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Polycystic liver disease: Classification, diagnosis, treatment process, and clinical management

Polycystic liver disease (PLD) is a rare hereditary disease that independently exists in isolated PLD, or as an accompanying symptom of autosomal dominant polycystic kidney disease and autosomal recessive polycystic kidney disease with complicated mechanisms. PLD currently lacks a unified diagnostic...

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Autores principales: Zhang, Ze-Yu, Wang, Zhi-Ming, Huang, Yun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Baishideng Publishing Group Inc 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7097502/
https://www.ncbi.nlm.nih.gov/pubmed/32231761
http://dx.doi.org/10.4254/wjh.v12.i3.72
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author Zhang, Ze-Yu
Wang, Zhi-Ming
Huang, Yun
author_facet Zhang, Ze-Yu
Wang, Zhi-Ming
Huang, Yun
author_sort Zhang, Ze-Yu
collection PubMed
description Polycystic liver disease (PLD) is a rare hereditary disease that independently exists in isolated PLD, or as an accompanying symptom of autosomal dominant polycystic kidney disease and autosomal recessive polycystic kidney disease with complicated mechanisms. PLD currently lacks a unified diagnostic standard. The diagnosis of PLD is usually made when the number of hepatic cysts is more than 20. Gigot classification and Schnelldorfer classification are now commonly used to define severity in PLD. Most PLD patients have no clinical symptoms, and minority with severe complications need treatments. Somatostatin analogues, mammalian target of rapamycin inhibitor, ursodeoxycholic acid and vasopressin-2 receptor antagonist are the potentially effective medical therapies, while cyst aspiration and sclerosis, transcatheter arterial embolization, fenestration, hepatic resection and liver transplantation are the options of invasion therapies. However, the effectiveness of these therapies except liver transplantation are still uncertain. Furthermore, there is no unified strategy to treat PLD between medical centers at present. In order to better understand recent study progresses on PLD for clinical practice and obtain potential directions for future researches, this review mainly focuses on the recent progress in PLD classification, clinical manifestation, diagnosis and treatment. For information, we also provided medical treatment processes of PLD in our medical center.
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spelling pubmed-70975022020-03-31 Polycystic liver disease: Classification, diagnosis, treatment process, and clinical management Zhang, Ze-Yu Wang, Zhi-Ming Huang, Yun World J Hepatol Review Polycystic liver disease (PLD) is a rare hereditary disease that independently exists in isolated PLD, or as an accompanying symptom of autosomal dominant polycystic kidney disease and autosomal recessive polycystic kidney disease with complicated mechanisms. PLD currently lacks a unified diagnostic standard. The diagnosis of PLD is usually made when the number of hepatic cysts is more than 20. Gigot classification and Schnelldorfer classification are now commonly used to define severity in PLD. Most PLD patients have no clinical symptoms, and minority with severe complications need treatments. Somatostatin analogues, mammalian target of rapamycin inhibitor, ursodeoxycholic acid and vasopressin-2 receptor antagonist are the potentially effective medical therapies, while cyst aspiration and sclerosis, transcatheter arterial embolization, fenestration, hepatic resection and liver transplantation are the options of invasion therapies. However, the effectiveness of these therapies except liver transplantation are still uncertain. Furthermore, there is no unified strategy to treat PLD between medical centers at present. In order to better understand recent study progresses on PLD for clinical practice and obtain potential directions for future researches, this review mainly focuses on the recent progress in PLD classification, clinical manifestation, diagnosis and treatment. For information, we also provided medical treatment processes of PLD in our medical center. Baishideng Publishing Group Inc 2020-03-27 2020-03-27 /pmc/articles/PMC7097502/ /pubmed/32231761 http://dx.doi.org/10.4254/wjh.v12.i3.72 Text en ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved. http://creativecommons.org/licenses/by-nc/4.0/ This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial.
spellingShingle Review
Zhang, Ze-Yu
Wang, Zhi-Ming
Huang, Yun
Polycystic liver disease: Classification, diagnosis, treatment process, and clinical management
title Polycystic liver disease: Classification, diagnosis, treatment process, and clinical management
title_full Polycystic liver disease: Classification, diagnosis, treatment process, and clinical management
title_fullStr Polycystic liver disease: Classification, diagnosis, treatment process, and clinical management
title_full_unstemmed Polycystic liver disease: Classification, diagnosis, treatment process, and clinical management
title_short Polycystic liver disease: Classification, diagnosis, treatment process, and clinical management
title_sort polycystic liver disease: classification, diagnosis, treatment process, and clinical management
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7097502/
https://www.ncbi.nlm.nih.gov/pubmed/32231761
http://dx.doi.org/10.4254/wjh.v12.i3.72
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