Polycystic Disease
Polycystic disease of the kidneys means that the substance of the kidneys contains a large number of fluid filled spaces. There are two forms of polycystic kidney disease - an infantile form which inherited as an autosomal recessive disorder (both your parents need to carry an abnormal gene, and you inherit both of them) and a so-called adult form which is transmitted in an autosomal dominant trait (which means that you only have to have one defective gene to develop the condition, and that if you have it statistically 50 per cent of your offspring will also develop it). In the infantile form the cysts can be seen on antenatal screening by ultrasound, and may be associated with abnormalities in the liver and lungs. This is often fatal in the neonatal period. In the adult form the cysts sometimes don’t appear until the 3rd or 4th decade, but can appear during childhood. The kidney substance is progressively replaced by cysts, causing abdominal pain, urinary tract infections, haematuria and possibly progressive renal failure. There may also be cysts in the liver and pancreas, and in some cases there are associated aneurysms affecting arteries in the brain. The treatment is to treat the complications as they arise, but endstage renal failure may require dialysis and transplantation.
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