C-04: Polycystic kidney diseases
H- 10: criteria for living donor transplantation
Renal transplantation in autosomal dominant polycystic kidney
disease
Nada Kanaan,1, Olivier Devuyst1, & Yves Pirson1,
Nature Reviews Nephrology 10, 455–465 (2014)
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
COMMENTS
Renal and nonrenal complications in kidney transplant recipients with ADPKD (figure 4 of the
article)
Owing to excellent patient and graft survival rates, renal transplantation is the preferred
treatment option for patients with ADPKD and ESRD. In the pretransplant evaluation, issues
related to native nephrectomy, cystic liver involvement and screening for intracranial
aneurysms must be considered. The authors recommend prophylactic native nephrectomy
only in symptomatic patients with severe pain, early satiety, recurrent bleeding, infections or
stones and in asymptomatic patients in whom space is required to implant the graft. Liver
involvement must be assessed by imaging to detect the presence of cysts and evaluate liver
volume; measurement of pretransplant serum CA19-9s level could be valuable in case of
subsequent hepatic cyst infection. Screening for intracranial aneurysms follows the same
indications recommended in patients with ADPKD in general.
Living related-donors at risk of developing ADPKD should undergo careful screening to
exclude the disease. In patients aged ≥40 years, the predictive value of ultrasonography is
excellent: normal kidneys or a single renal cyst rule out the diagnosis. For patients aged 30–
39 years, ultrasonography has a false-negative rate of 0.7%, therefore negative MRI findings
are required to rule out ADPKD. In donor candidates aged <30 years, molecular genetic
testing should be carried out to exclude the disease and permit donation if ultrasonography
and MRI findings are normal.
After transplantation, the volume of native ADPKD kidneys usually decreases markedly.
Conversely, liver cysts continue to grow, and somatostatin analogue therapy should be
considered in patients with massive symptomatic liver disease. Cerebrovascular events only
marginally affect morbidity and mortality, whereas cardiac morbidity and mortality is not
increased.
Pr. Jacques CHANARD
Professor of Nephrology