Skip to main content

Pathogenesis of Cysts and Cystic Kidneys

  • Chapter
The Cystic Kidney

Part of the book series: Developments in Nephrology ((DINE,volume 27))

Abstract

Although the formation of fluid-filled renal cysts is a fairly common event that has received attention for more than a century, the mechanisms involved in their development are not well understood. In this chapter we consider a series of model explanations to which we add or subtract until we arrive at one with the greatest number of favorable traits. It is agreed that renal cysts develop in pre-existing nephrons and collecting ducts, and that they can develop in the absence of distal tubule obstruction, large transmural pressure or solute gradients, abnormally stretchable basement membranes, or permanent connection to their tubule of origin. It is decided, however, that there is an absolute requirement for diffuse hyperplasia of the lining epithelium that keeps pace exactly with the enlarging surface area of the cyst cavity. At the same time, the cyst cavity is always filled with fluid, and that probably requires the new development of a net secretory mechanism. As to the question of which comes first, the cellular proliferation or the accumulation of fluid, the two in fact must occur simultaneously and are perhaps consequences of the same cause.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Subscribe and save

Springer+ Basic
$34.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or eBook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 39.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Similar content being viewed by others

References

  1. Amis ES Jr, Cronan JJ, Yoder IC, Pfister RC and Newhouse JH. Renal cysts: curios and caveats. Urological Radiology 1982, 4, 199 – 209.

    Article  Google Scholar 

  2. Avner ED. Renal cystic disease. Insights from recent experimental investigations. Nephron 1988, 48, 89 – 93.

    Article  PubMed  CAS  Google Scholar 

  3. Avner ED, Piesco NP, Sweeney WE, Studnicki FM, Fetterman GH and Ellis D. Hydro- cortisone-induced cystic metanephric maldevelopment in serum-free organ culture. Laboratory Investigation 1984, 50, 208 – 218.

    PubMed  CAS  Google Scholar 

  4. Baert L. Hereditary polycystic kidney disease (adult form): A microdissection study of two cases at an early stage of the disease. Kidney International 1978, 13, 519 – 525.

    Article  PubMed  CAS  Google Scholar 

  5. Baxter TJ. Cysts arising in the renal tubules. A microdissection study. Archives of Diseases in Childhood 1965, 40, 464 – 473.

    Article  CAS  Google Scholar 

  6. Bernstein J, Evan AP and Gardner KF Jr. Human cystic kidney diseases: epithelial hyperplasia in the pathogenesis of cysts and tumors. Pediatric Nephrology 1987, 1, 393 – 396.

    Article  PubMed  CAS  Google Scholar 

  7. Bialestock D. The morphogenesis of renal cysts in the stillborn studied by micro-dissection techniques. Journal of Pathology and Bacteriology 1956, 71, 51 – 59.

    Article  PubMed  CAS  Google Scholar 

  8. Bjerle P, Lindqvist B and Michaelson G. Pressure measurements in renal cysts. Scandinavian Journal of Clinical Laboratory Investigations 1971, 27, 135 – 138.

    Article  CAS  Google Scholar 

  9. Brunette DM. Mechanical stretching increases the number of epithelial cells synthesizing DNA in culture. Journal of Cellular Science 1984, 69, 35 – 45.

    CAS  Google Scholar 

  10. Butkowski RJ, Carone FA, Grantham JJ and Hudson BG. Tubular basement membrane changes in 2-amino-2,4-diphenylthiazole-induced polycystic disease. Kidney International 1985, 28, 744 – 751.

    Article  PubMed  CAS  Google Scholar 

  11. Carone FA, Makino H and Kanwar YS. Basement membrane antigens in renal polycystic disease. American Journal of Pathology 1988, 130, 466 – 471.

    PubMed  CAS  Google Scholar 

  12. Carone FA, Rowland RG, Perlman SG and Ganote CE. The pathogenesis of drug-induced renal cystic disease. Kidney International 1974, 5, 411 – 421.

    Article  PubMed  CAS  Google Scholar 

  13. Cortell S, Gennari FJ, Davidson M, Bossert WH and Schwartz WB. A definition of proximal and distal tubule compliance: Practical and theoretical implications. Journal of Clinical Investigation 1973, 52, 2330 – 2339.

    Article  PubMed  CAS  Google Scholar 

  14. Crocker JFS, Blecher SR, Givner ML and McCarthy SC. Polycystic kidneys and liver disease and corticosterone changes in the cpk mouse. Kidney International 1987, 31, 1088 – 1091.

    Article  PubMed  CAS  Google Scholar 

  15. Cuppage FE, Huseman RA, Chapman A and Grantham JJ. Ultrastructure and function of cysts from human adult polycystic kidneys. Kidney International 1980, 17, 373 – 381.

