Excellent survival after liver transplantation for isolated polycystic liver disease: an European Liver Transplant Registry study.

Patients with end-stage isolated polycystic liver disease (PCLD) suffer from incapacitating symptoms because of very large liver volumes. Liver transplantation (LT) is the only curative option. This study assesses the feasibility of LT in PCLD.

Patients with isolated polycystic liver disease referred to liver centres: clinical characterization of 137 cases.

Background And Aim: Isolated polycystic liver disease (PCLD) is characterized by the presence of multiple cysts in the liver in the absence of polycystic kidneys. The clinical profile of PCLD is poorly defined and we set up a study for the clinical characteristics of PCLD.

Methods: We collected clinical data on 188 PCLD patients (defined as >10 liver cysts) from five tertiary referral centres, and 137 patients were selected for the purpose of this study.

Lanreotide reduces the volume of polycystic liver: a randomized, double-blind, placebo-controlled trial.

Background & Aims: Therapy for polycystic liver is invasive, expensive, and has disappointing long-term results. Treatment with somatostatin analogues slowed kidney growth in patients with polycystic kidney disease (PKD) and reduced liver and kidney volume in a PKD rodent model. We evaluated the effects of lanreotide, a somatostatin analogue, in patients with polycystic liver because of autosomal-dominant (AD) PKD or autosomal-dominant polycystic liver disease (PCLD).

Carbohydrate antigen 19-9 is extremely elevated in polycystic liver disease.

Background/aims: Carbohydrate antigen 19-9 (CA19-9) is used as a biomarker to differentiate benign from malignant gastrointestinal disorders. We examined the value of CA19-9 measurement in polycystic livers after observing high CA19-9 cyst fluid levels in a benign polycystic liver case.

Methods: We determined CA19-9 levels in serum (n=120) and hepatic cyst fluid (n=81), from patients with polycystic livers (n=109) and simple hepatic cysts (n=24).

Aspiration-sclerotherapy results in effective control of liver volume in patients with liver cysts.

Purpose: To study the extent to which aspiration-sclerotherapy reduces liver volume and whether this therapy results in relief of symptoms.

Results: Four patients, group I, with isolated large liver cysts, and 11 patients, group II, with polycystic livers, underwent aspiration-sclerotherapy. Average volume of aspirated cyst fluid was 1,044 ml (range 225-2,000 ml) in group I and 1,326 ml (range 40-4,200 ml) in group II.