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  • Review Article
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Renal transplantation in autosomal dominant polycystic kidney disease

Nature Reviews Nephrology volume 10, pages 455–465 (2014)Cite this article

Subjects

Key Points

  • In patients with autosomal dominant polycystic kidney disease (ADPKD), rates of patient and graft survival following kidney transplantation are excellent

  • Prophylactic native nephrectomy is restricted to patients with severe pain, early satiety, recurrent bleeding, infections or stones and those in whom space must be made to implant the graft

  • Patients with liver involvement require pretransplant imaging; combined liver–kidney transplantation should be considered in cases of symptomatic hepatomegaly or recurrent cholangitis if the glomerular filtration rate is ≤30 ml/min/1.73 m2

  • General recommendations for the screening of intracranial aneurysms in patients with ADPKD should be used to select patients for pretransplant screening

  • Genetic testing should be done in living related-donor candidates aged <30 years when imaging is normal or equivocal

  • No reason exists to preferentially use mammalian target of rapamycin inhibitors as immunosuppressive agents in kidney transplant recipients with ADPKD

Abstract

In patients with autosomal dominant polycystic kidney disease (ADPKD) evaluated for kidney transplantation, issues related to native nephrectomy, cystic liver involvement, screening for intracranial aneurysms and living-related kidney donation deserve special consideration. Prophylactic native nephrectomy is restricted to patients with a history of cyst infection or recurrent haemorrhage or to those in whom space must be made to implant the graft. Patients with liver involvement require pretransplant imaging. Selection of patients for pretransplant screening of intracranial aneurysms should follow the general recommendations for patients with ADPKD. In living related-donor candidates aged <30 years and at-risk of ADPKD, molecular genetic testing should be carried out when ultrasonography and MRI findings are normal or equivocal. After kidney transplantation, patient and graft survival rates are excellent and the volume of native kidneys decreases. However, liver cysts continue to grow and treatment with a somatostatin analogue should be considered in patients with massive cyst involvement. Cerebrovascular events have a marginal effect on post-transplant morbidity and mortality. An increased risk of new-onset diabetes mellitus and nonmelanoma skin cancers has been reported, but several studies have challenged these findings. Finally, no data currently support the preferential use of mammalian target of rapamycin inhibitors as immunosuppressive agents in transplant recipients with ADPKD.

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Figure 1: Criteria for kidney donation by family members of patients with ADPKD.
Figure 2: Liver cyst infection in a patient with autosomal polycystic kidney disease.
Figure 3: Pretransplant screening and follow-up monitoring for intracranial aneurysms in patients with ADPKD aged <60 years.
Figure 4: Renal and nonrenal complications in kidney transplant recipients with ADPKD.

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References

  1. Torres, V. E., Harris, P. C. & Pirson, Y. Autosomal dominant polycystic kidney disease. Lancet 369, 1287–1301 (2007).

    Article  PubMed  Google Scholar 

  2. Harris, P. C. & Torres, V. E. Polycystic kidney disease. Annu. Rev. Med. 60, 321–337 (2009).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Ong, A. C. & Harris, P. C. Molecular pathogenesis of ADPKD: the polycystin complex gets complex. Kidney Int. 67, 1234–1247 (2005).

    Article  CAS  PubMed  Google Scholar 

  4. Iglesias, C. G. et al. Epidemiology of adult polycystic kidney disease, Olmsted County, Minnesota: 1935–1980 Am. J. Kidney Dis. 2, 630–639 (1983).

    Article  CAS  PubMed  Google Scholar 

  5. Davies, F. et al. Polycystic kidney disease re-evaluated: a population-based study. Q. J. Med. 79, 477–485 (1991).

    CAS  PubMed  Google Scholar 

  6. Collins, A. J. et al. Excerpts from the United States Renal Data System 2004 annual data report: atlas of end-stage renal disease in the United States. Am. J. Kidney Dis. 45 (Suppl. 1), A5–A7, S1–S280 (2005).

    Article  PubMed  Google Scholar 

  7. Pirson, Y. Extrarenal manifestations of autosomal dominant polycystic kidney disease. Adv. Chronic. Kidney Dis. 17, 173–180 (2010).

    Article  PubMed  Google Scholar 

  8. Luciano, R. L. & Dahl, N. K. Extra-renal manifestations of autosomal dominant polycystic kidney disease (ADPKD): considerations for routine screening and management. Nephrol. Dial. Transplant. 29, 247–254 (2014).

    Article  PubMed  Google Scholar 

  9. Torra, R. et al. Prevalence of cysts in seminal tract and abnormal semen parameters in patients with autosomal dominant polycystic kidney disease. Clin. J. Am. Soc. Nephrol. 3, 790–793 (2008).

