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End-Stage Renal Disease in Patients with Autosomal Dominant Polycystic Kidney Disease

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Polycystic Kidney Disease

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) accounts for approximately 5–10% of end-stage renal disease (ESRD) cases and is the fourth leading cause for kidney failure worldwide. Half of patients with ADPKD reach ESRD by age 60 requiring the need for some form of renal replacement therapy. This chapter describes the changing incidence and prevalence of kidney dialysis and transplantation for patients with ADPKD and discusses the various issues around renal replacement therapy, focusing on aspects related to clinical management. The choice of dialysis modality, management of anemia and anticoagulation, hypertension, and ongoing renal and extrarenal complications of ADPKD are some potential concerns for those starting dialysis. Although hemodialysis and peritoneal dialysis are both appropriate options, kidney transplantation remains the preferred choice if possible. Transplant-related issues for patients with ADPKD include the selection of living kidney donors from relatives, the risk of alloimmunization, the indication and timing for native nephrectomy, and the choice of immunosuppression. Overall, the outcomes of patients with ADPKD are favorable on dialysis and kidney transplantation compared to other patients reaching ESRD.

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References

  1. Collins AJ, Foley RN, Herzog C, et al. US renal data system 2012 annual data report. Am J Kidney Dis. 2013;61(A7):e1–476.

    Google Scholar 

  2. Orskov B, Romming Sorensen V, Feldt-Rasmussen B, Strandgaard S. Improved prognosis in patients with autosomal dominant polycystic kidney disease in Denmark. Clin J Am Soc Nephrol. 2010;5:2034–9.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Haynes R, Kheradmand F, Winearls CG. Survival after starting renal replacement treatment in patients with autosomal dominant polycystic kidney disease: a single-centre 40-year study. Nephron Clin Pract. 2012;120:c42–7.

    Article  PubMed  Google Scholar 

  4. Perrone R, Ruthazer R, Terrin N. Survival after end-stage renal disease in autosomal dominant polycystic kidney disease: contribution of extrarenal complications to mortality. Am J Kidney Dis. 2001;38:777–84.

    Article  CAS  PubMed  Google Scholar 

  5. Wolfe R, Ashby V, Milford E, et al. Comparison of mortality in all patients on dialysis, patients on dialysis awaiting transplantation, and recipients of a first cadaveric transplant. New Eng J Med. 1999;341:1725–30.

    Article  CAS  PubMed  Google Scholar 

  6. 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;353:103–7.

    Article  CAS  PubMed  Google Scholar 

  7. Perl J, Bargman JM. The importance of residual kidney function for patients on dialysis: a critical review. Am J Kidney Dis. 2009;53:1068–81.

    Article  PubMed  Google Scholar 

  8. Pandya BK, Friede T, Williams JD. A comparison of peritonitis in polycystic and non-polycystic patients on peritoneal dialysis. Perit Dial Int. 2004;24:79–81.

    PubMed  Google Scholar 

  9. Hadimeri H, Johansson AC, Haraldsson B, Nyberg G. CAPD in patients with autosomal dominant polycystic kidney disease. Perit Dial Int. 1998;18:429–32.

    PubMed  CAS  Google Scholar 

  10. Kumar S, Fan SL, Raftery MJ, Yaqoob MM. Long term outcome of patients with autosomal dominant polycystic kidney diseases receiving peritoneal dialysis. Kidney Int. 2008;74:946–51.

    Article  PubMed  Google Scholar 

  11. Nolph KD, Cutler SJ, Steinberg SM, Novak JW. Continuous ambulatory peritoneal dialysis in the United States: a three-year study. Kidney Int. 1985;28:198–205.

    Article  CAS  PubMed  Google Scholar 

  12. Lobbedez T, Touam M, Evans D, Ryckelynck JP, Knebelman B, Verger C. Peritoneal dialysis in polycystic kidney disease patients. Report from the French peritoneal dialysis registry (RDPLF). Nephrol Dial Transplant. 2011;26:2332–9.

    Article  PubMed  Google Scholar 

  13. Portoles JM, Tato AM, Lopez-Sanchez P. Peritoneal dialysis for patients with polycystic kidney disease in Spain. Am J Kidney Dis. 2011;58:493. author reply 4

    Article  PubMed  Google Scholar 

  14. Li L, Szeto CC, Kwan BC, Chow KM, Leung CB, Kam-Tao LP. Peritoneal dialysis as the first-line renal replacement therapy in patients with autosomal dominant polycystic kidney disease. Am J Kidney Dis. 2011;57:903–7.

