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Cystic kidney disease refers to a wide range of hereditary, developmental, and acquired conditions [1] and with the inclusion of neoplasms with cystic changes, over 40 classifications and subtypes have been identified. Depending on the disease classification, the presentation may be at birth, or much later into adult life.
Medullary cystic kidney disease (MCKD) is an autosomal dominant kidney disorder characterized by tubulointerstitial sclerosis leading to end-stage renal disease.Because the presence of cysts is neither an early nor a typical diagnostic feature of the disease, and because at least four different gene mutations may give rise to the condition, the name autosomal dominant tubulointerstitial kidney ...
Polycystic kidney disease (PKD or PCKD, also known as polycystic kidney syndrome) is a genetic disorder [5] [6] in which the renal tubules become structurally abnormal, resulting in the development and growth of multiple cysts within the kidney. [7] These cysts may begin to develop in utero, in infancy, in childhood, or in adulthood. [8]
Affected individuals have small kidneys with several cysts and their risk of renal cell carcinoma is 30 times higher than people without ACKD. Bloody urine and flank pain in a person with end-stage kidney disease raise suspicion for ACKD-associated renal cell carcinoma. Although people with ACKD have a substantially higher risk of renal cell ...
Glomerulocystic kidney disease (GCKD) is a renal cystic disease that can be classified in five major groups [3] including familial, familial/sporadic heritable, glomerulocystic kidneys as a component of other cystic disease, sporadic, and acquired.
Among American patients on the kidney-transplant waiting list (as of December 2011), 7256 (8.4%) were listed due to cystic kidney disease and of the 16,055 renal transplants performed in 2011, 2057 (12.8%) were done for patients with cystic kidney disease, with 1,189 from deceased donors and 868 from living donors.