Autosomal recessive polycystic kidney disease (ARPKD)

Introduction

Autosomal recessive polycystic kidney disease (ARPKD) is a genetic condition. It damages the kidneys by causing cysts to grow in them. Both kidneys are affected.

Genetic conditions are inherited, which means they run in the family.

The kidneys are towards the back of the abdomen, on either side of the spine. They help to keep the body healthy. The kidneys filter the blood, for example, to:

  • Get rid of waste materials, which are passed out in urine.
  • Keep a normal balance of water and salts in the body by adjusting the levels in the blood.

ARPKD can cause cysts to develop in other parts of the body as well as the kidneys. The liver is usually affected, often more severely than the kidneys. Other organs are sometimes affected, such as the spleen (an organ that helps filter impurities from your blood).

ARPKD is a chronic condition that cannot be cured. It is sometimes called infantile PKD or childhood PKD.

Who does ARPKD affect?

Symptoms of ARPKD can develop:

  • In unborn babies.
  • During a baby's first month of life – this is most common.
  • In older children and teenagers.

Symptoms can be very severe and sometimes progress rapidly.

ARPKD is rare. It affects about one in 20,000 people.

Some research suggests that the condition is more frequent in people from some groups. For example, Finnish people and Afrikaans-speaking people in South Africa.

Autosomal dominant polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) is a different condition to ARPKD.

ADPKD is nearly always inherited. Symptoms develop mainly in adults, usually between the ages of 30-60. ADPKD is much more common than ARPKD.

 

Cyst

A cyst is a fluid-filled sac or cavity in the body.

 

 

Kidney


Kidneys are a pair of bean-shaped organs located at the back of the abdomen, which remove waste and extra fluid from the blood and pass them out of the body as urine.

 

 

Genetic

Genetic is a term that refers to genes- the characteristics inherited from a family member.

Spine


The spine supports the skeleton, and surrounds and protects the delicate spinal cord and nerves. It is made up of 33 bones called the vertebrae.

Blood


Blood supplies oxygen to the body and removes carbon dioxide. It is pumped around the body by the heart.

Chronic


Chronic usually means a condition that continues for a long time or keeps coming back.

 

Last updated: 04 October 2011

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