    Article  PubMed  PubMed Central  Google Scholar 

  10. Kumar, S., Adeva, M., King, B. F., Kamath, P. S. & Torres, V. E. Duodenal diverticulosis in autosomal dominant polycystic kidney disease. Nephrol. Dial. Transplant. 21, 3576–3578 (2006).

    Article  PubMed  Google Scholar 

  11. Driscoll, J. A., Bhalla, S., Liapis, H., Ibricevic, A. & Brody, S. L. Autosomal dominant polycystic kidney disease is associated with an increased prevalence of radiographic bronchiectasis. Chest 133, 1181–1188 (2008).

    Article  PubMed  Google Scholar 

  12. Harris, P. C. 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. 17, 3013–3019 (2006).

    Article  CAS  PubMed  Google Scholar 

  13. Dobin, A. et al. Segregation analysis of autosomal dominant polycystic kidney disease. Genet. Epidemiol. 10, 189–200 (1993).

    Article  CAS  PubMed  Google Scholar 

  14. The International Polycystic Kidney Disease Consortium. Polycystic kidney disease: the complete structure of the PKD1 gene and its protein. Cell 81, 289–298 (1995).

  15. Hughes, J. et al. The polycystic kidney disease 1 (PKD1) gene encodes a novel protein with multiple cell recognition domains. Nat. Genet. 10, 151–160 (1995).

    Article  CAS  PubMed  Google Scholar 

  16. Mochizuki, T. et al. PKD2, a gene for polycystic kidney disease that encodes an integral membrane protein. Science 272, 1339–1342 (1996).

    Article  CAS  PubMed  Google Scholar 

  17. Yoder, B. K. Role of primary cilia in the pathogenesis of polycystic kidney disease. J. Am. Soc. Nephrol. 18, 1381–1388 (2007).

    Article  CAS  PubMed  Google Scholar 

  18. Hateboer, N. et al. Comparison of phenotypes of polycystic kidney disease types 1 and 2. European PKD1-PKD2 Study Group. Lancet 353, 103–107 (1999).

    Article  CAS  PubMed  Google Scholar 

  19. Cornec-Le, G. E. et al. Type of PKD1 mutation influences renal outcome in ADPKD. J. Am. Soc. Nephrol. 24, 1006–1013 (2013).

    Article  CAS  Google Scholar 

  20. Paterson, A. D. 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, 755–762 (2005).

    Article  PubMed  Google Scholar 

  21. Fain, P. R. et al. Modifier genes play a significant role in the phenotypic expression of PKD1. Kidney Int. 67, 1256–1267 (2005).

    Article  CAS  PubMed  Google Scholar 

  22. Magistroni, R. et al. Genotype-renal function correlation in type 2 autosomal dominant polycystic kidney disease. J. Am. Soc. Nephrol. 14, 1164–1174 (2003).

    Article  PubMed  Google Scholar 

  23. Persu, A. et al. Comparison between siblings and twins supports a role for modifier genes in ADPKD. Kidney Int. 66, 2132–2136 (2004).

    Article  CAS  PubMed  Google Scholar 

  24. Alam, A. & Perrone, R. D. Management of ESRD in patients with autosomal dominant polycystic kidney disease. Adv. Chronic. Kidney Dis. 17, 164–172 (2010).

    Article  PubMed  Google Scholar 

  25. Haynes, R., Kheradmand, F. & Winearls, C. G. Survival after starting renal replacement treatment in patients with autosomal dominant polycystic kidney disease: a single-centre 40-year study. Nephron Clin. Pract. 120, c42–c47 (2012).

    Article  PubMed  Google Scholar 

  26. Mosconi, G. et al. Renal transplant in patients with polycystic disease: the Italian experience. Transplant. Proc. 45, 2635–2640 (2013).

    Article  CAS  PubMed  Google Scholar 

  27. Jacquet, A. et al. Outcomes of renal transplantation in patients with autosomal dominant polycystic kidney disease: a nationwide longitudinal study. Transpl. Int. 24, 582–587 (2011).

    Article  PubMed  Google Scholar 

  28. 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. 5, 2034–2039 (2010).

    Article  PubMed  PubMed Central  Google Scholar 

  29. Perrone, R. D., Ruthazer, R. & Terrin, N. C. Survival after end-stage renal disease in autosomal dominant polycystic kidney disease: contribution of extrarenal complications to mortality. Am. J. Kidney Dis. 38, 777–784 (2001).

    Article  CAS  PubMed  Google Scholar 

  30. Spithoven, E. M. et al. Renal replacement therapy for ADPKD in Europe: prevalence and survival. An analysis of data from the ERA-EDTA Registry. Nephrol. Dial. Transplant. (in press).

  31. Kirkman, M. A. et al. Native nephrectomy for autosomal dominant polycystic kidney disease: before or after kidney transplantation? BJU Int. 108, 590–594 (2011).

