Celiprolol in Patients With Ehlers-Danlos Syndrome, Vascular Type

Phase 4CompletedNCT00190411

Assistance Publique - Hôpitaux de Paris54 enrolled

Overview

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

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.

Study Type

INTERVENTIONAL

Allocation

RANDOMIZED

Purpose

TREATMENT

Masking

NONE

Enrollment

Conditions

EHLERS-DANLOS SYNDROME, TYPE IV, AUTOSOMAL DOMINANT CHROMOSOME 2q31.2 DELETION SYNDROME

Interventions

celiprololDRUG

celiprolol

ControlDRUG

Untreated controls excluding betablockers

Eligibility

Sex: ALLMin age: 15 YearsMax age: 65 Years

Medical Language ↔ Plain English

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. Criteria of not inclusion for both groups: * Refusal to participate in the study. * Impossibility to move. * Pregnancy * Woman in age to procreate without means of effective contraception.

Outcomes

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

Secondary Outcomes

Tolerance, effect of drug on arterial parameters : diameter, IMT, stiffness.

Time frame: during the study

Data from ClinicalTrials.gov

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