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Polycystic Kidney Disease, Pediatric

Paul R. Brakeman, MD, PhD Reviewed 10/2018
 


BASICS

DESCRIPTION

Polycystic kidney disease (PKD) is a heritable disorder with diffuse cystic involvement of both kidneys without other dysplastic elements. The term PKD is generally used to describe ...

DIAGNOSIS

HISTORY

  • ADPKD

    • Detailed family history is essential.

    • Most common presenting complaint in adults is pain.

    • Hypertension, polyuria, gross hematuria, nephrolithiasis, and UTIs are common.

  • ARPKD

    • Oligohy...

TREATMENT

GENERAL MEASURES

  • No currently approved targeted treatments to cure or slow progression

  • Medical management is supportive.

  • Pain is the most common symptom in ADPKD and can be difficult to treat.

MEDICATION

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

A pediatric nephrologist should be involved in the care of children with PKD. 

DIET

  • In both conditions, dietary changes depend on the degree of renal failure.

  • Sodium...

ADDITIONAL READING

  • Dell KM. The spectrum of polycystic kidney disease in children. Adv Chronic Kidney Dis.  2011;18(5):339–347. [View Abstract on OvidInsights]

  • Hoyer PF. Clinical manifestations of...

CODES

ICD9

  • 753.12 Polycystic kidney, unspecified type

  • 753.13 Polycystic kidney, autosomal dominant

  • 753.14 Polycystic kidney, autosomal recessive

ICD10

  • Q61.3 Polycystic kidney, unspecified

  • Q61.19 Other polyc...

FAQ

  • Q: What can be done to slow the progression of renal insufficiency in ADPKD?

  • A: Well-controlled BP and rapid treatment of UTIs may decrease the progression of renal failure.

  • Q: Should asymptomatic o...

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