17 research outputs found

    Scientific Knowledge of the Human Side of Information Security as a Basis for Sustainable Trainings in Organizational Practices

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    Comprehensive digitization leads to new chal-lenges because of cybercrime and related security countermeasures. There is no doubt that this will fundamentally affect our lives and is leading to an increase in the importance of information security (IS). However, technology solutions alone are not sufficient to ensure IS countermeasures. The human side of security is important to protect organizational assets like user information and systems. The paper illustrates these relationships in terms of information security awareness (ISA), examining its goals and the factors influencing it through the systematic analysis and review of scientific literature and the transfer of scientific knowledge for practical purposes. We reviewed the publications of leading academic journals in the field of IS over the past decade

    Mudd’s disease (MAT I/III deficiency): a survey of data for MAT1A homozygotes and compound heterozygotes

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    The phenotypic spectrum of organic acidurias and urea cycle disorders. Part 1: the initial presentation.

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    BACKGROUND: The clinical presentation of patients with organic acidurias (OAD) and urea cycle disorders (UCD) is variable; symptoms are often non-specific. AIMS/METHODS: To improve the knowledge about OAD and UCD the E-IMD consortium established a web-based patient registry. RESULTS: We registered 795 patients with OAD (n = 452) and UCD (n = 343), with ornithine transcarbamylase (OTC) deficiency (n = 196), glutaric aciduria type 1 (GA1; n = 150) and methylmalonic aciduria (MMA; n = 149) being the most frequent diseases. Overall, 548 patients (69 %) were symptomatic. The majority of them (n = 463) presented with acute metabolic crisis during (n = 220) or after the newborn period (n = 243) frequently demonstrating impaired consciousness, vomiting and/or muscular hypotonia. Neonatal onset of symptoms was most frequent in argininosuccinic synthetase and lyase deficiency and carbamylphosphate 1 synthetase deficiency, unexpectedly low in male OTC deficiency, and least frequently in GA1 and female OTC deficiency. For patients with MMA, propionic aciduria (PA) and OTC deficiency (male and female), hyperammonemia was more severe in metabolic crises during than after the newborn period, whereas metabolic acidosis tended to be more severe in MMA and PA patients with late onset of symptoms. Symptomatic patients without metabolic crises (n = 94) often presented with a movement disorder, mental retardation, epilepsy and psychiatric disorders (the latter in UCD only). CONCLUSIONS: The initial presentation varies widely in OAD and UCD patients. This is a challenge for rapid diagnosis and early start of treatment. Patients with a sepsis-like neonatal crisis and those with late-onset of symptoms are both at risk of delayed or missed diagnosis
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