Polycystic kidney is a genetic disease that mainly manifests as multiple cysts in both kidneys in the shape of grapes, eventually causing renal failure uremic syndrome, accounting for about 10% of all uremic patients. It is divided into autosomal dominant (ADPKD) and autosomal recessive (ARPKD), the former both occurring mainly in adults and the latter both occurring mainly in pediatric patients. Although the pathogenesis of the two diseases differs, with the former showing focal lesions and the latter cysts showing spindle expansion, the pathogenesis is the same at the cellular level, such as poor differentiation of the epithelium, repetitive expression of proteins, increased proliferation and apoptosis, abnormal protein action and abnormalities in transport and fluid transport. ADPKD is genetically heterogeneous and two genes have been identified: PKD1, located at 16p13.3; in 85% of patients; and PKD2, located at 4q21, in 15% of patients. In ARPKD, one gene, PKHD1, was identified and is present in all patients. polycystin1 (460 kDa) and polycystin2 (110 kDa), the proteins encoded by PKD1 and PKD2, are two transmembrane proteins with 11 and 6 domains, respectively, thus forming a functional complex. polycystin2 is a calcium channel that can sense ARPKD proteins have a transmembrane channel and a large extracellular region. studies on the etiology of PKD are mainly from animal models, either due to primary defects or due to pleiotropic causes. Studies over the past 7 years have shown that the pathogenesis of polycystic kidney in humans and rodents is related to primary cilia and the basal body. Primary cilia are single hair-like organelles that are found on the surface of most mammalian cells. Like the tubular epithelium of the kidney, the cilia protrude from the lumen and are thought to have a sensory role. Primary cilia have a microtubular structure of 9+0 and are usually inactive. Active cilia have 9+2 microtubular structures that allow microtubules to move, and special nodular cilia are present in embryonic structures. The basal body starts from the centriole of the centrosome and the cilia start from the basal body. ADPKD is a late-onset and focal lesion that usually starts in adulthood although the patient carries a genetic mutation and the cyst only involves no more than 5% of the renal tubules. This phenomenon can be explained by the second strike theory: one of the pkd allele pairs (pkd1 or pkd2) has an embryonic mutation due to parental inheritance or spontaneous mutation, and all renal epithelial cells carry this mutation, but it is not sufficient to cause renal cysts. In some individuals, due to genetic factors and acquired environmental effects, another normal haploid of the renal tubule also has a somatic mutation that completely inactivates the pkd gene, leading to the formation of cysts. Thus, although ADPKD is inherited in a dominant manner, it is inherited in a recessive manner at the molecular level. The second strike theory has been confirmed by several foreign studies, the strongest evidence of which is the monoclonal origin of cystic epithelial cells and the pkd2. mouse model (the presence of a duplication series of the pkd2 gene makes it susceptible to gene rearrangement and inactivation). However, it has also been shown that haploinsufficiency (mutation of only 1 allele) or crossover heterozygosity (e.g. pkd2 somatic mutation based on embryonic mutation pkdpkd1) can also cause cystogenesis and that cellular heterogeneity can be observed in the early and middle stages of cystogenesis.