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Autosomal dominant polycystic kidney disease: updated perspectives

Authors Rastogi A, Ameen KM, Al-Baghdadi M, Shaffer K, Nobakht N, Kamgar M, Lerma EV

Received 31 May 2019

Accepted for publication 1 August 2019

Published 26 August 2019 Volume 2019:15 Pages 1041—1052


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Professor Garry Walsh

Anjay Rastogi,1 Khalid Mohammed Ameen,1 Maha Al-Baghdadi,1 Kelly Shaffer,1 Niloofar Nobakht,1 Mohammad Kamgar,1 Edgar V Lerma2

1Department of Medicine, Division of Nephrology, David Geffen School of Medicine, Los Angeles, CA, USA; 2Department of Medicine, Divison of Nephrology, University of Illinois at Chicago/Advocate Christ Medical Center, Section of Nephrology, Oak Lawn, IL, USA

Correspondence: Edgar V Lerma
University of Illinois at Chicago/Advocate Christ Medical Center, 4400 W 95th, Oak Lawn, IL 60453, USA
Tel +1 708 227 7305

Abstract: Autosomal dominant polycystic kidney disease (ADPKD) is an inherited multisystem disorder, characterized by renal and extra-renal fluid-filled cyst formation and increased kidney volume that eventually leads to end-stage renal disease. ADPKD is considered the fourth leading cause of end-stage renal disease in the United States and globally. Care of patients with ADPKD was, for a long time, limited to supportive lifestyle measures, due to the lack of therapeutic strategies targeting the main pathways involved in the pathophysiology of ADPKD. As the first FDA approved treatment of ADPKD, Vasopressin (V2) receptor blocking agent, tolvaptan, is an urgently awaited advance for ADPKD patients. In our review, we also shed some lights on what is beyond Tolvaptan as there are other medications in the pipeline and many medications have been or are currently being studied in clinical trials such as Tesevatinib, Metformin and Pravastatin, with the goal of slowing the rate of progression of ADPKD by reducing the increase in total kidney volume or maintaining eGFR. Here, we review updates in the perspectives and management of ADPKD.

Keywords: vasopressin receptor antagonist, tolvaptan, metformin, total kidney volume, chronic kidney disease, hypertension

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