Molecular Pathogenesis of Cholestasis

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Publisher : Springer Science & Business Media
ISBN 13 : 9780306482403
Total Pages : 388 pages
Book Rating : 4.4/5 (824 download)

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Book Synopsis Molecular Pathogenesis of Cholestasis by : Michael Trauner

Download or read book Molecular Pathogenesis of Cholestasis written by Michael Trauner and published by Springer Science & Business Media. This book was released on 2004 with total page 388 pages. Available in PDF, EPUB and Kindle. Book excerpt: Knowledge of hepatobiliary transport is increasing rapidly. This book provides a cutting-edge overview of hepatobiliary transport and the molecular pathogenesis of cholestasis. Topics range from basic mechanisms of transport and regulation to general molecular and cellular concepts of cholestatic liver injury to specific molecular mechanisms of hereditary and acquired cholestatic liver injury, their complications and treatment. Basic researchers, academic physicians and students in hepatology, genetics, molecular and cell biology, pharmacology, pathology, gastroenterology and endocrinology will find this book instructive and stimulating.

The Liver

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Publisher : John Wiley & Sons
ISBN 13 : 1119436826
Total Pages : 1156 pages
Book Rating : 4.1/5 (194 download)

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Book Synopsis The Liver by : Irwin M. Arias

Download or read book The Liver written by Irwin M. Arias and published by John Wiley & Sons. This book was released on 2020-03-09 with total page 1156 pages. Available in PDF, EPUB and Kindle. Book excerpt: Bridging the gap between basic scientific advances and the understanding of liver disease — the extensively revised new edition of the premier text in the field. The latest edition of The Liver: Biology and Pathobiology remains a definitive volume in the field of hepatology, relating advances in biomedical sciences and engineering to understanding of liver structure, function, and disease pathology and treatment. Contributions from leading researchers examine the cell biology of the liver, the pathobiology of liver disease, the liver’s growth, regeneration, metabolic functions, and more. Now in its sixth edition, this classic text has been exhaustively revised to reflect new discoveries in biology and their influence on diagnosing, managing, and preventing liver disease. Seventy new chapters — including substantial original sections on liver cancer and groundbreaking advances that will have significant impact on hepatology — provide comprehensive, fully up-to-date coverage of both the current state and future direction of hepatology. Topics include liver RNA structure and function, gene editing, single-cell and single-molecule genomic analyses, the molecular biology of hepatitis, drug interactions and engineered drug design, and liver disease mechanisms and therapies. Edited by globally-recognized experts in the field, this authoritative volume: Relates molecular physiology to understanding disease pathology and treatment Links the science and pathology of the liver to practical clinical applications Features 16 new “Horizons” chapters that explore new and emerging science and technology Includes plentiful full-color illustrations and figures The Liver: Biology and Pathobiology, Sixth Edition is an indispensable resource for practicing and trainee hepatologists, gastroenterologists, hepatobiliary and liver transplant surgeons, and researchers and scientists in areas including hepatology, cell and molecular biology, virology, and drug metabolism.

Liver Disease in Children

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Author :
Publisher : Cambridge University Press
ISBN 13 : 1108911374
Total Pages : 875 pages
Book Rating : 4.1/5 (89 download)

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Book Synopsis Liver Disease in Children by : Frederick J. Suchy

Download or read book Liver Disease in Children written by Frederick J. Suchy and published by Cambridge University Press. This book was released on 2021-03-18 with total page 875 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.

Cholestatic Liver Disease

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Publisher : Springer
ISBN 13 : 1493910132
Total Pages : 265 pages
Book Rating : 4.4/5 (939 download)

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Book Synopsis Cholestatic Liver Disease by : Elizabeth J. Carey

Download or read book Cholestatic Liver Disease written by Elizabeth J. Carey and published by Springer. This book was released on 2014-07-18 with total page 265 pages. Available in PDF, EPUB and Kindle. Book excerpt: Since the publication of the first edition, there have been advances in both the diagnosis and the management of many of the cholestatic liver diseases. Cholestatic Liver Disease, Second Edition thoroughly updates the topics previously addressed, such as primary biliary cirrhosis, primary sclerosing cholangitis and cholestatic variants of drug hepatotoxicity and viral disease. New treatments, such as the development of the farnesoid X receptor agonists for the treatment of PBC, are highlighted. Current guidelines and areas of uncertainty are also covered. Additionally, new chapters have been added to reflect the changing landscape of cholestatic liver disease. Cholestatic Liver Disease, Second Edition is a concise yet comprehensive summary of the current status of the field and is of value to clinicians and researchers interested in patients with cholestatic liver disease provide that will help to guide patient management and stimulate investigative efforts.

