Ielasi L, Tonnini M, Piscaglia F, Serio I. Current guidelines for diagnosis and management of hepatic involvement in hereditary hemorrhagic teleangiectasia. World J Hepatol 2023; 15(5): 675-687 [PMID: 37305373 DOI: 10.4254/wjh.v15.i5.675]
Corresponding Author of This Article
Luca Ielasi, MD, Doctor, Department of Medical and Surgical Sciences, University of Bologna, Via Albertoni, 15, Bologna 40138, Italy. luca.ielasi.kr@gmail.com
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Minireviews
Open-Access Policy of This Article
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. See: http://creativecommons.org/licenses/by-nc/4.0/
Luca Ielasi, Matteo Tonnini, Fabio Piscaglia, Department of Medical and Surgical Sciences, University of Bologna, Bologna 40138, Italy
Luca Ielasi, Department of Internal Medicine, Ospedale per gli Infermi di Faenza, Faenza 48018, Italy
Matteo Tonnini, Fabio Piscaglia, Ilaria Serio, Division of Internal Medicine, Hepatobiliary and Immunoallergic Diseases, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna 40138, Italy
Author contributions: Ielasi L and Tonnini M conceived the manuscript, reviewed the literature and wrote the original draft; Serio I reviewed and edited the manuscript; Piscaglia F supervised; all authors read and agreed to the published version of the manuscript.
Conflict-of-interest statement: All authors declare no conflict of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (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. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Luca Ielasi, MD, Doctor, Department of Medical and Surgical Sciences, University of Bologna, Via Albertoni, 15, Bologna 40138, Italy. luca.ielasi.kr@gmail.com
Received: February 7, 2023 Peer-review started: February 7, 2023 First decision: March 22, 2023 Revised: April 4, 2023 Accepted: April 12, 2023 Article in press: April 12, 2023 Published online: May 27, 2023 Processing time: 105 Days and 13.7 Hours
Abstract
Hereditary hemorrhagic teleangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is the most common cause of hepatic vascular malformations in adults. Different vascular shunts (arteriovenous, arterioportal or portovenous) lead to different clinical manifestations. Even though no hepatic-related symptoms are reported in the majority of cases, the severity of liver disease could lead to refractory medical conditions, in some cases requiring liver transplantation. The aim of this manuscript is to provide an updated overview of the current evidence regarding the diagnosis and treatment of HHT liver involvement and liver-related complications.
Core Tip: Hereditary hemorrhagic teleangiectasia (HHT) is the most common cause of hepatic vascular malformation in adults. Although liver involvement is common in HHT, most patients do not present any hepatic-related symptoms. Unfortunately, some patients have severe forms of disease with refractory medical conditions related to the hepatic vascular malformations. For those patients the only definitive treatment available at present is liver transplantation.