    Article  PubMed  Google Scholar 

  32. Bennett, W. M. Peritransplant management of retained native kidneys in autosomal dominant polycystic kidney disease. Nephrol. Dial. Transplant. 28, 245–246 (2013).

    Article  PubMed  Google Scholar 

  33. Kasiske, B. L. et al. The evaluation of renal transplant candidates: clinical practice guidelines. Patient Care and Education Committee of the American Society of Transplant Physicians. J. Am. Soc. Nephrol. 6, 1–34 (1995).

    CAS  PubMed  Google Scholar 

  34. European Renal Best Practice Transplantation Guideline Development Group. ERBP Guideline on the Management and Evaluation of the Kidney Donor and Recipient. Nephrol. Dial. Transplant. 28 (Suppl. 2), ii1–ii71 (2013).

  35. Yarimizu, S. N. et al. Mortality and morbidity in pretransplant bilateral nephrectomy: analysis of 305 cases. Urology 12, 55–58 (1978).

    Article  CAS  PubMed  Google Scholar 

  36. Mendelssohn, D. C., Harding, M. E., Cardella, C. J., Cook, G. T. & Uldall, P. R. Management of end-stage autosomal dominant polycystic kidney disease with hemodialysis and transplantation. Clin. Nephrol. 30, 315–319 (1988).

    CAS  PubMed  Google Scholar 

  37. Patel, P. et al. Native nephrectomy in transplant patients with autosomal dominant polycystic kidney disease. Ann. R. Coll. Surg. Engl. 93, 391–395 (2011).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  38. Pirson, Y. in Oxford Textbook of Clinical Nephrology (Ed. Davison, A. M.) 2304–2323 (Liepman, 2005).

    Google Scholar 

  39. Neeff, H. P. et al. One hundred consecutive kidney transplantations with simultaneous ipsilateral nephrectomy in patients with autosomal dominant polycystic kidney disease. Nephrol. Dial. Transplant. 28, 466–471 (2013).

    Article  PubMed  Google Scholar 

  40. Cornelis, F. et al. Embolization of polycystic kidneys as an alternative to nephrectomy before renal transplantation: a pilot study. Am. J. Transplant. 10, 2363–2369 (2010).

    Article  CAS  PubMed  Google Scholar 

  41. Wagner, M. D., Prather, J. C. & Barry, J. M. Selective, concurrent bilateral nephrectomies at renal transplantation for autosomal dominant polycystic kidney disease. J. Urol. 177, 2250–2254 (2007).

    Article  PubMed  Google Scholar 

  42. Kramer, A. et al. Simultaneous bilateral native nephrectomy and living donor renal transplantation are successful for polycystic kidney disease: the University of Maryland experience. J. Urol. 181, 724–728 (2009).

    Article  PubMed  Google Scholar 

  43. Martin, A. D. et al. Laparoscopic bilateral native nephrectomies with simultaneous kidney transplantation. BJU Int. 110, E1003–E1007 (2012).

    Article  PubMed  Google Scholar 

  44. Verhoest, G. et al. Transperitoneal laparoscopic nephrectomy for autosomal dominant polycystic kidney disease. JSLS 16, 437–442 (2012).

    Article  PubMed  PubMed Central  Google Scholar 

  45. Gevers, T. J. & Drenth, J. P. Diagnosis and management of polycystic liver disease. Nat. Rev. Gastroenterol. Hepatol. 10, 101–108 (2013).

    Article  CAS  PubMed  Google Scholar 

  46. Strazzabosco, M. & Somlo, S. Polycystic liver diseases: congenital disorders of cholangiocyte signaling. Gastroenterology 140, 1855–1859. e1 (2011).

    Article  CAS  PubMed  Google Scholar 

  47. Bae, K. T. et al. Magnetic resonance imaging evaluation of hepatic cysts in early autosomal-dominant polycystic kidney disease: the Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease cohort. Clin. J. Am. Soc. Nephrol. 1, 64–69 (2006).

    Article  PubMed  Google Scholar 

  48. Alvaro, D. et al. Estrogens and insulin-like growth factor 1 modulate neoplastic cell growth in human cholangiocarcinoma. Am. J. Pathol. 169, 877–888 (2006).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  49. Chapman, A. B. Cystic disease in women: clinical characteristics and medical management. Adv. Ren. Replace. Ther. 10, 24–30 (2003).

    Article  PubMed  Google Scholar 

  50. Sherstha, R. et al. Postmenopausal estrogen therapy selectively stimulates hepatic enlargement in women with autosomal dominant polycystic kidney disease. Hepatology 26, 1282–1286 (1997).

