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Polycystic kidney disease

  • Definition
    • Polycystic kidney disease is a kidney disorder passed down through families. In this disease, many cysts form in the kidneys, causing them to become enlarged.

  • Alternative Names
    • Cysts - kidneys; Kidney - polycystic; Autosomal dominant polycystic kidney disease; ADPKD

  • Causes
    • Polycystic kidney disease (PKD) is passed down through families (inherited). The 2 inherited forms of PKD are autosomal dominant and autosomal recessive.

      People with PKD have many clusters of cysts in the kidneys. What exactly triggers the cysts to form is unknown.

      PKD is associated with the following conditions:

      As many as half of people with PKD have cysts in the liver.

  • Symptoms
  • Exams and Tests
  • Treatment
    • The goal of treatment is to control symptoms and prevent complications. Treatment may include:

      • Blood pressure medicines
      • Diuretics (water pills)
      • Low-salt diet

      Any urinary tract infection should be treated quickly with antibiotics.

      Cysts that are painful, infected, bleeding, or causing a blockage may need to be drained. There are usually too many cysts to make it practical to remove each cyst.

      Surgery to remove 1 or both kidneys may be needed. Treatments for end-stage kidney disease may include dialysis or a kidney transplant.

  • Support Groups
    • You can often ease the stress of an illness by joining a support group where members share common experiences and problems.

  • Outlook (Prognosis)
    • The disease gets worse slowly. Eventually, it may lead to end-stage kidney failure. It is also associated with liver disease, including infection of liver cysts.

      Treatment may relieve symptoms for many years.

      People with PKD who don't have other diseases may be good candidates for a kidney transplant.

  • Possible Complications
    • Health problems that may result from PKD include:

  • When to Contact a Medical Professional
    • Call your health care provider if:

      • You have symptoms of PKD
      • You have a family history of PKD or related disorders and you are planning to have children (you may want to have genetic counseling)
  • Prevention
    • Currently, no treatment can prevent the cysts from forming or enlarging.

  • References
    • Arnaout MA. Cystic kidney diseases. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 129.

      Porter CC, Avner Ed. Anatomic abnormalities associated with hematuria. In: Kliegman RM, Stanton BF, St. Geme JW III, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 515.

      Somlo S, Torres VE, Caplan MJ. Autosomal dominant polycystic kidney disease. In: Alpern RJ, Moe OW, Caplan MJ, eds. Seldin and Giebisch's The Kidney. 5th ed. Philadelphia, PA: Elsevier; 2013:chap 80.

      Torres VE, Grantham JJ. Cystic diseases of the kidney. In: Taal MW, Chertow GM, Marsden PA, Skorecki K, Yu ASL, Brenner BM, eds. Brenner and Rector's The Kidney. 9th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 45.

      Torres VE, Grantham JJ. Cystic diseases of the kidney. In: Brenner BM, ed. Brenner and Rector's the Kidney. 8th ed. Philadelphia, PA: Elsevier Saunders; 2007:chap 41.