Polycystic Kidney Disease

Polycystic kidney disease is a serious health condition that most often is passed down in families. Polycystic kidney disease is a lifelong condition.

The doctors at MaineHealth provide treatment options that can help patients control the debilitating symptoms of PKD.

What is polycystic kidney disease?

Polycystic kidney disease (PKD) causes multiple cysts to form on the kidneys. PKD most often is caused by a genetic mutation, which means it is inherited.

Kidney cysts are fluid-filled and can be as small as a pinhead and grow into the size of a grapefruit.

Cysts occasionally spread to the liver, spleen, ovaries and large intestines.

PKD can sometimes lead to kidney failure, especially if left untreated.

Polycystic kidney disease symptoms

People may have PKD for years without having symptoms. Early symptoms include:

  • High blood pressure
  • Back or side pain
  • Increase in abdomen size
  • Blood in urine
  • Frequent bladder or kidney infections
  • Frequent urination
  • Fluttering or pounding in chest
  • Frequent bruising
  • Pale skin tone
  • Joint pain
  • Nails that do not look normal

PKD screening and diagnosis

Your provider will look at your family history, because polycystic kidney disease is most often inherited. Your provider also will order tests:

  • Blood test to check for anemia or signs of infection
  • Urinalysis to look for blood, bacteria or protein
  • Imaging tests to look for cysts
  • Genetic testing can also determine the presence of PKD.

PKD treatment

There is no cure for PKD, but symptoms can be treated.

  • Reducing salt intake can help with high blood pressure.
  • Immediate treatment of kidney or bladder infections can prevent further complications of PKD.
  • Cysts may be drained surgically or using interventional radiology techniques to provide relief.
  • Dialysis and kidney transplant can be needed in advanced stages of polycystic kidney disease.

Polycystic disease types

The providers at MaineHealth diagnose and treat the three forms of polycystic disease:

  • Autosomal dominant polycystic kidney disease (ADPKD)
  • Autosomal recessive polycystic kidney disease (ARPKD)
  • Acquired cystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD)

Autosomal dominant polycystic kidney disease (ADPKD) also is known as adult PKD.

It is the most common form of polycystic kidney disease.

People who have one parent with polycystic kidney disease have a 50 percent chance of developing ADPKD.

Symptoms most often occur in adulthood, though sometimes it is diagnosed in childhood.

Autosomal recessive polycystic kidney disease (ARPKD)

Autosomal recessive polycystic kidney disease (ARPKD) is rare. It is passed down from both parents.

People with ARPKD inherit two genes, one from each parent.

ARPKD can be present at birth, or it develops in very young children. Children with autosomal recessive PKD may have symptoms that include:

  • High blood pressure,
  • Urinary tract infections,
  • Need to urinate often.

Improved sonogram technology often allows doctors to diagnose ARPKD before birth, allowing for immediate care from specialists at birth.

Acquired cystic kidney disease

Acquired cystic kidney disease develops in people who have other serious kidney problems.

Acquired cystic kidney disease is not inherited. It is a rare disorder.

When acquired cystic kidney disease does occur, it most often is diagnosed in people with kidney failure or on dialysis.

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