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Autosomal Dominant Polycystic Kidney Disease
Bryan R. Foster, MD; Alessandro Furlan, MD
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10

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, renal failure, 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

                                                  • Reporting Tips

                                                    Selected References

                                                    1. Gaur P et al: ADPKD-what the radiologist should know. Br J Radiol. 92(1098):20190078, 2019
                                                    2. Murray N et al: Dual-energy CT in evaluation of the acute abdomen. Radiographics. 39(1):264-86, 2019
                                                    3. Jeudy J et al: Spectrum of coronary artery aneurysms: from the radiologic pathology archives. Radiographics. 38(1):11-36, 2018
                                                    4. Dillman JR et al: Hereditary renal cystic disorders: imaging of the kidneys and beyond. Radiographics. 37(3):924-46, 2017
                                                    5. Kim B et al: Inherited renal cystic diseases. Abdom Radiol (NY). 41(6):1035-51, 2016
                                                    6. Kim JA et al: Pancreatic cysts in autosomal dominant polycystic kidney disease: prevalence and association with PKD2 gene mutations. Radiology. 280(3):762-70, 2016
                                                    7. Harvey CJ et al: Role of US contrast agents in the assessment of indeterminate solid and cystic lesions in native and transplant kidneys. Radiographics. 35(5):1419-30, 2015
                                                    8. Hogan MC et al: Liver involvement in early autosomal-dominant polycystic kidney disease. Clin Gastroenterol Hepatol. 13(1):155-64.e6, 2015
                                                    9. Reig B et al: Seminal megavesicle in autosomal dominant polycystic kidney disease. Clin Imaging. 39(2):289-92, 2015
                                                    10. Tellman MW et al: Management of pain in autosomal dominant polycystic kidney disease and anatomy of renal innervation. J Urol. 193(5):1470-8, 2015
                                                    11. Wood CG 3rd et al: CT and MR imaging for evaluation of cystic renal lesions and diseases. Radiographics. 35(1):125-41, 2015
                                                    12. 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
                                                    13. Kanaan N et al: Renal transplantation in autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 10(8):455-65, 2014
                                                    14. Patel MS et al: Trends in the management and outcomes of kidney transplantation for autosomal dominant polycystic kidney disease. J Transplant. 2014:675697, 2014
                                                    15. 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
                                                    16. 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
                                                    17. 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
                                                    18. Grantham JJ et al: Why kidneys fail in autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 7(10):556-66, 2011
                                                    19. Bae KT et al: Imaging for the prognosis of autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 6(2):96-106, 2010
                                                    20. Katabathina VS et al: Adult renal cystic disease: a genetic, biological, and developmental primer. Radiographics. 30(6):1509-23, 2010
                                                    21. Gonçalves S et al: Autosomal-dominant polycystic kidney disease and kidney transplantation: experience of a single center. Transplant Proc. 41(3):887-90, 2009
                                                    22. Harris PC et al: Polycystic kidney disease. Annu Rev Med. 60:321-37, 2009
                                                    23. Pei Y et al: Unified criteria for ultrasonographic diagnosis of ADPKD. J Am Soc Nephrol. 20(1):205-12, 2009
                                                    24. Grantham JJ: Clinical practice. Autosomal dominant polycystic kidney disease. N Engl J Med. 359(14):1477-85, 2008
                                                    25. 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
                                                    26. Grantham JJ et al: Volume progression in polycystic kidney disease. N Engl J Med. 354(20):2122-30, 2006
                                                    27. 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
                                                    28. Morgan DE et al: Polycystic liver disease: multimodality imaging for complications and transplant evaluation. Radiographics. 26(6):1655-68; quiz 1655, 2006
                                                    29. 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
                                                    30. Nascimento AB et al: Rapid MR imaging detection of renal cysts: age-based standards. Radiology 221: 628-32, 2001
                                                    31. 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
                                                    32. 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
                                                    33. Nicolau C et al: Autosomal dominant polycystic kidney disease types 1 and 2: Assessment of US sensitivity for diagnosis. Radiology 213: 273-6, 1999
                                                    34. 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, renal failure, 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

