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The interfacing of soft and hard electronics is a key challenge for flexible hybrid electronics. Currently, a multisubstrate approach is employed, where soft and hard devices are fabricated or assembled on separate substrates, and bonded or interfaced using connectors; this hinders the flexibility of the device and is prone to interconnect issues. Here, a single substrate interfacing approach is reported, where soft devices, i.e., sensors, are directly printed on Kapton polyimide substrates that are widely used for fabricating flexible printed circuit boards (FPCBs). Utilizing a process flow compatible with the FPCB assembly process, a wearable sensor patch is fabricated composed of inkjet-printed gold electrocardiography (ECG) electrodes and a stencil-printed nickel oxide thermistor. The ECG electrodes provide 1 mVp–p ECG signal at 4.7 cm electrode spacing and the thermistor is highly sensitive at normal body temperatures, and demonstrates temperature coefficient, α ≈ –5.84% K–1 and material constant, β ≈ 4330 K. This sensor platform can be extended to a more sophisticated multisensor platform where sensors fabricated using solution processable functional inks can be interfaced to hard electronics for health and performance monitoring, as well as internet of things applications.

Triggered by the recent inquiries into the Fukushima nuclear disaster, this article reflects on the challenges of developing and operating risk regulation and crisis management regimes in an era of highly complex and tightly interconnected socio-technical systems. These challenges are not just technical and professional but fundamentally institutional and cultural. The article identifies three key paradoxes and challenges of contemporary risk and crisis management, signals a range of recurrent problems in governments' efforts to cope with these challenges, problematizes current patterns of societal learning from crises, and sketches an agenda for public administration research in this area.

The February 2009 bushfires have prompted a debate with regard to contemporary arrangements for dealing with large-scale disasters. In this article, we seek to contribute to that debate by culling lessons learned from the literature on crisis and disaster management. We discuss what constitutes an effective disaster response system, we identify some key barriers to the effective functioning of such a system, and offer some suggestions for improvement.

The article investigates an important recent dispute within systematic theology over the interpretation of Thomas Aquinas. John Milbank has defended the view that the doctrine of analogy in Aquinas is peculiarly implicated with his entire ontology, that it cannot be understood in merely semantic terms, and that it involves a less “agnostic” position on knowledge of God than is often assumed. The article critically engages this position in two ways. It offers an archaeology of the prior polemical context out of which the claim arose, for the meaning and purpose of Milbank's claim are illuminated once it is seen as the vigorous repudiation of a “grammatical” or “linguistic” interpretation of Thomas on analogy which had been proffered earlier by Nicholas Lash. It will also provide a close investigation of the citations and interpretations of Aquinas texts that Milbank uses to ground his position, in order to adjudicate the dispute with Lash. The result will be to call strongly into question the plausibility of Milbank's readings of Aquinas. The article also indicates at several points the way in which those readings are shaped by an overriding anti-Kantian thrust in Milbank's entire approach to the discussion. In conclusion, it adumbrates the larger and older question which subtends the entire dispute: to what degree is some kind of vision or intuitive grasp of being as such, or of God's being, granted human beings in this life?

A pilot study of high dose coenzyme Q(10) (CoQ(10))/vitamin E therapy in Friedreich's ataxia (FRDA) patients resulted in significant clinical improvements in most patients. This study investigated the potential for this treatment to modify clinical progression in FRDA in a randomized double blind trial.Fifty FRDA patients were randomly divided into high or low dose CoQ(10)/ vitamin E groups. The change in International Co-operative Ataxia Ratings Scale (ICARS) was assessed over 2 years as the primary end-point. A post hoc analysis was made using cross-sectional data.At baseline serum CoQ(10) and vitamin E levels were significantly decreased in the FRDA patients (P < 0.001). During the trial CoQ(10) and vitamin E levels significantly increased in both groups (P < 0.01). The primary and secondary end-points were not significantly different between the therapy groups. When compared to cross-sectional data 49% of all patients demonstrated improved ICARS scores. This responder group had significantly lower baseline serum CoQ(10) levels.A high proportion of FRDA patients have a decreased serum CoQ(10) level which was the best predictor of a positive clinical response to CoQ(10)/vitamin E therapy. Low and high dose CoQ(10)/vitamin E therapies were equally effective in improving ICARS scores.

