Abstract:
Polycystic kidney disease (PKD) is one of the most common hereditary kidney diseases, which is characterized by progressive cyst growth and secondary hypertension. In addition to cystogenesis and renal abnormalities, patients with PKD can develop vascular abnormalities and cardiovascular complications. Progressive cyst growth substantially alters renal structure and culminates into end-stage renal disease. There remains no cure beyond renal transplantation, and treatment options remain largely limited to chronic renal replacement therapy. In addition to end-stage renal disease, patients with PKD also present with hypertension and cardiovascular disease, yet the timing and interactions between the cardiovascular and renal effects of PKD progression are understudied. Here, we review the vascular dysfunction found in clinical and preclinical models of PKD, including the clinical manifestations and relationship to hypertension, stroke, and related cardiovascular diseases. Finally, our discussion also highlights the critical questions and emerging areas in vascular research in PKD.
摘要:
多囊肾病(PKD)是最常见的遗传性肾脏疾病之一。其特征是进行性囊肿生长和继发性高血压。除了膀胱发生和肾脏异常,PKD患者可发生血管异常和心血管并发症。囊肿的进行性生长大大改变了肾脏结构,并最终导致终末期肾脏疾病。除了肾移植,还没有治愈方法,和治疗选择仍然很大程度上限于慢性肾脏替代疗法。除了终末期肾病,PKD患者还存在高血压和心血管疾病,然而PKD进展对心血管和肾脏影响的时间和相互作用仍未得到充分研究.这里,我们回顾了在PKD的临床和临床前模型中发现的血管功能障碍,包括临床表现和与高血压的关系,中风,和相关的心血管疾病。最后,我们的讨论还强调了PKD血管研究中的关键问题和新兴领域.