Fat Absorption

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Publisher : CRC Press
ISBN 13 : 1351088890
Total Pages : 582 pages
Book Rating : 4.3/5 (51 download)

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Book Synopsis Fat Absorption by : Arnis Kuksis

Download or read book Fat Absorption written by Arnis Kuksis and published by CRC Press. This book was released on 2018-01-18 with total page 582 pages. Available in PDF, EPUB and Kindle. Book excerpt: This book provides an authoritative and comprehensive source of information on the biochemical an metabolic aspects of digestion and absorption of different dietary fats and other lipids, with minimal discussion of the physical chemistry of the process, which has been covered in great detail in previous reviews. It is intended for both researchers and practitioners in the biomedical field who require detailed knowledge of the biomedical and metabolic transformations involed in the intestinal digestion and resynthesis of dietary fats and other lipids.

Itch

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Publisher : CRC Press
ISBN 13 : 1466505435
Total Pages : 482 pages
Book Rating : 4.4/5 (665 download)

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Book Synopsis Itch by : E. Carstens

Download or read book Itch written by E. Carstens and published by CRC Press. This book was released on 2014-02-25 with total page 482 pages. Available in PDF, EPUB and Kindle. Book excerpt: Advances in itch research have elucidated differences between itch and pain but have also blurred the distinction between them. There is a long debate about how somatic sensations including touch, pain, itch, and temperature sensitivity are encoded by the nervous system. Research suggests that each sensory modality is processed along a fixed, direct-line communication system from the skin to the brain. Itch: Mechanisms and Treatment presents a timely update on all aspects of itch research and the clinical treatment of itch that accompanies many dermatological conditions including psoriasis, neuropathic itch, cutaneous t-cells lymphomas, and systemic diseases such as kidney and liver disease and cancer. Composed of contributions from distinguished researchers around the world, the book explores topics such as: Neuropathic itch Peripheral neuronal mechanism of itch The role of PAR-2 in neuroimmune communication and itch Mrgprs as itch receptors The role of interleukin-31 and oncostatin M in itch and neuroimmune communication Spinal coding of itch and pain Spinal microcircuits and the regulation of itch Examining new findings on cellular and molecular mechanisms, the book is a compendium of the most current research on itch, its prevalence in society, and the problems associated with treatment.

Osteosarcopenia: Bone, Muscle and Fat Interactions

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Author :
Publisher : Springer Nature
ISBN 13 : 3030258904
Total Pages : 383 pages
Book Rating : 4.0/5 (32 download)

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Book Synopsis Osteosarcopenia: Bone, Muscle and Fat Interactions by : Gustavo Duque

Download or read book Osteosarcopenia: Bone, Muscle and Fat Interactions written by Gustavo Duque and published by Springer Nature. This book was released on 2019-11-06 with total page 383 pages. Available in PDF, EPUB and Kindle. Book excerpt: This edited work presents the most current evidence on osteosarcopenia from bench to bedside, which is expected to facilitate the understanding of this syndrome and to develop preventive and therapeutic strategies. With our aging population, chronic diseases such as osteoporosis and sarcopenia are becoming highly prevalent. Fortunately, our understanding of the bone and muscle interactions has increased in recent years. This has allowed to the coining of the term osteosarcopenia to describe a syndrome in which these two diseases overlap. This overlap between osteoporosis and sarcopenia has major negative effects not only on our older adults but also on health systems worldwide. Readers will find a highly translational approach that starts with a summary of recent discoveries on stem cells biology, muscle and bone interactions – including the role of local bone and muscle fat – followed by comprehensive reviews on myokines (i.e. myostatin), osteokines (i.e. osteocalcin) and adipokines (i.e. interleukins) as major players and determinants of bone and muscle loss with aging. In addition, the role of sex steroids (i.e. estrogens, androgens), and calciotropic hormones (i.e. parathyroid hormone, vitamin D) in the pathogenesis of this syndrome is also reviewed. Moreover, using practical diagnostic and therapeutic tips, this book summarizes the clinical characteristics of osteosarcopenic patients thus facilitating the diagnosis and treatment of this syndrome in clinical practice. Finally, the book presents the case for the Falls and Fractures Clinic as the optimal model of care for this syndrome, aimed to avoid fragmentation and optimize osteosarcopenia care, and simultaneously prevent falls and fractures in older persons. This book offers relevant information on the mechanisms of osteosarcopenia, and a practical guide on how to identify and treat this geriatric syndrome and its adverse outcomes, which are dramatically affecting our aging population. The work is written by leaders in the field and is especially suited not only to any researcher in the musculoskeletal arena but also to medical specialists and allied health professionals involved in the care of older persons.

