link
Bookmarks
Polycystic Kidney Disease, Autosomal Dominant
Sara M. O'Hara, MD, FAAP
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
19
0
11

KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Autosomal dominant polycystic kidney disease (ADPKD), adult polycystic kidney disease
          • Definitions

            • Hereditary ciliopathy characterized by multiple renal cysts & various other systemic manifestations
            • Cystic organ involvement
              • Kidneys (100%), liver (50%), pancreas (9%), brain/ovaries/testis (1%)
            • Noncystic manifestations
              • Cardiac valve (26%), hernias (25%), colonic diverticula
              • Aneurysms: Cerebral "berry" aneurysms (5-10%); aortic or coronary aneurysms less common

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      Selected References

                                      1. Cadnapaphornchai MA et al: Effect of pravastatin on total kidney volume, left ventricular mass index, and microalbuminuria in pediatric autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 9(5):889-96, 2014
                                      2. 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
                                      3. Sweeney WE Jr et al: Diagnosis and management of childhood polycystic kidney disease. Pediatr Nephrol. 26(5):675-92, 2011
                                      4. Barua M et al: Diagnosis of autosomal-dominant polycystic kidney disease: an integrated approach. Semin Nephrol. 30(4):356-65, 2010
                                      5. Katabathina VS et al: Adult renal cystic disease: a genetic, biological, and developmental primer. Radiographics. 30(6):1509-23, 2010
                                      6. Walz G et al: Everolimus in patients with autosomal dominant polycystic kidney disease. N Engl J Med. 2010 Aug 26;363(9):830-40. Epub 2010 Jun 26. Erratum in: N Engl J Med. 2010 Sep 16;363(12):1190. N Engl J Med. 363(20):1977, 2010
                                      7. de Mattos AM et al: Autosomal-dominant polycystic kidney disease as a risk factor for diabetes mellitus following renal transplantation. Kidney Int. 67(2):714-20, 2005
                                      8. Lang EK et al: Autosomal dominant polycystic disease with renal cell carcinoma. J Urol. 173(3):987, 2005
                                      9. Nagaba Y et al: Spontaneous rupture of a left gastroepiploic artery aneurysm in a patient with autosomal-dominant polycystic kidney disease. Clin Nephrol. 63(2):163-6, 2005
                                      10. Paterson AD et al: Progressive loss of renal function is an age-dependent heritable trait in type 1 autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 16(3):755-62, 2005
                                      Related Anatomy
                                      Loading...
                                      Related Differential Diagnoses
                                      Loading...
                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Pathology

                                            • Clinical Issues

                                              TERMINOLOGY

                                              • Synonyms

                                                • Autosomal dominant polycystic kidney disease (ADPKD), adult polycystic kidney disease
                                              • Definitions

                                                • Hereditary ciliopathy characterized by multiple renal cysts & various other systemic manifestations
                                                • Cystic organ involvement
                                                  • Kidneys (100%), liver (50%), pancreas (9%), brain/ovaries/testis (1%)
                                                • Noncystic manifestations
                                                  • Cardiac valve (26%), hernias (25%), colonic diverticula
                                                  • Aneurysms: Cerebral "berry" aneurysms (5-10%); aortic or coronary aneurysms less common

                                              IMAGING

                                              • General Features

                                                • Radiographic Findings

                                                  • CT Findings

                                                    • MR Findings

                                                      • Ultrasonographic Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Gross Pathologic & Surgical Features

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          Selected References

                                                                          1. Cadnapaphornchai MA et al: Effect of pravastatin on total kidney volume, left ventricular mass index, and microalbuminuria in pediatric autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 9(5):889-96, 2014
                                                                          2. 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
                                                                          3. Sweeney WE Jr et al: Diagnosis and management of childhood polycystic kidney disease. Pediatr Nephrol. 26(5):675-92, 2011
                                                                          4. Barua M et al: Diagnosis of autosomal-dominant polycystic kidney disease: an integrated approach. Semin Nephrol. 30(4):356-65, 2010
                                                                          5. Katabathina VS et al: Adult renal cystic disease: a genetic, biological, and developmental primer. Radiographics. 30(6):1509-23, 2010
                                                                          6. Walz G et al: Everolimus in patients with autosomal dominant polycystic kidney disease. N Engl J Med. 2010 Aug 26;363(9):830-40. Epub 2010 Jun 26. Erratum in: N Engl J Med. 2010 Sep 16;363(12):1190. N Engl J Med. 363(20):1977, 2010
                                                                          7. de Mattos AM et al: Autosomal-dominant polycystic kidney disease as a risk factor for diabetes mellitus following renal transplantation. Kidney Int. 67(2):714-20, 2005
                                                                          8. Lang EK et al: Autosomal dominant polycystic disease with renal cell carcinoma. J Urol. 173(3):987, 2005
                                                                          9. Nagaba Y et al: Spontaneous rupture of a left gastroepiploic artery aneurysm in a patient with autosomal-dominant polycystic kidney disease. Clin Nephrol. 63(2):163-6, 2005
                                                                          10. Paterson AD et al: Progressive loss of renal function is an age-dependent heritable trait in type 1 autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 16(3):755-62, 2005