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Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases
by K Michael Gibson Nenad Blau Marinus Duran Carlo Dionisi ViciThis book, combining and updating two previous editions, is a unique source of information on the diagnosis, treatment, and follow-up of metabolic diseases. The clinical and laboratory data characteristic of rare metabolic conditions can be bewildering for both clinicians and laboratory personnel. Reference laboratory data are scattered, and clinical descriptions may be obscure. The Physician's Guide documents the features of more than five hundred conditions, grouped according to type of disorder, organ system affected (e. g. liver, kidney, etc) or phenotype (e. g. neurological, hepatic, etc). Relevant clinical findings are provided and pathological values for diagnostic metabolites highlighted. Guidance on appropriate biochemical genetic testing is provided. Established experimental therapeutic protocols are described, with recommendations on follow-up and monitoring. The authors are acknowledged experts, and the book will be a valuable desk reference for all who deal with inherited metabolic diseases.
Structural Nanocomposites
by James NjugunaAdvancements in the nanotechnology industry promise to offer improvements in capabilities across a spectrum of applications. This is of immense strategic importance to the high performance sector which has historically leveraged technological advances. The use of polymer nanocomposites in structures have several predictable impacts on structural design and applications, primarily by providing a safer, faster, and eventually cheaper transportation in the future. This books comprises reviews on the state-of-the-art, new research results, perspectives of future developments, and innovative applications relevant to structural materials, engineering structures, nanocomposites, modelling and simulations, and their related application areas.
Medizinprodukte in der Anwendung: Alle machen mit, keiner haftet?
by Springer New York Arbeitsgemeinschaft Afghanistan StaffMedizinprodukte kommen in der medizinischen Praxis immer häufiger zur Anwendung. Aufgrund der sehr komplexen Haftungsgrundlage des Medizinprodukterechtes wird es immer undurchsichtiger, wer im Falle eines Schadens zur Haftung zu ziehen ist. Um das komplexe System des Medizinprodukterechtes aus haftungsrechtlicher Sicht etwas durchschaubarer zu machen, haben sich die Autoren von verschiedensten Seiten dem Medizinprodukterecht genähert. Die rechtlichen Grundlagen des Medizinprodukterechtes werden ebenso wie die Grundlagen zur Inanspruchnahme bei Medizinprodukten dargestellt. Zugleich werden auch prozessuale Besonderheiten hinsichtlich des Medizinprodukterechtes in einem haftungsrechtlichen Rechtsstreit ausgeführt.
Microperimetry and Multimodal Retinal Imaging
by Edoardo MidenaMicroperimetry allows precise functional examination of the retina and optic nerve and is especially valuable for evaluation of the macular area. This fully up-to-date textbook on microperimetry, written by leading experts in the field, will also serve as an atlas and diagnostic reference. It is designed for use by both ophthalmologists and optometrists in daily clinical practice and clinical research, and the contents thus range from basic technical information to the most advanced clinical applications. Past and current microperimetry techniques are reviewed, and the role of microperimetry in the context of multimodal imaging scenario is explained. The full range of clinical applications is then discussed, including age-related macular degeneration, diabetes, myopia, retinal dystrophies, disorders of the vitreoretinal interface, inflammatory chorioretinal diseases, toxic retinopathies, glaucoma, and low vision.
The History and Science of the Manhattan Project
by Bruce Cameron ReedThe development of atomic bombs under the auspices of the U. S. Army's Manhattan Project during World War II is considered to be the outstanding news story of the twentieth century. In this book, a physicist and expert on the history of the Project presents a comprehensive overview of this momentous achievement. The first three chapters cover the history of nuclear physics from the discovery of radioactivity to the discovery of fission, and would be ideal for instructors of a sophomore-level "Modern Physics" course. Student-level exercises at the ends of the chapters are accompanied by answers. Chapter 7 covers the physics of first-generation fission weapons at a similar level, again accompanied by exercises and answers. For the interested layman and for non-science students and instructors, the book includes extensive qualitative material on the history, organization, implementation, and results of the Manhattan Project and the Hiroshima and Nagasaki bombing missions. The reader also learns about the legacy of the Project as reflected in the current world stockpiles of nuclear weapons.
