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Polycystic Kidney Disease

Reviewed 06/2019
 


BASICS

DESCRIPTION

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

  • The most frequent are two genetically distinct conditions: autosomal dominant polycystic kidney disease (ADPKD) 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 cu...

TREATMENT

GENERAL MEASURES

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

  • Medications: ACE inhibitors; angiotensin receptor blockers (ARBs)

  • Pain: narcotics and other analgesics; b...

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
Schrier RW, Brosnahan G, Cadnapaphornchai MA, et al. Predictors of autosomal dominant polycystic disease progression. J Am Soc Nephrol.  2014;25(11):2399–2418. [View Abstract on OvidM...

ADDITIONAL READING

  • Chapman AB, Bost JE, Torres VE, et al. Kidney volume and functional outcomes in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol.  2012;7(3):479–486. [View A...

CODES

ICD10

  • Q61.3 Polycystic kidney, unspecified

  • Q61.19 Other polycystic kidney, infantile type

  • Q61.2 Polycystic kidney, adult type

  • Q61.11 Cystic dilatation of collecting ducts

ICD9

  • 753.12 Polycystic kidney...

CLINICAL PEARLS

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

  • Patien...

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