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Mechanism of disease

Multiple factors contribute to the formation and growth of renal cysts in autosomal dominant polycystic kidney disease (ADPKD)
 

The 2 main mechanisms leading to cyst formation and expansion are fluid secretion and cell proliferation. Over time, these cysts contribute to an increase in total kidney volume (TKV), destruction of kidney tissue, and diminishing kidney function.1-5

While much is known about the etiology and pathophysiology of ADPKD, advancement of the disease can often go unnoticed, as normal kidney function can mask the severity of disease progression until irreversible damage has already occurred.6,7

Uncover the science behind ADPKD

  1. Chapin HC, Caplan MJ. The cell biology of polycystic kidney disease. J Cell Biol. 2010;191(4):701-710.

  2. Yamaguchi T, Pelling JC, Ramaswamy NT, et al. cAMP stimulates the in vitro proliferation of renal cyst epithelial cells by activating the extracellular signal-regulated kinase pathway. Kidney Int. 2000;57(4):1460-1471.

  3. Patel V, Chowdhury R, Igarashi P. Advances in the pathogenesis and treatment of polycystic kidney disease. Curr Opin Nephrol Hypertens. 2009;18(2):99-106. doi:10.1097/MNH.0b013e3283262ab0

  4. Grantham JJ, Mulamalla S, Swenson-Fields KI. Why kidneys fail in autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 2011;7(10):556-566.

  5. Grantham JJ, Torres VE. The importance of total kidney volume in evaluating progression of polycystic kidney disease. Nat Rev Nephrol. 2016;12(11):667-677.

  6. Grantham JJ, Chapman AB, Torres VE. Volume progression in autosomal dominant polycystic kidney disease: the major factor determining clinical outcomes. Clin J Am Soc Nephrol. 2006;1(1):148-157.

  7. Braun WE. Autosomal dominant polycystic kidney disease: emerging concepts of pathogenesis and new treatments. Cleve Clin J Med. 2009;76(2):97-104.