    CAS  PubMed  Google Scholar 

  51. Caroli, A. et al. Reducing polycystic liver volume in ADPKD: effects of somatostatin analogue octreotide. Clin. J. Am. Soc. Nephrol. 5, 783–789 (2010).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  52. Hogan, M. C. et al. Randomized clinical trial of long-acting somatostatin for autosomal dominant polycystic kidney and liver disease. J. Am. Soc. Nephrol. 21, 1052–1061 (2010).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  53. van Keimpema, L. et al. Lanreotide reduces the volume of polycystic liver: a randomized, double-blind, placebo-controlled trial. Gastroenterology 137, 1661–1668 (2009).

    Article  CAS  PubMed  Google Scholar 

  54. Grunfeld, J. P. et al. Liver changes and complications in adult polycystic kidney disease. Adv. Nephrol. Necker Hosp. 14, 1–20 (1985).

    CAS  PubMed  Google Scholar 

  55. Jouret, F. et al. Positron-emission computed tomography in cyst infection diagnosis in patients with autosomal dominant polycystic kidney disease. Clin. J. Am. Soc. Nephrol. 6, 1644–1650 (2011).

    Article  PubMed  Google Scholar 

  56. Bleeker-Rovers, C. P. et al. Diagnosis of renal and hepatic cyst infections by 18F-fluorodeoxyglucose positron emission tomography in autosomal dominant polycystic kidney disease. Am. J. Kidney Dis. 41, E18–E21 (2003).

    Article  PubMed  Google Scholar 

  57. Jouret, F. et al. Diagnosis of cyst infection in patients with autosomal dominant polycystic kidney disease: attributes and limitations of the current modalities. Nephrol. Dial. Transplant. 2, 3746–3751 (2012).

    Article  CAS  Google Scholar 

  58. Sallee, M. et al. Cyst infections in patients with autosomal dominant polycystic kidney disease. Clin. J. Am. Soc. Nephrol. 4, 1183–1189 (2009).

    Article  PubMed  PubMed Central  Google Scholar 

  59. Telenti, A. et al. Hepatic cyst infection in autosomal dominant polycystic kidney disease. Mayo Clin. Proc. 65, 933–942 (1990).

    Article  CAS  PubMed  Google Scholar 

  60. Chauveau, D., Fakhouri, F. & Grunfeld, J. P. Liver involvement in autosomal-dominant polycystic kidney disease: therapeutic dilemma. J. Am. Soc. Nephrol. 11, 1767–1775 (2000).

    CAS  PubMed  Google Scholar 

  61. Kanaan, N., Goffin, E., Pirson, Y., Devuyst, O. & Hassoun, Z. Carbohydrate antigen 19-9 as a diagnostic marker for hepatic cyst infection in autosomal dominant polycystic kidney disease. Am. J. Kidney Dis. 55, 916–922 (2010).

    Article  PubMed  Google Scholar 

  62. Waanders, E. et al. Carbohydrate antigen 19-9 is extremely elevated in polycystic liver disease. Liver Int. 29, 1389–1395 (2009).

    Article  CAS  PubMed  Google Scholar 

  63. van, K. L. et al. Patients with isolated polycystic liver disease referred to liver centres: clinical characterization of 137 cases. Liver Int. 31, 92–98 (2011).

    Article  Google Scholar 

  64. Torres, V. E. & Harris, P. C. Autosomal dominant polycystic kidney disease: the last 3 years. Kidney Int. 76, 149–168 (2009).

    Article  PubMed  PubMed Central  Google Scholar 

  65. Hoevenaren, I. A. et al. Polycystic liver: clinical characteristics of patients with isolated polycystic liver disease compared with patients with polycystic liver and autosomal dominant polycystic kidney disease. Liver Int. 28, 264–270 (2008).

    Article  PubMed  Google Scholar 

  66. Van, K. L. & Hockerstedt, K. Treatment of polycystic liver disease. Br. J. Surg. 96, 1379–1380 (2009).

    Article  Google Scholar 

  67. Ruggenenti, P. et al. Safety and efficacy of long-acting somatostatin treatment in autosomal-dominant polycystic kidney disease. Kidney Int. 68, 206–216 (2005).

    Article  CAS  PubMed  Google Scholar 

  68. Hogan, M. C. et al. Somatostatin analog therapy for severe polycystic liver disease: results after 2 years. Nephrol. Dial. Transplant. 27, 3532–3539 (2012).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  69. Russell, R. T. & Pinson, C. W. Surgical management of polycystic liver disease. World J. Gastroenterol. 13, 5052–5059 (2007).

    Article  PubMed  PubMed Central  Google Scholar 

  70. Schnelldorfer, T., Torres, V. E., Zakaria, S., Rosen, C. B. & Nagorney, D. M. Polycystic liver disease: a critical appraisal of hepatic resection, cyst fenestration, and liver transplantation. Ann. Surg. 250, 112–118 (2009).

