Polycystic Kidney Disease Questions & Answers

1. Goksu SY, Khattar D. Cystic Kidney Disease. 2020 Jan. [Medline]. [Full Text].

2. Schrier RW. Renal volume, renin-angiotensin-aldosterone system, hypertension, and left ventricular hypertrophy in patients with autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 2009 Sep. 20(9):1888-93. [Medline].

3. Cadnapaphornchai MA, McFann K, Strain JD, et al. Prospective change in renal volume and function in children with ADPKD. Clin J Am Soc Nephrol. 2009 Apr. 4(4):820-9. [Medline]. [Full Text].

4. Pei Y. Diagnostic approach in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2006 Sep. 1(5):1108-14. [Medline].

5. Ravine D, Gibson RN, Walker RG, et al. Evaluation of ultrasonographic diagnostic criteria for autosomal dominant polycystic kidney disease 1. Lancet. 1994 Apr 2. 343(8901):824-7. [Medline].

6. Pei Y, Obaji J, Dupuis A, Paterson AD, Magistroni R, Dicks E, et al. Unified criteria for ultrasonographic diagnosis of ADPKD. J Am Soc Nephrol. 2009 Jan. 20(1):205-12. [Medline]. [Full Text].

7. Huston J 3rd, Torres VE, Wiebers DO, et al. Follow-up of intracranial aneurysms in autosomal dominant polycystic kidney disease by magnetic resonance angiography. J Am Soc Nephrol. 1996 Oct. 7(10):2135-41. [Medline].

8. Irazabal MV, Huston J 3rd, Kubly V, Rossetti S, Sundsbak JL, Hogan MC, et al. Extended follow-up of unruptured intracranial aneurysms detected by presymptomatic screening in patients with autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2011 Jun. 6(6):1274-85. [Medline]. [Full Text].

9. Wilson PD. Polycystic kidney disease. N Engl J Med. 2004 Jan 8. 350(2):151-64. [Medline].

10. Pirson Y. Extrarenal Manifestations of Autosomal Dominant Polycystic Kidney Disease. Adv Chronic Kidney Dis. 2010 Mar. 17(2):173-180. [Medline].

11. Tufan F, Uslu B, Cekrezi B, Uysal M, Alpay N, Turkmen K, et al. Assessment of Adrenal Functions in Patients with Autosomal Dominant Polycystic Kidney Disease. Exp Clin Endocrinol Diabetes. 2010 Feb 9. [Medline].

12. Porath B, Gainullin VG, Cornec-Le Gall E, Dillinger EK, Heyer CM, Hopp K, et al. Mutations in GANAB, Encoding the Glucosidase IIα Subunit, Cause Autosomal-Dominant Polycystic Kidney and Liver Disease. Am J Hum Genet. 2016 Jun 2. 98 (6):1193-207. [Medline].

13. Torres VE. Vasopressin antagonists in polycystic kidney disease. Kidney Int. 2005 Nov. 68(5):2405-18. [Medline]. [Full Text].

14. Ong AC, Wheatley DN. Polycystic kidney disease--the ciliary connection. Lancet. 2003 Mar 1. 361(9359):774-6. [Medline].

15. Cornec-Le Gall E, Audrézet MP, Chen JM, Hourmant M, Morin MP, Perrichot R, et al. Type of PKD1 mutation influences renal outcome in ADPKD. J Am Soc Nephrol. 2013 May. 24 (6):1006-13. [Medline].

16. Qian F, Watnick TJ, Onuchic LF, et al. The molecular basis of focal cyst formation in human autosomal dominant polycystic kidney disease type I. Cell. 1996 Dec 13. 87(6):979-87. [Medline].

17. Akoh JA. Current management of autosomal dominant polycystic kidney disease. World J Nephrol. 2015 Sep 6. 4 (4):468-79. [Medline].

18. Fary Ka E, Seck SM, Niang A, et al. Patterns of autosomal dominant polycystic kidney diseases in black Africans. Saudi J Kidney Dis Transpl. 2010 Jan. 21(1):81-6. [Medline].

19. Grantham JJ, Chapman AB, Torres VE. Volume progression in autosomal dominant polycystic kidney disease: the major factor determining clinical outcomes. Clin J Am Soc Nephrol. 2006 Jan. 1(1):148-57. [Medline].

20. Grantham JJ, Torres VE, Chapman AB, et al. Volume progression in polycystic kidney disease. N Engl J Med. 2006 May 18. 354(20):2122-30. [Medline]. [Full Text].

21. Idrizi A, Barbullushi M, Petrela E, et al. The influence of renal manifestations to the progression of autosomal dominant polycystic kidney disease. Hippokratia. 2009 Jul. 13(3):161-4. [Medline]. [Full Text].

22. Hateboer N, v Dijk MA, Bogdanova N, et al. Comparison of phenotypes of polycystic kidney disease types 1 and 2. European PKD1-PKD2 Study Group. Lancet. 1999 Jan 9. 353(9147):103-7. [Medline].

23. Torra R, Badenas C, Darnell A, et al. Linkage, clinical features, and prognosis of autosomal dominant polycystic kidney disease types 1 and 2. J Am Soc Nephrol. 1996 Oct. 7(10):2142-51. [Medline].

