Many inherited diseases and non-hereditary disorders have in common the development of renal cystic disease. The most common, autosonal dominant polycystic kidney disease, is responsible for 5-10% of end-stage renal failure treated by dialysis or transplantation. Since the publication six years ago of the last book on polycystic kidney disease, a number of genes causing the disease have been identified, mapped, or sequenced; new experimental models and the application of molecular biology techniques have provided new insights into the pathogenesis R polycystic kidney disease; novel clinical studies have provided valuable information for the prevention, evaluation, and treatment of the complications of this disease. This book provides an updated,state-of-the-art review of the genetics, pathophysiology, evaluation, and management of these diseases and will be of interest to both basic researchers and clinicians in nephrology.
Part I:: Cystic renal disease:: experimental models and pathology; Principles of molecular biology as applied to the study of disease; In vitro models in the study of renal cystogenesis; Mouse models of polycystic kidney disease; In vivo models in non-murine species; Pathogenesis of polycystic kidney disease:: basement membrane and extracellular matric; Pathogenesis of polycystic kidney disease:: altered cellular function; Part II:: Cystic renal disease:: clinical spectrum; Classification of cystic kidneys; Diagnostic imaging of renal cystic diseases; Autosomal recessive polycystic kidney disease:: clinical and genetic profiles; Acquired renal cystic disease; Tuberous sclerosis; complex; Von Hippel-Lindau Disease; Part III:: Adult polycystic kidney disease:: natural history and genetics; Definition and natural history of autosomal dominant polycystic kidney disease; Cloning strategies and genetics of type 1 autosomal dominant polycystic kidney disease; Part IV:: Adult polycystic kidney disease:: clinical features; Hypertension in polycystic kidney disease; Progression to renal insufficiency; Management of end-stage renal failure and problems of transplantation in autosomal dominant polycystic kidney disease; Chronic pain and its medical and surgical management in renal cystic disease; Part V:: Adult polycystic kidney disease:: complications; Miscellaneous renal and systemic complications of autosomal dominant polycystic kidney disease including infection; Polycystic liver disease; Intracranial aneurysms in autosomal dominant polycystic kidney disease; Particular problems in childhood and adolescents in autosomal dominant polycystic kidney disease; Counselling and ethical considerations in autosomal polycystic kidney disease;
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