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Site reviewed:

June 2008

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Autosomal Recessive Polycystic Kidney Disease (ARPKD) is a rare genetic form of PKD affecting approximately 1 in 6,000 to 1 in 40,000 people in the general population (although there is a higher incidence in certain populations e.g. Finnish and Afrikaaner).

ARPKD is usually an infantile disease; the baby inherits two copies of the defective gene, one from each parent.

Sadly, 30% to 50% of ARPKD infants die at birth or shortly after, primarily as the result of underdeveloped lungs.

High blood pressure is a very common complication in 80% of the children. They also have enlarged kidneys with or without cysts and liver enlargement.

Improvements in the treatment of newborn babies, control of blood pressure and liver complications, and management of renal failure are resulting in children with ARPKD living longer with a much better quality of life into adulthood. 

Click here to download more information on ARPKD (PDF file).

Click here to visit the US based ARPKD website which provides support and information to people around the world.