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Autosomal Dominant Polycystic Kidney Disease
Alessandro Furlan, MD
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11

KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Autosomal dominant polycystic kidney disease (ADPKD)
              • Definitions

                • Inherited polycystic renal disease characterized by progressive cystic growth and multiple systemic manifestations

              IMAGING

              • General Features

                • Ultrasonographic Findings

                  • CT Findings

                    • MR Findings

                      • Other Renal Findings

                        • Extrarenal Manifestations

                          • Imaging Recommendations

                            DIFFERENTIAL DIAGNOSIS

                              PATHOLOGY

                              • General Features

                                • Staging, Grading, & Classification

                                  • Gross Pathologic & Surgical Features

                                    • Microscopic Features

                                      CLINICAL ISSUES

                                      • Presentation

                                        • Demographics

                                          • Natural History & Prognosis

                                            • Treatment

                                              DIAGNOSTIC CHECKLIST

                                              • Consider

                                                • Image Interpretation Pearls

                                                  Selected References

                                                  1. Hogan MC et al: Liver involvement in early autosomal-dominant polycystic kidney disease. Clin Gastroenterol Hepatol. 13(1):155-64.e6, 2015
                                                  2. Reig B et al: Seminal megavesicle in autosomal dominant polycystic kidney disease. Clin Imaging. 39(2):289-92, 2015
                                                  3. Tellman MW et al: Management of Pain in Autosomal Dominant Polycystic Kidney Disease and Anatomy of Renal Innervation. J Urol. 193(5):1470-1478, 2015
                                                  4. Wood CG 3rd et al: CT and MR Imaging for Evaluation of Cystic Renal Lesions and Diseases. Radiographics. 35(1):125-41, 2015
                                                  5. Chung EM et al: From the radiologic pathology archives: pediatric polycystic kidney disease and other ciliopathies: radiologic-pathologic correlation. Radiographics. 34(1):155-78, 2014
                                                  6. Kanaan N et al: Renal transplantation in autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 10(8):455-65, 2014
                                                  7. Patel MS et al: Trends in the management and outcomes of kidney transplantation for autosomal dominant polycystic kidney disease. J Transplant. 2014:675697, 2014
                                                  8. Rahbari-Oskoui F et al: Renal relevant radiology: radiologic imaging in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 9(2):406-15, 2014
                                                  9. Chapman AB et al: Kidney volume and functional outcomes in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 7(3):479-86, 2012
                                                  10. Grantham JJ et al: Detected renal cysts are tips of the iceberg in adults with ADPKD. Clin J Am Soc Nephrol. 7(7):1087-93, 2012
                                                  11. Grantham JJ et al: Why kidneys fail in autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 7(10):556-66, 2011
                                                  12. Bae KT et al: Imaging for the prognosis of autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 6(2):96-106, 2010
                                                  13. Katabathina VS et al: Adult renal cystic disease: a genetic, biological, and developmental primer. Radiographics. 30(6):1509-23, 2010
                                                  14. Gonçalves S et al: Autosomal-dominant polycystic kidney disease and kidney transplantation: experience of a single center. Transplant Proc. 41(3):887-90, 2009
                                                  15. Harris PC et al: Polycystic kidney disease. Annu Rev Med. 60:321-37, 2009
                                                  16. Pei Y et al: Unified criteria for ultrasonographic diagnosis of ADPKD. J Am Soc Nephrol. 20(1):205-12, 2009
                                                  17. Grantham JJ: Clinical practice. Autosomal dominant polycystic kidney disease. N Engl J Med. 359(14):1477-85, 2008
                                                  18. Zhao X et al: Molecular diagnostics in autosomal dominant polycystic kidney disease: utility and limitations. Clin J Am Soc Nephrol. 3(1):146-52, 2008
                                                  19. Grantham JJ et al: Volume progression in polycystic kidney disease. N Engl J Med. 354(20):2122-30, 2006
                                                  20. Harris PC et al: Cyst number but not the rate of cystic growth is associated with the mutated gene in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 17(11):3013-9, 2006
                                                  21. Morgan DE et al: Polycystic liver disease: multimodality imaging for complications and transplant evaluation. Radiographics. 26(6):1655-68; quiz 1655, 2006
                                                  22. Truong LD et al: Renal cystic neoplasms and renal neoplasms associated with cystic renal diseases: pathogenetic and molecular links. Adv Anat Pathol. 10(3):135-59, 2003
                                                  23. Nascimento AB et al: Rapid MR imaging detection of renal cysts: Age-based standards. Radiology 221: 628-32, 2001
                                                  24. Perrone RD et al: Survival after end-stage renal disease in autosomal dominant polycystic kidney disease: contribution of extrarenal complications to mortality. Am J Kidney Dis. 38(4):777-84, 2001
                                                  25. Hateboer N et al: Comparison of phenotypes of polycystic kidney disease types 1 and 2. European PKD1-PKD2 Study Group. Lancet. 353(9147):103-7, 1999
                                                  26. Nicolau C et al: Autosomal dominant polycystic kidney disease types 1 and 2: Assessment of US sensitivity for diagnosis. Radiology 213: 273-6, 1999
                                                  27. Franz KA et al: Rate of functional deterioration in polycystic kidney disease. Kidney Int. 23(3):526-9, 1983
                                                  Related Anatomy
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                                                  Related Differential Diagnoses
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                                                  References
                                                  Tables

                                                  Tables

                                                  KEY FACTS

                                                  • Terminology

                                                    • Imaging

                                                      • Top Differential Diagnoses

                                                        • Pathology

                                                          • Clinical Issues

                                                            • Diagnostic Checklist

                                                              TERMINOLOGY

                                                              • Abbreviations

                                                                • Autosomal dominant polycystic kidney disease (ADPKD)
                                                              • Definitions