24. Chauveau D, Pirson Y, Verellen-Dumoulin C, et al. Intracranial aneurysms in autosomal dominant polycystic kidney disease. Kidney Int. 1994 Apr. 45(4):1140-6. [Medline].

25. Rahman E, Niaz FA, Al-Suwaida A, et al. Analysis of causes of mortality in patients with autosomal dominant polycystic kidney disease: A single center study. Saudi J Kidney Dis Transpl. 2009 Sep-Oct. 20(5):806-10. [Medline].

26. Chapman AB, Devuyst O, Eckardt KU, Gansevoort RT, Harris T, Horie S, et al. Autosomal-dominant polycystic kidney disease (ADPKD): executive summary from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int. 2015 Jul. 88 (1):17-27. [Medline].

27. Cornec-Le Gall E, Audrézet MP, Rousseau A, et al. The PROPKD Score: A New Algorithm to Predict Renal Survival in Autosomal Dominant Polycystic Kidney Disease. J Am Soc Nephrol. 2016 Mar. 27 (3):942-51. [Medline]. [Full Text].

28. Sato M, Kataoka H, Ushio Y, Manabe S, Watanabe S, Akihisa T, et al. High Serum Phosphate Level as a Risk Factor to Determine Renal Prognosis in Autosomal Dominant Polycystic Kidney Disease: A Retrospective Study. Medicines (Basel). 2020 Mar 12. 7 (3):[Medline]. [Full Text].

29. Baker A, King D, Marsh J, Makin A, Carr A, Davis C, et al. Understanding the physical and emotional impact of early-stage ADPKD: experiences and perspectives of patients and physicians. Clin Kidney J. 2015 Oct. 8 (5):531-7. [Medline]. [Full Text].

30. Kistler AD, Serra AL, Siwy J, Poster D, Krauer F, Torres VE, et al. Urinary proteomic biomarkers for diagnosis and risk stratification of autosomal dominant polycystic kidney disease: a multicentric study. PLoS One. 2013. 8(1):e53016. [Medline]. [Full Text].

31. Srivastava A, Patel N. Autosomal dominant polycystic kidney disease. Am Fam Physician. 2014 Sep 1. 90 (5):303-7. [Medline]. [Full Text].

32. Perrone RD, Malek AM, Watnick T. Vascular complications in autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 2015 Oct. 11 (10):589-98. [Medline].

33. Sawicki M, Walecka A, Rozanski J, et al. Doppler sonography measurements of renal vascular resistance in autosomal-dominant polycystic kidney disease. Med Sci Monit. 2009 Aug. 15(8):MT101-4. [Medline].

34. Spithoven EM, van Gastel MD, Messchendorp AL, Casteleijn NF, Drenth JP, Gaillard CA, et al. Estimation of total kidney volume in autosomal dominant polycystic kidney disease. Am J Kidney Dis. 2015 Nov. 66 (5):792-801. [Medline].

35. Schrier RW, Abebe KZ, Perrone RD, Torres VE, Braun WE, Steinman TI, et al. Blood pressure in early autosomal dominant polycystic kidney disease. N Engl J Med. 2014 Dec 11. 371 (24):2255-66. [Medline].

36. Rozenfeld MN, Ansari SA, Mohan P, Shaibani A, Russell EJ, Hurley MC. Autosomal Dominant Polycystic Kidney Disease and Intracranial Aneurysms: Is There an Increased Risk of Treatment?. AJNR Am J Neuroradiol. 2015 Sep 3. [Medline].

37. Torres VE, Chapman AB, Devuyst O, Gansevoort RT, Perrone RD, Koch G, et al. Tolvaptan in Later-Stage Autosomal Dominant Polycystic Kidney Disease. N Engl J Med. 2017 Nov 16. 377 (20):1930-1942. [Medline].

38. Torres VE, Chapman AB, Devuyst O, Gansevoort RT, Grantham JJ, Higashihara E, et al. Tolvaptan in patients with autosomal dominant polycystic kidney disease. N Engl J Med. 2012 Dec 20. 367 (25):2407-18. [Medline].

39. Torres VE, Chapman AB, Devuyst O, Gansevoort RT, Perrone RD, Dandurand A, et al. Multicenter, open-label, extension trial to evaluate the long-term efficacy and safety of early versus delayed treatment with tolvaptan in autosomal dominant polycystic kidney disease: the TEMPO 4:4 Trial. Nephrol Dial Transplant. 2018 Mar 1. 33 (3):477-489. [Medline].

40. Taler SJ, Agarwal R, Bakris GL, Flynn JT, Nilsson PM, Rahman M, et al. KDOQI US commentary on the 2012 KDIGO clinical practice guideline for management of blood pressure in CKD. Am J Kidney Dis. 2013 Aug. 62 (2):201-13. [Medline]. [Full Text].

41. Schrier RW. Optimal care of autosomal dominant polycystic kidney disease patients. Nephrology (Carlton). 2006 Apr. 11(2):124-30. [Medline].

