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KIDNEY, POLYCYSTIC

DESCRIPTION

Polycystic kidney disease is an inherited kidney disorder in which cysts develop in the kidneys. The cysts enlarge the kidney and reduce its function. This is not cancerous. Most cases show no symptoms until adulthood. Then symptoms progress slowly for up to 20 years.
Appropriate health care includes:
  • Self-care after diagnosis.
  • Physician's monitoring of general condition and medications.
  • Surgery to perform a kidney transplant (rare).
  • Hospitalization for dialysis (rare).

    SIGNS & SYMPTOMS

  • Early stages: blood in the urine that may be visible only by microscopic examination; repeated kidney infections; a mass in the abdomen; no symptoms (frequently) until the cysts replace so much normal kidney structure that kidney failure occurs.
  • Symptoms of kidney failure: pain in the lower back; frequent urination; increasing fatigue and weakness; headache; bad breath; hypertension; nausea, vomiting, or diarrhea; fluid retention, especially swelling around the ankles or eyes; shortness of breath; chest pain; itching skin; cessation of menstruation in females of childbearing age.

    CAUSES
    This disease is inherited; the cause is unknown.

    RISK FACTORS
    Family history of polycystic disease.

    PREVENTING COMPLICATIONS OR RECURRENCE

    Cannot be prevented at present. If polycystic kidney disease runs in your family, consult your doctor for tests to discover if your child has kidney cysts. Even if your child feels well and doesn't have the disease, schedule regular checkups. If you have a family history of polycystic kidney disease, seek genetic counseling before starting a family.

    BASIC INFORMATION

    MEDICAL TESTS

  • Your own observation of symptoms.
  • Medical history and physical exam by a doctor.
  • X-rays and ultrasonography of kidneys and other parts of the urinary tract.
  • Special studies that may include ultrasonography, CAT or CT scan, MRI, and radionuclide scan (See Glossary for all).

    POSSIBLE COMPLICATIONS

  • Urinary-tract infections.
  • Chronic kidney failure.

    PROBABLE OUTCOME
    Polycystic kidney disease is currently considered incurable, but persons with it have a normal life expectancy. Your doctor may slow the progressive kidney damage by treating complications as they arise. Scientific research into causes and treatment continues. This offers hope for increasingly effective treatment and eventual cure.

    TREATMENT

    HOME CARE

    There is no specific treatment for polycystic kidney disease. The treatment described below applies primarily to patients with polycystic disease who have chronic kidney failure.

    MEDICATION

  • Without complications, medicine usually is not necessary for this disorder.
  • If necessary, your doctor may prescribe antibiotics for infection or anti-hypertensives to control high blood pressure.
  • Most drugs are excreted by the kidney. If your child has chronic kidney failure and takes prescription drugs, the dose may need adjustment because of this disorder.

    ACTIVITY
    Your child should take short, frequent rest periods during the day. Otherwise, advise your child to stay as active as strength allows.

    DIET & FLUIDS

  • Encourage your child to eat a low-salt (See Appendix 29), low-protein diet.
  • Urge your child to drink at least 8 glasses of fluid every day.
  • Iron and multiple-vitamin supplements may be necessary to ensure good nutrition because of the dietary restrictions. Your doctor may also prescribe calcium and vitamin D supplements for your child to prevent softening of the bones (osteoporosis) that may occur later in life.

    OK TO GO TO SCHOOL?

    Yes.

    CALL YOUR DOCTOR IF

  • Your child has symptoms of polycystic kidney disease.
  • Your child has symptoms of kidney failure.
  • Your child has fever or other signs of infection.
  • Urination decreases. ‡
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