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Polycystic Kidney Disease: A Review from the Cleveland Clinic

September 21, 2009 - 4:24am 926 reads 3 comments

Polycystic kidney disease, or PKD, is the most common inherited renal disease. In the United States, 5,000 to 6,000 new cases are diagnosed each year. Typically 90% to 95% have a family history of the disease. There is currently no cure, but the progression of the disease can be slowed with treatment. Research studies are in progress.

The main feature of PKD is multiple cysts, or fluid-filled sacks, in the kidneys. Cysts may also develop in other organs. About 80% of PKD patients also get cysts in the liver, and 10% get cysts in the pancreas. Other symptoms of this disease are cardiac valve abnormalities, hernias (umbilical, hiatal, and inguinal, as well as diverticulae of the colon), and intracranial aneurysms. A “sentinel” or “thunderclap” headache signals a leaking aneurysm causing irritation of the brain tissue. The aneurysm may rupture soon after the start of this severely painful headache, so PKD patients should seek emergency care immediately.

There are two major types of PKD, caused by mutations in different genes. About 85% of PKD is attributed to mutations in the gene PKD1. For these patients, the cysts first appear in their 20s, and the disease progresses to end stage renal disease by their 50s.

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Anonymous

Thanks to dedicated doctors like Dr. William Braun at the CCF we are learning more about this giving disease (genetic - generation afflicted). Hope is just around the corner. To learn more contact the PKD Foundation @ PKDCURE.org
Nan Robison, Canton, OH

Linda Fugate

Thank you for this information. Another useful site is
https://www.google.com/health/ref/Polycystic+kidney+disease

Anonymous

Anything that can be done to help with this disease would be wonderful. I have PKD and have lost a father three brothers an aunt and most recently a son. I was fortunate enough to receive a transplant 25 years ago at the Cleveland Clinic and am grateful for that. I have another son who's been on dialysis for two and a half years now. Like so many families, we are grateful for Dr. Braun and the Cleveland Clinic and for the Polycystic Kidney Foundation.

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