A Practical Approach to Polycystic Liver Disease

Abstract

http://aasldpubs.onlinelibrary.wiley.com/hub/journal/10.1002/(ISSN)2046-2484/video/14-5-reading-patel a video presentation of this article http://aasldpubs.onlinelibrary.wiley.com/hub/journal/10.1002/(ISSN)2046-2484/video/14-5-interview-patel the interview with the author

Current and Evolving Indications for Simultaneous Liver Kidney Transplantation

“…Polycystic liver disease (with or without kidney involvement) is broadly defined as greater than 20 epithelial-lined biliary cysts. 40 The most common form, autosomal dominant polycystic kidney disease (ADPKD), results from mutations most often in the PKD1 or PKD2 genes (although in many cases no mutation is identified). Less common is the autosomal recessive polycystic kidney disease associated with congenital hepatic fibrosis.…”

“…Massive hepatomegaly and compressive symptoms may occur, including esophageal reflux, nausea, vomiting, early satiety, weight loss, abdominal distention or pain, and dyspnea. 40 The hepatic severity is graded A (mildest) through D (most severe) based on the degree of symptoms and the number of hepatic segments that contain cysts. 41 The presence of diffuse hepatic cysts with severe symptoms designates class C, whereas class D also includes the presence of portal or hepatic vein occlusion due to the cysts.…”

Polycystic Liver Disease: A Case Report

“…PLD may be part of autosomal dominant polycystic liver disease (ADPLD) or extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD); with the latter is more common. 1,2 Several diagnostic criteria have been published to differentiate between ADPLD and ADPKD. Distinguishing ADPKD with ADPLD is important as the monitoring protocol, management, and prognosis may vary greatly.…”

“…2 ADPKD is a multisystemic and progressive disease characterized by cyst formation in kidney and other organs which warranted routine renal function monitoring because the risk of developing end stage renal disease. 1 Evaluation regarding the burden of PLD is warranted; because PLD is often mistakenly as benign disease and no treatment required. Treatment is indicated in symptomatic patients.…”

“…Liver transplantation is the only definitive cure for PLD. [1][2][3] Until now, there is no specific guideline published for PLD. Here, we present a recently symptomatic 73-year-old-female with PLD and acute cholecystitis.…”