Radiology for PET/CT Reporting
by Stefano Fanti Cristina Nanni Lucia ZanoniReading PET/CT scans is sometimes challenging. Not infrequently, abnormal findings on CT images are functionally silent and therefore difficult for nuclear medicine practitioners to interpret. Furthermore, in general only a low-dose CT scan is produced as part of the combined PET/CT study, and the resulting CT images may prove suboptimal for image interpretation. This atlas is designed to enable nuclear medicine practitioners who routinely read PET/CT scans to recognize the most common CT abnormalities. Slice-by-slice descriptions are provided of anatomical structures as visualized on CT scans obtained in PET/CT studies. The CT findings that may be detected while reviewing PET/CT scans of various body regions and conditions are then illustrated and fully described. The concluding section of the book is devoted to the principal MRI findings in diseases which cannot be evaluated using PETs/CTs.
The Physiology of Characean Cells
by Mary J. Beilby Michelle T. CasanovaThis book describes the unique characean experimental system, which provides a simplified model for many aspects of the physiology, transport and electrophysiology of higher plants. The first chapter offers a thorough grounding in the morphology, taxonomy and ecology of Characeae plants. Research on characean detached cells in steady state is summarised in Chapter 2, and Chapter 3 covers characean detached cells subjected to calibrated and mostly abiotic types of stress: touch, wounding, voltage clamp to depolarised and hyperpolarised potential difference levels, osmotic and saline stress. Chapter 4 highlights cytoplasmic streaming, cell-to-cell transport, gravitropism, cell walls and the role of Characeae in phytoremediation. The book is intended for researchers and students using the characean system and will also serve as an invaluable reference resource for electrophysiologists working on higher plants.
Pathologies of Calcium Channels
by Norbert Weiss Alexandra KoschakCalcium ions represent Mother Nature's 'ion-of-choice' for regulating fundamental physiological functions, as they initiate a new life at the time of fertilization and guide subsequent developmental and physiological functions of the human body. Calcium channels, which act as gated pathways for the movement of calcium ions across the membranes, play a central part in the initiation of calcium signals, and defects in calcium channel function have been found to result in a plethora of human diseases, referred to as the calcium channelopathies. Pathologies of Calcium Channels brings together leading international experts to discuss our current understanding of human diseases associated with the various calcium channels, from their molecular basis to potential future therapeutic targeting of calcium channels.
Knowledge as a Driver of Regional Growth in the Russian Federation
by Jens Kai PerretThe Russian Federation has a history of more than twenty years of transformation to a market economy, but as well to a knowledge society, to look back on. This study takes a look at the knowledge generation, knowledge transmission and knowledge use inside the Federation since the early 1990s. Furthermore, in light of the high dependence of the Russian economy on the oil and gas sectors this study analyzes the impact knowledge related factors have on regional income generation following thereby in the direction of Schumpeterian growth theory. The study combines descriptive with empirical analyses to paint a picture as detailed as possible of the Russian knowledge society and its innovative potential.
Reforming the Common European Asylum System -- Legislative developments and judicial activism of the European Courts
by Samantha VellutiIn June 2013, after lengthy and complex negotiations the EU adopted the recast "asylum package" which represents a significant step forward in the future development of CEAS. In this timely study Velluti provides fresh insights into recent legislative and judicial developments in asylum and through the "lens" of sovereignty she looks at some of the contemporary challenges faced by the EU protection regime, with a particular focus on asylum-seekers' rights. The volume assesses whether the EU provides an adequate framework for protecting those seeking international protection from the opposing perspectives of effectiveness and fairness. It shows that, despite the newly adopted "second-generation" legislative acts which include changes aimed at ensuring a stronger level of protection for asylum-seekers, the reform process at European level does not adequately ensure an equal standard of protection across all Member States. Through a comparative analysis of selected ECtHR and ECJ asylum cases the book also examines the constitutional relationship between the two European Courts and how it impacts on the human rights of asylum-seekers and on the future of EU asylum law. Ultimately, the book shows that real progress in the development of the human rights dimension of CEAS will be achieved largely through the European and domestic courts.
