Vnitr Lek 2021, 67(6):339-344 | DOI: 10.36290/vnl.2021.090

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

Zdeněk Adam1, Dagmar Brančíková1, Gabriela Romanová2, Luděk Pour1, Marta Krejčí1, Jiří König3, Tomáš Nebeský4, Zuzana Adamová5, Martin Štork1, Martin Krejčí1, Sabina Ševčíková4, Michal Eid1, Zdeněk Král1
1 Interní hematologická a onkologická klinika LF MU a FN Brno
2 Oddělení klinické hematologie FN Brno
3 Oddělní krční, nosní ušní FN Brno
4 Ústav patologické fyziologie LF MU a FN Brno
5 Chirurgické oddělení nemocnice Vsetín

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.

Published: October 13, 2021  Show citation

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Adam Z, Brančíková D, Romanová G, Pour L, Krejčí M, König J, et al.. Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) Part I. Pathophysiology, clinical symptoms and recommend screening for vascular malformations. Vnitr Lek. 2021;67(6):339-344. doi: 10.36290/vnl.2021.090.
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