Polycystic kidney disease - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Polycystic kidney disease (PKD) is an inherited renal cystic disease, of which autosomal-dominant polycystic kidney disease is the more common form.
Characterized by renal cysts, extrarenal cysts, intracranial aneurysms, aortic root dilation and aneurysms, mitral valve prolapse, and abdominal wall hernias.
Interfamilial and intrafamilial variability is explained to a large extent by its genetic heterogeneity and modifier genes.
Long-term complications include hypertension; increased cardiovascular morbidity and mortality; chronic kidney disease; intracranial, coronary, thoracic, and abdominal aneurysms; liver and pancreatic cysts; and end-stage renal disease.
Efforts to slow the progression of the disease and delay the need for renal replacement therapy are being investigated, and in some countries therapies that slow kidney and severe liver disease are available for use.

MRI of abdomen and pelvis of patient with symptomatic polycystic kidney disease

From collection of Dr M. Hogan

Quick Reference

History & Exam
- Key Factors
- Other Factors
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Diagnostics Tests
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Treatment Options
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Definition

Epidemiology

Etiology

Pathophysiology

Images

Gross pathology of polycystic kidneys
CT scan of abdomen and pelvis of patient with mild disease
MRI of abdomen and pelvis of patient with symptomatic polycystic kidney disease

Citations

Key Articles

Bergmann C, Guay-Woodford LM, Harris PC, et al. Polycystic kidney disease. Nat Rev Dis Primers. 2018 Dec 6;4(1):50.[Abstract][Full Text]
Cornec-Le Gall E, Alam A, Perrone RD. Autosomal dominant polycystic kidney disease. Lancet. 2019 Mar 2;393(10174):919-35.[Abstract]
Pei Y, Obaji J, Dupuis A, et al. Unified criteria for ultrasonographic diagnosis of ADPKD. J Am Soc Nephrol. 2009 Jan;20(1):205-12.[Abstract][Full Text]
Torres VE, Chapman AB, Devuyst O, et al; TEMPO 3:4 Trial Investigators. Tolvaptan in patients with autosomal dominant polycystic kidney disease. N Engl J Med. 2012 Dec 20;367(25):2407-18.[Abstract][Full Text]
Schrier RW, Abebe KZ, Perrone RD, et al; HALT-PKD Trial Investigators. Blood pressure in early autosomal dominant polycystic kidney disease. N Engl J Med. 2014 Dec 11;371(24):2255-66.[Abstract]
Torres VE, Abebe KZ, Chapman AB, et al; HALT-PKD Trial Investigators. Angiotensin blockade in late autosomal dominant polycystic kidney disease. N Engl J Med. 2014 Dec 11;371(24):2267-76.[Abstract]

