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Polycystic kidney disease--a truly pediatric problem

M R Ogborn1

  • 1Department of Pediatrics and Child Health, University of Manitoba, Winnipeg, Canada.

Pediatric Nephrology (Berlin, Germany)
|December 1, 1994
PubMed
Summary

Polycystic kidney disease (PKD) is a common inherited cause of chronic kidney failure. Early diagnosis and intervention in children may significantly impact the prevalence of adult renal failure.

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Area of Science:

  • Pediatric Nephrology
  • Renal Pathophysiology

Background:

  • Polycystic kidney disease (PKD) is the most common inherited cause of chronic renal failure.
  • While uncommon in childhood, PKD can cause significant renal disease symptoms.

Purpose of the Study:

  • To explore the pathogenesis of PKD, focusing on tubular epithelium growth regulation.
  • To highlight the potential for early diagnosis and intervention in pediatric PKD.

Main Methods:

  • Review of current research into PKD pathogenesis.
  • Analysis of animal and in vitro studies on cyst growth modification.

Main Results:

  • PKD pathogenesis involves disturbed regulation of tubular epithelium growth and development.
  • Cystic epithelium exhibits altered extracellular matrix, abnormal cell proliferation, and persistent secretory transport.
  • Studies show cyst growth modification via dietary protein reduction, amiloride, EGF receptor antagonism, anti-inflammatory therapy, and taxol.

Conclusions:

  • Early diagnosis and intervention in pediatric PKD are crucial.
  • Childhood intervention may significantly reduce adult chronic renal failure prevalence.

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