                                                                                                    • Reporting Tips

                                                                                                      Selected References

                                                                                                      1. Gaur P et al: ADPKD-what the radiologist should know. Br J Radiol. 92(1098):20190078, 2019
                                                                                                      2. Murray N et al: Dual-energy CT in evaluation of the acute abdomen. Radiographics. 39(1):264-86, 2019
                                                                                                      3. Jeudy J et al: Spectrum of coronary artery aneurysms: from the radiologic pathology archives. Radiographics. 38(1):11-36, 2018
                                                                                                      4. Dillman JR et al: Hereditary renal cystic disorders: imaging of the kidneys and beyond. Radiographics. 37(3):924-46, 2017
                                                                                                      5. Kim B et al: Inherited renal cystic diseases. Abdom Radiol (NY). 41(6):1035-51, 2016
                                                                                                      6. Kim JA et al: Pancreatic cysts in autosomal dominant polycystic kidney disease: prevalence and association with PKD2 gene mutations. Radiology. 280(3):762-70, 2016
                                                                                                      7. Harvey CJ et al: Role of US contrast agents in the assessment of indeterminate solid and cystic lesions in native and transplant kidneys. Radiographics. 35(5):1419-30, 2015
                                                                                                      8. Hogan MC et al: Liver involvement in early autosomal-dominant polycystic kidney disease. Clin Gastroenterol Hepatol. 13(1):155-64.e6, 2015
                                                                                                      9. Reig B et al: Seminal megavesicle in autosomal dominant polycystic kidney disease. Clin Imaging. 39(2):289-92, 2015
                                                                                                      10. Tellman MW et al: Management of pain in autosomal dominant polycystic kidney disease and anatomy of renal innervation. J Urol. 193(5):1470-8, 2015
                                                                                                      11. Wood CG 3rd et al: CT and MR imaging for evaluation of cystic renal lesions and diseases. Radiographics. 35(1):125-41, 2015
                                                                                                      12. 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
                                                                                                      13. Kanaan N et al: Renal transplantation in autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 10(8):455-65, 2014
                                                                                                      14. Patel MS et al: Trends in the management and outcomes of kidney transplantation for autosomal dominant polycystic kidney disease. J Transplant. 2014:675697, 2014
                                                                                                      15. 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
                                                                                                      16. 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
                                                                                                      17. 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
                                                                                                      18. Grantham JJ et al: Why kidneys fail in autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 7(10):556-66, 2011
                                                                                                      19. Bae KT et al: Imaging for the prognosis of autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 6(2):96-106, 2010
                                                                                                      20. Katabathina VS et al: Adult renal cystic disease: a genetic, biological, and developmental primer. Radiographics. 30(6):1509-23, 2010
                                                                                                      21. Gonçalves S et al: Autosomal-dominant polycystic kidney disease and kidney transplantation: experience of a single center. Transplant Proc. 41(3):887-90, 2009
                                                                                                      22. Harris PC et al: Polycystic kidney disease. Annu Rev Med. 60:321-37, 2009
                                                                                                      23. Pei Y et al: Unified criteria for ultrasonographic diagnosis of ADPKD. J Am Soc Nephrol. 20(1):205-12, 2009
                                                                                                      24. Grantham JJ: Clinical practice. Autosomal dominant polycystic kidney disease. N Engl J Med. 359(14):1477-85, 2008
                                                                                                      25. 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
                                                                                                      26. Grantham JJ et al: Volume progression in polycystic kidney disease. N Engl J Med. 354(20):2122-30, 2006
                                                                                                      27. 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
                                                                                                      28. Morgan DE et al: Polycystic liver disease: multimodality imaging for complications and transplant evaluation. Radiographics. 26(6):1655-68; quiz 1655, 2006
                                                                                                      29. 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
                                                                                                      30. Nascimento AB et al: Rapid MR imaging detection of renal cysts: age-based standards. Radiology 221: 628-32, 2001
                                                                                                      31. 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
                                                                                                      32. 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
                                                                                                      33. Nicolau C et al: Autosomal dominant polycystic kidney disease types 1 and 2: Assessment of US sensitivity for diagnosis. Radiology 213: 273-6, 1999
                                                                                                      34. Franz KA et al: Rate of functional deterioration in polycystic kidney disease. Kidney Int. 23(3):526-9, 1983