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Related Experiment Videos

Polycystic kidney disease: huge kidneys, huge problems, huge progress

J J Grantham1

  • 1Department of Medicine, University of Kansas Medical Center, Kansas City 66160, USA.

Transactions of the American Clinical and Climatological Association
|January 1, 1996
PubMed
Summary

Autosomal dominant polycystic kidney disease (ADPKD) arises from gene mutations, causing kidney enlargement due to cell proliferation and fluid buildup. Understanding ADPKD

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Area of Science:

  • Nephrology
  • Genetics
  • Cell Biology

Background:

  • Polycystic kidney disorders are common, causing significant morbidity and mortality.
  • Autosomal dominant polycystic kidney disease (ADPKD) is a leading inherited kidney disease.
  • Recent advances have improved understanding of renal cystic disorder pathogenesis.

Purpose of the Study:

  • To review the pathogenesis of renal cystic disorders, focusing on ADPKD.
  • To discuss the genetic basis and cellular mechanisms underlying kidney enlargement in ADPKD.
  • To explore factors influencing cyst progression and renal insufficiency.

Main Methods:

  • Review of current literature on ADPKD pathogenesis.
  • Analysis of genetic mutations (chromosomes 16 and 4) and their cellular consequences.

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  • Discussion of endocrine, paracrine, and autocrine factors in cyst growth.

    • Main Results:

    • ADPKD results from mutations causing kidney enlargement via epithelial cell proliferation, fluid accumulation, and matrix remodeling.
    • Focal onset suggests clonal growth and a potential somatic "second hit" in cyst development.
    • Cyst enlargement is driven by factors promoting proliferation and fluid secretion.

    Conclusions:

    • ADPKD pathogenesis involves complex cellular and molecular mechanisms.
    • Interstitial inflammation, apoptosis, and fibrosis contribute to renal insufficiency in about half of ADPKD patients.
    • Further research is needed to explain why some patients do not develop renal failure.