| Autor: |
K.T. Ong, J. Perdu, H. Plauchu, J. De Backer, A. De Paepe, J. Emmerich, X. Jeunemaitre, D. Germain, P. Collignon, G. Georgesco, E. Bozec, J.S. Hulot, S. Laurent, P. Boutouyrie |
| Jazyk: |
angličtina |
| Rok vydání: |
2009 |
| Předmět: |
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| Zdroj: |
Artery Research, Vol 3, Iss 4 (2009) |
| Druh dokumentu: |
article |
| ISSN: |
1876-4401 |
| DOI: |
10.1016/j.artres.2009.10.154 |
| Popis: |
Background: Vascular Ehlers-Danlos syndrome (vEDS) is a rare severe genetic disease which results from mutations in the gene encoding type III procollagen (COL3A1), characterized by vascular and/or hollow organic ruptures. No treatment is yet validated. We tested the ability of celiprolol, a beta1-adrenoceptor antagonist with a beta2-adrenoceptor agonist action, for preventing the complications of vEDS in a prospective, randomized, open, blinded endpoints trial. Methods: Fifty three previously untreated vEDS patients were randomized to a 5-year treatment with either celiprolol (n=25) or no treatment (n=28). The two groups were matched for demographic, medical historic and clinical characteristics. Celiprolol was up-titrated from 100 to 400mg by steps of 100mg every 6 months. The primary end-point was an arterial event (rupture or dissection, fatal or not) occurring during follow-up. Secondary endpoints were intestinal or uterine rupture or major clinical events, related to vEDS, judged by the event committee. Results: Mean duration of follow-up was 47 (± 15) months. The study was ended prematurely by the safety monitoring board since significant differences were reached between two groups. The primary endpoint was reached by 5 patients (20%) in the celiprolol group and by 14 patients (50%) in the control group (hazard ratio, 0.36; 95% CI, 0.15 to 0.88; P=0.04). Primary plus secondary endpoints occurred in 6 patients (24%) in the celiprolol group and in 17 patient (61%) in the control group (hazard ratio, 0.31; 95% CI, 0.14 to 0.71; P=0.0097). Conclusions: Celiprolol effectively reduced both vascular complications and organic ruptures in vEDS patients. |
| Databáze: |
Directory of Open Access Journals |
| Externí odkaz: |
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