Medullary cystic kidney disease

Medullary cystic kidney disease (MCKD) is an autosomal dominant kidney disorder characterized by tubulointerstitial sclerosis leading to end-stage renal disease. While the name is misleading, recent research has shown that the presence cysts is not pathognomonic of the disease. For this reason, it has been recently referred as the more accurate, medullary kidney disease. Importantly, if cysts are found in the medullary collecting ducts they can result in a shrunken kidney, unlike that of polycystic kidney disease. There are two known forms medullary cystic kidney disease, MKD1 and MKD2