Clinicians require scientifically rigorous, clinically meaningful rating scales to evaluate the health impact of disease and treatment that cannot be measured using conventional laboratory instruments. This study evaluated the psychometric properties of the International Cooperative Ataxia Rating Scale (ICARS), a commonly used clinician-rated measure, in Friedreich's ataxia (FRDA). People with confirmed FRDA were assessed by using the ICARS. Two assumptions of its measurement model were tested: the legitimacy of reporting ICARS scores in FRDA, and the acceptability, reliability, and validity of total and subscale scores. Seventy-seven people with FRDA were assessed. The ICARS total score effectively satisfied all psychometric criteria tested. The posture and gait disturbances subscale also performed well. The other three subscales did not pass standard criteria for tests of scaling assumptions, reliability, and validity. This small study recommends only the use of the ICARS total score as a measure of FRDA. However, the extent to which this score quantifies the true extent of FRDA remains uncertain as our validity testing was limited, partly by the lack of appropriate validating measures. Further validity testing, and examination of responsiveness, is required before the ICARS can be recommended as an outcome measure for treatment trials of FDRA.

Crisis management (prevention, preparedness, response, and reconstruction) is a tough task for political and bureaucratic leaders. This article documents the persistent tensions between the expectations and realities of crisis leadership. It explores the popular notion that crises provide key opportunities for reform. The very occurrence of a crisis is then thought to expose the status quo as problematic, making it easier to gain momentum for alternative policies and institutions. We argue that the opportunities for reform in the wake of crisis are smaller than often thought. The prime reason is that the requisites of crisis leadership are at odds with the requirements of effective reform.

Friedreich's ataxia (FA) is the most common form of autosomal recessive spinocerebellar ataxia and is often associated with a cardiomyopathy. The disease is caused by an expanded intronic GAA repeat, which results in deficiency of a mitochondrial protein called frataxin. In the yeast YFH1 knockout model of the disease there is evidence that frataxin deficiency leads to a severe defect of mitochondrial respiration, intramitochondrial iron accumulation, and associated production of oxygen free radicals. Recently, the analysis of FA cardiac and skeletal muscle samples and in vivo phosphorus magnetic resonance spectroscopy (31P-MRS) has confirmed the deficits of respiratory chain complexes in these tissues. The role of oxidative stress in FA is further supported by the accumulation of iron and decreased aconitase activities in cardiac muscle. We used 31P-MRS to evaluate the effect of 6 months of antioxidant treatment (Coenzyme Q10 400 mg/day, vitamin E 2,100 IU/day) on cardiac and calf muscle energy metabolism in 10 FA patients. After only 3 months of treatment, the cardiac phosphocreatine to ATP ratio showed a mean relative increase to 178% (p = 0.03) and the maximum rate of skeletal muscle mitochondrial ATP production increased to 139% (p = 0.01) of their respective baseline values in the FA patients. These improvements, greater in prehypertrophic hearts and in the muscle of patients with longer GAA repeats, were sustained after 6 months of therapy. The neurological and echocardiographic evaluations did not show any consistent benefits of the therapy after 6 months. This study demonstrates partial reversal of a surrogate biochemical marker in FA with antioxidant therapy and supports the evaluation of such therapy as a disease-modifying strategy in this neurodegenerative disorder.

Huntington's disease is a progressive neurodegenerative disease caused by an abnormally expanded (>36) CAG repeat within the ITI5 gene encoding a widely expressed 349-kd protein, huntingtin. The medium spiny neurons of the caudate preferentially degenerate in Huntington's disease, with the presence of neuronal intranuclear inclusions. Excitotoxicity is thought to be important in the pathogenesis of Huntington's disease; the recently described mitochondrial respiratory chain and aconitase defects in Huntington's disease brain are consistent with this hypothesis. A transgenic mouse model (R6/2) of Huntington's disease develops a movement disorder, muscle wasting, and premature death at about 14 to 16 weeks. Selective neuronal death in these mice is not seen until 14 weeks. Biochemical analysis of R6/2 mouse brain at 12 weeks demonstrated a significant reduction in aconitase and mitochondrial complex IV activities in the striatum and a decrease in complex IV activity in the cerebral cortex. Increased immunostaining for inducible nitric oxide synthase and nitrotyrosine was seen in the transgenic mouse model but not control mouse brains. These results extend the parallels between Huntington's disease and the transgenic mouse model to biochemical events and suggest complex IV deficiency and elevated nitric oxide and superoxide radical generation precede neuronal death in the R6/2 mouse and contribute to pathogenesis.

