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Polycystic Kidney Disease

Anila Khaliq, MD Reviewed 04/2024
 


BASICS

DESCRIPTION

  • A group of monogenic disorders that results in renal cyst development.

  • Autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD).

  • A...

DIAGNOSIS

HISTORY

ADPKD 
  • Positive family history (15% are de novo mutations.)

  • Flank pain: 60%

  • Hematuria

  • UTI

  • HTN: 50% aged 20 to 34 years; 100% with ESKD

  • Renal failure

  • Presymptomatic screening of ADPKD is not ...

TREATMENT

GENERAL MEASURES

  • HTN: moderate sodium restriction, weight control, and regular exercise

  • Aggressive blood pressure control (<110/75) mmHg in subjects younger than 50 years of age with preser...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

None in early stages of the disease; avoid vigorous activity if disease advances. Recurrent gross hematuria is secondary to trauma, associated with faster decline...

REFERENCES

1
Chapman  AB, Devuyst  O, Eckardt  KU, et al. Autosomal-dominant polycystic kidney disease (ADPKD): executive summary from a Kidney Disease: Improving Global Outcome...

SEE ALSO

Chronic Kidney Disease 

CODES

ICD10

  • Q61.3 Polycystic kidney, unspecified

  • Q61.2 Polycystic kidney, adult type

  • Q61.1 Polycystic kidney, infantile type

  • Q61.19 Other polycystic kidney, infantile type

  • Q61.9 Cystic kidney disease, unsp...

CLINICAL PEARLS

  • Most PKD patients eventually develop ESKD. No specific treatment has been proven to prevent ESKD, but hydration and control of BP are reasonable goals.

  • Patients may benefit from a nephr...

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