Electrocaloric Materials
by Tatiana Correia Qi ZhangSince the 1997 Kyoto protocol of reduction of greenhouse gas emissions, the development of novel refrigerators has been a priority within the scientific community. Although magnetocaloric materials are promising candidates, they still need a large magnetic field to induce a giant ΔT as well as powerful and costly magnets. However, in electrocaloric materials (ECMs) a temperature change may be achieved by applying or removing an electric field. Since a giant electrocaloric effect on ferroelectric thin films was reported in Science in 2006, researchers have been inspired to explore such effect in different ferroelectric thin films. This book reviews electrocaloric effects observed in bulk materials as well as recent promising advances in thin films, with special emphasis on the ferroelectric, antiferroelectric and relaxor nature of ECMs. It reports a number of considerations about the future of ECMs as a means of achieving an efficient, ecologically sustainable and low cost refrigerator.
Public Procurement, Innovation and Policy
by Veiko Lember Rainer Kattel Tarmo KalvetThis book maps the latest developments in public procurement of innovation policy in various contexts and analyzes the evolution and development of the various policy solutions in broader institutional contexts. In doing so, it addresses significant theoretical and practical gaps: On the one hand, there is an emerging interest in public procurement as a policy tool for spurring innovation; yet on the other hand, the current theory, with some notable exceptions, is guided and often constrained by historical applications, above all in the defence industries. By carefully examining the cases of eleven countries, the book points to the existence of much more nuanced public procurement on the innovation policy landscape than has been acknowledged in the academic and policy debates to date.
Soft Computing for Image and Multimedia Data Processing
by Ujjwal Maulik Siddhartha BhattacharyyaProper analysis of image and multimedia data requires efficient extraction and segmentation techniques. Among the many computational intelligence approaches, the soft computing paradigm is best equipped with several tools and techniques that incorporate intelligent concepts and principles. This book is dedicated to object extraction, image segmentation, and edge detection using soft computing techniques with extensive real-life application to image and multimedia data. The authors start with a comprehensive tutorial on the basics of brain structure and learning, and then the key soft computing techniques, including evolutionary computation, neural networks, fuzzy sets and fuzzy logic, and rough sets. They then present seven chapters that detail the application of representative techniques to complex image processing tasks such as image recognition, lighting control, target tracking, object extraction, and edge detection. These chapters follow a structured approach with detailed explanations of the problems, solutions, results, and conclusions. This is both a standalone textbook for graduates in computer science, electrical engineering, system science, and information technology, and a reference for researchers and engineers engaged with pattern recognition, image processing, and soft computing.
Thymic Development and Selection of T Lymphocytes
by Thomas Boehm Yousuke TakahamaThe thymus is an evolutionarily ancient primary lymphoid organ common to all vertebrates in which T cell development takes place. Failing thymus function is associated with immunodeficiency and/or autoimmunity. In this volume, leading experts provide a comprehensive overview of recent advances in thymopoiesis research. The chapters cover the development of the thymic epithelial microenvironment, address the formation of a diverse and self-tolerant repertoire of T cell receptors as the basis for cellular immunity, discuss the mechanisms by which progenitor cells colonize the thymus and detail the molecular basis for T lineage decisions. The reviews illustrate the important role of the multifaceted process of thymopoiesis for adaptive immunity.
Electrodialysis and Water Reuse
by Andréa Moura Bernardes Marco Antônio Siqueira Rodrigues Jane Zoppas FerreiraThis book presents novel techniques to evaluate electrodialysis processes, to synthesize ionic membranes and to characterize their properties. It shows the potential use of membrane process to the treatment of effluents generated in many industrial sectors such as refineries, leather industries, mining and electroplating processes. The book is based on the results obtained by the author's research group during the past decade. It is useful for students, researchers and engineers interested in membrane technologies for water reuse.