Books reviewed in this essay: K.J. Bade and M. Weiner (Eds), Migration Past, Migration Future: Germany and the United States R. Munz and M. Weiner (Eds), Migrants, Refugees, and Foreign Policy: U.S. and German Policies Towards Countries of Origin K. Hailbronner and D.A. Martin (Eds), Immigration Admissions: The Search for Workable Policies in Germany and the United States K. Hailbronner, D.A. Martin and H. Motomura (Eds), Immigration Controls: The Search for Workable Policies in Germany and the United States P. Schuck and R. Munz (Eds), Paths to Inclusion: The Integration of Migrants in the United States and Germany

Bureaucratic politics—for example, the “political” interaction between individuals and organizations in the executive branch of government—is a potentially significant force affecting the course and outcomes of governmental policymaking processes. In this article, an empirical and normative model and operationalization of bureaucratic politics is developed, treating it as a variable rather than as an immutable (and by definition problematic) property of policy decision-making. A conceptual model, linking the nature and intensity of bureaucratic politics in advisory systems to the characteristics of the political leaders served by them, is also developed. On the basis of earlier studies of presidential leadership (e.g., Preston, 1996), the leader's need for power, cognitive complexity, and prior policy experience/expertise are hypothesized to affect the organization and operation of the advisory system in terms of bureaucratic politics. Using Hermann's (1983) Personality Assessment-at-a-Distance (PAD) coding technique, theoretical assertions are illustrated in a detailed case study of two critical episodes in U.S. decision-making in the conduct of the Vietnam war in 1965 and 1968.

The understanding of mitochondrial biology and, subsquently, the role of mitochondrial pathology in human disease has increased exponentially over the past 30 years. As insight has increased, so attention has begun to shift to the possibilities for treating mitochondrially based disorders. There are a number of archetypal mitochondrial diseases, each associated with specific mitochondrial DNA mutations, deletions, or depletions. In addition there are a number of disorders, mainly neurodegenerative in nature, in which mitochondrial dysfunction appears to play a pivotal role. Mitochondrial structure and function are discussed. Treatment of the archetypal mitochondrial disorders and other neurogenerative conditions is reviewed, with specific emphasis on the prospects for neuroprotection. (C) 1998 Wiley-Liss, Inc.

This article describes and reflects upon practical experiences in using simulations to increase the crisis preparedness of policy makers and government agencies in the Netherlands. Basic issues in crisis simulation design are discussed in view of the various functions that simulations may perform in crisis management planning. A concrete example of simulation is provided to explain its design and modus operandi. The article concludes with a number of practical recommendations.

There is a systematic deflation in governmental rhetoric of the developments that call attention to the unequal distribution of goods and services and a systematic inflation of the forms of threat that legitimize and expand authority. The latter are defined as crises, the former as problems. As crises recur and problems persist, so does a governmental dramaturgy of coping. (Edelman, 1977: 49) This article proposes a more power-critical approach to the analysis of crisis management and, in this respect, explores the possible contribution of research on political and organizational symbolism. Viewed in terms of symbolic action, attention is drawn to the opportunity spaces that crises entail for policy makers and other crisis actors. To exploit these, it is important for decision elites to influence collective definitions of the situation in such a way as to highlight preferred courses of action and to selectively obscure alternative interpretations. Three types of symbolic ‘crisis handling devices’ (framing, ritualization and masking) are presented and illustrated. In conclusion, the need for a broader perspective on the nature of the politics of crisis management is emphasised.

Bureau-political tensions and competition are an often neglected, yet crucial element in crisis management. Bureau-politics in crisis management runs counter to pervasive notions that portray centralization and concentration of power as the dominant mode of administrative response to crisis. This article presents empirical evidence suggesting the importance of bureau-politics in the planning, response and post-crisis stages of crisis management at both strategic and operational levels of action. Again contrary to conventional wisdom, it is argued that such interagency tensions may fulfill various positive functions: they put crisis agencies to the test; they serve to counteract ‘groupthink tendencies; they foster a certain degree of openness; and they may facilitate democratic control of far-reaching crisis management policies.