    Article  Google Scholar 

  16. Derezic D and Cecuk L. Hydrostatic pressure within renal cysts. Journal of Urology 1982, 54, 93 – 94.

    Article  CAS  Google Scholar 

  17. Dunnill MS, Millard PR and Oliver D. Acquired cystic disease of the kidneys: a hazard of long-term intermittent maintenance hemodialysis. Journal of Clinical Pathology 1977, 30, 868 – 877.

    Article  PubMed  CAS  Google Scholar 

  18. Ebihara I, Killen PD, Laurie GW, Huang T, Yamada T, Martin GR and Brown KS. Altered mRNA expression of basement membrane components in a murine model of polycystic kidney disease. Laboratory Investigation 1988, 58, 262 – 269.

    PubMed  CAS  Google Scholar 

  19. Evan AP and Gardner KD Jr. Nephron obstruction in nordihydroguaiaretic acid-induced renal cystic disease. Kidney International 1979, 15, 7 – 19.

    Article  PubMed  CAS  Google Scholar 

  20. Evan AP, Gardner KD Jr and Bernstein J. Polypoid and papillary epithelial hyperplasia: a potential cause of ductal obstruction in adult polycystic disease. Kidney International 1979, 16, 743 – 750.

    Article  PubMed  CAS  Google Scholar 

  21. Folkman J and Moscona A. Role of cell shape in growth control. Nature 1978, 273, 345 – 349.

    Article  PubMed  CAS  Google Scholar 

  22. Gardner KD Jr. Composition of fluid in twelve cysts of a polycystic kidney. New England Journal of Medicine 1969, 281, 985 – 988.

    Article  PubMed  CAS  Google Scholar 

  23. Gardner KD Jr. Pathogenesis of human cystic renal disease. Annual Review of Medicine 1988, 39, 185 – 191.

    Article  PubMed  Google Scholar 

  24. Gardner KD and Evan AP. Renal cystic disease induced by diphenylthiazole. Kidney International 1983, 24, 43 – 52.

    Article  PubMed  CAS  Google Scholar 

  25. Gardner KD Jr, Evan AP and Reed WP. Accelerated renal cyst development in decon- ditioned germ-free rats. Kidney International 1986, 29, 1116 – 1123.

    Article  PubMed  Google Scholar 

  26. Gardner KD Jr, Reed WP, Evan AP, Zedalis J, Hylarides MD and Leon AA. Endotoxin provocation of experimental renal cystic disease. Kidney International 1987, 32, 329 – 334.

    Article  PubMed  CAS  Google Scholar 

  27. Gardner KD Jr, Solomon S, Fitzgerald WW and Evan AP. Function and structure in the diphenylamine-exposed kidney. Journal of Clinical Investigation 1976, 57, 796 – 806.

    Article  PubMed  Google Scholar 

  28. Grantham JJ, Donoso VS, Evan AP, Carone FA and Gardner KD Jr. Viscoelastic properties of tubule basement membranes in experimental renal cystic disease. Kidney International 1987, 32, 187 – 197.

    Article  PubMed  CAS  Google Scholar 

  29. Grantham JJ, Geiser JL and Evan AP. Cyst formation and growth in autosomal dominant polycystic kidney disease. Kidney International 1987, 31, 1145 – 1152.

    Article  PubMed  CAS  Google Scholar 

  30. Grantham JJ, Qualizza PB and Irwin RL. Net fluid secretion in proximal straight renal tubules in vitro: Role of PAH. American Journal of Physiology 1974, 266, 191 – 197.

    Google Scholar 

  31. Gregoire JR, Torrest VE, Holley KE and Farrow GM. Renal epithelial hyperplastic and neoplastic proliferation in autosomal dominant polycystic kidney disease (ARPKD). American Journal of Kidney Disease 1987, 9, 27 – 38.

    CAS  Google Scholar 

  32. Haverty TP and Neilson EG. Basement membrane gene expression in polycystic kidney disease. Laboratory Investigation 1988, 58, 245 – 248.

    PubMed  CAS  Google Scholar 

  33. Heggo O. A microdissection study of cystic disease of the kidneys in adults. Journal of Pathology and Bacteriology 1966, 91, 311 – 315.

    Article  PubMed  CAS  Google Scholar 

  34. Huseman R, Grady D, Welling D and Grantham J. Macropuncture study of polycystic disease in adult human kidneys. Kidney International 1980, 18, 375 – 385.

    Article  PubMed  CAS  Google Scholar 

  35. Jensen PK and Steven K. Influence of intratubular pressure on proximal tubular compliance and capillary diameter in the rat kidney. Pflugers Archiv 1979, 382, 179 – 187.

    PubMed  CAS  Google Scholar 

  36. Kanwar YS and Carone FA. Reversible changes of tubular cell and basement membrane in drug-induced renal cystic disease. Kidney International 1984, 26, 35 – 43.

    Article  PubMed  CAS  Google Scholar 

  37. Lambert PP. Polycystic disease of the kidney. A review. Archives of Pathology 1947, 44, 34 – 58.

    PubMed  CAS  Google Scholar 

  38. McAteer JA, Evan AP and Gardner KD. Morphogenetic clonal growth of kidney epithelial cell line MDCK. Anatomical Record 1987, 217, 229 – 239.