Molecular Pathology of Liver Diseases

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Publisher : Springer Science & Business Media
ISBN 13 : 1441971076
Total Pages : 920 pages
Book Rating : 4.4/5 (419 download)

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Book Synopsis Molecular Pathology of Liver Diseases by : Satdarshan P. S. Monga

Download or read book Molecular Pathology of Liver Diseases written by Satdarshan P. S. Monga and published by Springer Science & Business Media. This book was released on 2010-12-14 with total page 920 pages. Available in PDF, EPUB and Kindle. Book excerpt: Cellular and Molecular Pathology of the Liver is extensive, complex and ranges from the understanding the basic molecular mechanisms that dictate everything from liver homeostasis to liver disease. Molecular Pathology of the liver is complicated due to some of the important functions inherent and unique to the Liver, including its innate ability to regenerate and the multitude of functions it plays for the wellbeing of an organism. With all this in mind, Molecular Pathology of Liver Diseases is organized in different sections, which will coherently and cohesively present the molecular basis of hepatic physiology and pathology. The first two sections are key to understanding the liver anatomy and physiology at a cellular level and go on to define the molecular mechanics in various liver cell types. These sections also cover the existing paradigms in liver development, regeneration and growth. The next section is key to understanding the Molecular Pathology unique to liver diseases and associated phenotypes. The final sections are geared towards the existing knowledge of the molecular basis of many common and uncommon liver diseases in both neoplastic and non-neoplastic areas including pathologies associated with intra-hepatic and extra-hepatic biliary tree. Thus, this textbook is a one-stop reference for comprehending the molecular mechanisms of hepatic pathobiology. It is clearly unique in its format, readability and information and thus will be an asset to many in the field of Pathology and other disciplines.

Inborn Errors of Development

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Publisher : Oxford University Press, USA
ISBN 13 : 019514502X
Total Pages : 1110 pages
Book Rating : 4.1/5 (951 download)

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Book Synopsis Inborn Errors of Development by : Charles J. Epstein

Download or read book Inborn Errors of Development written by Charles J. Epstein and published by Oxford University Press, USA. This book was released on 2004 with total page 1110 pages. Available in PDF, EPUB and Kindle. Book excerpt: In this book, the clinical chapters are organized into sections by defined developmental pathways or gene families, and each section is preceded by a general overview. For each disorder the authors cover the disease-causing genes, the role of these genes in development as elucidated in model organisms, the human mutations that have been identified, and the developmental pathogenesis of the condition. Clinical descriptions, along with discussions of therapy and counseling, are provided. This book will be an invaluable resource for physicians, dentists, and other health professionals and for basic scientists interested in developmental processes and genetic perturbations that affect them.

Sherlock's Diseases of the Liver and Biliary System

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Author :
Publisher : John Wiley & Sons
ISBN 13 : 1119237548
Total Pages : 832 pages
Book Rating : 4.1/5 (192 download)

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Book Synopsis Sherlock's Diseases of the Liver and Biliary System by : James S. Dooley

