Polycystic Kidney/Liver Disease

Rebecca Roediger; Douglas Dieterich; Pramodh Chanumolu; Priya Deshpande

doi:10.1016/j.cld.2022.01.009

Polycystic Kidney/Liver Disease

Clin Liver Dis. 2022 May;26(2):229-243. doi: 10.1016/j.cld.2022.01.009. Epub 2022 Apr 1.

Authors

Rebecca Roediger¹, Douglas Dieterich², Pramodh Chanumolu³, Priya Deshpande³

Affiliations

¹ Division of Liver Disease, Department of Medicine, Icahn School of Medicine, 1 Gustave L Levy Place, Box 1123, New York, NY 10029, USA. Electronic address: Rebecca.roediger@mssm.edu.
² Division of Liver Disease, Department of Medicine, Icahn School of Medicine, 1 Gustave L Levy Place, Box 1123, New York, NY 10029, USA.
³ Division of Nephrology, Department of Medicine, Icahn School of Medicine, 1 Gustave L Levy Place, Box 1123, New York, NY 10029, USA.

PMID: 35487607
DOI: 10.1016/j.cld.2022.01.009

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder that leads to chronic kidney disease and end-stage kidney disease (ESKD). Polycystic liver disease (PCLD) is the most common extrarenal manifestation of ADPKD. Though isolated PCLD and PCLD due to ADPKD are genetically distinct, they follow a similar clinical course of hepatomegaly from multiple cysts with preserved liver function. Tolvaptan use in ADPKD can slow down the deterioration of renal function and growth of cysts. Somatostatin analogs can slow the growth of polycystic livers but the effect is short-lived. The only curative therapy for PCLD is liver transplantation. Renal transplantation can significantly improve survival in patients with ESKD due to ADPKD.

Keywords: Autosomal dominant polycystic kidney disease; End-stage kidney disease; Polycystic liver disease; Polycystic renal disease.

Publication types

Review

MeSH terms

Cysts* / therapy
Female
Humans
Kidney / physiology
Liver Diseases* / etiology
Liver Diseases* / therapy
Male
Polycystic Kidney Diseases* / therapy
Polycystic Kidney, Autosomal Dominant* / therapy

Supplementary concepts

Polycystic liver disease