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Primary mitochondrial disease is a highly heterogeneous but collectively common inherited metabolic disorder, affecting at least one in 4300 individuals. Therapeutic management of mitochondrial disease typically involves empiric prescription of enzymatic cofactors, antioxidants, and amino acid and other nutrient supplements, based on biochemical reasoning, historical experience, and consensus expert opinion. As the field continues to rapidly advance, we review here the preclinical and clinical evidence, and specific dosing guidelines, for common mitochondrial medicine therapies to guide practitioners in their prescribing practices.

Since publication of Mitochondrial Medicine Society guidelines for mitochondrial medicine therapies management in 2009, data has emerged to support consideration for using additional therapeutic agents and discontinuation of several previously used agents. Preclinical animal modeling data have indicated a lack of efficacy for vitamin C as an antioxidant for primary mitochondriaetabolic function to improve cellular resiliency and slow clinical disease progression and/or prevent acute decompensation.

We provide a comprehensive update on mitochondrial medicine therapies based on current evidence and our single-center clinical experience to support or refute their use, and provide detailed dosing guidelines, for the clinical management of mitochondrial disease. The overarching goal of empiric mitochondrial medicines is to utilize therapies with favorable benefit-to-risk profiles that may stabilize and enhance residual metabolic function to improve cellular resiliency and slow clinical disease progression and/or prevent acute decompensation.

The purpose of this review is to describe ethical and legal issues that arise in the management of patients with disorders of consciousness ranging from the minimally conscious state to the coma state, as well as brain death.

The recent literature highlights dilemmas created by diagnostic and prognostic uncertainties in patients with disorders of consciousness. The discussion also reveals the challenges experienced by the disability community, which includes individuals with severe brain injury who are classified as having a disorder of consciousness. We review current guidelines for management of patients with disorders of consciousness including discussions around diagnosis, prognosis, consideration of neuropalliation, and decisions around life sustaining medical treatment.

In the setting of uncertainty, this review describes the utility of applying a disability rights perspective and shared decision-making process to approach medical decision-making for patients with disorders of consciousness. Selleck AM580 We outline approaches to identifying surrogate decision makers, standards for decision-making and decision-making processes, specifically addressing the concept of futility as a less useful framework for making decisions. We also highlight special considerations for research, innovative and controversial care, brain death, organ donation, and child abuse and neglect.

In the setting of uncertainty, this review describes the utility of applying a disability rights perspective and shared decision-making process to approach medical decision-making for patients with disorders of consciousness. We outline approaches to identifying surrogate decision makers, standards for decision-making and decision-making processes, specifically addressing the concept of futility as a less useful framework for making decisions. We also highlight special considerations for research, innovative and controversial care, brain death, organ donation, and child abuse and neglect.An augmented reality (AR) platform combines several technologies in a system that can render individual "digital objects" that can be manipulated for a given purpose. In the audio domain, these may, for example, be generated by speaker separation, noise suppression, and signal enhancement. Access to the "digital objects" could be used to augment auditory objects that the user wants to hear better. Such AR platforms in conjunction with traditional hearing aids may contribute to closing the gap for people with hearing loss through multimodal sensor integration, leveraging extensive current artificial intelligence research, and machine-learning frameworks. This could take the form of an attention-driven signal enhancement and noise suppression platform, together with context awareness, which would improve the interpersonal communication experience in complex real-life situations. In that sense, an AR platform could serve as a frontend to current and future hearing solutions. The AR device would enhance the signals to be attended, but the hearing amplification would still be handled by hearing aids. In this article, suggestions are made about why AR platforms may offer ideal affordances to compensate for hearing loss, and how research-focused AR platforms could help toward better understanding of the role of hearing in everyday life.A range of new technologies have the potential to help people, whether traditionally considered hearing impaired or not. These technologies include more sophisticated personal sound amplification products, as well as real-time speech enhancement and speech recognition. They can improve user's communication abilities, but these new approaches require new ways to describe their success and allow engineers to optimize their properties. Speech recognition systems are often optimized using the word-error rate, but when the results are presented in real time, user interface issues become a lot more important than conventional measures of auditory performance. For example, there is a tradeoff between minimizing recognition time (latency) by quickly displaying results versus disturbing the user's cognitive flow by rewriting the results on the screen when the recognizer later needs to change its decisions. This article describes current, new, and future directions for helping billions of people with their hearing. Theases, the technologies turn the hearing assistance problem on its head, and thus require new ways to measure their performance.

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