Referenced Articles

1. Bergmann C, Guay-Woodford LM, Harris PC, et al. Polycystic kidney disease. Nat Rev Dis Primers. 2018 Dec 6;4(1):50.[Abstract][Full Text]
2. Lavu S, Vaughan LE, Senum SR, et al. The value of genotypic and imaging information to predict functional and structural outcomes in ADPKD. JCI Insight. 2020 Aug 6;5(15):e138724.[Abstract][Full Text]
3. Shukoor SS, Vaughan LE, Edwards ME, et al. Characteristics of patients with end-stage kidney disease in ADPKD. Kidney Int Rep. 2021 Mar;6(3):755-67.[Abstract][Full Text]
4. Cornec-Le Gall E, Alam A, Perrone RD. Autosomal dominant polycystic kidney disease. Lancet. 2019 Mar 2;393(10174):919-35.[Abstract]
5. Guay-Woodford LM, Bissler JJ, Braun MC, et al. Consensus expert recommendations for the diagnosis and management of autosomal recessive polycystic kidney disease: report of an international conference. J Pediatr. 2014 Sep;165(3):611-7.[Abstract][Full Text]
6. AlNuaimi D, AlKetbi R, AlFalahi A, et al. Ruptured berry aneurysm as the initial presentation of polycystic kidney disease: a case report and review of literature. J Radiol Case Rep. 2018 Sep;12(9):1-8.[Abstract][Full Text]
7. Gimpel C, Bergmann C, Bockenhauer D, et al. International consensus statement on the diagnosis and management of autosomal dominant polycystic kidney disease in children and young people. Nat Rev Nephrol. 2019 Nov;15(11):713-26.[Abstract][Full Text]
8. Lanktree MB, Haghighi A, Guiard E, et al. Prevalence estimates of polycystic kidney and liver disease by population sequencing. J Am Soc Nephrol. 2018 Oct;29(10):2593-600.[Abstract][Full Text]
9. Willey CJ, Blais JD, Hall AK, et al. Prevalence of autosomal dominant polycystic kidney disease in the European Union. Nephrol Dial Transplant. 2017 Aug 1;32(8):1356-63.[Abstract][Full Text]
10. Willey C, Gauthier-Loiselle M, Cloutier M, et al. Regional variations in prevalence and severity of autosomal dominant polycystic kidney disease in the United States. Curr Med Res Opin. 2021 Jul;37(7):1155-62.[Abstract][Full Text]
11. Suwabe T, Shukoor S, Chamberlain AM, et al. Epidemiology of autosomal dominant polycystic kidney disease in Olmsted County. Clin J Am Soc Nephrol. 2020 Jan 7;15(1):69-79.[Abstract][Full Text]
12. Solazzo A, Testa F, Giovanella S, et al. The prevalence of autosomal dominant polycystic kidney disease (ADPKD): a meta-analysis of European literature and prevalence evaluation in the Italian province of Modena suggest that ADPKD is a rare and underdiagnosed condition. PLoS One. 2018;13(1):e0190430.[Abstract][Full Text]
13. Neumann HP, Jilg C, Bacher J, et al. Epidemiology of autosomal-dominant polycystic kidney disease: an in-depth clinical study for south-western Germany. Nephrol Dial Transplant. 2013 Jun;28(6):1472-87.[Abstract][Full Text]
14. Willey C, Kamat S, Stellhorn R, et al. Analysis of nationwide data to determine the incidence and diagnosed prevalence of autosomal dominant polycystic kidney disease in the USA: 2013-2015. Kidney Dis (Basel). 2019 Mar;5(2):107-17.[Abstract][Full Text]
15. United States Renal Data System. 2020 annual data report: epidemiology of kidney disease in the United States. 2020 [internet publication].[Full Text]
16. Harris PC, Torres VE. Polycystic kidney disease, autosomal dominant. GeneReviews. July 2018 [internet publication].[Abstract][Full Text]
17. Porath B, Gainullin VG, Cornec-Le Gall E, et al. Mutations in GANAB, encoding the glucosidase IIalpha subunit, cause autosomal-dominant polycystic kidney and liver disease. Am J Hum Genet. 2016 Jun 2;98(6):1193-207.[Abstract][Full Text]
18. Cornec-Le Gall E, Olson RJ, Besse W, et al. Monoallelic mutations to DNAJB11 cause atypical autosomal-dominant polycystic kidney disease. Am J Hum Genet. 2018 May 3;102(5):832-44.[Abstract][Full Text]
