Brief Introduction to Renal Cystic Disease

Renal cystic disease comprises a wide range of disease entities. They can be classified as either 

(1) hereditary or acquired
or
(2) systemic or renal confined diseases

that have the common feature of multiple renal cysts. Each disease entity differs in its presentation, prognosis, and management.

Renal cysts are smooth-walled, fluid-filled circular structures formed by focal outpouching of renal tubules. The pathogenesis of cyst formation has not been entirely elucidated. However, tremendous strides have been made in recent years. For autosomal dominant and autosomal recessive polycystic kidney diseases (ADPKD and ARPKD), a picture is starting to emerge. Defects in the primary ciliary sensing mechanisms, intracellular calcium regulation, and cellular cyclic AMP (cAMP) accumulation all seem to play a role in the altered cellular phenotype and functions.