    Article  PubMed  CAS  Google Scholar 

  39. Milutinovic J and Agodoa LY. Potential causes and pathogenesis in autosomal dominant polycystic kidney disease. Nephron 1983, 33, 139 – 144.

    Article  PubMed  CAS  Google Scholar 

  40. Norris RJ and Herman L. The pathogenesis of polycystic kidneys: Reconstruction of cystic elements in four cases. Journal of Urology 1941, 46, 147 – 176.

    Google Scholar 

  41. Osathanondh V and Potter EL. Pathogenesis of polycystic kidneys: Type 1 due to hyperplasia of interstitial portions of collecting tubules. Archives of Pathology 1964, 77, 466 – 473.

    PubMed  CAS  Google Scholar 

  42. Osathanondh V and Potter EL. Pathogenesis of polycystic kidneys: Type 2 due to inhibition of ampullary activity. Archives of Pathology 1964, 77, 474 – 484.

    PubMed  CAS  Google Scholar 

  43. Osathanondh V and Potter EL. Pathogenesis of polycystic kidneys: Type 3 due to multiple abnormalities of development. Archives of Pathology 1964, 77, 485 – 502.

    PubMed  CAS  Google Scholar 

  44. Osathanondh V and Potter EL. Pathogenesis of polycystic kidneys: Type 4 due to urethral occlusion. Archives of Pathology 1964, 77, 502 – 509.

    PubMed  CAS  Google Scholar 

  45. Perrone RD. In vitro function of cyst epithelium from human polycystic kidney. Journal of Clinical Investigation 1985, 76, 1688 – 1691.

    Article  PubMed  CAS  Google Scholar 

  46. Potter EL. Normal and abnormal development of the kidney. Chicago Year Book Medical Publishers, Inc., 1972.

    Google Scholar 

  47. Rall JE and Odel HM. Congenital polycystic disease of the kidney: Review of the literature, and data on 207 cases. American Journal of Medical Sciences 1949, 218, 399 – 407.

    Article  CAS  Google Scholar 

  48. Resnick JS, Brown DM and Vernier RL. Normal development and experimental models of cystic renal disease. In: Gardner KD Jr, ed, Cystic Diseases of the Kidney. New York: John Wiley & Sons, 1976, 221 – 241.

    Google Scholar 

  49. Torres VE, Berndt TJ, Okamura M, Nesbit JW, Holley KE, Carone FA, Knox FG and Romero JC. Mechanisms affecting the development of renal cystic disease induced by diephenylthiazole. Kidney International 1988, 33, 1130 – 1139.

    Article  PubMed  CAS  Google Scholar 

  50. Uchic ME, Donoso VS, Kornhaus J, Cragoe EJ and Grantham JJ. Alteration of MDCK cyst enlargement in hydrated collagen gel by inhibitors of sodium transport. (abstract) Kidney International 1988, 33, 386.

    Google Scholar 

  51. Welling LW and Grantham JJ. Physical properties of isolated perfused renal tubules and tubular basement membranes. Journal of Clinical Investigation 1972, 51, 1063 – 1075.

    Article  PubMed  CAS  Google Scholar 

  52. Welling LW and Welling DJ. Physical properties of isolated perfused basement membranes from rabbit loop of Henle. American Journal of Physiology 1978, 234, F54 – F58.

    PubMed  CAS  Google Scholar 

  53. Welling LW and Welling DJ. Kinetics of cyst development in cystic renal disease. In: Cummings NB and Klahr S, eds, Chronic Renal Disease. New York: Plenum Medical Book Company, 1985, 95 – 103.

    Google Scholar 

  54. Welling LW and Welling DJ. Theoretical models of cyst formation and growth. Scanning Electron Microscopy 1988, 2, 1097 – 1102.

    CAS  Google Scholar 

  55. Wickre CG and Bennett WM. Renal cyst epithelial transport in non-uremic polycystic kidney disease. Kidney International 1983, 23, 514 – 518.

    Article  PubMed  CAS  Google Scholar 

  56. Wilson PD, Schrier RW, Breckon RD and Gabow PA. A new method for studying human polycystic kidney disease epithelia in culture. Kidney International 1986, 30, 371 – 378.

    Article  PubMed  CAS  Google Scholar 

Download references

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 1990 Kluwer Academic Publishers

About this chapter

Cite this chapter

Welling, L.W. (1990). Pathogenesis of Cysts and Cystic Kidneys. In: Gardner, K.D., Bernstein, J. (eds) The Cystic Kidney. Developments in Nephrology, vol 27. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-0457-6_5

Download citation

  • DOI: https://doi.org/10.1007/978-94-009-0457-6_5

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-010-6690-7

  • Online ISBN: 978-94-009-0457-6

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics