Polycystic liver disease

A predominantly autosomal dominant hereditary condition characterized by multiple cysts of various sizes in the LIVER of affected patients. May also occur together with AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE. Germline mutations in PRKCSH and SEC63 genes have been identified. OMIM: 174050

Also Known As:

Isolated autosomal dominant polycystic liver disease; Isolated polycystic liver disease

Networked: 335 relevant articles (18 outcomes, 23 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Autosomal Dominant Polycystic Kidney (ADPKD)
2.	Cysts
3.	Polycystic Kidney Diseases (Polycystic Kidney Disease)
4.	Disease Progression
5.	Sclerosis

Experts

1.	Drenth, Joost P H: 45 articles (01/2022 - 04/2004)
2.	Torres, Vicente E: 20 articles (01/2020 - 01/2003)
3.	LaRusso, Nicholas F: 17 articles (01/2022 - 12/2008)
4.	Gevers, Tom J G: 16 articles (01/2022 - 05/2011)
5.	Hogan, Marie C: 15 articles (01/2021 - 06/2010)
6.	Masyuk, Tatyana V: 14 articles (01/2022 - 03/2007)
7.	Nevens, Frederik: 13 articles (12/2019 - 10/2009)
8.	Masyuk, Anatoliy I: 12 articles (01/2022 - 03/2007)
9.	Kievit, Wietske: 11 articles (12/2019 - 11/2014)
10.	Ubara, Yoshifumi: 10 articles (01/2022 - 06/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Polycystic liver disease:

1.	1-dodecylpyridoxal (PLD)IBA 10/01/2022 - "Purpose: We investigated the long-term safety and efficacy of hepatic transarterial embolization (TAE) in patients with symptomatic polycystic liver disease (PLD). " 09/25/2023 - "Factors related to the development and severity of polycystic liver disease (PLD) have not been well established. " 05/01/2023 - "Polycystic liver disease (PLD) is characterized by the progressive development of polycystic lesions in the kidney and the liver, possibly resulting in dual organ failure. " 01/01/2023 - "Polycystic liver disease (PLD) causes symptoms resulting from cystic volume expansion. " 01/01/2023 - "Cholangiocytes transit from quiescence to hyperproliferation during cystogenesis in polycystic liver disease (PLD), the severity of which displays prominent sex differences. "
2.	Somatostatin (Somatotropin Release-Inhibiting Factor)IBA 12/06/2021 - "The effects of somatostatin analogues on liver volume and quality of life in polycystic liver disease: a meta-analysis of randomized controlled trials." 09/01/2015 - "We pooled data from two randomized, double-blind, placebo-controlled trials that evaluated HRQL using the Short-Form 36 (SF-36) in 96 polycystic liver disease patients treated 6-12 months with somatostatin analogues or placebo. " 09/01/2015 - "Somatostatin analogues improve health-related quality of life in polycystic liver disease: a pooled analysis of two randomised, placebo-controlled trials." 03/01/2014 - "Recent clinical studies indicate that somatostatin analogues are promising for treating polycystic liver disease and potentially also for the kidney phenotype. " 11/01/2012 - "Although there are no curative medical options for polycystic liver disease, somatostatin analogs hold promise and have shown minimal efficacy in human studies. "
3.	lanreotide (Somatuline)FDA Link 08/01/2013 - "Safety and efficacy of different lanreotide doses in the treatment of polycystic liver disease: pooled analysis of individual patient data." 01/01/2019 - "Further Evidence That Lanreotide Reduces Liver Growth in Patients With Polycystic Liver Disease, But Not the End of the Story." 01/01/2012 - "The long-term outcome of patients with polycystic liver disease treated with lanreotide." 05/01/2015 - "The aim of this open-label clinical trial (RESOLVE trial) was to assess the efficacy of 6-month lanreotide treatment, 120 mg, subcutaneously every 4 weeks in ADPKD patients with symptomatic polycystic liver disease. " 01/01/2019 - "In this subanalysis of a randomized trial of patients with polycystic liver disease due to ADPKD, lanreotide for 120 weeks reduced the growth of liver and combined liver and kidney volume. "
4.	Octreotide (Sandostatin)FDA LinkGeneric 08/01/2015 - "Efficacy of 4 Years of Octreotide Long-Acting Release Therapy in Patients With Severe Polycystic Liver Disease." 04/01/2016 - "We included 153 polycystic liver disease patients (86% female, median liver volume 4974 ml) from three international centres, all treated with octreotide (n = 70) or lanreotide (n = 83). " 03/01/2007 - "Octreotide inhibits hepatic cystogenesis in a rodent model of polycystic liver disease by reducing cholangiocyte adenosine 3',5'-cyclic monophosphate." 09/01/2012 - "We showed in a randomized double-blinded placebo-controlled clinical trial that octreotide long-acting repeatable depot.® (OctLAR(®)) for 12 months reduces kidney and liver growth in autosomal dominant polycystic kidney patients with severe polycystic liver disease (PLD) and liver growth in patients with severe isolated PLD. " 08/01/2015 - "To observe the effect on total liver volume (TLV) on and off therapy in selected symptomatic patients with autosomal dominant polycystic kidney disease (ADPKD) or autosomal dominant polycystic liver disease (PLD) who received octreotide long-acting release (OctLAR) for up to 4 years. "
5.	Tolvaptan FDA Link 01/01/2020 - "The management of polycystic liver disease by tolvaptan." 05/01/2017 - "Tolvaptan for the Treatment of Enlarged Polycystic Liver Disease." 01/01/2022 - "Potential effect of tolvaptan on polycystic liver disease for patients with ADPKD meeting the Japanese criteria of tolvaptan use." 11/01/2016 - "Liver transplantation (LT) is the only curative treatment for patients with severe polycystic liver disease associated with ADPKD, but considering that liver injuries should be avoided particularly in patients who underwent LT, we must be careful to start tolvaptan in post-LT ADPKD patients. " 05/01/2017 - "The patient's abdominal fullness began to improve and liver volume, indicating advanced polycystic liver disease (PLD), decreased from 9,750 mL to 8,345 mL after 17 months of tolvaptan treatment, though there was no significant change in kidney volume. "
6.	Leuprolide (Lupron)FDA LinkGeneric 02/25/2022 - "Protocol for a randomized controlled multicenter trial assessing the efficacy of leuprorelin for severe polycystic liver disease: the AGAINST-PLD study."
7.	pasireotideIBA 01/01/2020 - "Individuals with severe polycystic liver disease were assigned in a 2:1 ratio in a 1-year, double-blind, randomized trial to receive pasireotide long-acting release or placebo. " 01/01/2020 - "Pasireotide LAR in Severe Polycystic Liver Disease, NCT01670110 PODCAST: This article contains a podcast at https://www.asn-online.org/media/podcast/CJASN/2020_08_28_CJN13661119.mp3." 01/01/2020 - "We assessed safety and efficacy of another somatostatin receptor analog, pasireotide long-acting release, in severe polycystic liver disease and autosomal dominant polycystic kidney disease. "
8.	ethanolamine oleate (Ethamolin)FDA Link 12/01/2009 - "Percutaneous aspiration and ethanolamine oleate sclerotherapy for sustained resolution of symptomatic polycystic liver disease: an initial experience." 12/01/2009 - "This initial experience with a single session of percutaneous aspiration and ethanolamine oleate sclerosis resulted in sustained resolution of symptomatic polycystic liver disease with minimal morbidity, avoidance of surgery, and improvement in quality of life." 12/01/2009 - "The purpose of this study was to evaluate percutaneous aspiration with ethanolamine oleate sclerosis in the management of symptomatic polycystic liver disease. "
9.	Somatostatin Receptors (Somatostatin Receptor)IBA 10/01/2017 - "TGR5 contributes to hepatic cystogenesis by increasing cAMP and enhancing cholangiocyte proliferation; our data suggest that a TGR5 antagonist alone or concurrently with somatostatin receptor agonists represents a potential therapeutic approach in polycystic liver disease. " 01/01/2018 - "Combination of a Histone Deacetylase 6 Inhibitor and a Somatostatin Receptor Agonist Synergistically Reduces Hepatorenal Cystogenesis in an Animal Model of Polycystic Liver Disease." 01/01/2020 - "We assessed safety and efficacy of another somatostatin receptor analog, pasireotide long-acting release, in severe polycystic liver disease and autosomal dominant polycystic kidney disease. "
10.	Minocycline (Cyclops)FDA LinkGeneric 01/01/1998 - "Single-shot injection of minocycline hydrochloride is an effective treatment for symptomatic solitary hepatic cysts but is less effective in polycystic liver disease." 04/01/2017 - "The medical records of patients with autosomal dominant polycystic kidney disease or isolated polycystic liver disease, who underwent minocycline hydrochloride cyst aspiration sclerotherapy for their symptomatic PLD, were retrospectively analyzed. "

