2016年10月27日星期四

Process of Renal Fibrosis in Polycystic Kidney Disease

How does cysts of PKD happen and why they can cause renal function injury? PKD belongs to hereditary Kidney Diseases. Injuring process of PKD is mainly the process of Renal Fibrosis.

Congenital gene mutation can cause cystic gene secretion which can enlarge cysts of Polycystic Kidney. Under the action of cystic wall, tubular basement membrane become abnormal, and continuously secrete growth factors, like EGF, TGF-β, IGF,etc, which can increase proliferation and secretion of epithelial cells on tubular wall of Polycystic Kidney. Proliferation of epithelial cells can cause renal tubule obstruction which can lead to fluid retention in upper tubular, and then cysts forms.

Besides, proliferation of epithelial cells can also lead to semi-obstruction at cysts neck, which causes difficulties for fluid to go out of human body. For this reason, cysts can keep growing.

With the development of Renal Fibrosis, synthesis of Extracellular Matrix increases, with less degradation, which causes an unbalance between ECM synthesis and degradation. Massive ECM deposit and accumulate in human body, resulting in Glomerular Sclerosis and renal tubular atrophy. Renal fibrosis tissue replaces healthy nephron gradually, and finally Renal Fibrosis forms.

According to pathogenesis of congenital Polycystic Kidney Disease, there are three main causes of cysts forming and developing. Firstly, cystic gene of PKD. Secondly, proliferation and secretion of renal tubule and epithelial cells on cyst wall. Thirdly, composition changes of ECM as well as development of Renal Fibrosis.


There are very few discomforts in the early stage of Polycystic Kidney Disease which is the main reason why the disease is always neglected by patients. Patients are suggested to accept treatment as early as possible, in order to avoid renal function injury and prevent Renal Fibrosis developing into cicatricial tissue, aggravating to Uremia.

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