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

Provides a comprehensive review of PKD mutations, pathophysiologic mechanisms of PKD, renal and extrarenal manifestations of PKD, clinical trials and emerging therapies in PKD, and appropriate management of patients with PKD

Written by top professionals in the field

Well-illustrated resource serves both clinician and researcher

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.