    Article  PubMed  Google Scholar 

  15. Christophe JL, van Ypersele de Strihou C, Pirson Y. Complications of autosomal dominant polycystic kidney disease in 50 haemodialysed patients. A case-control study. The U.C.L. collaborative group. Nephrol Dial Transplant. 1996;11:1271–6.

    Article  CAS  PubMed  Google Scholar 

  16. Yoo DJ, Agodoa L, Yuan CM, Abbott KC, Nee R. Risk of intracranial hemorrhage associated with autosomal dominant polycystic kidney disease in patients with end stage renal disease. BMC Nephrol. 2014;15:39.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Eckardt K, Mollmann M, Neumann R, et al. Erythropoietin in polycystic kidneys. J Clin Invest. 1989;84:1160–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Abbott KC, Agodoa LY. Polycystic kidney disease in patients on the renal transplant waiting list: trends in hematocrit and survival. BMC Nephrol. 2002;3:7.

    Article  PubMed  PubMed Central  Google Scholar 

  19. Shah A, Molnar MZ, Lukowsky LR, Zaritsky JJ, Kovesdy CP, Kalantar-Zadeh K. Hemoglobin level and survival in hemodialysis patients with polycystic kidney disease and the role of administered erythropoietin. Am J Hematol. 2012;87:833–6.

    Article  CAS  PubMed  Google Scholar 

  20. Gabow PA, Chapman AB, Johnson AM, et al. Renal structure and hypertension in autosomal dominant polycystic kidney disease. Kidney Int. 1990;38:1177–80.

    Article  CAS  PubMed  Google Scholar 

  21. Schrier RW, Abebe KZ, Perrone RD, et al. Blood pressure in early autosomal dominant polycystic kidney disease. N Engl J Med. 2014;371:2255–66.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Torres VE, Abebe KZ, Chapman AB, et al. Angiotensin blockade in late autosomal dominant polycystic kidney disease. N Engl J Med. 2014;371:2267–76.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Molnar MZ, Lukowsky LR, Streja E, et al. Blood pressure and survival in long-term hemodialysis patients with and without polycystic kidney disease. J Hypertens. 2010;28:2475–84.

    PubMed  PubMed Central  CAS  Google Scholar 

  24. Lukowsky LR, Molnar MZ, Zaritsky JJ, et al. Mineral and bone disorders and survival in hemodialysis patients with and without polycystic kidney disease. Nephrol Dial Transplant. 2012;27:2899–907.

    Article  CAS  PubMed  Google Scholar 

  25. U.S. Renal Data System. USRDS 2008 annual data report: atlas of chronic kidney disease and end-stage renal disease in the United States. National Institutes of Health, National Institute of Diabetes and Digestive and Kidney Diseases 2008; Bethesda.

    Google Scholar 

  26. Yabu JM, Anderson MW, Kim D, et al. Sensitization from transfusion in patients awaiting primary kidney transplant. Nephrol Dial Transplant. 2013;28:2908–18.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. Irazabal MV, Huston J 3rd, Kubly V, 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;6:1274–85.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Pei Y, Hwang YH, Conklin J, et al. Imaging-based diagnosis of autosomal dominant polycystic kidney disease. J Am Soc Nephrol JASN. 2015;26:746–53.

    Article  CAS  PubMed  Google Scholar 

  29. Kanaan N, Devuyst O, Pirson Y. Renal transplantation in autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 2014;10:455–65.

    Article  CAS  PubMed  Google Scholar 

  30. Patel P, Horsfield C, Compton F, Taylor J, Koffman G, Olsburgh J. Native nephrectomy in transplant patients with autosomal dominant polycystic kidney disease. Ann R Coll Surg Engl. 2011;93:391–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Martin AD, Mekeel KL, Castle EP, et al. Laparoscopic bilateral native nephrectomies with simultaneous kidney transplantation. BJU Int. 2012;110:E1003–7.

    Article  PubMed  Google Scholar 

  32. Neeff HP, Pisarski P, Tittelbach-Helmrich D, et al. One hundred consecutive kidney transplantations with simultaneous ipsilateral nephrectomy in patients with autosomal dominant polycystic kidney disease. Nephrol Dial Transplant. 2013;28:466–71.

    Article  PubMed  Google Scholar 

  33. Lucas SM, Mofunanya TC, Goggins WC, Sundaram CP. Staged nephrectomy versus bilateral laparoscopic nephrectomy in patients with autosomal dominant polycystic kidney disease. J Urol. 2010;184:2054–9.

    Article  PubMed  Google Scholar 

  34. Skauby MH, Oyen O, Hartman A, Leivestad T, Wadstrom J. Kidney transplantation with and without simultaneous bilateral native nephrectomy in patients with polycystic kidney disease: a comparative retrospective study. Transplantation. 2012;94:383–8.

