Polycystic kidney disease affects around 1:1000.
Type 1 is inherited on chromosome 16
Type 2 is inherited on chromosome 4
The US criteria for diagnosing polycystic kidney disease in a patient with a positive family history is:
- 2 cysts if aged less than 30
- 2 cysts in both kidneys if aged 30-59
- 4 cysts in both kidneys if aged greater than 60
Signs/symptoms of polycystic kidney disease include:
- renal enlargement
- abdominal pain
- hypertension
- renal failure
Associated features include:
- liver cysts
- subarachnoid haemorrhage
- mitral valve prolapse
Other causes of renal cysts include:
- autosomal recessive polycystic kidney disease
- chromosome 6
- tend to develop end stage renal failure in childhood
- fibrosis of liver
- Von-Hippel-Lindau (see battle 15.2)
- autosomal dominant on chromosome 3
- pre-maligant
- tuberous sclerosis
- auto dom on chromosome 9 or 16
- simple cysts
- occur in less than 2% of under 50s but up to 20% of over 70s.
Now for some renal tubular acidosis...