Download or read book Sherlock's Diseases of the Liver and Biliary System written by James S. Dooley and published by John Wiley & Sons. This book was released on 2018-08-06 with total page 832 pages. Available in PDF, EPUB and Kindle. Book excerpt: A new, fully updated edition of the world’s most famous book on liver diseases—with updating of all areas and inclusion of new specific topics, by internationally renowned specialists This brand new edition of the classic book on hepatology provides a concise, clearly presented and well-structured review across the whole spectrum of hepatobiliary diseases by some of the world’s leading hepatologists and hepatobiliary specialists. Where many other hepatology textbooks provide detailed accounts of basic science and clinical management, Sherlock's Diseases of the Liver and Biliary System, 13th Edition takes a different approach. Concentrating on the clinical decisions to be taken and the relevant supporting data, it is written and edited to maintain Sheila Sherlock's unique approach, in particular the clarity and layout of the text, and the explanatory figures and tables. The book is thus concise, highly accessible, and generously illustrated with over 700 attractive color figures. There is a pithy approach to each disease based both on evidence and on the authors’ experience, the hallmark of this book. Based on these elements, the 12th edition was awarded first prize in the 2012 British Medical Association Book Awards in the Internal Medicine category. Sherlock's Diseases of the Liver and Biliary System begins by introducing the anatomy and function of the liver to readers, continuing then with in-depth coverage of liver biopsy techniques and interpretation, and fibrogenesis and its assessment. There are then chapters on all aspects of liver and biliary disease including acute liver failure, cirrhosis, portal hypertension, hepatic encephalopathy, ascites, hepatitis B and C, alcohol and the liver, non-alcoholic fatty liver disease, drug related liver reactions, cholestatic, autoimmune and genetic liver diseases, benign and malignant tumours and not least liver transplantation. There are also chapters on the liver in pregnancy, in the neonate, infancy and childhood, in systemic diseases and in infections. This new edition also features four new individual chapters focusing on coagulation, non-invasive assessment of fibrosis and cirrhosis; vascular diseases of the liver and portal vein thrombosis, and nutrition in liver disease. Digital downloads of the figures from this edition are offered on a companion website. Internationally recognized and loved, world-renowned hepatology book, first published in 1955 Takes a one-of-a-kind, clinical approach maintaining Sheila Sherlock’s clarity and legacy of presentation Full colour throughout with 700 illustrative figures Wide faculty of international contributors Sherlock's Diseases of the Liver and Biliary System, 13th Edition is an ideal primer in hepatology for students and trainees in hepatology and gastroenterology, and a valuable resource for all specialist gastroenterologists and hepatologists, paediatricians, pathologists, radiologists, general physicians and specialist nurses.

Primary Biliary Cirrhosis

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Publisher : Springer Science & Business Media
ISBN 13 : 9401148848
Total Pages : 187 pages
Book Rating : 4.4/5 (11 download)

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Book Synopsis Primary Biliary Cirrhosis by : Keith D. Lindor

Download or read book Primary Biliary Cirrhosis written by Keith D. Lindor and published by Springer Science & Business Media. This book was released on 2012-12-06 with total page 187 pages. Available in PDF, EPUB and Kindle. Book excerpt: The condition of prolonged obstructive jaundice with patent bile ducts was first described in 1851 by Addison and Gull of Guy's Hospital, London. The term primary biliary cirrhosis (PBC) was defined in 1950 by Ahrens and colleagues of the Rockefeller Institute, New York. The condition was considered rare but this changed in 1965 with the discovery of a definitive diagnostic serum mitochondrial antibody test and the recognition that a raised serum alkaline phosphatase value, often discovered incidentally, could be a diagnostic pointer. If the diagnosis is made earlier, the end stages are rarely reached as death is replaced by liver transplantation. On November 6th 1997, in Chicago, an International Faculty discussed in depth the clinical features, pathogenesis and treatment of PBC, no longer considered a rare disease. The course of PBC is long, but some 18 years after the discovery of a positive mitochondrial antibody test in a symptom free patient with normal serum biochemistry, 83% will have developed abnormal tests and 76% will be symptomatic. Identification of those who will progress rapidly is difficult. The serum antimitochondrial profile may be useful but this is a very specialist technique. Mathematical prognostic models are useful in therapeutic trials and in the selection and timing of patients for liver transplantation but have limited value in individual patients. An increasing serum bilirubin level remains the most important indicator of rapid progression. Its value however can be negated by the use of ursodeoxycholic acid which has a bilirubin-lowering effect.

