KIDNEY, POLYCYSTIC

General Information

DEFINITION--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. It is the most common hereditary disease in the U.S.

BODY PARTS INVOLVED--Kidneys.

SEX OR AGE MOST AFFECTED--Both sexes; all ages.

SIGNS & SYMPTOMS

Early stages:

• Blood in the urine that may be visible only by microscopic examination.
• Repeated kidney infections.
• A mass in the abdomen.
• Hypertension.
• No symptoms (frequently) until the cysts replace so much normal kidney structure that kidney failure occurs.

Symptoms of kidney failure are:

• Pain in the lower back.
• Frequent urination.
• Increasing fatigue and weakness.
• Headache
• Bad breath.
• Nausea, vomiting or diarrhea.
• Fluid retention, especially swelling around the ankles or eyes.
• Shortness of breath.
• Chest pain.
• Itching skin.
• Cessation of menstruation in women of childbearing age.

CAUSES--This disease is inherited; the cause is unknown.

RISK INCREASES WITH--Family history of polycystic disease.

HOW TO PREVENT--Cannot be prevented at present. If polycystic kidney disease runs in your family, consult your doctor for tests to discover if you have kidney cysts. Even if you feel well and don't have the disease, get regular checkups. If you have a family history of polycystic kidney, seek genetic counseling before starting a family.

What To Expect

DIAGNOSTIC MEASURES--

• Your own observation of symptoms.
• Medical history and physical exam by a doctor.
• Diagnostic tests may include laboratory studies of blood, serum creatinine, renal concentration ability; CT scan and ultrasound (See Glossary for both).

APPROPRIATE HEALTH CARE

• Self-care after diagnosis.
• Doctor's treatment.
• Home dialysis or hospitalization for dialysis (rare).
• Surgery to perform a kidney transplant (rare).

POSSIBLE COMPLICATIONS

• Progression to kidney failure.
• Kidney stones.
• Infection or rupture of cysts.

PROBABLE OUTCOME--Polycystic kidney disease is currently considered incurable. Medical care 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.

How To Treat

GENERAL MEASURES--

• Treatment is aimed at preventing complications and preserving renal function.
• Prompt treatment of any infection is important.
• See Resources for Additional Information.

MEDICATION--

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

ACTIVITY--Take short, frequent rest periods during the day. Otherwise, stay as active as your strength allows.

DIET--

• Eat a low-salt, low-protein diet (ask your doctor).
• 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. Calcium and vitamin D supplements may be recommended to prevent softening of the bones (osteoporosis).

Call Your Doctor If

• You have symptoms of polycystic kidney.
• You have symptoms of kidney failure.
• You have fever or other signs of infection.
• Urination decreases.