Nobakht Niloofar, Hanna Ramy M, Al-Baghdadi Maha, Ameen Khalid Mohammed, Arman Farid, Nobahkt Ehsan, Kamgar Mohammad, Rastogi Anjay
Division of Nephrology, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA.
Division of Nephrology, Department of Medicine, University of California Irvine, Orange, CA.
Kidney Med. 2020 Feb 22;2(2):196-208. doi: 10.1016/j.xkme.2019.11.009. eCollection 2020 Mar-Apr.
Polycystic kidney disease (PKD) is a multiorgan disorder resulting in fluid-filled cyst formation in the kidneys and other systems. The replacement of kidney parenchyma with an ever-increasing volume of cysts eventually leads to kidney failure. Recently, increased understanding of the pathophysiology of PKD and genetic advances have led to new approaches of treatment targeting physiologic pathways, which has been proven to slow the progression of certain types of the disease. We review the pathophysiologic patterns and recent advances in the clinical pharmacotherapy of autosomal dominant PKD. A multipronged approach with pharmacologic and nonpharmacologic treatments can be successfully used to slow down the rate of progression of autosomal dominant PKD to kidney failure.
多囊肾病(PKD)是一种多器官疾病,可导致肾脏和其他系统中形成充满液体的囊肿。肾实质被不断增多的囊肿所取代最终会导致肾衰竭。最近,对PKD病理生理学的深入了解和遗传学进展带来了针对生理途径的新治疗方法,事实证明这些方法可减缓某些类型疾病的进展。我们综述了常染色体显性PKD的病理生理模式和临床药物治疗的最新进展。采用药物和非药物治疗的多管齐下方法可成功用于减缓常染色体显性PKD发展至肾衰竭的速度。