    Article  PubMed  Google Scholar 

  35. Bennett WM. Peritransplant management of retained native kidneys in autosomal dominant polycystic kidney disease. Nephrol Dial Transplant. 2013;28:245–6.

    Article  PubMed  Google Scholar 

  36. Yamamoto T, Watarai Y, Kobayashi T, et al. Kidney volume changes in patients with autosomal dominant polycystic kidney disease after renal transplantation. Transplantation. 2012;93:794–8.

    Article  PubMed  Google Scholar 

  37. Tao Y, Kim J, Schrier RW, Edelstein CL. Rapamycin markedly slows disease progression in a rat model of polycystic kidney disease. J Am Soc Nephrol: JASN. 2005;16:46–51.

    Article  CAS  PubMed  Google Scholar 

  38. Wahl PR, Serra AL, Le Hir M, Molle KD, Hall MN, Wuthrich RP. Inhibition of mTOR with sirolimus slows disease progression in Han:SPRD rats with autosomal dominant polycystic kidney disease (ADPKD). Nephrol Dial Transplant. 2006;21:598–604.

    Article  CAS  PubMed  Google Scholar 

  39. 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 U S A. 2006;103:5466–71.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  40. Qian Q, Du H, King BF, et al. Sirolimus reduces polycystic liver volume in ADPKD patients. J Am Soc Nephrol: JASN. 2008;19:631–8.

    Article  CAS  PubMed  Google Scholar 

  41. Serra AL, Poster D, Kistler AD, et al. Sirolimus and kidney growth in autosomal dominant polycystic kidney disease. N Engl J Med. 2010;363:820–9.

    Article  CAS  PubMed  Google Scholar 

  42. Walz G, Budde K, Mannaa M, et al. Everolimus in patients with autosomal dominant polycystic kidney disease. N Engl J Med. 2010;363:830–40.

    Article  CAS  PubMed  Google Scholar 

  43. Sherstha R, McKinley C, Russ P, et al. Postmenopausal estrogen therapy selectively stimulates hepatic enlargement in women with autosomal dominant polycystic kidney disease. Hepatology. 1997;26:1282–6.

    PubMed  CAS  Google Scholar 

  44. Chauveau D, Fakhouri F, Grunfeld JP. Liver involvement in autosomal-dominant polycystic kidney disease: therapeutic dilemma. J Am Soc Nephrol: JASN. 2000;11:1767–75.

    PubMed  CAS  Google Scholar 

  45. Hadimeri H, Norden G, Friman S, Nyberg G. Autosomal dominant polycystic kidney disease in a kidney transplant population. Nephrol Dial Transplant. 1997;12:1431–6.

    Article  CAS  PubMed  Google Scholar 

  46. Jacquet A, Pallet N, Kessler M, et al. Outcomes of renal transplantation in patients with autosomal dominant polycystic kidney disease: a nationwide longitudinal study. Transpl Int: Off J Eur Soc Organ Transplant. 2011;24:582–7.

    Article  Google Scholar 

  47. Oliveras A, Roquer J, Puig JM, et al. Stroke in renal transplant recipients: epidemiology, predictive risk factors and outcome. Clin Transpl. 2003;17:1–8.

    Article  Google Scholar 

  48. Abedini S, Holme I, Fellstrom B, et al. Cerebrovascular events in renal transplant recipients. Transplantation. 2009;87:112–7.

    Article  PubMed  Google Scholar 

  49. Niemczyk M, Niemczyk S, Paczek L. Autosomal dominant polycystic kidney disease and transplantation. Ann Transplant: Q Pol Transplant Soc. 2009;14:86–90.

    Google Scholar 

  50. Stiasny B, Ziebell D, Graf S, Hauser IA, Schulze BD. Clinical aspects of renal transplantation in polycystic kidney disease. Clin Nephrol. 2002;58:16–24.

    Article  CAS  PubMed  Google Scholar 

  51. Wetmore JB, Calvet JP, Yu AS, et al. Polycystic kidney disease and cancer after renal transplantation. J Am Soc Nephrol: JASN. 2014;25:2335–41.

    Article  PubMed  Google Scholar 

  52. Vichot AA, Geller DS, Perazella MA. Progression of polycystic kidney disease in a kidney transplant. Kidney Int. 2013;83:533.

    Article  PubMed  Google Scholar 

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Correspondence to Ahsan Alam MD, MS, FRCPC .

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Al Khunaizi, A., Alam, A. (2018). End-Stage Renal Disease in Patients with Autosomal Dominant Polycystic Kidney Disease. In: Cowley, Jr., B., Bissler, J. (eds) Polycystic Kidney Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-7784-0_14

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  • DOI: https://doi.org/10.1007/978-1-4939-7784-0_14

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