                                                                • Inherited polycystic renal disease characterized by progressive cystic growth and multiple systemic manifestations

                                                              IMAGING

                                                              • General Features

                                                                • Ultrasonographic Findings

                                                                  • CT Findings

                                                                    • MR Findings

                                                                      • Other Renal Findings

                                                                        • Extrarenal Manifestations

                                                                          • Imaging Recommendations

                                                                            DIFFERENTIAL DIAGNOSIS

                                                                              PATHOLOGY

                                                                              • General Features

                                                                                • Staging, Grading, & Classification

                                                                                  • Gross Pathologic & Surgical Features

                                                                                    • Microscopic Features

                                                                                      CLINICAL ISSUES

                                                                                      • Presentation

                                                                                        • Demographics

                                                                                          • Natural History & Prognosis

                                                                                            • Treatment

                                                                                              DIAGNOSTIC CHECKLIST

                                                                                              • Consider

                                                                                                • Image Interpretation Pearls

                                                                                                  Selected References

                                                                                                  1. Hogan MC et al: Liver involvement in early autosomal-dominant polycystic kidney disease. Clin Gastroenterol Hepatol. 13(1):155-64.e6, 2015
                                                                                                  2. Reig B et al: Seminal megavesicle in autosomal dominant polycystic kidney disease. Clin Imaging. 39(2):289-92, 2015
                                                                                                  3. Tellman MW et al: Management of Pain in Autosomal Dominant Polycystic Kidney Disease and Anatomy of Renal Innervation. J Urol. 193(5):1470-1478, 2015
                                                                                                  4. Wood CG 3rd et al: CT and MR Imaging for Evaluation of Cystic Renal Lesions and Diseases. Radiographics. 35(1):125-41, 2015
                                                                                                  5. Chung EM et al: From the radiologic pathology archives: pediatric polycystic kidney disease and other ciliopathies: radiologic-pathologic correlation. Radiographics. 34(1):155-78, 2014
                                                                                                  6. Kanaan N et al: Renal transplantation in autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 10(8):455-65, 2014
                                                                                                  7. Patel MS et al: Trends in the management and outcomes of kidney transplantation for autosomal dominant polycystic kidney disease. J Transplant. 2014:675697, 2014
                                                                                                  8. Rahbari-Oskoui F et al: Renal relevant radiology: radiologic imaging in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 9(2):406-15, 2014
                                                                                                  9. Chapman AB et al: Kidney volume and functional outcomes in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 7(3):479-86, 2012
                                                                                                  10. Grantham JJ et al: Detected renal cysts are tips of the iceberg in adults with ADPKD. Clin J Am Soc Nephrol. 7(7):1087-93, 2012
                                                                                                  11. Grantham JJ et al: Why kidneys fail in autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 7(10):556-66, 2011
                                                                                                  12. Bae KT et al: Imaging for the prognosis of autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 6(2):96-106, 2010
                                                                                                  13. Katabathina VS et al: Adult renal cystic disease: a genetic, biological, and developmental primer. Radiographics. 30(6):1509-23, 2010
                                                                                                  14. Gonçalves S et al: Autosomal-dominant polycystic kidney disease and kidney transplantation: experience of a single center. Transplant Proc. 41(3):887-90, 2009
                                                                                                  15. Harris PC et al: Polycystic kidney disease. Annu Rev Med. 60:321-37, 2009
                                                                                                  16. Pei Y et al: Unified criteria for ultrasonographic diagnosis of ADPKD. J Am Soc Nephrol. 20(1):205-12, 2009
                                                                                                  17. Grantham JJ: Clinical practice. Autosomal dominant polycystic kidney disease. N Engl J Med. 359(14):1477-85, 2008
                                                                                                  18. Zhao X et al: Molecular diagnostics in autosomal dominant polycystic kidney disease: utility and limitations. Clin J Am Soc Nephrol. 3(1):146-52, 2008
                                                                                                  19. Grantham JJ et al: Volume progression in polycystic kidney disease. N Engl J Med. 354(20):2122-30, 2006
                                                                                                  20. Harris PC et al: Cyst number but not the rate of cystic growth is associated with the mutated gene in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 17(11):3013-9, 2006
                                                                                                  21. Morgan DE et al: Polycystic liver disease: multimodality imaging for complications and transplant evaluation. Radiographics. 26(6):1655-68; quiz 1655, 2006
                                                                                                  22. Truong LD et al: Renal cystic neoplasms and renal neoplasms associated with cystic renal diseases: pathogenetic and molecular links. Adv Anat Pathol. 10(3):135-59, 2003
                                                                                                  23. Nascimento AB et al: Rapid MR imaging detection of renal cysts: Age-based standards. Radiology 221: 628-32, 2001
                                                                                                  24. Perrone RD et al: Survival after end-stage renal disease in autosomal dominant polycystic kidney disease: contribution of extrarenal complications to mortality. Am J Kidney Dis. 38(4):777-84, 2001
                                                                                                  25. Hateboer N et al: Comparison of phenotypes of polycystic kidney disease types 1 and 2. European PKD1-PKD2 Study Group. Lancet. 353(9147):103-7, 1999
                                                                                                  26. Nicolau C et al: Autosomal dominant polycystic kidney disease types 1 and 2: Assessment of US sensitivity for diagnosis. Radiology 213: 273-6, 1999
                                                                                                  27. Franz KA et al: Rate of functional deterioration in polycystic kidney disease. Kidney Int. 23(3):526-9, 1983