Biosensors: Essentials (Lecture Notes in Chemistry #84)
by Gennady EvtugynToday, biosensors are broadly applied in research, clinical diagnosis and monitoring, as well as in pharmaceutical, environmental or food analysis. In this work, the author presents the essentials that advanced students and researchers need to know in order to make full use of this technology. This includes a description of biochemical recognition elements, such as enzymes, antibodies, aptamers or even whole cells. Various signal transducers such as electrochemical and optical transducers, luminescence devices and advanced techniques such as quartz crystal microbalances and MEMS systems are covered as well. Current applications are introduced through various case studies, rounded out by a forward-looking chapter on the prospects for biosensor development offered by nanotechnology, lab-on-a-chip, and biomimetic systems.
Pathogenesis of Mycobacterium tuberculosis and its Interaction with the Host Organism
by Jean Pieters John D. MckinneyMycobacterium tuberculosis is one of the most notorious pathogens on earth, causing the death of approximately 1. 5 million people annually. A major problem in the fight against tuberculosis is the emergence of strains that have acquired resistance to all available antibiotics. One key to the success of M. tuberculosis as a pathogen is its ability to circumvent host immune responses at different levels. This is not only a result of the special makeup of M. tuberculosis in terms of genetic diversity and DNA metabolism and its possession of specialized secretion systems, but also of its ability to hijack the host's innate immune defence mechanisms. In this volume, researchers from different disciplines provide a topical overview of the diverse mechanisms that contribute to the virulence of M. tuberculosis, ranging from their genetic, metabolic and molecular makeup, as well as the complex strategies these bacteria utilize to escape immune destruction within infected hosts.
Mut zu Innovationen
by Wolfgang Scholl Frank Schmelzer Sebastian Kunert Stephan Bedenk Jens Hüttner Julia Pullen Sandra TirreWie können Innovationsprojekte erfolgreicher gestaltet werden? Anspruch und Wirklichkeit des Innovationsmanagements liegen oft weit auseinander: Zwar werden zunehmend Innovationen begonnen, viele Projekte bleiben aber erfolglos. Untersuchungen in über 100 Unternehmen machen deutlich: Innovationen sind zu verschieden und zu komplex, um sie nach Checklisten und Ablaufplänen durchführen zu können. Es gibt jedoch flexible Formen der Zusammenarbeit und förderliche Organisationskulturen, mit denen sich die Erfolgsrate von Innovationen deutlich erhöhen lässt. Das vorliegende Buch stellt aus wissenschaftlicher und praktischer Perspektive Möglichkeiten zur Förderung von Innovationen vor: Identifikation von Treibern und Hemmnissen bei Innovationen Konkrete Fälle zeigen wie Unternehmen Innovationen verankern ,,Innovationspromotoren" können die Geschäftsleitung bei der Überwindung von Innovationshürden unterstützen Worauf Firmen beim Engagement von Beratern achten sollten
Nanophotonic Information Physics
by Makoto NaruseThis book provides a new direction in the field of nano-optics and nanophotonics from information and computing-related sciences and technology. Entitled by "Information Physics and Computing in NanosScale Photonics and Materials", IPCN in short, the book aims to bring together recent progresses in the intersection of nano-scale photonics, information, and enabling technologies. The topic will include (1) an overview of information physics in nanophotonics, (2) DNA self-assembled nanophotonic systems, (3) Functional molecular sensing, (4) Smart fold computing, an architecture for nanophotonics, (5) semiconductor nanowire and its photonic applications, (6) single photoelectron manipulation in imaging sensors, (6) hierarchical nanophotonic systems, (8) photonic neuromorphic computing, and (9) SAT solver and decision making based on nanophotonics.
Carl Adam Petri: Eine Biographie
by Einar SmithDer Autor präsentiert Leben, Werk und Wirken eines der weltweit bekanntesten deutschen Informatiker: Carl Adam Petri. Die Stadien seiner Entwicklung sind lebendig und kurzweilig beschrieben, gehen aber so weit in die Tiefe, dass der Leser ein gutes Verständnis für die Hintergründe von Petris wichtigstem Beitrag zur Informatik bekommt, die nach ihm benannten Petrinetze. Das Buch kann so als Einführung in die Petrinetze gelesen werden, erörtert aber auch die theoretischen, physikalischen und philosophischen Grundlagen der Netze, die erst einen umfassenden Zugang zu Petris Werk ermöglichen. Das Buch wendet sich dank seiner verständlichen Darstellung aber auch an Leser, die einfach nur eine bemerkenswerte Persönlichkeit der Zeitgeschichte kennenlernen möchten.