19. Mallawaarachchi AC, Lundie B, Hort Y, et al. Genomic diagnostics in polycystic kidney disease: an assessment of real-world use of whole-genome sequencing. Eur J Hum Genet. 2021 May;29(5):760-70.[Abstract]
20. Iliuta IA, Kalatharan V, Wang K, et al. Polycystic kidney disease without an apparent family history. J Am Soc Nephrol. 2017 Sep;28(9):2768-76.[Abstract][Full Text]
21. Lanktree MB, Haghighi A, di Bari I, et al. Insights into autosomal dominant polycystic kidney disease from genetic studies. Clin J Am Soc Nephrol. 2021 May 8;16(5):790-9.[Abstract][Full Text]
22. 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.[Abstract][Full Text]
23. Hanaoka K, Qian F, Boletta A, et al. Co-assembly of polycystin-1 and -2 produces unique cation-permeable currents. Nature. 2000 Dec 21-28;408(6815):990-4.[Abstract]
24. Nauli SM, Alenghat FJ, Luo Y, et al. Polycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells. Nat Genet. 2003 Feb;33(2):129-37.[Abstract]
25. Douguet D, Patel A, Honoré E. Structure and function of polycystins: insights into polycystic kidney disease. Nat Rev Nephrol. 2019 Jul;15(7):412-22.[Abstract]
26. 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.[Abstract][Full Text]
27. Chebib FT, Torres VE. Autosomal dominant polycystic kidney disease: core curriculum 2016. Am J Kidney Dis. 2016 May;67(5):792-810.[Abstract][Full Text]
28. Hunsicker LG, Adler S, Caggiula A, et al. Predictors of the progression of renal disease in the Modification of Diet in Renal Disease Study. Kidney Int. 1997 Jun;51(6):1908-19.[Abstract]
29. Harris PC, Bae KT, Rossetti S, 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. 2006 Nov;17(11):3013-9.[Abstract][Full Text]
30. Cornec-Le Gall E, Audrézet MP, Renaudineau E, et al. PKD2-related autosomal dominant polycystic kidney disease: prevalence, clinical presentation, mutation spectrum, and prognosis. Am J Kidney Dis. 2017 Oct;70(4):476-85.[Abstract][Full Text]
31. Chapman AB, Devuyst O, Eckardt KU, 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.[Abstract][Full Text]
32. Rossetti S, Harris PC. The genetics of vascular complications in autosomal dominant polycystic kidney disease (ADPKD). Curr Hypertens Rev. 2013 Feb;9(1):37-43.[Abstract][Full Text]
33. Gunay-Aygun M, Font-Montgomery E, Lukose L, et al. Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease. Gastroenterology. 2013 Jan;144(1):112-21.e2.[Abstract]
34. Pirson Y, Chauveau D, Torres V. Management of cerebral aneurysms in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 2002 Jan;13(1):269-76.[Abstract][Full Text]
35. Chebib FT, Torres VE. Recent advances in the management of autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2018 Nov 7;13(11):1765-76.[Abstract][Full Text]
36. Grantham JJ. Clinical practice. Autosomal dominant polycystic kidney disease. N Engl J Med. 2008 Oct 2;359(14):1477-85.[Abstract]
37. Zhang ZY, Wang ZM, Huang Y. Polycystic liver disease: classification, diagnosis, treatment process, and clinical management. World J Hepatol. 2020 Mar 27;12(3):72-83.[Abstract][Full Text]
38. Mikolajczyk AE, Te HS, Chapman AB. Gastrointestinal manifestations of autosomal-dominant polycystic kidney disease. Clin Gastroenterol Hepatol. 2017 Jan;15(1):17-24.[Abstract][Full Text]
39. 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.[Abstract][Full Text]
40. Torres VE, Grantham JJ, Chapman AB, et al. Potentially modifiable factors affecting the progression of autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2011 Mar;6(3):640-7.[Abstract][Full Text]
41. Torres VE, Erickson SB, Smith LH, et al. The association of nephrolithiasis and autosomal dominant polycystic kidney disease. Am J Kidney Dis. 1988 Apr;11(4):318-25.[Abstract]
42. Pei Y, Obaji J, Dupuis A, et al. Unified criteria for ultrasonographic diagnosis of ADPKD. J Am Soc Nephrol. 2009 Jan;20(1):205-12.[Abstract][Full Text]
43. Pei Y, Hwang YH, Conklin J, et al. Imaging-based diagnosis of autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 2015 Mar;26(3):746-53.[Abstract][Full Text]
