12 research outputs found

    Complex craniosynostoses: a review of the prominent clinical features and the related management strategies

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    The protocols for clinical evaluation and management of children with complex craniosynostoses are significantly different from those used in single suture forms. The time at which the various anatomical and functional anomalies observed in the affected subjects become clinically relevant varies from patient to patient, consequently requiring a tailored approach. The clinical course is variable and influenced by multiple factors, acting at different steps of the children growth. Intracranial hypertension is a major concern already in the first months of life; active cerebrospinal fluid (CSF) dynamics disorders, venous hypertension, and progressive craniocerebral disproportion are considered the main pathogenetic factors. Cranial vault and skull base sutures synostoses account for the frequently observed increased venous pressure. Skull base abnormalities lead to upper airways obstruction, which, on one side, might create significant upper airways obstructive problems and, on the other, contribute to the increase in the intracranial pressure. Secondary Chiari malformation is common and considered as a progressive disorder, mainly due to progressive craniocerebral disproportion, venous hypertension, and CSF dynamics disorders. Optic nerve and orbit-related eye-globe diseases are also a major concern. Papilledema is mostly related to increased intracranial pressure. The skull base synostotic process is the base of significant abnormalities of the orbital space, ending in the common feature of significant proptosis with the consequent risk of corneal ulcers. Aims of this paper are to analyze the physiopathogenetic mechanisms at the base of the clinical manifestations presented by children with complex craniosynostoses, and the therapeutic options currently available

    Evidence based EU criminal policy making: in search of valid data

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    EU criminal policy making is a relatively new policy domain and its credibility is said to be undermined by the lack of an evidence base. Because the EU claims to pursue evidence based policy making, this justifies reviewing the mechanisms put in place to that end. To properly evaluate the evidence base in EU criminal policy making, an assessment is made of the availability of comparable crime statistics. Crime statistics, a vital data source for criminal policy making, are considered highly problematic at EU level due to (amongst other reasons) the differences in the definition of the offences. In spite of the good intentions that can be read into the repeated acknowledgement of the importance of crime statistics and the efforts to commonly define EU worthy offences, a thorough empirical analysis leads to the conclusion that we are still in search of valid EU level data with respect to the EU level offences. The EU as a policy maker does not take its responsibility to ensure the availability of the necessary comparable crime statistical data serious enough

    Genetics of Human Hydrocephalus

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