42. Patch C, Charlton J, Roderick PJ, Gulliford MC. Use of antihypertensive medications and mortality of patients with autosomal dominant polycystic kidney disease: a population-based study. Am J Kidney Dis. 2011 Jun. 57(6):856-62. [Medline].

43. Doulton TW, Saggar-Malik AK, He FJ, Carney C, Markandu ND, Sagnella GA, et al. The effect of sodium and angiotensin-converting enzyme inhibition on the classic circulating renin-angiotensin system in autosomal-dominant polycystic kidney disease patients. J Hypertens. 2006 May. 24(5):939-45. [Medline].

44. Russell RT, Pinson CW. Surgical management of polycystic liver disease. World J Gastroenterol. 2007 Oct 14. 13(38):5052-9. [Medline].

45. Sallee M, Rafat C, Zahar JR, et al. Cyst infections in patients with autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2009 Jul. 4(7):1183-9. [Medline]. [Full Text].

46. Gansevoort RT, Arici M, Benzing T, Birn H, Capasso G, Covic A, et al. Recommendations for the use of tolvaptan in autosomal dominant polycystic kidney disease: a position statement on behalf of the ERA-EDTA Working Groups on Inherited Kidney Disorders and European Renal Best Practice. Nephrol Dial Transplant. 2016 Mar. 31 (3):337-48. [Medline].

47. FDA Drug Safety Communication: FDA limits duration and usage of Samsca (tolvaptan) due to possible liver injury leading to organ transplant or death. U.S. Food & Drug Administration. Available at https://www.fda.gov/Drugs/DrugSafety/ucm350062.htm. January 15, 2016; Accessed: March 31, 2017.

48. Rossetti S, Harris PC. Genotype-phenotype correlations in autosomal dominant and autosomal recessive polycystic kidney disease. J Am Soc Nephrol. 2007 May. 18(5):1374-80. [Medline].

49. Torres VE, Grantham JJ, Chapman AB, Mrug M, Bae KT, King BF Jr, et al. Potentially Modifiable Factors Affecting the Progression of Autosomal Dominant Polycystic Kidney Disease. Clin J Am Soc Nephrol. 2011 Mar. 6(3):640-647. [Medline].

50. Masoumi A, Reed-Gitomer B, Kelleher C, et al. Potential pharmacological interventions in polycystic kidney disease. Drugs. 2007. 67(17):2495-510. [Medline].

51. Walz G. Therapeutic approaches in autosomal dominant polycystic kidney disease (ADPKD): is there light at the end of the tunnel?. Nephrol Dial Transplant. 2006 Jul. 21(7):1752-7. [Medline].

52. Bolignano D, Palmer SC, Ruospo M, Zoccali C, Craig JC, Strippoli GF. Interventions for preventing the progression of autosomal dominant polycystic kidney disease. Cochrane Database Syst Rev. 2015 Jul 14. 7:CD010294. [Medline].

53. Torres VE, Harris PC. Mechanisms of Disease: autosomal dominant and recessive polycystic kidney diseases. Nat Clin Pract Nephrol. 2006 Jan. 2(1):40-55; quiz 55. [Medline]. [Full Text].

54. Torres VE, Harris PC. Polycystic kidney disease: genes, proteins, animal models, disease mechanisms and therapeutic opportunities. J Intern Med. 2007 Jan. 261(1):17-31. [Medline].

55. Torres VE, Chapman AB, Devuyst O, Gansevoort RT, Grantham JJ, Higashihara E, et al. Tolvaptan in Patients with Autosomal Dominant Polycystic Kidney Disease. N Engl J Med. 2012 Nov 3. [Medline].

56. Caroli A, Perico N, Perna A, et al, for the ALADIN study group. Effect of longacting somatostatin analogue on kidney and cyst growth in autosomal dominant polycystic kidney disease (ALADIN): a randomised, placebo-controlled, multicentre trial. Lancet. 2013 Aug 20. [Medline].

Roser Torra, MD, PhD Consulting Staff, Hereditary Renal Diseases, Department of Nephrology, Fundacio Puigvert, Spain

Roser Torra, MD, PhD is a member of the following medical societies: American Society of Nephrology, International Society of Nephrology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

George R Aronoff, MD Director, Professor, Departments of Internal Medicine and Pharmacology, Section of Nephrology, Kidney Disease Program, University of Louisville School of Medicine

George R Aronoff, MD is a member of the following medical societies: American Federation for Medical Research, American Society of Nephrology, Kentucky Medical Association, National Kidney Foundation

Disclosure: Nothing to disclose.

Vecihi Batuman, MD, FASN Huberwald Professor of Medicine, Section of Nephrology-Hypertension, Interim Chair, Deming Department of Medicine, Tulane University School of Medicine

Vecihi Batuman, MD, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, International Society of Nephrology, Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

Laura Lyngby Mulloy, DO, FACP Professor of Medicine, Chief, Section of Nephrology, Hypertension, and Transplantation Medicine, Glover/Mealing Eminent Scholar Chair in Immunology, Medical College of Georgia, Georgia Regents University

Disclosure: Nothing to disclose.

• Print this section
• Print the entire contents of
• Print the entire contents of article