44. Irazabal MV, Rangel LJ, Bergstralh EJ, et al. Imaging classification of autosomal dominant polycystic kidney disease: a simple model for selecting patients for clinical trials. J Am Soc Nephrol. 2015 Jan;26(1):160-72.[Abstract][Full Text]
45. Kuo IY, Chapman AB. Polycystins, ADPKD, and cardiovascular disease. Kidney Int Rep. 2020 Apr;5(4):396-406.[Abstract][Full Text]
46. Grampsas SA, Chandhoke PS, Fan J, et al. Anatomic and metabolic risk factors for nephrolithiasis in patients with autosomal dominant polycystic kidney disease. Am J Kidney Dis. 2000 Jul;36(1):53-7.[Abstract]
47. Hoh BL, Ko NU, Amin-Hanjani S, et al. 2023 Guideline for the management of patients with aneurysmal subarachnoid hemorrhage: a guideline from the American Heart Association/American Stroke Association. Stroke. 2023 Jul;54(7):e314-70.[Abstract][Full Text]
48. Chung DY, Abdalkader M, Nguyen TN. Aneurysmal subarachnoid hemorrhage. Neurol Clin. 2021 May;39(2):419-42.[Abstract]
49. Lavi R, Yarnitsky D, Rowe JM, et al. Standard vs atraumatic Whitacre needle for diagnostic lumbar puncture: a randomized trial. Neurology. 2006 Oct 24;67(8):1492-4.[Abstract]
50. Arendt K, Demaerschalk BM, Wingerchuk DM, et al. Atraumatic lumbar puncture needles: after all these years, are we still missing the point? Neurologist. 2009 Jan;15(1):17-20.[Abstract]
51. Nath S, Koziarz A, Badhiwala JH, et al. Atraumatic versus conventional lumbar puncture needles: a systematic review and meta-analysis. Lancet. 2018 Mar 24;391(10126):1197-204.[Abstract]
52. Rochwerg B, Almenawer SA, Siemieniuk RAC, et al. Atraumatic (pencil-point) versus conventional needles for lumbar puncture: a clinical practice guideline. BMJ. 2018 May 22;361:k1920.[Abstract][Full Text]
53. Ahmed SV, Jayawarna C, Jude E. Post lumbar puncture headache: diagnosis and management. Postgrad Med J. 2006 Nov;82(973):713-6.[Abstract][Full Text]
54. Arevalo-Rodriguez I, Ciapponi A, Roqué i Figuls M, et al. Posture and fluids for preventing post-dural puncture headache. Cochrane Database Syst Rev. 2016 Mar 7;(3):CD009199.[Abstract][Full Text]
55. Dad T, Abebe KZ, Bae KT, et al. Longitudinal assessment of left ventricular mass in autosomal dominant polycystic kidney disease. Kidney Int Rep. 2018 May;3(3):619-24.[Abstract][Full Text]
56. 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.[Abstract][Full Text]
57. Jouret F, Lhommel R, Beguin C, et al. Positron-emission computed tomography in cyst infection diagnosis in patients with autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2011 Jul;6(7):1644-50.[Abstract]
58. 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.[Abstract]
59. Israel GM, Bosniak MA. An update of the Bosniak renal cyst classification system. Urology. 2005 Sep;66(3):484-8.[Abstract]
60. Graumann O, Osther SS, Osther PJ. Characterization of complex renal cysts: a critical evaluation of the Bosniak classification. Scand J Urol Nephrol. 2011 Mar;45(2):84-90.[Abstract]
61. Chen YZ, Gao Q, Zhao XZ, et al. Systematic review of TCF2 anomalies in renal cysts and diabetes syndrome/maturity onset diabetes of the young type 5. Chin Med J (Engl). 2010 Nov;123(22):3326-33.[Abstract]
62. Kelleher CL, McFann KK, Johnson AM, et al. Characteristics of hypertension in young adults with autosomal dominant polycystic kidney disease compared with the general U.S. population. Am J Hypertens. 2004 Nov;17(11 Pt 1):1029-34.[Abstract]
63. Sanchis IM, Shukoor S, Irazabal MV, et al. Presymptomatic screening for intracranial aneurysms in patients with autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2019 Aug 7;14(8):1151-60.[Abstract][Full Text]
64. Cagnazzo F, Gambacciani C, Morganti R, et al. Intracranial aneurysms in patients with autosomal dominant polycystic kidney disease: prevalence, risk of rupture, and management - a systematic review. Acta Neurochir (Wien). 2017 May;159(5):811-21.[Abstract]
