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Celiprolol in Patients With Ehlers-Danlos Syndrome, Vascular Type

key information

study id #: NCT00190411

condition: Ehlers-danlos syndrome, type iv, autosomal dominant Chromosome 2q31.2 deletion syndrome

status: Completed


Ehlers-Danlos syndrome vascular type (EDS-IV) is caused by a genetic defect of collagen type III. Patient die (median 40 yrs) of vascular complications. There is no treatment. We showed that arteries are thin and overloaded in this patients. We test the protective effect of celiprolol on cardiovascular events in a 5 years, randomized, PROBE design

intervention: Drug: celiprolol celiprolol Other Names: - celiprolol? Dose ranging 100 to 400 mg, dose adaptation - every 6 months by increment of 100 mg, based on tolerance Drug: Control Untreated controls excluding betablockers


  • Ong KT, Perdu J, De Backer J, Bozec E, Collignon P, Emmerich J, Fauret AL, Fiessinger JN, Germain DP, Georgesco G, Hulot JS, De Paepe A, Plauchu H, Jeunemaitre X, Laurent S, Boutouyrie P. Effect of celiprolol on prevention of cardiovascular events in vascular Ehlers-Danlos syndrome: a prospective randomised, open, blinded-endpoints trial. Lancet. 2010 Oct 30;376(9751):1476-84. doi: 10.1016/S0140-6736(10)60960-9. Epub 2010 Sep 7. Erratum in: Lancet. 2016 Aug 6;388(10044):564. Dosage error in published abstract; MEDLINE/PubMed abstract corrected; Dosage error in article text.

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