Advances in Autosomal Dominant Polycystic Kidney Disease: A Clinical Review

Niloofar Nobakht; Ramy M Hanna; Maha Al-Baghdadi; Khalid Mohammed Ameen; Farid Arman; Ehsan Nobahkt; Mohammad Kamgar; Anjay Rastogi

doi:10.1016/j.xkme.2019.11.009

Advances in Autosomal Dominant Polycystic Kidney Disease: A Clinical Review

Kidney Med. 2020 Feb 22;2(2):196-208. doi: 10.1016/j.xkme.2019.11.009. eCollection 2020 Mar-Apr.

Authors

Niloofar Nobakht¹, Ramy M Hanna^{1

2}, Maha Al-Baghdadi^{1

3}, Khalid Mohammed Ameen¹, Farid Arman^{1

4}, Ehsan Nobahkt⁵, Mohammad Kamgar¹, Anjay Rastogi¹

Affiliations

¹ Division of Nephrology, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA.
² Division of Nephrology, Department of Medicine, University of California Irvine, Orange, CA.
³ Department of Medicine, University of Alabama Birmingham Huntsville Regional Campus, Huntsville, AL.
⁴ Department of Medicine, University of Pennsylvania Medical Center, Philadelphia, PA.
⁵ Division of Renal Diseases and Hypertension, Department of Medicine, George Washington University, Washington, DC.

Abstract

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.

Keywords: ADH; ADPKD; Polycystic kidney disease; TKV; autosomal dominant polycystic kidney disease; tolvaptan.

Publication types

Review