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This comprehensive guide to polycystic kidney disease captures the growing knowledge of this common, potentially-fatal and hereditary disease. The first two sections of the book provide an overview of PKD gene structures, mutations and pathophysiologic mechanisms. This is followed by chapters focused on PKD´s clinical features, including renal and extrarenal manifestations, and appropriate management of patients. The final section covers current clinical trials and emerging therapies in PKD. Authored by experts in the field, this book provides the clinician and researcher with critical information on basic and translational science and clinical approaches in one concise resource.

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PART 1. POLYCYSTIC KIDNEY DISEASE GENES & PROTEINS
1. Classical Polycystic Kidney Disease (PKD): Gene Structures and Mutations and Protein Structures and Functions

2. Bardet-Biedl Syndrome
3. Cystic Kidney Diseases Associated with Increased Cancer Risk: Tuberous Sclerosis Complex, Von Hippel Lindau, and Birt Hogg Dubé
PART 2. POLYCYCSTIC KIDNEY DISEASE: MECHANISMS OF DISEASE

4. Aberrant Cellular Pathways in PKD
5. Cilia and Polycystic Kidney Disease
6 . The Role of Inflammation and Fibrosis in Cystic Kidney Disease

PART 3. CLINICAL FEATURES

7. Imaging-based Diagnosis of Autosomal Dominant Polycystic Kidney Disease

8. Renal Structural Involvement in Autosomal Dominant Polycystic Kidney Disease: Cyst Growth and Total Kidney Volume: Lessons from the Consortium for Radiologic Imaging of Polycystic Kidney Disease (CRISP)
9. Renal Complications: Pain, Infection and Nephrolithiasis 10. ADPKD Extrarenal Manifestations: Polycystic Liver Disease
11. Extrarenal Manifestations of Autosomal Dominant Polycystic Kidney Disease

PART 4. CLINICAL TRIALS AND EMERGING THERAPIES

12. Role of Renin- Angiotensin- Aldosterone System Inhibition in Autosomal Dominant Polycystic Kidney Disease

13. Vasopressin Receptor Antagonism

14. End-stage Renal Disease in Patients with Autosomal Dominant Polycystic Kidney Disease

15. Polycystic Kidney Disease: Translating Mechanisms into Therapy Management of ADPKD Today

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John J. Bissler, MD

Federal Express Chair of Excellence, Professor and Chief, Division of Nephrology, Department of Pediatrics, Director of the Tuberous Sclerosis Center of Excellence at Le Bonheur Children´s Hospital, Medical Director of Nephrology, St. Jude Children´s Research Hospital, University of Tennessee Health Science Center

University of Tennessee Health Science Center

Children´s Foundation Research Center

Memphis, TN




Benjamin D. Cowley, Jr., M.D., F.A.C.P., F.A.S.N.

Professor of Medicine

Chief, Nephrology & Hypertension

John Gammill Professor in Polycystic Kidney Disease

University of Oklahoma Health Sciences Center



Oklahoma City, OK

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