The Road to a Modern IT Factory
by Ferri AbolhassanIT is currently going through one of its most critical phases of transformation. IT vendors and IT service organizations are revolutionizing their production and service processes, adopting industrial practices. It is only through the consistent transformation into factory-like structures that quality, effectiveness and efficiency can be increased. By integrating professional concepts and methods taken from the context of industrial production, it is possible to meet functional and qualitative requirements from the departments and therefore from the end user. On top of that this new paradigm enables the implementation of optimal processes in the organization. An interdisciplinary team of authors addresses the current challenges for global IT services organizations and describes the process of IT industrialization. The transformation of the IT industry towards the model of an IT factory is the core theme of this book, which takes the latest findings from applied research, consulting and IT business practices and combines them into a consistent and innovative approach to IT services.
Methodological Cognitivism
by Riccardo VialeThis book deals with the cognitive foundation of the theory of social action. The social sciences are still guided by models of social action, far from the empirical reality of the psychology of action. While economics seems to have made greater progress in accepting the changes to the theory of action derived from cognitive science (see, for example, the 2002 Nobel prize for economics awarded to Daniel Kahneman), sociology is still being oriented on the dualism of hermeneutics vs. structuralism, which leaves very little room for a cognitive theory of social action. The unique features of the book are its combination of epistemology, philosophy of mind and cognitive science in order to renew and overcome the limits of the current methodologies of social science and in particular methodological individualism. Methodological cognitivism is proposed as an alternative to the holistic character of structuralism, to the intentionalist and rationalist features of methodological individualism, and to the relativistic character of hermeneutics and ethnomethodology.
Fundamentals of Software Engineering
by Farhad Arbab Marjan SirjaniThis book constitutes the proceedings of the 5th IPM International Conference on Fundamentals of Software Engineering, FSEN 2013, held in Tehran, Iran, in April 2013. The 17 full papers presented in this volume were carefully reviewed and selected from 65 submissions. The topics of interest in FSEN span over all aspects of formal methods, especially those related to advancing the application of formal methods in software industry and promoting their integration with practical engineering techniques.
Modeling of Land-Use and Ecological Dynamics
by Dan Malkinson Danny Czamanski Itzhak BenensonAs cities are rapidly expanding and encroaching into agricultural and natural areas, a question of primary concern is how this expansion affects surrounding agriculture and natural landscapes. This book presents a wide spectrum of both theoretical and empirical approaches to simulation and assessment of landscape dynamics. The first part presents state-of-the-art modelling approaches pertaining to land-use changes entailed by the urban sprawl, at different spatial resolutions and temporal time scales. The second part is dedicated to case studies of the effects and consequences of the emerging urban-agriculture open space patterns.
Discrete and Topological Models in Molecular Biology
by Nataša Jonoska Masahico SaitoTheoretical tools and insights from discrete mathematics, theoretical computer science, and topology now play essential roles in our understanding of vital biomolecular processes. The related methods are now employed in various fields of mathematical biology as instruments to "zoom in" on processes at a molecular level. This book contains expository chapters on how contemporary models from discrete mathematics - in domains such as algebra, combinatorics, and graph and knot theories - can provide perspective on biomolecular problems ranging from data analysis, molecular and gene arrangements and structures, and knotted DNA embeddings via spatial graph models to the dynamics and kinetics of molecular interactions. The contributing authors are among the leading scientists in this field and the book is a reference for researchers in mathematics and theoretical computer science who are engaged with modeling molecular and biological phenomena using discrete methods. It may also serve as a guide and supplement for graduate courses in mathematical biology or bioinformatics, introducing nontraditional aspects of mathematical biology.