Naslov
Vascular Ehlers-Danlos syndrome – our centre’s experience

Autori
Vrkić Kirhmajer, Majda ; Crkvenac Gregorek, Andrea ; Huljev Frković, Sanda

Vrsta, podvrsta i kategorija rada
Poglavlja u knjigama, znanstveni

Knjiga
Kaj je treba vedeti!

Urednik/ci
Kozak, Matija ; Boc, Vinko ; Krevel, Barbara ; Blinc, Aleš

Izdavač
Slovensko zdravniško društvo

Grad
Ljubljana

Godina
2023

Raspon stranica
102-108

ISBN
978-961-7092-43-1

Ključne riječi
vascular Ehlers-Danlos syndrome ; celiprolol

Sažetak
The vascular type of Ehlers – Danlos syndrome (vEDS) is considered as the most severe form of EDS because of its typical life-threatening complications in young adults: spontaneous rupture of arteries and hollow organs. Regarding shortened life expectancy in these patients and different forms of clinical presentation, accurate diagnosis confirmed by genetic testing is crucial. Due to the complexity of this disorder, the management of vEDS patients should be reserved to experienced hospital centres. Apart from surgical or endovascular treatment of serious vascular complications, medical strategy based on celiprolol provides a valuable and protective role in the reduction of arterial complications. We report clinical experience with vEDS in UHC Zagreb.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

POVEZANOST RADA