    Article  PubMed  Google Scholar 

  71. Nadim, M. K., Davis, C. L., Sung, R., Kellum, J. A. & Genyk, Y. S. Simultaneous liver–kidney transplantation: a survey of US transplant centers. Am. J. Transplant. 12, 3119–3127 (2012).

    Article  CAS  PubMed  Google Scholar 

  72. Davis, C. L. Impact of pretransplant renal failure: when is listing for kidney-liver indicated? Liver Transpl. 11, S35–S44 (2005).

    Article  Google Scholar 

  73. Drenth, J. P., Chrispijn, M., Nagorney, D. M., Kamath, P. S. & Torres, V. E. Medical and surgical treatment options for polycystic liver disease. Hepatology 52, 2223–2230 (2010).

    Article  PubMed  Google Scholar 

  74. Fong, T. L., Bunnapradist, S., Jordan, S. C., Selby, R. R. & Cho, Y. W. Analysis of the United Network for Organ Sharing database comparing renal allografts and patient survival in combined liver–kidney transplantation with the contralateral allografts in kidney alone or kidney–pancreas transplantation. Transplantation 76, 348–353 (2003).

    Article  PubMed  Google Scholar 

  75. Kirchner, G. I. et al. Outcome and quality of life in patients with polycystic liver disease after liver or combined liver–kidney transplantation. Liver Transpl. 12, 1268–1277 (2006).

    Article  PubMed  Google Scholar 

  76. Torra, R. et al. Autosomal dominant polycystic kidney disease with anticipation and Caroli's disease associated with a PKD1 mutation. Kidney Int. 52, 33–38 (1997).

    Article  CAS  PubMed  Google Scholar 

  77. Shedda, S. & Robertson, A. Caroli's syndrome and adult polycystic kidney disease. ANZ J. Surg. 77, 292–294 (2007).

    Article  PubMed  Google Scholar 

  78. Jordon, D., Harpaz, N. & Thung, S. N. Caroli's disease and adult polycystic kidney disease: a rarely recognized association. Liver 9, 30–35 (1989).

    Article  CAS  PubMed  Google Scholar 

  79. Aguilar, M., Meterissian, S., Levesque, S. & Andonian, S. Nephrectomy in patients with Caroli's and ADPKD may be associated with increased morbidity. Can. Urol. Assoc. J. 5, E19–E22 (2011).

    Article  PubMed  PubMed Central  Google Scholar 

  80. Habib, S. et al. Caroli's disease and orthotopic liver transplantation. Liver Transpl. 12, 416–421 (2006).

    Article  PubMed  Google Scholar 

  81. Yamamoto, T. et al. Kidney volume changes in patients with autosomal dominant polycystic kidney disease after renal transplantation. Transplantation 93, 794–798 (2012).

    Article  PubMed  Google Scholar 

  82. Temmerman, F. et al. Safety and efficacy of different lanreotide doses in the treatment of polycystic liver disease: pooled analysis of individual patient data. Aliment. Pharmacol. Ther. 38, 397–406 (2013).

    Article  CAS  PubMed  Google Scholar 

  83. Pirson, Y., Chauveau, D. & Torres, V. Management of cerebral aneurysms in autosomal dominant polycystic kidney disease. J. Am. Soc. Nephrol. 13, 269–276 (2002).

    PubMed  Google Scholar 

  84. Irazabal, M. 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. 6, 1274–1285 (2011).

    Article  PubMed  PubMed Central  Google Scholar 

  85. Chauveau, D. et al. Intracranial aneurysms in autosomal dominant polycystic kidney disease. Kidney Int. 45, 1140–1146 (1994).

    Article  CAS  PubMed  Google Scholar 

  86. Rinkel, G. J. Natural history, epidemiology and screening of unruptured intracranial aneurysms. J. Neuroradiol. 35, 99–103 (2008).

    Article  CAS  PubMed  Google Scholar 

  87. Broderick, J. P. et al. Greater rupture risk for familial as compared to sporadic unruptured intracranial aneurysms. Stroke 40, 1952–1957 (2009).

    Article  PubMed  PubMed Central  Google Scholar 

  88. Wiebers, D. O. et al. Unruptured intracranial aneurysms: natural history, clinical outcome, and risks of surgical and endovascular treatment. Lancet 362, 103–110 (2003).

    Article  PubMed  Google Scholar 

  89. Ishibashi, T. et al. Unruptured intracranial aneurysms: incidence of rupture and risk factors. Stroke 40, 313–316 (2009).

    Article  PubMed  Google Scholar 

  90. Vlak, M. H., Algra, A., Brandenburg, R. & Rinkel, G. J. Prevalence of unruptured intracranial aneurysms, with emphasis on sex, age, comorbidity, country, and time period: a systematic review and meta-analysis. Lancet Neurol. 10, 626–636 (2011).

