目次

PART 1.  POLYCYSTIC KIDNEY DISEASE GENES & PROTEINS1. 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