Polycystic Kidney Disease Questions & Answers

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

3. Boerrigter MM, Bongers EMHF, Lugtenberg D, Nevens F, Drenth JPH. Polycystic liver disease genes: Practical considerations for genetic testing. Eur J Med Genet. 2021 Mar. 64 (3):104160. [QxMD MEDLINE Link]. [Full Text].

4. 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. [QxMD MEDLINE Link].

5. 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. [QxMD MEDLINE Link]. [Full Text].

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

7. 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. [QxMD MEDLINE Link].

8. 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. [QxMD MEDLINE Link]. [Full Text].

9. 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. [QxMD MEDLINE Link].

10. 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. [QxMD MEDLINE Link]. [Full Text].

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

12. 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. [QxMD MEDLINE Link].

13. 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. [QxMD MEDLINE Link].

14. Huynh VT, Audrézet MP, Sayer JA, et al. Clinical spectrum, prognosis and estimated prevalence of DNAJB11-kidney disease. Kidney Int. 2020 Aug. 98 (2):476-487. [QxMD MEDLINE Link].

15. Besse W, Chang AR, Luo JZ, Triffo WJ, Moore BS, Gulati A, et al. ALG9 Mutation Carriers Develop Kidney and Liver Cysts. J Am Soc Nephrol. 2019 Nov. 30 (11):2091-2102. [QxMD MEDLINE Link].

16. Schönauer R, Baatz S, Nemitz-Kliemchen M, Frank V, Petzold F, Sewerin S, et al. Matching clinical and genetic diagnoses in autosomal dominant polycystic kidney disease reveals novel phenocopies and potential candidate genes. Genet Med. 2020 Aug. 22 (8):1374-1383. [QxMD MEDLINE Link]. [Full Text].

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

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

19. 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. [QxMD MEDLINE Link].

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

21. 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. [QxMD MEDLINE Link].

22. 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. [QxMD MEDLINE Link].

23. 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. [QxMD MEDLINE Link]. [Full Text].

24. 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. [QxMD MEDLINE Link]. [Full Text].

25. 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. [QxMD MEDLINE Link].

26. 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. [QxMD MEDLINE Link].

27. 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. [QxMD MEDLINE Link].

28. 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. [QxMD MEDLINE Link].

29. 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. [QxMD MEDLINE Link].

30. 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. [QxMD MEDLINE Link]. [Full Text].

31. 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):[QxMD MEDLINE Link]. [Full Text].

32. 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. [QxMD MEDLINE Link]. [Full Text].

33. 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. [QxMD MEDLINE Link]. [Full Text].

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

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

36. 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. [QxMD MEDLINE Link].

37. 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. [QxMD MEDLINE Link].

38. 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. [QxMD MEDLINE Link].

39. 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. [QxMD MEDLINE Link].

40. JYNARQUE [package insert]. Otsuka Pharmaceutical Co., Ltd. April 2018. Available at [Full Text].

41. 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. [QxMD MEDLINE Link].

42. 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. [QxMD MEDLINE Link].

43. 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. [QxMD MEDLINE Link].

44. 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. [QxMD MEDLINE Link].

45. Perrone RD, Abebe KZ, Watnick TJ, Althouse AD, Hallows KR, Lalama CM, et al. Primary results of the randomized trial of metformin administration in polycystic kidney disease (TAME PKD). Kidney Int. 2021 Sep. 100 (3):684-696. [QxMD MEDLINE Link].

46. Ong ACM, Gansevoort RT. TAMEing ADPKD with metformin: safe and effective?. Kidney Int. 2021 Sep. 100 (3):513-515. [QxMD MEDLINE Link].

47. Suwabe T, Barrera FJ, Rodriguez-Gutierrez R, Ubara Y, Hogan MC. Somatostatin analog therapy effectiveness on the progression of polycystic kidney and liver disease: A systematic review and meta-analysis of randomized clinical trials. PLoS One. 2021. 16 (9):e0257606. [QxMD MEDLINE Link]. [Full Text].

48. Leonhard WN, Song X, Kanhai AA, Iliuta IA, Bozovic A, Steinberg GR, et al. Salsalate, but not metformin or canagliflozin, slows kidney cyst growth in an adult-onset mouse model of polycystic kidney disease. EBioMedicine. 2019 Sep. 47:436-445. [QxMD MEDLINE Link]. [Full Text].

49. 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. [QxMD MEDLINE Link]. [Full Text].

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

51. 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. [QxMD MEDLINE Link].

52. 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. [QxMD MEDLINE Link].

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

54. 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. [QxMD MEDLINE Link]. [Full Text].

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.

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