ABSTRACT
Both the incidence and prevalence of chronic kidney disease are rising rapidly all over the world, which brings heavy economic and mental burden to the patients and the society. A retrospective study of the European Renal Association _ European Dialysis and Transplant Association( ERA _ EDTA) Registry showed cystic kidney disease was one of the three most prevalent rare diseases leading to renal replacement therapy before the age of 20 years. Renal cystic disease encompasses a variety of diseases that cause single or multiple cysts in the kidneys,and can be divided into 3 categories:cystic kidney disease due to fetal renal malformations,hereditary cystic kidney diseases and acquired cystic kidney diseases. Acquired renal cysts rarely occur in the general pediatric population. Renal cystic disease can present during fetus,infancy,childhood or adulthood. The onset of the disease is occult,and the clinical manifestations are heterogeneous. The manifestations may be confined to the kidney or as part of a syndrome. On renal imaging,the kidney size is normal,enlarged or small,and cysts can involve unilateral or bilateral kidneys. Pamily history investigation should be performed when collecting first_hand clinical data. Genetic testing can confirm the diagnosis of hereditary renal cystic disease. Cystic kidney disease is currently treated symptomatically,and patients with end_stage renal disease require renal replacement therapy.