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Clinical and Translational Perspectives on Wilson Disease brings together the genetics, cell and structural biology of Wilson Disease into one contemporary, easy to navigate handbook. Created to meet the diverse needs of the clinical and research communities surrounding Wilson Disease, this reference provides a worldwide approach that is concise and translational. Specifically, it provides a basis for clinicians to appreciate ‘basic science’ aspects of Wilson disease, presenting a guide for researchers to understand the clinical disorder on which their research is focused and fostering constructive dialogue and progress for this puzzling disorder. Delivers numerous, succinct, expert chapters with summaries designed for quick reference Includes a 'How-to appendix' for diagnosis and management tips Contains access to a companion website with a self-help teaching module, links to key resources, and an extended reference list
Liver disease in children is increasing in prevalence, placing a huge burden on healthcare systems and often requiring long-term management. Offering an integrative approach to the science and clinical practice of pediatric hepatology, this is the definitive reference text for improved diagnosis and treatment strategies. In the new edition of this authoritative text, chapters have been thoroughly revised in line with major advances in the field, such as recognizing the increased frequency of fatty liver disease, and how genetic testing has the potential to establish earlier diagnoses for a variety of diseases. Disorders covered include cholestasis, metabolic disorders and hepatitis, with their presentation across the spectrum of infancy, childhood and adolescence discussed. The indications and surgical aspects of liver transplant are explained and post-transplant care is described in detail. This is a valuable resource for pediatricians, hepatologists, gastroenterologists and all clinicians involved in the care of children with liver diseases.
Neuroacanthocytosis Syndromes is the first comprehensive review of a field that has not yet received the attention it deserves. Affecting the brain as well as the circulating red cells, these multi-system disorders in the past had often been mistaken for Huntington's disease. Recent breakthroughs have now identified the molecular basis of several of these. This volume grew out of the first international scientific meeting ever devoted to neuroacanthocytosis and provides in-depth information about the state of the art. Its thirty chapters were written by the leading authorities in the field to cover the clinical as well as the basic science perspective, including not only molecular genetics but also experimental pharmacology and cell membrane biology, among others. The book vehemently poses the question of how the membrane deformation of circulating red blood cells relates to degeneration of nerve cells in the brain, the basal ganglia, in particular. It provides a wealth of data that will help to solve an intriguing puzzle and ease the suffering of those affected by one of the neuroacanthocytosis syndromes.
This pocket guide provides a concise yet comprehensive overview of the pathogenesis, treatment, and management of Wilson disease. Drawing upon expertise from the Center of Excellence at Yale University in liver disease, neurology, and psychiatry for adult and pediatric patients, as well as dieticians, clinical trial coordinators, representatives from the Wilson Disease Association and outside experts from the US and UK as well, this guide provides a balanced and easy to digest approach to understanding treatment and care for Wilson disease patients. The text reviews different treatment options and other care needed to address clinical symptoms, as well as the testing needed for monitoring efficacy and adherence. Furthermore, the volume discusses these issues in the context of a best care model, which is an integrative care model where patients and their caregivers partner with Centers of Excellence with expertise in this rare disorder, to achieve best long-term outcomes. Written by experts in the field, Management of Wilson Disease: A Pocket Guide is a valuable resource for clinicians and primary care physicians who treat patients with Wilson disease.
Wilson Disease: Pathogenesis, Molecular Mechanisms, Diagnosis, Treatment and Monitoring translates both clinical and experimental findings into a comprehensive approach for anyone involved in research and patient care. While the clinical variability of Wilson Disease poses a challenge from a diagnostic approach, the book uses the translational impact of new research findings to relate to new treatment concepts. Comprehensive chapters include common knowledge, guideline consensus statements, and discussions of clinical evidence. This is a must-have reference for researchers and clinicians in translational research. Delivers a substantial overview of evidence driven diagnostic pathways and treatment concepts for Wilson disease Provides an understanding of the translational impact of new research findings in Wilson Disease Reveals the current controversies in treatment decisions for Wilson disease, providing the best decisions for optimal patient care
Current Advances in Osteosarcoma edited by Dr. Eugenie S. Kleinerman summarizes molecular and genetic characteristics, new therapeutic ideas, and biological characteristics that have been uncovered in the past 10 years. Osteosarcoma is an aggressive malignant neoplasm and it is also the most common histological form of bone cancer. It accounts for approximately 56% of new bone tumors, making it the most primary malignant bone tumor in children and adolescents. The lungs are the most common site of metastases and once osteosarcoma spreads to the lungs, it is very difficult to treat. To improve the outcome of this disease, the biology of osteosarcoma needs to be better understood. There are numerous investigators around the world who have made seminal discoveries about the important molecular pathways and genetic alterations that contribute to the development and metastases of osteosarcoma. Other investigators have proposed novel therapeutic strategies including some based on the molecular and genetic phenotype of the disease. Current Advances in Osteosarcoma summarizes all of these new discoveries in one singular text, which will help move the field forward.
