Autosomal Dominant Polycystic Kidney Disease

Prim Care. 2020 Dec;47(4):673-689. doi: 10.1016/j.pop.2020.08.010.

Abstract

Autosomal Dominant Polycystic Kidney Disease is an inherited multisystemic disorder of the renal tubules with subsequent formation of multiple cysts and enlargement of the kidney, affecting various organs. Diagnosis is initially suspected in those with family history and/or individuals who develop hypertension early on (secondary hypertension) or certain symptoms. Renal function is initially preserved for years secondary to compensatory mechanisms. Associated conditions include: liver cysts, berry aneurysms, kidney stones, etc. The disease course is variable, but patients often progress to end-stage renal failure by age 60. There is no known cure, however, risk factor modification at early stages is critical. Renal transplant is the optimal treatment in ESRD.

Keywords: Berry aneurysm; Dialysis; End-stage renal disease; Hematuria; Liver cysts; Polycystic kidney disease; Secondary hypertension; Transplant.

Publication types

  • Review

MeSH terms

  • Age Factors
  • Antidiuretic Hormone Receptor Antagonists / therapeutic use
  • Disease Progression
  • Humans
  • Kidney Failure, Chronic / etiology
  • Kidney Failure, Chronic / physiopathology*
  • Polycystic Kidney, Autosomal Dominant / complications
  • Polycystic Kidney, Autosomal Dominant / diagnosis
  • Polycystic Kidney, Autosomal Dominant / physiopathology*
  • Polycystic Kidney, Autosomal Dominant / therapy
  • Primary Health Care
  • Referral and Consultation
  • Risk Factors
  • Sex Factors

Substances

  • Antidiuretic Hormone Receptor Antagonists