65. Thompson BG, Brown RD Jr, Amin-Hanjani S, et al. Guidelines for the management of patients with unruptured intracranial aneurysms: a guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2015 Aug;46(8):2368-400.[Abstract][Full Text]
66. Belz MM, Fick-Brosnahan GM, Hughes RL, et al. Recurrence of intracranial aneurysms in autosomal-dominant polycystic kidney disease. Kidney Int. 2003 May;63(5):1824-30.[Abstract][Full Text]
67. Gibbs GF, Huston J 3rd, Qian Q, et al. Follow-up of intracranial aneurysms in autosomal-dominant polycystic kidney disease. Kidney Int. 2004 May;65(5):1621-7.[Abstract]
68. Schrier RW, Belz MM, Johnson AM, et al. Repeat imaging for intracranial aneurysms in patients with autosomal dominant polycystic kidney disease with initially negative studies: a prospective ten-year follow-up. J Am Soc Nephrol. 2004 Apr;15(4):1023-8.[Abstract][Full Text]
69. Chebib FT, Torres VE. Assessing risk of rapid progression in autosomal dominant polycystic kidney disease and special considerations for disease-modifying therapy. Am J Kidney Dis. 2021 Aug;78(2):282-92.[Abstract]
70. Gansevoort RT, Arici M, Benzing T, 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.[Abstract][Full Text]
71. Lanktree MB, Guiard E, Li W, et al. Intrafamilial variability of ADPKD. Kidney Int Rep. 2019 Jul;4(7):995-1003.[Abstract][Full Text]
72. Torres VE, Meijer E, Bae KT, et al. Rationale and design of the TEMPO (tolvaptan efficacy and safety in management of autosomal dominant polycystic kidney disease and its outcomes) 3-4 study. Am J Kidney Dis. 2011 May;57(5):692-9.[Abstract]
73. Torres VE, Chapman AB, Devuyst O, et al; TEMPO 3:4 Trial Investigators. Tolvaptan in patients with autosomal dominant polycystic kidney disease. N Engl J Med. 2012 Dec 20;367(25):2407-18.[Abstract][Full Text]
74. Torres VE, Chapman AB, Devuyst O, et al; REPRISE Trial Investigators. Tolvaptan in later-stage autosomal dominant polycystic kidney disease. N Engl J Med. 2017 Nov 16;377(20):1930-42.[Abstract]
75. Schrier RW, Abebe KZ, Perrone RD, et al; HALT-PKD Trial Investigators. Blood pressure in early autosomal dominant polycystic kidney disease. N Engl J Med. 2014 Dec 11;371(24):2255-66.[Abstract]
76. Torres VE, Abebe KZ, Chapman AB, et al; HALT-PKD Trial Investigators. Angiotensin blockade in late autosomal dominant polycystic kidney disease. N Engl J Med. 2014 Dec 11;371(24):2267-76.[Abstract]
77. Torres VE, Wilson DM, Burnett JC Jr, et al. Effect of inhibition of converting enzyme on renal hemodynamics and sodium management in polycystic kidney disease. Mayo Clin Proc. 1991 Oct;66(10):1010-7.[Abstract]
78. Watson ML, Macnicol AM, Allan PL, et al. Effects of angiotensin converting enzyme inhibition in adult polycystic kidney disease. Kidney Int. 1992 Jan;41(1):206-10.[Abstract]
79. Lantinga MA, Casteleijn NF, Geudens A, et al. Management of renal cyst infection in patients with autosomal dominant polycystic kidney disease: a systematic review. Nephrol Dial Transplant. 2017 Jan 1;32(1):144-50.[Abstract][Full Text]
80. European Medicines Agency. Quinolone- and fluoroquinolone-containing medicinal products. March 2019 [internet publication].[Full Text]
81. US Food and Drug Administration. FDA reinforces safety information about serious low blood sugar levels and mental health side effects with fluoroquinolone antibiotics; requires label changes. July 2018 [internet publication].[Full Text]
82. US Food and Drug Administration. FDA warns about increased risk of ruptures or tears in the aorta blood vessel with fluoroquinolone antibiotics in certain patients. December 2018 [internet publication].[Full Text]
83. Sallée 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.[Abstract][Full Text]
84. Millar M, Tanagho YS, Haseebuddin M, et al. Surgical cyst decortication in autosomal dominant polycystic kidney disease. J Endourol. 2013 May;27(5):528-34.[Abstract][Full Text]
