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childhood polycystic kidney disease

Genetics: - autosomal recessive Pathology: 1) renal medullary ductal ectasia 2) may be associated with hepatic fibrosis 3) portal hypertension Clinical manifestations: 1) presents in childhood 2) progressive, severe renal failure at an early age Complications: 1) adenomas in 5-10% 2) adenocarcinomas in 1.5% Management: - treatment of hypertension a) dietary salt restriction, DASH diet b) ACE inhibitor or ARB [1]

Interactions

disease interactions

General

genetic disease of the kidney polycystic kidney disease

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 11, 15, American College of Physicians, Philadelphia 1998, 2009
  2. Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1324