Therapies and Procedures

1.	Therapeutics 09/19/2023 - "Higher need for polycystic liver disease therapy in female patients: sex-specific association between liver volume and need for therapy." 01/01/2023 - "A polycystic liver disease-specific questionnaire can be used as a guide on when to start a volume reduction therapy and to assess the effect of treatment." 01/01/2023 - "Expanding the clinical application of the polycystic liver disease questionnaire: determination of a clinical threshold to select patients for therapy." 01/01/2016 - "Medical therapy for polycystic liver disease." 01/01/2016 - "Current therapies for polycystic liver disease are invasive and have high recurrence risks. "
2.	Sclerotherapy 12/01/2022 - "Trans sigmoid colon NOTES sclerotherapy for polycystic liver disease." 03/01/2015 - "Ultrasound-guided percutaneous drainage and sclerotherapy in a patient with isolated autosomal dominant polycystic liver disease." 05/01/1995 - "Management of symptomatic polycystic liver disease: laparoscopy adjuvant with alcohol sclerotherapy." 11/20/1993 - "In patients with complicated polycystic liver disease various treatments, such as needle aspiration, alcohol sclerotherapy or surgery, must be considered." 06/14/2007 - "Results of percutaneous sclerotherapy and surgical treatment in patients with symptomatic simple liver cysts and polycystic liver disease."
3.	Microspheres (Microsphere) 10/01/2022 - "The Safety and Efficacy of Hepatic Transarterial Embolization Using Microspheres and Microcoils in Patients with Symptomatic Polycystic Liver Disease." 01/01/2013 - "[Preliminary experience of transcatheter hepatic artery embolization using microspheres for polycystic liver disease]."
4.	Length of Stay 09/01/1998 - "Selected patients with symptomatic but uncomplicated polycystic liver disease and favourable anatomy benefit from laparoscopic fenestration of the cysts with low morbidity and hospital stay." 12/01/2014 - "In all three periods analysed, the laparoscopic approach showed a statistically significant reduction in operative time (p = 0.009) and hospital stay (p = 0.001) and a significant (p < 0.05) reduction rate in symptomatic recurrences in patients with polycystic liver disease (25 %) as compared with simple liver cysts (7.5 %). "
5.	Operative Time 12/01/2014 - "In all three periods analysed, the laparoscopic approach showed a statistically significant reduction in operative time (p = 0.009) and hospital stay (p = 0.001) and a significant (p < 0.05) reduction rate in symptomatic recurrences in patients with polycystic liver disease (25 %) as compared with simple liver cysts (7.5 %). "