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Kidney anatomy
Kidney anatomy
Kidney cyst with gallstones, CT scan
Kidney cyst with gallstones, CT scan
Kidney - blood and urine flow
Kidney - blood and urine flow


Medullary cystic kidney disease

Alternative Names:
Familial juvenile nephrophthisis; Senior-Loken syndrome

Treatment:

There is no cure for this disease. At first, treatment focuses on controlling symptoms, minimizing complications, and slowing the progression of the disease. Because of the loss of water and salt, the patient will need a liberal intake of both to avoid dehydration.

As the disease progresses, kidney failure develops and appropriate changes will be made:

  • If high blood pressure develops, anti-hypertensive medications may be needed.
  • Anemia may require erythopoeitin treatment to build red blood cells.
  • Diet will be modified to limit phosphorous- and potassium-containing foods.

Once end-stage kidney disease approaches, dialysis may be started. If a suitable donor becomes available, a kidney transplant may be performed. Kidney transplant is the preferred treatment. It has excellent results.



Expectations (prognosis):

There is no cure for chronic kidney failure resulting from medullary cystic disease. Untreated, it usually progresses to end-stage kidney disease. Lifelong treatment may control the symptoms of chronic kidney failure.



Complications:


Calling your health care provider:

Call for an appointment with your health care provider if you have any symptoms of medullary cystic disease.




Review Date: 2/3/2006
Reviewed By: Charles Silberberg, DO, Private Practice specializing in Nephrology, Affiliated with New York Medical College, Division of Nephrology, Valhalla, NY. Review provided by VeriMed Healthcare Network.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2004 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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