66 research outputs found

    Varieties of Homeland Security: An Assessment of US State-level Definitions

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    Homeland Security continues to struggle to define itself as a field of practice and scholarship. The difficulty in defining the field has led to a variety of conflicts over membership, content, and focus. This article reviews some of the prominent debates over the meaning of homeland security as a field of study and practice. It then defines a simple schema for definitions of homeland security inspired by the academic and legislative debates over the issue. A frequency cataloging of definitions from US state agencies illustrates the continued relevance of a “partial membership” approach to defining the field. The article concludes with a discussion of the implications of the diversity of homeland security definitions for the development of the field.Ye

    Efficacy and safety of asfotase alfa in infants and young children with hypophosphatasia : a phase 2 open-label study

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    Context Long-term data on enzyme replacement treatment of hypophosphatasia (HPP) are limited. Objective To evaluate efficacy and safety of asfotase alfa in patients aged ≤5 years with HPP followed for up to 6 years. Design Phase 2 open-label study (July 2010 to September 2016). Setting Twenty-two sites; 12 countries. Participants Sixty-nine patients [median (range) age: 16.0 (0.02 to 72) months] with severe HPP and sign/symptom onset before age 6 months. Intervention Asfotase alfa 2 mg/kg three times/week or 1 mg/kg six times/week subcutaneously. Main Outcome Measures Primary efficacy measure: Radiographic Global Impression of Change (RGI-C) score [−3 (severe worsening) to +3 (complete/near-complete healing)]. Additional outcome measures: respiratory status, growth, and safety. Post hoc analysis: characteristics of radiographic responders vs nonresponders at Year 1 (RGI-C: ≥+2 vs <+2). Results During median (minimum, maximum) 2.3 (0.02, 5.8) years of treatment, RGI-C scores improved significantly at Month 6 [+2.0 (−1.7, +3.0)], Year 1 [+2.0 (−2.3, +3.0)], and Last Assessment [+2.3 (−2.7, +3.0); P < 0.0001 all]. Of 24 patients requiring respiratory support at Baseline, 11 (46%) no longer needed support. Height/weight z scores generally increased. Nine patients died (13%). All patients experienced at least one adverse event; pyrexia was most common. Compared with responders [n = 50 (72%)], nonresponders [n = 19 (28%)] had more severe disease at Baseline and a higher rate of neutralizing antibodies (NAbs) at Last Assessment. Conclusions Most infants/young children given asfotase alfa showed early radiographic and clinical improvement sustained up to 6 years; radiographic nonresponders had more severe disease and more frequent NAbs at Last Assessment
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