Hepatobiliary Transport: From Bench to Bedside

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Publisher : Springer Science & Business Media
ISBN 13 : 9780792387718
Total Pages : 196 pages
Book Rating : 4.3/5 (877 download)

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Book Synopsis Hepatobiliary Transport: From Bench to Bedside by : S. Matern

Download or read book Hepatobiliary Transport: From Bench to Bedside written by S. Matern and published by Springer Science & Business Media. This book was released on 2001-12-31 with total page 196 pages. Available in PDF, EPUB and Kindle. Book excerpt: There has been a tremendous amount of scientific progress in our understanding of the molecular mechanisms of transport processes in the liver within the last few years. Cloning of various members of organic anion and cation transporters has provided the necessary tools to study their regulation under physiological and pathophysiological conditions and has advanced our knowledge about bile formation. Mutations of various hepatic organic anion transporters have been identified in humans as hereditary defects leading to the heterogenous syndrome of progressive familial intrahepatic cholestasis (PFIC). Various mouse models including knockout animals have given us the opportunity to gain insight into lipid transport by the liver and the genetics of cholesterol gallstone formation. The physiology of bile duct cells and the molecular mechanisms leading to various cholangiopathies have been a main scientific focus in hepatology in recent years. Drug targeting to the liver by hepatic organic anion transporters represents an attractive way of selective delivery of pharmaceutical agents in humans. Ursodeoxycholic acid is successfully used in the treatment of patients with chronic cholestatic liver disease and major advances have been made in understanding its mode of action in liver and bile duct cells. This book, the proceedings of the Falk Workshop held in Aachen, Germany, on 25-26 January 2001, contains chapters on all important aspects of biliary transport by well-known experts in this field. It is an essential resource for new developments in the field of biliary transport, both in basic science and clinical medicine.

Intrahepatic Cholangiocarcinoma

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Publisher : Springer
ISBN 13 : 9783030222604
Total Pages : 0 pages
Book Rating : 4.2/5 (226 download)

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Book Synopsis Intrahepatic Cholangiocarcinoma by : Timothy M. Pawlik

Download or read book Intrahepatic Cholangiocarcinoma written by Timothy M. Pawlik and published by Springer. This book was released on 2020-08-13 with total page 0 pages. Available in PDF, EPUB and Kindle. Book excerpt: This book provides a comprehensive review of the epidemiology, molecular pathogenesis, diagnosis and treatment of intrahepatic cholangiocarcinoma (ICC). It brings together an impressive group of international experts in cholangiocarcinoma research and clinical care. The book was organized and written to aid the clinician’s understanding of emerging research in cholangiocarcinoma and its application to the clinical care of patients with ICC. Each chapter details the scientific evidence to support clinical decisions that are needed to care for these complex patients. Focused chapters detail the epidemiology, diagnostic evaluation, as well as staging and prognosis of this disease. In addition to dedicated chapters on surgical management of ICC, a broad emphasis on locoregional therapies, including percutaneous ablation and transarterial therapies, is included. An up-to-date overview of the molecular pathogenesis and pathological assessment of ICC is detailed prior to chapters focusing on systemic chemotherapy and emerging novel therapy options. Intrahepatic Cholangiocarcinoma is not only an invaluable resource for many as they seek to provide the best multidisciplinary cancer care to patients with ICC, but also an opportunity to identify new avenues of scientific discovery that lead to significant advances in the diagnosis and management of ICC.

Pediatric Hepatology and Liver Transplantation

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Publisher : Springer
ISBN 13 : 3319964003
Total Pages : 829 pages
Book Rating : 4.3/5 (199 download)

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Book Synopsis Pediatric Hepatology and Liver Transplantation by : Lorenzo D'Antiga

Download or read book Pediatric Hepatology and Liver Transplantation written by Lorenzo D'Antiga and published by Springer. This book was released on 2019-04-29 with total page 829 pages. Available in PDF, EPUB and Kindle. Book excerpt: This book is the first to provide balanced examination of both pediatric liver disease and liver transplantation – two topics that are inherently related, given that most chronic liver disorders eventually require organ replacement. The different forms of liver disease encountered in the pediatric age group are first discussed in a series of disease-specific chapters that have a reader-friendly, uniform structure covering pathophysiology, diagnostic and treatment algorithms, clinical cases, and transition to adult care. Key topics in the field of liver transplantation are then addressed. Examples include indications and contraindications, surgical techniques and complications, immunosuppression, in pediatric liver transplantation, acute and chronic rejection and allograft dysfunction, and CMV and EBV infection in transplant recipients, long-term graft injury and tolerance. A section on pediatric hepatology across the world includes chapters presenting the features and management of pediatric liver disease in South-America, Africa and Asia. A closing section considers what the future holds for pediatric liver disease and its management, including novel genetic testing, cell therapy and gene therapy. Pediatric Hepatology and Liver Transplantation will be of value for a range of practitioners, from residents making their first approach to pediatric liver disease through to specialists working in transplantation centers.