    Article  PubMed  Google Scholar 

  91. Li, L. M., Bulters, D. O. & Kirollos, R. W. A mathematical model of utility for single screening of asymptomatic unruptured intracranial aneurysms at the age of 50 years. Acta Neurochir. (Wien.) 154, 1145–1152 (2012).

    Article  Google Scholar 

  92. Schrier, R. W. 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. 15, 1023–1028 (2004).

    Article  PubMed  Google Scholar 

  93. Belz, M. M. et al. Recurrence of intracranial aneurysms in autosomal-dominant polycystic kidney disease. Kidney Int. 63, 1824–1830 (2003).

    Article  PubMed  Google Scholar 

  94. Gibbs, G. F. et al. Follow-up of intracranial aneurysms in autosomal-dominant polycystic kidney disease. Kidney Int. 65, 1621–1627 (2004).

    Article  PubMed  Google Scholar 

  95. Oliveras, A. et al. Stroke in renal transplant recipients: epidemiology, predictive risk factors and outcome. Clin. Transplant. 17, 1–8 (2003).

    Article  PubMed  Google Scholar 

  96. Abedini, S. et al. Cerebrovascular events in renal transplant recipients. Transplantation 87, 112–117 (2009).

    Article  PubMed  Google Scholar 

  97. Hadimeri, H., Norden, G., Friman, S. & Nyberg, G. Autosomal dominant polycystic kidney disease in a kidney transplant population. Nephrol. Dial. Transplant. 12, 1431–1436 (1997).

    Article  CAS  PubMed  Google Scholar 

  98. Fick, G. M., Johnson, A. M., Hammond, W. S. & Gabow, P. A. Causes of death in autosomal dominant polycystic kidney disease. J. Am. Soc. Nephrol. 5, 2048–2056 (1995).

    CAS  PubMed  Google Scholar 

  99. Yoo, D. J. Agodoa, L., Yuan, C. M., Abbott, K. C. & Nee, R. Risk of intracranial hemorrhage associated with autosomal dominant polycystic kidney disease in patients with end stage renal disease. BMC Nephrology 15, 39–46 (2014).

    Article  PubMed  PubMed Central  Google Scholar 

  100. Ravine, D. et al. Evaluation of ultrasonographic diagnostic criteria for autosomal dominant polycystic kidney disease 1. Lancet 343, 824–827 (1994).

    Article  CAS  PubMed  Google Scholar 

  101. Pei, Y. et al. Unified criteria for ultrasonographic diagnosis of ADPKD. J. Am. Soc. Nephrol. 20, 205–212 (2009).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  102. Peral, B. et al. Evidence of linkage disequilibrium in the Spanish polycystic kidney disease I population. Am. J. Hum. Genet. 54, 899–908 (1994).

    CAS  PubMed  PubMed Central  Google Scholar 

  103. Zhao, X. et al. Molecular diagnostics in autosomal dominant polycystic kidney disease: utility and limitations. Clin. J. Am. Soc. Nephrol. 3, 146–152 (2008).

    Article  PubMed  PubMed Central  Google Scholar 

  104. Rossetti, S. et al. Comprehensive molecular diagnostics in autosomal dominant polycystic kidney disease. J. Am. Soc. Nephrol. 18, 2143–2160 (2007).

    Article  CAS  PubMed  Google Scholar 

  105. Garcia-Gonzalez, M. A. et al. Evaluating the clinical utility of a molecular genetic test for polycystic kidney disease. Mol. Genet. Metab. 92, 160–167 (2007).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  106. Harris, P. C. & Rossetti, S. Molecular diagnostics for autosomal dominant polycystic kidney disease. Nat. Rev. Nephrol. 6, 197–206 (2010).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  107. Audrezet, M. P. et al. Autosomal dominant polycystic kidney disease: comprehensive mutation analysis of PKD1 and PKD2 in 700 unrelated patients. Hum. Mutat. 33, 1239–1250 (2012).

    Article  CAS  PubMed  Google Scholar 

  108. Barua, M. et al. Family history of renal disease severity predicts the mutated gene in ADPKD. J. Am. Soc. Nephrol. 20, 1833–1838 (2009).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  109. Fitzpatrick, P. M. et al. Long-term outcome of renal transplantation in autosomal dominant polycystic kidney disease. Am. J. Kidney Dis. 15, 535–543 (1990).

    Article  CAS  PubMed  Google Scholar 

  110. Florijn, K. W., Chang, P. C., van der Woude, F. J., van Bockel, J. H. & van Saase, J. L. Long-term cardiovascular morbidity and mortality in autosomal dominant polycystic kidney disease patients after renal transplantation. Transplantation 57, 73–81 (1994).

