Renal transplantation in autosomal dominant polycystic kidney disease

Nat Rev Nephrol. 2014 Aug;10(8):455-65. doi: 10.1038/nrneph.2014.104. Epub 2014 Jun 17.

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.

Publication types

  • Review

MeSH terms

  • Adult
  • Aged
  • Aneurysm, Ruptured / prevention & control
  • Cysts / etiology
  • Genetic Testing
  • Graft Survival
  • Humans
  • Intracranial Aneurysm / diagnosis
  • Kidney Failure, Chronic / etiology
  • Kidney Failure, Chronic / surgery*
  • Kidney Transplantation* / adverse effects
  • Liver Diseases / etiology
  • Living Donors
  • Middle Aged
  • Nephrectomy
  • Polycystic Kidney, Autosomal Dominant / complications*
  • Polycystic Kidney, Autosomal Dominant / diagnostic imaging
  • Polycystic Kidney, Autosomal Dominant / surgery*
  • TRPP Cation Channels / genetics
  • Ultrasonography

Substances

  • TRPP Cation Channels
  • polycystic kidney disease 1 protein
  • polycystic kidney disease 2 protein