Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) Part I. Pathophysiology, clinical symptoms and recommend screening for vascular malformations

Czech version

Authors: Zdeněk Adam ¹; Dagmar Brančiková ¹; Gabriela Romanová ²; Luděk Pour ¹; Marta Krejčí ¹; Jiří König ³; Tomáš Nebeský ⁴; Zuzana Adamová ⁵; Martin Štork ¹; Martin Krejčí ¹; Sabina Ševčíková ⁴; Michal Eid ¹; Zdeněk Král ¹
Authors‘ workplace: Interní hematologická a onkologická klinika LF MU a FN Brno ¹; Oddělení klinické hematologie FN Brno ²; Oddělní krční, nosní ušní FN Brno ³; Ústav patologické fyziologie LF MU a FN Brno ⁴; Chirurgické oddělení nemocnice Vsetín ⁵
Published in: Vnitř Lék 2021; 67(6): 339-344
Category: Review Articles

Overview

Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is an autosomal dominant disorder that causes abnormal blood vessel formation. Patients with HHT may have telangiectasias and later may develop arteriovenous malformations in various organs. Pacients suffer from many complications caused by the malformations and therefore by patients with HHT must by performed screening of this arteriovenous malformations. Optimal treatment of this malformations is best delivered throught a multidisciplinary approach. Farmacological treatment is described in next paper.

Keywords:

hereditary hemorrhagic teleangiectasia – arteriovenous malformations

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