    Article  CAS  PubMed  Google Scholar 

  111. Valderrábano, F., Jones, E. H. & Mallick, N. P. Report on management of renal failure in Europe, XXIV, 1993. Nephrol. Dial.Transplant. 10 (Suppl. 5), 1–25 (1995).

    Article  PubMed  Google Scholar 

  112. Pirson, Y., Christophe, J. L. & Goffin, E. Outcome of renal replacement therapy in autosomal dominant polycystic kidney disease. Nephrol. Dial. Transplant. 11 (Suppl. 6), 24–28 (1996).

    Article  PubMed  Google Scholar 

  113. Thaysen, J. H., Thomsen, H. S., Sass, A. & Kristensen, J. K. Volume changes in polycystic kidneys during chronic dialysis and after renal transplantation. Acta Med. Scand. 217, 197–204 (1985).

    Article  CAS  PubMed  Google Scholar 

  114. Ishikawa, I., Tateishi, K., Kitada, H. & Shinoda, A. Regression of adult type polycystic kidneys during chronic intermittent hemodialysis. Is it a universal phenomenon? Nephron 36, 147 (1984).

    Article  CAS  PubMed  Google Scholar 

  115. Ho, T. A. et al. Autosomal dominant polycystic kidney disease is associated with central and nephrogenic defects in osmoregulation. Kidney Int. 82, 1121–1129 (2012).

    Article  CAS  PubMed  Google Scholar 

  116. Ecder, T. & Schrier, R. W. Hypertension in autosomal-dominant polycystic kidney disease: early occurrence and unique aspects. J. Am. Soc. Nephrol. 12, 194–200 (2001).

    CAS  PubMed  Google Scholar 

  117. Ecder, T. & Schrier, R. W. Cardiovascular abnormalities in autosomal-dominant polycystic kidney disease. Nat. Rev. Nephrol. 5, 221–228 (2009).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  118. Hossack, K. F., Leddy, C. L., Johnson, A. M., Schrier, R. W. & Gabow, P. A. Echocardiographic findings in autosomal dominant polycystic kidney disease. N. Engl. J. Med. 319, 907–912 (1988).

    Article  CAS  PubMed  Google Scholar 

  119. Lumiaho, A. et al. Mitral valve prolapse and mitral regurgitation are common in patients with polycystic kidney disease type 1. Am. J. Kidney Dis. 38, 1208–1216 (2001).

    Article  CAS  PubMed  Google Scholar 

  120. Qian, Q., Hartman, R. P., King, B. F. & Torres, V. E. Increased occurrence of pericardial effusion in patients with autosomal dominant polycystic kidney disease. Clin. J. Am. Soc. Nephrol. 2, 1223–1227 (2007).

    Article  PubMed  Google Scholar 

  121. Ducloux, D. et al. Polycystic kidney disease as a risk factor for post-transplant diabetes mellitus. Nephrol. Dial. Transplant. 14, 1244–1246 (1999).

    Article  CAS  PubMed  Google Scholar 

  122. de Mattos, A. M. et al. Autosomal-dominant polycystic kidney disease as a risk factor for diabetes mellitus following renal transplantation. Kidney Int. 67, 714–720 (2005).

    Article  PubMed  Google Scholar 

  123. Hamer, R. A., Chow, C. L., Ong, A. C. & McKane, W. S. Polycystic kidney disease is a risk factor for new-onset diabetes after transplantation. Transplantation 83, 36–40 (2007).

    Article  PubMed  Google Scholar 

  124. Pietrzak-Nowacka, M. et al. Autosomal dominant polycystic kidney disease is not a risk factor for post-transplant diabetes mellitus. Matched-pair design multicenter study. Arch. Med. Res. 39, 312–319 (2008).

    Article  CAS  PubMed  Google Scholar 

  125. Ruderman, I. et al. New onset diabetes after kidney transplantation in autosomal dominant polycystic kidney disease: a retrospective cohort study. Nephrology (Carlton) 17, 89–96 (2012).

    Article  CAS  Google Scholar 

  126. Sharp, C. K., Zeligman, B. E., Johnson, A. M., Duley, I. & Gabow, P. A. Evaluation of colonic diverticular disease in autosomal dominant polycystic kidney disease without end-stage renal disease. Am. J. Kidney Dis. 34, 863–868 (1999).

    Article  CAS  PubMed  Google Scholar 

  127. Lederman, E. D., McCoy, G., Conti, D. J. & Lee, E. C. Diverticulitis and polycystic kidney disease. Am. Surg. 66, 200–203 (2000).

    CAS  PubMed  Google Scholar 

  128. Sarkio, S., Halme, L., Kyllonen, L. & Salmela, K. Severe gastrointestinal complications after 1,515 adult kidney transplantations. Transpl. Int. 17, 505–510 (2004).