This report is the twelfth assessment of the National Institutes of Health National Research Service Awards program. The research training needs of the country in basic biomedical, clinical, and behavioral and social sciences are considered. Also included are the training needs of oral health, nursing, and health services research. The report has been broadly constructed to take into account the rapidly evolving national and international health care needs. The past and present are analyzed, and predictions with regard to future needs are presented.
Temporomandibular disorders (TMDs), are a set of more than 30 health disorders associated with both the temporomandibular joints and the muscles and tissues of the jaw. TMDs have a range of causes and often co-occur with a number of overlapping medical conditions, including headaches, fibromyalgia, back pain and irritable bowel syndrome. TMDs can be transient or long-lasting and may be associated with problems that range from an occasional click of the jaw to severe chronic pain involving the entire orofacial region. Everyday activities, including eating and talking, are often difficult for people with TMDs, and many of them suffer with severe chronic pain due to this condition. Common social activities that most people take for granted, such as smiling, laughing, and kissing, can become unbearable. This dysfunction and pain, and its associated suffering, take a terrible toll on affected individuals, their families, and their friends. Individuals with TMDs often feel stigmatized and invalidated in their experiences by their family, friends, and, often, the health care community. Misjudgments and a failure to understand the nature and depths of TMDs can have severe consequences - more pain and more suffering - for individuals, their families and our society. Temporomandibular Disorders: Priorities for Research and Care calls on a number of stakeholders - across medicine, dentistry, and other fields - to improve the health and well-being of individuals with a TMD. This report addresses the current state of knowledge regarding TMD research, education and training, safety and efficacy of clinical treatments of TMDs, and burden and costs associated with TMDs. The recommendations of Temporomandibular Disorders focus on the actions that many organizations and agencies should take to improve TMD research and care and improve the overall health and well-being of individuals with a TMD.
This book provides an excellent platform for understanding the chemical processes involved in food transformation. Starting with the examination of major food components, such as water, carbohydrates, lipids, proteins and minerals, the author further introduces the biochemistry of digestion and energy metabolism of food ingredients. The last section of the book is devoted to modern food technologies and their future perspectives.
Healthcare decision makers in search of reliable information that compares health interventions increasingly turn to systematic reviews for the best summary of the evidence. Systematic reviews identify, select, assess, and synthesize the findings of similar but separate studies, and can help clarify what is known and not known about the potential benefits and harms of drugs, devices, and other healthcare services. Systematic reviews can be helpful for clinicians who want to integrate research findings into their daily practices, for patients to make well-informed choices about their own care, for professional medical societies and other organizations that develop clinical practice guidelines. Too often systematic reviews are of uncertain or poor quality. There are no universally accepted standards for developing systematic reviews leading to variability in how conflicts of interest and biases are handled, how evidence is appraised, and the overall scientific rigor of the process. In Finding What Works in Health Care the Institute of Medicine (IOM) recommends 21 standards for developing high-quality systematic reviews of comparative effectiveness research. The standards address the entire systematic review process from the initial steps of formulating the topic and building the review team to producing a detailed final report that synthesizes what the evidence shows and where knowledge gaps remain. Finding What Works in Health Care also proposes a framework for improving the quality of the science underpinning systematic reviews. This book will serve as a vital resource for both sponsors and producers of systematic reviews of comparative effectiveness research.