Journal article

Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia

Marie E Faughnan, Johannes J Mager, Steven W Hetts, Valerie A Palda, Kelly Lang-Robertson, Elisabetta Buscarini, Erik Deslandres, Raj S Kasthuri, Andrea Lausman, David Poetker, Felix Ratjen, Mark S Chesnutt, Marianne Clancy, Kevin J Whitehead, Hanny Al-Samkari, Murali Chakinala, Miles Conrad, Daniel Cortes, Claudia Crocione, Jama Darling Show all

Annals of Internal Medicine | AMER COLL PHYSICIANS | Published : 2020

Abstract

Description: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with an estimated prevalence of 1 in 5000 that is characterized by the presence of vascular malformations (VMs). These result in chronic bleeding, acute hemorrhage, and complications from shunting through VMs. The goal of the Second International HHT Guidelines process was to develop evidence-based consensus guidelines for the management and prevention of HHT-related symptoms and complications. Methods: The guidelines were developed using the AGREE II (Appraisal of Guidelines for Research and Evaluation II) framework and GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodol..

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