research outputs found

    Women and trusts

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    Variant transthyretin amyloidosis (ATTRv) polyneuropathy in Greece: a broad overview with a focus on non-endemic unexplored regions of the country

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    Comprehensive data on variant transthyretin amyloidosis polyneuropathy (ATTRv-PN) in Greece are lacking. We presently provide an overview of ATTRv-PN in Greece, focusing on unexplored non-endemic regions of the country. In total, we identified 57 cases of ATTRv-PN diagnosed over the past 25 years, including 30 from the island of Crete, an apparent endemic region. Patients carried 10 different TTR mutations (C10R; P24S; V30M; R34G; R34T; I68L; A81T; E89Q; E89K and V94A). Carriers of the common V30M mutation constituted 54.3 % of the cohort. A known founder effect for the V30M mutation was present on the island of Crete. Non-endemic cases identified outside the island of Crete are presently reported in more detail. The age of onset ranged from 25 to 77 years, with a mean of 51.1 years. A mean diagnostic delay of 3.2 years was observed. V30M patients had earlier onset and less cardiac involvement than patients carrying other mutations. Genotype-phenotype correlations were largely consistent with published data. We conclude that, with the exception of the Cretan cluster, ATTRv-PN is not endemic in the Greek population. This makes timely diagnosis more challenging, yet absolutely essential given the availability of therapies that can alter the long-term course of the disease. © 2021 Elsevier B.V

    Child sexual abuse in institutional and non-institutional contexts

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    This chapter first focuses on major challenges confronting child and youth-serving organisations and high-risk settings. It then discusses the nature, key findings and major recommendations of Australia’s Royal Commission Into Institutional Responses to Child Sexual Abuse, a landmark public inquiry into institutional sexual abuse, including a special focus on the Roman Catholic Church. The chapter then focuses on several recent examples of progress in responses to major problems presented by child sexual abuse in institutional and non-institutional settings. Developments from Australia in particular, but also from other countries, will show how new public health law responses, including through civil law, and with various emphases on primary prevention and secondary prevention, can create frameworks for enhanced prevention, identification, and response to cases of child sexual abuse. Some of these responses, such as redress schemes, reportable conduct schemes and child safe standards legislation, have specific application to institutional settings. Other responses, such as the abolition of statutes of limitation for civil claims for injuries caused by sexual abuse, and other kinds of legislative reporting duties, have broader application across society, as they apply to sexual abuse in all settings, whether within institutions, families, private settings, or other community settings. These responses are of broad application regarding prevention of child sexual abuse, early identification of child sexual offending, and ensuring appropriate responses once it is known or suspected. They are particularly relevant when dealing with high risk institutional settings and prolific individual offenders, both of which present especially urgent examples of the need for an appropriate societal approach to child sexual abuse informed by public health and social justice
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