Drug-Induced Liver Injury

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Author :
Publisher : Academic Press
ISBN 13 : 0128173173
Total Pages : 288 pages
Book Rating : 4.1/5 (281 download)

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Book Synopsis Drug-Induced Liver Injury by :

Download or read book Drug-Induced Liver Injury written by and published by Academic Press. This book was released on 2019-07-13 with total page 288 pages. Available in PDF, EPUB and Kindle. Book excerpt: Drug-Induced Liver Injury, Volume 85, the newest volume in the Advances in Pharmacology series, presents a variety of chapters from the best authors in the field. Chapters in this new release include Cell death mechanisms in DILI, Mitochondria in DILI, Primary hepatocytes and their cultures for the testing of drug-induced liver injury, MetaHeps an alternate approach to identify IDILI, Autophagy and DILI, Biomarkers and DILI, Regeneration and DILI, Drug-induced liver injury in obesity and nonalcoholic fatty liver disease, Mechanisms of Idiosyncratic Drug-Induced Liver Injury, the Evaluation and Treatment of Acetaminophen Toxicity, and much more. Includes the authority and expertise of leading contributors in pharmacology Presents the latest release in the Advances in Pharmacology series

Liver Pathophysiology

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Publisher : Academic Press
ISBN 13 : 0128043210
Total Pages : 914 pages
Book Rating : 4.1/5 (28 download)

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Book Synopsis Liver Pathophysiology by : Pablo Muriel

Download or read book Liver Pathophysiology written by Pablo Muriel and published by Academic Press. This book was released on 2017-03-02 with total page 914 pages. Available in PDF, EPUB and Kindle. Book excerpt: Liver Pathophysiology: Therapies and Antioxidants is a complete volume on morphology, physiology, biochemistry, molecular biology and treatment of liver diseases. It uses an integral approach towards the role of free radicals in the pathogenesis of hepatic injury, and how their deleterious effects may be abrogated by the use of antioxidants. Written by the most prominent authors in the field, this book will be of use to basic and clinical scientists and clinicians working in the biological sciences, especially those dedicated to the study and treatment of liver pathologies. Presents the most recent advances in hepatology, with a special focus on the role of oxidative stress in liver injury. Provides in vivo and in vitro models to study human liver pathology. Explains the beneficial effects of antioxidants on liver diseases. Contains the most recent and modern treatments of hepatic pathologies, including, but not limited to, stem cells repopulation, gene therapy and liver transplantation.

Metabolic Diseases

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Publisher : IOS Press
ISBN 13 : 1614997187
Total Pages : 960 pages
Book Rating : 4.6/5 (149 download)

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Book Synopsis Metabolic Diseases by : E. Gilbert-Barness

Download or read book Metabolic Diseases written by E. Gilbert-Barness and published by IOS Press. This book was released on 2017-01-06 with total page 960 pages. Available in PDF, EPUB and Kindle. Book excerpt: The 2nd Edition of Metabolic Diseases provides readers with a completely updated description of the Foundations of Clinical Management, Genetics, and Pathology. A distinguished group of 31 expert authors has contributed 25 chapters as a tribute to Enid Gilbert-Barness and the late Lewis Barness--- both pioneers in this topic. Enid’s unique perspectives on the pathology of genetic disorders and Lew’s unsurpassed knowledge of metabolism integrated with nutrition have inspired the contributors to write interdisciplinary descriptions of generally rare, and always challenging, hereditary metabolic disorders. Discussions of these interesting genetic disorders are organized in the perspective of molecular abnormalities leading to morphologic disturbances with distinct pathology and clinical manifestations. The book emphasizes recent advances such as development of improved diagnostic methods and discovery of new, more effective therapies for many of the diseases. It includes optimal strategies for diagnosis and information on access to specialized laboratories for specific testing. The target audience is a wide variety of clinicians, including pediatricians, neonatologists, obstetricians, maternal-fetal specialists, internists, pathologists, geneticists, and laboratorians engaged in prenatal and/or neonatal screening. In addition, all scientists and health science professionals interested in metabolic diseases will find the comprehensive, integrated chapters informative on the latest discoveries. It is our hope that the 2nd Edition will open new avenues and vistas for our readers and that they will share with us the interest, excitement and passion of the research into all these challenging disorders.