Saturday, February, 11, 2012

Medullary cystic kidney disease

Table of Contents

Alternative Names

Familial juvenile nephrophthisis; Senior-Loken syndrome


Treatment

There is no cure for this disease. At first, treatment focuses on controlling symptoms, reducing complications, and slowing the progression of the disease. Because of the loss of water and salt, the patient will need to drink plenty of fluids and take salt supplements to avoid dehydration.

As the disease gets worse, kidney failure develops. Treatment may involve medications and diet changes to limit foods containing phosphorus and potassium. Dialysis and a kidney transplant may be needed. For detailed information on treatment, see the articles on chronic kidney failure and end-stage kidney disease.


Support Groups


Expectations (prognosis)

Most persons with MCKD reach end-stage kidney disease between the ages of 30 and 50. Lifelong treatment may control the symptoms of chronic kidney failure. The cysts associated with MCKD may be very small, but large numbers of them can lead to kidney impairment.


Complications
  • Anemia
  • Bone weakening and fractures
  • Cardiac tamponade
  • Changes in glucose metabolism
  • Changes in electrolyte levels ( hyperkalemia)
  • Congestive heart failure
  • End-stage kidney disease
  • Gastrointestinal bleeding, ulcers
  • Hemorrhage
  • High blood pressure
  • Hyponatremia (low blood sodium level)
  • Hyperkalemia (too much potassium in the blood)
  • Infertility
  • Menstrual irregularities
  • Miscarriage
  • Pericarditis
  • Peripheral neuropathy
  • Permanent skin coloring changes
  • Platelet dysfunction with easy bruising
  • Skin coloring changes
  • Weakening of the bones, fractures, joint disorders

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: 11/30/2009
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; Herbert Y. Lin, MD, PHD, Nephrologist, Massachusetts General Hospital; Associate Professor of Medicine, Harvard Medical School. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)