Kidney Transplant

Kidney Transplant for Polycystic Kidney Disease at Mayo Clinic

World-leading Care for Polycystic Kidney Disease

Patients with polycystic kidney disease (PKD) come to Mayo Clinic for world-leading care. A multidisciplinary team of physicians with a major clinical interest and expertise in PKD — kidney and liver specialists, molecular geneticists, radiologists, urologists, neurologists and neurosurgeons, as well as liver and transplant surgeons — work together as an integrated team to serve the needs of each patient. Doctors at Mayo have treated thousands of people with PKD over the years, and have a vast depth of experience in managing this disorder. In addition, Mayo Clinic is at the forefront of research into medications and therapies for managing the disease.

Treatment and Management

Mayo Clinic has developed a comprehensive team approach to managing PKD, involving:

1. Genetic counseling
2. Maximizing medical management in an effort to extend the life of the kidneys
3. Early detection and treatment of complications
4. Multidisciplinary approach to manage complex clinical problems, such as massive polycystic liver disease and chronic disabling renal pain
5. Basic research to understand how mutations in the PKD genes cause cyst development in the kidneys and the liver, as well as hypertension and aneurysms
6. Clinical trials of novel medications aimed at delaying the progression of the disease
7. Appropriate referral and management of kidney transplantation.

Kidney transplant for PKD

Up to 50 percent of people with PKD will eventually suffer kidney failure. Fortunately, most people with PKD remain relatively healthy, making them good candidates for kidney transplantation. While PKD is a genetic disease that can occur in many family members, Mayo Clinic has been able to perform living donor kidney transplants in a high percentage of people with PKD. A living donor kidney transplant offers many advantages over waiting for a deceased donor. In many instances, the recipient can be transplanted without ever having to go on dialysis, and the transplanted kidney functions immediately. In addition, living donor transplants typically experience better long-term graft survival.

Mayo Clinic research has shown that performing the kidney transplant without removing the polycyctic kidneys is preferable, if possible. If the original kidneys do need to be removed (either before or after transplant), surgeons can generally use a laparoscopic technique to remove them through a much smaller incision than previously used.

About PKD

PKD is an inherited disorder in which multiple cysts develop, primarily in a person's kidneys; it manifests in two types:

• Autosomal dominant polycystic kidney disease (ADPKD) affects from between 1 and 400 to 1 in 1000 individuals, and is caused by an abnormal gene inherited from either parent. Children of a parent with ADPKD have a 50 percent chance of inheriting the disease.
• Autosomal recessive polycystic kidney disease (ARPKD) affects from between 1 in 20,000 to 1 in 40,000 individuals, and is caused by abnormal genes inherited from both parents. Siblings of a child with ARPKD also have a 25 percent chance of inheriting the disease.

Up to 50 percent of patients with ADPKD will require dialysis or a kidney transplant during their lifetime.

PKD Research

Mayo Clinic researchers discovered the main gene causing ADPKD (PKD1), the gene causing ARPKD (PKHD1), and a gene causing a rare form of polycystic kidney disease associated with multiple malformations (Meckel-Gruber syndrome, MKS3). In collaboration with investigators at Yale University, they have also discovered two genes responsible for isolated polycystic liver disease (PRKCSH and Sec63). Laboratory-based research at Mayo has focused on explaining how mutations in the PKD genes lead to cyst development and on finding effective therapies for PKD. These studies have shown that a new agent, an antagonist of the V2 vasopressin receptor (tolvaptan), can dramatically slow the development and progression of PKD in several animal models. In addition, Mayo researchers have shown that another drug (octreotide) delays the development of both PKD and polycystic liver disease. Both medications are now in clinical trials.

Mayo Clinic also conducts observational studies and clinical trials into PKD, including:

• The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP, NIH funded)
• HALT Progression of Polycystic Kidney Disease (HALT PKD , NIH funded, http://clinicaltrials.gov/ct/show/NCT00283686)
• Tolvaptan Efficacy and Safety in Management of Polycystic Kidney Disease and Its Outcomes (TEMPO, funded by Otsuka Pharmaceutical, http://clinicaltrials.gov/ct/show /NCT00428948 )
• Octreotide in Severe Polycystic Liver Disease (funded by Mayo Foundation and Novartis, http://clinicaltrials.gov/ct/show/NCT00426153)

For more information on PKD, see: http://www.mayoclinic.com

Appointments

The most efficient approach for most patients is simply to contact Central appointments, who can schedule an evaluation:

Arizona

For appointments or more information, call the Central Appointment Office at (800) 446-2279 between 8 a.m. and 5 p.m. Mountain time Monday through Friday, or complete an online appointment request form.

Florida

For appointments or more information, call the Central Appointment Office at (904) 953-0853 between 8 a.m. and 5 p.m. Eastern time Monday through Friday, or complete an online appointment request form.

Minnesota

For appointments or more information, call the Central Appointment Office at (507) 538-3270 7 a.m. to 7 p.m. Central time Monday through Thursday, 7 a.m. to 5 p.m. Friday.