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Extrarenal Manifestations of Autosomal Dominant Polycystic Kidney Disease: Polycystic Liver Disease

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Polycystic Kidney Disease

Abstract

Liver involvement is the most frequent extrarenal organ manifestation seen in autosomal dominant polycystic kidney disease (ADPKD). Most patients with polycystic liver disease (PLD) are asymptomatic and do not require interventions, but an important minority of individuals with symptomatic PLD can have challenging clinical problems resulting from massive hepatomegaly and its associated complications. These individuals can also experience a progressive decline in health status and quality of life. We discuss disease pathogenesis, prevalence, complications, and management. The current role of surgical interventions such as cyst fenestration, partial hepatectomy, liver transplantation, and recent advances in current and medical therapies is reviewed.

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Authors and Affiliations

  1. Mayo Medical School, Division of Nephrology & Hypertension, Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA

    Fouad T. Chebib MD & Marie C. Hogan MD, PhD

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  1. Fouad T. Chebib MD
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  2. Marie C. Hogan MD, PhD
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Correspondence to Marie C. Hogan MD, PhD .

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Editors and Affiliations

  1. John Gammill Professor in Polycystic Kidney Disease, Chief, Section of Nephrology and Hypertension, Department of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA

    Benjamin D. Cowley, Jr.

  2. Federal Express Chair of Excellence, Chief, Division of Nephrology, Department of Pediatrics, Director, Tuberous Sclerosis Center of Excellence at Le Bonheur Children’s Hospital, Medical Director, Nephrology St. Jude Children’s Research Hospital, University of Tennessee Health Science Center, Children’s Foundation Research Center, Memphis, Tennessee, USA

    John J. Bissler

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Chebib, F.T., Hogan, M.C. (2018). Extrarenal Manifestations of Autosomal Dominant Polycystic Kidney Disease: Polycystic Liver Disease. In: Cowley, Jr., B., Bissler, J. (eds) Polycystic Kidney Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-7784-0_10

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