    Article  PubMed  Google Scholar 

  129. Andreoni, K. A. et al. Increased incidence of gastrointestinal surgical complications in renal transplant recipients with polycystic kidney disease. Transplantation 67, 262–266 (1999).

    Article  CAS  PubMed  Google Scholar 

  130. Bretagnol, A. et al. Autosomal dominant polycystic kidney disease: risk factor for nonmelanoma skin cancer following kidney transplantation. Transpl. Int. 23, 878–886 (2010).

    PubMed  Google Scholar 

  131. Wetmore, J. B. et al. Polycystic kidney disease and cancer after renal transplantation. J. Am. Soc. Nephrol. http://dx.doi.org/10.1681/ASN.2013101122.

  132. Kasiske, B. L., Snyder, J. J., Gilbertson, D. T. & Wang, C. Cancer after kidney transplantation in the United States. Am. J. Transplant. 4, 905–913 (2004).

    Article  PubMed  Google Scholar 

  133. Otley, C. C. et al. Skin cancer in organ transplant recipients: effect of pretransplant end-organ disease. J. Am. Acad. Dermatol. 53, 783–790 (2005).

    Article  PubMed  Google Scholar 

  134. Shillingford, J. M. 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 103, 5466–5471 (2006).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  135. Qian, Q. et al. Sirolimus reduces polycystic liver volume in ADPKD patients. J. Am. Soc. Nephrol. 19, 631–638 (2008).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  136. Serra, A. L. et al. Sirolimus and kidney growth in autosomal dominant polycystic kidney disease. N. Engl. J. Med. 363, 820–829 (2010).

    Article  CAS  PubMed  Google Scholar 

  137. Walz, G. et al. Everolimus in patients with autosomal dominant polycystic kidney disease. N. Engl. J. Med. 363, 830–840 (2010).

    Article  CAS  PubMed  Google Scholar 

  138. Canaud, G. et al. Therapeutic mTOR inhibition in autosomal dominant polycystic kidney disease: What is the appropriate serum level? Am. J. Transplant. 10, 1701–1706 (2010).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  139. Novalic, Z. et al. Dose-dependent effects of sirolimus on mTOR signaling and polycystic kidney disease. J. Am. Soc. Nephrol. 23, 842–853 (2012).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  140. Chrispijn, M. et al. Everolimus does not further reduce polycystic liver volume when added to long acting octreotide: results from a randomized controlled trial. J. Hepatol. 59, 153–159 (2013).

    Article  CAS  PubMed  Google Scholar 

  141. Torres, V. E. et al. Prospects for mTOR inhibitor use in patients with polycystic kidney disease and hamartomatous diseases. Clin. J. Am. Soc. Nephrol. 5, 1312–1329 (2010).

    Article  CAS  PubMed  Google Scholar 

  142. Spees, E. K., Orlowski, J. P., Fitting, K. M., Temple, D. M. & Reynolds, C. P. Successful use of cadaver kidneys from diabetic donors for transplantation. Transplant. Proc. 22, 378–379 (1990).

    CAS  PubMed  Google Scholar 

  143. Siegal, B. The polycystic kidney donor. Transplantation 54, 1131 (1992).

    Article  CAS  PubMed  Google Scholar 

  144. Canaud, G. et al. Therapeutic mTOR inhibition in autosomal dominant polycystic kidney disease: What is the appropriate serum level? Am. J. Transplant. 10, 1701–1706 (2010).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  145. Howard, R. J. et al. Development of polycystic disease in a kidney 10 years after transplantation. Transplantation 68, 1620 (1999).

    Article  CAS  PubMed  Google Scholar 

  146. Koene, R. A. Should polycystic kidneys ever be used for renal transplantation? Nephrol. Dial. Transplant. 16, 227–229 (2001).

    Article  CAS  PubMed  Google Scholar 

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Acknowledgements

The authors thank M. Jadoul and Z. Hassoun for critically reviewing the manuscript before submission, and R. Lhommel for providing images of PET–CT.

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  1. Division of Nephrology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, 10 Avenue Hippocrate, Brussels, B-1200, Belgium

    Nada Kanaan, Olivier Devuyst & Yves Pirson

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  1. Nada Kanaan
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  2. Olivier Devuyst
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N.K., O.D. and Y.P. researched the data for the article. N.K. and Y.P. wrote the manuscript. All authors contributed to a discussion of the article content and edited the manuscript before submission.

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Correspondence to Yves Pirson.

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Kanaan, N., Devuyst, O. & Pirson, Y. Renal transplantation in autosomal dominant polycystic kidney disease. Nat Rev Nephrol 10, 455–465 (2014). https://doi.org/10.1038/nrneph.2014.104

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