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Medullary cystic kidney disease

Overview Symptoms Treatment Prevention
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.

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.
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
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