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An algorithmic approach to interpreting renal pathology, updated in light of recent advances in understanding and new classification schemes.
This is a book about renal cysts and cystic kidneys. Its contributors have created a resource of current information in a field that once aroused only curiosity, but that now stands at the leading edge of molecular nephrology. Its authorship includes 'oldtimers', who bring the wisdom of experience, and 'newcomers', whose presence attests to the contributions made by the investigative and technological advances of the past decade. Its text is organized to carry the reader from renal cyst to cystic renal disease. Each of its chapters defines or explores a challenge or an advance. Cells that line renal cysts are diverse in structure, type, and perhaps function. The cysts themselves lie within an interstitium that is not normal and may influence cyst development and growth. Experimental analogs of human disease offer increasing opportunities to basic researchers to examine, in sequence and under controlled circumstances, those events that favor nephron dilation, cyst growth and ultimate renal failure.
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a highly prevalent hereditary renal disorder in which fluid-filled cysts are appeared in both kidneys. Main causative genes of ADPKD are PKD1 and PKD2, encoding for polycystin-1 (PC1) and polycystin-2 (PC2) respectively. Those proteins are localized on primary cilia and function as mechanosensor in response to the fluid flow, translating mechanistic stimuli into calcium signaling. With mutations either of PKD1 or PKD2, hyper-activated renal tubular epithelial cell proliferation is observed, followed by disrupted calcium homeostasis and aberrant intracellular cyclic AMP (cAMP) accumulation. Increased cell proliferation with fluid secretion leads to the development of thousands of epithelial-lined, fluid-filled cysts in kidneys. It is also accompanied by interstitial inflammation, fibrosis, and finally reaching end-stage renal disease (ESRD). In human ADPKD, the age at which renal failure typically occurs is later in life, however no specific targeted medications are available to cure ADPKD. Recently, potential therapeutic targets or surrogate diagnostic biomarkers for ADPKD are proposed with the advances in the understanding of ADPKD pathogenesis, and some of them were attempted for clinical trials. Herein, we will summarize genetic and epi-genetic molecular mechanisms in ADPKD progression, and overview the currently available biomarkers or potential therapeutic reagents suggested.
Here is an extensive update of Pediatric Nephrology, which has become the standard reference text in the field. It is global in perspective and reflects the international group of editors, who are well-recognized experts in pediatric nephrology. Within this text, the development of kidney structure and function is followed by detailed and comprehensive chapters on all childhood kidney diseases.
This publication provides a synopsis of the rapid progress made in the field of renal cell biology during the last decade, progress which has resulted in a better conceptual understanding of the cellular and molecular mechanisms of fibrotic renal disease. These developments have provided new therapeutic choices and led to the discovery of gene-based therapeutic options. The topics covered in this book have been carefully selected from the immense number of aspects of the disease to provide essential information on the molecular basis of renal fibrosis. Individual chapters discuss topics such as proteinuria and tubulointerstitial injury, the roles and regulation of TGF-beta, chemokines, oxidant stress, matrix remodeling, significance of renal expression of NF-kappa, and the potential impact of cell death in renal fibrosis.Written so as to present the complex information as simply as possible, this publication will be a very useful tool for general health professionals involved in the fields of immunology and cell biology, as well as for clinicians and researchers within the fields of nephrology, pathology and matrix biology.
This book reviews important aspects of polycystic kidney diseases, the latest scientific understanding of the diseases and syndromes, along with the therapies being developed. Cystic kidney diseases comprise a spectrum of genetic syndromes defined by renal cyst formation and expansion with variable extrarenal manifestations. The most prevalent disorder is the autosomal dominant polycystic kidney disease (ADPKD). It is the most common monogenetic disorder in humans and accounts for 4.4% of end-stage renal disease (ESRD) cases in the U.S. Patients inevitably progress to ESRD and require renal replacement therapy in the form of dialysis or transplantation. Through advancements in genomics and proteomics approaches, novel genes responsible for cystic diseases have been identified, further expanding our understanding of basic mechanisms of disease pathogenesis. The hallmark among all cystic genetic syndromes is the formation and growth of fluid-filled cysts, which originate from tubular epithelia of nephron segments. Cysts are the disease, and treatment strategies are being developed to target prevention or delay of cyst formation and expansion at an early stage, however no such therapy is currently approved.
Significantly updated with the latest developments in diagnosis and treatment recommendations, Ferri’s Clinical Advisor 2020 features the popular "5 books in 1" format to organize vast amounts of information in a clinically relevant, user-friendly manner. This efficient, intuitive format provides quick access to answers on 1,000 common medical conditions, including diseases and disorders, differential diagnoses, and laboratory tests – all reviewed by experts in key clinical fields. Updated algorithms, along with hundreds of new figures, tables, and boxes, ensure that you stay current with today’s medical practice. Contains significant updates throughout, covering all aspects of current diagnosis and treatment. Features 27 all-new topics including chronic traumatic encephalopathy, medical marijuana, acute respiratory failure, gallbladder carcinoma, shift work disorder, radial tunnel syndrome, fertility preservation in women, fallopian tube cancer, primary chest wall cancer, large-bowel obstruction, inguinal hernia, and bundle branch block, among others. Includes a new appendix covering Physician Quality Reporting System (PQRS) Measures. Provides current ICD-10 insurance billing codes to help expedite insurance reimbursements. Patient Teaching Guides for many of the diseases and disorders are included, most available in both English and Spanish versions, which can be downloaded and printed for patients.
This manual will meet the everyday needs of the wide range of medical professionals who play a role in the treatment of children referred to hospital because of renal disease. It is an easy-to-use, portable guide that will assist pediatricians, residents, and trainees in making prompt first-level management decisions. It will also prove invaluable for the adult nephrologists who care for children in many developing countries, and will serve as a teaching guide for experts when training non-subspecialists. Individual sections are devoted to the evaluation of renal disease; fluid, electrolyte, and acid-base disorders; glomerular diseases; tubular disorders; congenital, inherited, and urological disorders; consequences of renal disease; and miscellaneous topics. The text is in a bulleted format with tables and algorithms wherever possible, making it straightforward and easy to read. An appendix includes further important information such as normal values, drug dosages, and drug nephrotoxicity.
A book such as this, correlating radiologic findings with the associated gross and microscopic pathologic findings, has never been offered to the medical community. It contains radiologic images, in a variety of formats (ultrasound, CT scan, MRI scan) correlated with gross photos and photomicrographs of a wide spectrum of pathologic entities, including their variants, occurring in the following organs or anatomic sites. This book would be of particular interest to radiologists and radiologists-in training, who naturally are very cognizant of radiologic abnormalities, but who rarely, if ever, encounter visual images of the pathologic lesions that they diagnose. It will also be of interest to pathologists and pathologists-in-training, urologists, GU radiation oncologists, and GU medical oncologists.
This volume focuses on the investigatory methods applied to autosomal dominant polycystic kidney disease (ADPKD), one of the most common human genetic diseases. ADPKD is caused by mutations in PKD1 and TRPP2, two integral membrane proteins that function as receptor/ion channels in primary cilia of tubular epithelial cells. Thus, ADPKD belongs to ciliopathies, a group of disorders caused by abnormal cilia formation or function. This proposed book will cover the state-of-the-art methods ranging from molecular biology, biochemistry, electrophysiology, to tools in model animal studies. Key Features Explores the role of cilia in polycystic kidney disease Focuses on myriad state-of-the-art methods and techniques Reviews specific mutations integral to this autosomal genetic disease Includes discussions of model systems