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Polycystic Kidney/Liver Disease.

Rebecca Roediger1, Douglas Dieterich1, Pramodh Chanumolu2

  • 1Division of Liver Disease, Department of Medicine, Icahn School of Medicine, 1 Gustave L Levy Place, Box 1123, New York, NY 10029, USA.

Clinics in Liver Disease
|April 29, 2022
PubMed
Summary
This summary is machine-generated.

Autosomal dominant polycystic kidney disease (ADPKD) causes kidney and liver cysts. Treatments like tolvaptan slow kidney issues, while liver transplant is the only cure for polycystic liver disease (PCLD).

Keywords:
Autosomal dominant polycystic kidney diseaseEnd-stage kidney diseasePolycystic liver diseasePolycystic renal disease

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Area of Science:

  • Nephrology
  • Hepatology
  • Genetics

Background:

  • Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder leading to chronic kidney disease and end-stage kidney disease (ESKD).
  • Polycystic liver disease (PCLD) is the most common extrarenal manifestation of ADPKD, characterized by multiple liver cysts and hepatomegaly, despite preserved liver function.
  • While isolated PCLD and ADPKD-related PCLD are genetically distinct, their clinical progression involving liver cyst growth is similar.

Purpose of the Study:

  • To review the current understanding of polycystic liver disease (PCLD) in the context of autosomal dominant polycystic kidney disease (ADPKD).
  • To discuss therapeutic strategies for managing cyst progression in both the kidneys and liver associated with ADPKD.
  • To highlight the role of transplantation in treating end-stage kidney disease (ESKD) and PCLD.

Main Methods:

  • Review of existing literature on ADPKD, PCLD, and their management.
  • Analysis of therapeutic options including pharmacological treatments and transplantation.
  • Synthesis of clinical information regarding disease progression and treatment efficacy.

Main Results:

  • Tolvaptan has demonstrated efficacy in slowing renal function decline and cyst growth in ADPKD patients.
  • Somatostatin analogs show a temporary effect in slowing polycystic liver growth but are not a long-term solution.
  • Liver transplantation remains the only curative option for PCLD, and renal transplantation significantly improves survival for ADPKD patients with ESKD.

Conclusions:

  • Management of ADPKD and PCLD requires a multi-faceted approach, addressing both renal and hepatic manifestations.
  • Pharmacological interventions offer symptomatic relief and disease modification, but transplantation is definitive for advanced disease.
  • Further research into targeted therapies for PCLD is warranted to improve patient outcomes and quality of life.