Polycystic Kidney Disease

Overview

Mayo Clinic is a recognized leader in the treatment of polycystic kidney disease (PKD). Each year, Mayo Clinic provides individualized care for more than 500 patients with polycystic kidney disease, providing treatment at all three Mayo Clinic locations. A team of experts from different medical specialties work together to diagnose and treat both adult and pediatric patients.

In Rochester, for example, patients are treated in the Inherited Renal Disease Clinic, which brings together kidney and liver specialists, medical geneticists, radiologists, urologists, neurologists and neurosurgeons, as well as liver and transplant surgeons. This team provides patients with a comprehensive medical evaluation and treatment plan.

Mayo Clinic is also at the forefront of research into medications and therapies for managing PKD. In fact, Mayo Clinic researchers have discovered many of the genes that cause the different forms of PKD. This ongoing research enhances patient care; physicians can apply the latest research findings to clinical management of the disease.

Autosomal dominant polycystic kidney disease (ADPKD) affects adults. Autosomal recessive polycystic kidney disease (ARPKD) affects infants and children. Mayo Clinic treats patients who have either type of PKD, although ARPKD is treated exclusively in Rochester.

Diagnosis

Mayo Clinic specialists use several diagnostic methods to detect the size and number of cysts and evaluate the amount of healthy kidney tissue. Genetic testing is also done to look for the presence of the disease in families and sometimes to confirm a diagnosis. Learn more about polycystic kidney disease diagnosis.

Treatment Options

While there is currently no cure for PKD, Mayo Clinic has developed a comprehensive team approach to managing the disease. Read more about Mayo Clinic's approach to polycystic kidney disease treatment options.

• Treatment in Arizona
• Treatment in Florida
• Treatment in Minnesota
• Treatment of Children & Adolescents

About Polycystic Kidney Disease

Polycystic kidney disease is an inherited disorder in which multiple cysts develop, primarily in a person's kidneys. These cysts are non-cancerous (benign), round sacs that contain water-like fluid. They vary in size from tiny sacs to sacs large enough to hold several quarts of fluid. A normal kidney is about the size of a fist; kidneys enlarged by cysts may be the size of a football and weigh up to 22 pounds.

There are two types of PKD:

• Autosomal dominant polycystic kidney disease (ADPKD) affects from 1 in 400 to 1 in 1,000 adults, 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, which manifests in adulthood. Up to 50 percent of patients with ADPKD will require dialysis or a kidney transplant during their lifetime. This form of the disease accounts for about 90 percent of PKD cases.
• Autosomal recessive polycystic kidney disease (ARPKD) affects from 1 in 20,000 to 1 in 40,000 infants and children, and is caused by abnormal genes inherited from both parents. Siblings of a child with PRPKD also have a 25 percent chance of inheriting the disease.

PKD is not limited to the kidneys, although the kidneys usually are the most severely affected organs. Liver damage is the second-most common manifestation of PKD. The disease can also cause cysts to develop in the pancreas, in the membranes that surround the brain and central nervous system, and in seminal vesicles. Abdominal hernias are also common in ADPKD patients; other complications may occur as well.

Approximately 50 percent of 20- to 34-year-old patients with PKD have hypertension, even though they retain normal kidney function. As patients progress to end-stage renal (kidney) disease, almost all will develop hypertension. Many people do not know they have the disease for years, as ADPKD usually does not cause symptoms or problems early in life. Regular checkups can help ensure timely treatments to reduce damage to the kidneys and manage hypertension.

Read more on polycystic kidney disease at www.MayoClinic.com (opens in new window)
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