85. Lifson BJ, Teichman JM, Hulbert JC. Role and long-term results of laparoscopic decortication in solitary cystic and autosomal dominant polycystic kidney disease. J Urol. 1998 Mar;159(3):702-5.[Abstract]
86. Tellman MW, Bahler CD, Shumate AM, et al. Management of pain in autosomal dominant polycystic kidney disease and anatomy of renal innervation. J Urol. 2015 May;193(5):1470-8.[Abstract]
87. Mufti UB, Nalagatla SK. Nephrolithiasis in autosomal dominant polycystic kidney disease. J Endourol. 2010 Oct;24(10):1557-61.[Abstract]
88. Mallett A, Patel M, Tunnicliffe DJ, et al. KHA-CARI autosomal dominant polycystic kidney disease guideline: management of renal stone disease. Semin Nephrol. 2015 Nov;35(6):603-6.[Abstract]
89. Xu Y, Bai Z, Ma D, et al. Laparoscopic ureterolithotomy, flexible ureteroscopic lithotripsy and percutaneous nephrolithotomy for treatment of upper urinary calculi in patients with autosomal dominant polycystic kidney disease. Clin Exp Nephrol. 2020 Sep;24(9):842-8.[Abstract]
90. Kalatharan V, Jandoc R, Grewal G, et al. Efficacy and safety of surgical kidney stone interventions in autosomal dominant polycystic kidney disease: a systematic review. Can J Kidney Health Dis. 2020;7:2054358120940433.[Abstract][Full Text]
91. Wiebers DO, Whisnant JP, Huston J 3rd, et al; International Study of Unruptured Intracranial Aneurysms Investigators. Unruptured intracranial aneurysms: natural history, clinical outcome, and risks of surgical and endovascular treatment. Lancet. 2003 Jul 12;362(9378):103-10.[Abstract]
92. Diringer MN. Management of aneurysmal subarachnoid hemorrhage. Crit Care Med. 2009 Feb;37(2):432-40.[Abstract][Full Text]
93. Salehipour M, Jalaeian H, Salahi H, et al. Are large nonfunctional kidneys risk factors for posttransplantation urinary tract infection in patients with end-stage renal disease due to autosomal dominant polycystic kidney disease? Transplant Proc. 2007 May;39(4):887-8.[Abstract]
94. Rozanski J, Kozlowska I, Myslak M, et al. Pretransplant nephrectomy in patients with autosomal dominant polycystic kidney disease. Transplant Proc. 2005 Mar;37(2):666-8.[Abstract]
95. Dunn MD, Portis AJ, Elbahnasy AM, et al. Laparoscopic nephrectomy in patients with end-stage renal disease and autosomal dominant polycystic kidney disease. Am J Kidney Dis. 2000 Apr;35(4):720-5.[Abstract]
96. Abbott KC, Agodoa LY. Polycystic kidney disease in patients on the renal transplant waiting list: trends in hematocrit and survival. BMC Nephrol. 2002 Aug 23;3:7.[Abstract][Full Text]
97. Shillingford JM, Murcia NS, Larson CH, et al. The mTOR pathway is regulated by polycystin-1, and its inhibition reverses renal cystogenesis in polycystic kidney disease. Proc Natl Acad Sci USA. 2006 Apr 4;103(14):5466-71.[Abstract][Full Text]
98. ClinicalTrials.gov. The ELiSA study: evaluation of lixivaptan in subjects with autosomal dominant polycystic kidney disease. November 2020 [internet publication].[Full Text]
99. van Keimpema L, Nevens F, Vanslembrouck R, et al. Lanreotide reduces the volume of polycystic liver: a randomized, double-blind, placebo-controlled trial. Gastroenterology. 2009 Nov;137(5):1661-8.e1-2.[Abstract]
100. Hogan MC, Masyuk TV, Page LJ, et al. Randomized clinical trial of long-acting somatostatin for autosomal dominant polycystic kidney and liver disease. J Am Soc Nephrol. 2010 Jun;21(6):1052-61.[Abstract]
101. Caroli A, Antiga L, Cafaro M, et al. Reducing polycystic liver volume in ADPKD: effects of somatostatin analogue octreotide. Clin J Am Soc Nephrol. 2010 May;5(5):783-9.[Abstract]
102. Caroli A, Perico N, Perna A, et al. 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 Nov 2;382(9903):1485-95.[Abstract]
