childhood polycystic kidney disease

Pathology

• renal medullary ductal ectasia
• may be associated with hepatic fibrosis
• portal hypertension

Genetics

• autosomal recessive

Clinical manifestations

• presents in childhood
• progressive, severe renal failure at an early age

Complications

• adenomas in 5-10%
• adenocarcinomas in 1.5%

Management

• treatment of hypertension
  • dietary salt restriction, DASH diet
  • ACE inhibitor or ARB

More general terms

• polycystic kidney disease
• genetic disease of the kidney