Celiprolol in Patients With Ehlers-Danlos Syndrome, Vascular Type | oneedsvoice

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completed

Celiprolol in Patients With Ehlers-Danlos Syndrome, Vascular Type

study id #: NCT00190411

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

status: Completed

purpose:

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

publications:

  • 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.

start date: October 2003

estimated completion: April 2011

last updated: January 16, 2013

phase of development: Phase 4

size / enrollment: 54

study design: Allocation: Randomized Intervention Model: Parallel Assignment Masking: None (Open Label) Primary Purpose: Treatment

study description: 100 patients with verified EDS-IV syndrome are included. Patients are randomized to either celiprolol (50 to 400 mg BID)or no treatment. Patients who are not randomized enter a longitudinal survey of events. The hypothesis is a 50% reduction in the occurrence of cardiovascular events under treatment, assessed by a blinded, independent events committee.

primary outcomes:

  • reduction in the rate of major cardiovascular events in the treated group during a 5 years follow-up [ Time Frame: during de study ] reduction in the rate of major cardiovascular events in the treated group during a 5 years follow-up

secondary outcomes:

  • Tolerance, effect of drug on arterial parameters : diameter, IMT, stiffness. [ Time Frame: during the study ] Tolerance, effect of drug on arterial parameters : diameter, IMT, stiffness.

inclusion criteria: Proven disease, No betablocker at inclusion if previous CV event

exclusion criteria: Criteria of not inclusion for the RIGHTEOUS group: Patient having already presented an arterial break or a dissection and treated(handled) by bétâ-blocking(surrounding). Against indication in the use of CELIPROLOL: Unchecked cardiac insufficiency by the treatment cardiogenic shock BAV of 2nd and 3rd not sailed degrees angor of Prinzmetal disease of the sine bradycardia pheochromocytoma untreated low blood pressure sentimentality in the CELIPROLOL Antecedent of anaphylactic reaction myasthenia treatment by FLOCTAFENINE ( Idarac), Sultopride ( interactions ) In these two cases, the patient can be included in the group followed by troop.

sponsor: Assistance Publique - Hôpitaux de Paris Aventis Pharmaceuticals

investigators: Pierre Boutouyrie, MD, PhD

locations: France