103. Meijer E, Visser FW, van Aerts RMM, et al. Effect of lanreotide on kidney function in patients with autosomal dominant polycystic kidney disease: the DIPAK 1 randomized clinical trial. JAMA. 2018 Nov 20;320(19):2010-9.[Abstract][Full Text]
104. Yheskel M, Patel V. Therapeutic microRNAs in polycystic kidney disease. Curr Opin Nephrol Hypertens. 2017 Jul;26(4):282-9.[Abstract][Full Text]
105. Lee EC, Valencia T, Allerson C, et al. Discovery and preclinical evaluation of anti-miR-17 oligonucleotide RGLS4326 for the treatment of polycystic kidney disease. Nat Commun. 2019 Sep 12;10(1):4148.[Abstract][Full Text]
106. ClinicalTrials.gov. A study of RGLS4326 in patients with autosomal dominant polycystic kidney disease. April 2021 [internet publication].[Full Text]
107. Serra AL, Poster D, Kistler AD, et al. Sirolimus and kidney growth in autosomal dominant polycystic kidney disease. N Engl J Med. 2010 Aug 26;363(9):820-9.[Abstract]
108. Walz G, Budde K, Mannaa M, et al. Everolimus in patients with autosomal dominant polycystic kidney disease. N Engl J Med. 2010 Aug 26;363(9):830-40.[Abstract][Full Text]
109. Perico N, Antiga L, Caroli A, et al. Sirolimus therapy to halt the progression of ADPKD. J Am Soc Nephrol. 2010 Jun;21(6):1031-40.[Abstract]
110. Liu YM, Shao YQ, He Q. Sirolimus for treatment of autosomal-dominant polycystic kidney disease: a meta-analysis of randomized controlled trials. Transplant Proc. 2014 Jan-Feb;46(1):66-74.[Abstract][Full Text]
111. Chrispijn M, Gevers TJ, Hol JC, et al. Everolimus does not further reduce polycystic liver volume when added to long acting octreotide: results from a randomized controlled trial. J Hepatol. 2013 Jul;59(1):153-9.[Abstract]
112. Franz DN, Belousova E, Sparagana S, et al. Efficacy and safety of everolimus for subependymal giant cell astrocytomas associated with tuberous sclerosis complex (EXIST-1): a multicentre, randomised, placebo-controlled phase 3 trial. Lancet. 2013 Jan 12;381(9861):125-32.[Abstract]
113. Bissler JJ, Kingswood JC, Radzikowska E, et al. Everolimus for angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis (EXIST-2): a multicentre, randomised, double-blind, placebo-controlled trial. Lancet. 2013 Mar 9;381(9869):817-24.[Abstract]
114. Xue C, Dai B, Mei C. Long-term treatment with mammalian target of rapamycin inhibitor does not benefit patients with autosomal dominant polycystic kidney disease: a meta-analysis. Nephron Clin Pract. 2013;124(1-2):10-6.[Abstract]
115. Herrera GA. C-erb B-2 amplification in cystic renal disease. Kidney Int. 1991 Sep;40(3):509-13.[Abstract]
116. Wilson SJ, Amsler K, Hyink DP, et al. Inhibition of HER-2(neu/ErbB2) restores normal function and structure to polycystic kidney disease (PKD) epithelia. Biochim Biophys Acta. 2006 Jul;1762(7):647-55.[Abstract]
117. Chapman AB, Johnson AM, Gabow PA. Pregnancy outcome and its relationship to progression of renal failure in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 1994 Nov;5(5):1178-85.[Abstract][Full Text]
118. Taskapan H, Tam P, Au V, et al. Improvement in eGFR in patients with chronic kidney disease attending a nephrology clinic. Int Urol Nephrol. 2008;40(3):841-8.[Abstract]
119. Winkelmayer WC, Glynn RJ, Levin R, et al. Determinants of delayed nephrologist referral in patients with chronic kidney disease. Am J Kidney Dis. 2001 Dec;38(6):1178-84.[Abstract]
120. Chan MR, Dall AT, Fletcher KE, et al. Outcomes in patients with chronic kidney disease referred late to nephrologists: a meta-analysis. Am J Med. 2007 Dec;120(12):1063-70.[Abstract]
121. McBride L, Wilkinson C, Jesudason S. Management of autosomal dominant polycystic kidney disease (ADPKD) during pregnancy: risks and challenges. Int J Womens Health. 2020 May 25;12:409-22.[Abstract][Full Text]
122. Belibi FA, Wallace DP, Yamaguchi T, et al. The effect of caffeine on renal epithelial cells from patients with autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 2002 Nov;13(11):2723-9.[Abstract][Full Text]