Information From Your Health Care Provider

KIDNEY DISEASE, POLYCYSTIC

Basic information

description

Polycystic kidney disease (PKD) is an inherited disorder in which cysts develop in the kidneys. Cysts are benign, round sacs that contain water-like fluid. The cysts replace normal kidney tissue and reduce kidney function. There are two forms of PKD.
- Autosomal dominant PKD (ADPKD) is the more common form. It is one of the most common inherited disorders in humans. Symptoms often don't occur until ages 30 to 40, but may begin in childhood.
- Autosomal recessive PKD is more rare and begins early in life. It is not discussed in this topic.

FREQUENT SIGNS & SYMPTOMS

A person with ADPKD often has no symptoms. Routine physical exams and blood and urine tests may not diagnose the disease in the early stages. Symptoms that occur may include pain in the side, back, or abdomen; increase in size of abdomen; less urine; blood in the urine; and high blood pressure.

causes

It is an inherited disorder. Autosomal dominant means if one parent has the disease, there is a 50% chance the disease gene will pass to a child. Two genes are known—called PKD1 (more severe disease) and PKD2. In a few cases, the disease results from a spontaneous gene mutation (non-inherited PKD).

risk increases with

Family history of polycystic kidney disease.

PREVENTIVE MEASURES

Cannot be prevented. If polycystic kidney disease runs in your family, get medical advice about tests to discover if you have kidney cysts. If you have a family history of polycystic kidney, seek genetic counseling before starting a family. Women with PKD can have a successful pregnancy, but some may develop preeclampsia.

expected outcomes

There is no cure for polycystic kidney disease. Medical care may slow the progressive kidney damage and improve quality of life.

possible complications

High blood pressure.
Aneurysms (ballooning of weak places in arteries).
Infection or rupture of cysts.
Cysts in the liver and other organs.
Heart valve problems.
Chronic pain.
Colon problems such as diverticulosis.
Kidney failure and end-stage renal disease (ESRD).
Need for dialysis or kidney transplantation.

diagnosis & treatment

general measures

Your health care provider will do a physical exam and ask questions about your family medical history. Medical tests may include blood studies, CT, MRI, and ultrasound. A genetic test may be done.
There is no specific treatment for the disorder. Treatment is aimed at prolonging life, maintaining kidney function, and managing complications.
Treatment may include drug therapy, lifestyle changes, surgery, and regular medical check-ups.
Check your blood pressure each day and keep a record. Don't smoke.
Surgery may be needed if kidney cysts rupture, large cysts cause pain, or if cysts in the liver cause problems.
Once kidney failure develops, dialysis or surgery to perform a kidney transplant may be recommended.
To learn more: PKD Foundation, 9221 Ward Parkway, Suite 400, Kansas City, MO 64114; (800) 753-2873; website www.pkdcure.org.

medications

Antibiotics for infection, drugs to control high blood pressure, and drugs for pain are often prescribed.
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.
Avoid nonprescription drugs (especially NSAIDs such as ibuprofen or naproxen) unless medically approved.
Vitamins and supplements may be recommended.

activity

Get rest as needed. Otherwise, stay as active as your strength allows. Try to exercise on a regular basis.
Avoid contact sports to reduce risk of kidney injury.

diet

A low-salt or low-protein or low-fat diet may be prescribed. Drink plenty of fluids each day. Avoid caffeine.

notify our office if

You or a family member has symptoms of polycystic kidney disease.
You have symptoms of kidney failure, signs of infection, urination decreases, or blood appears in urine.