Celiprolol in Patients With Ehlers-Danlos Syndrome, Vascular Type

NCT ID: NCT00190411

Last Updated: 2013-01-16

Basic Information

• Recruitment Status: COMPLETED
• Clinical Phase: PHASE4
• Total Enrollment: 54 participants
• Study Classification: INTERVENTIONAL
• Study Start Date: 2003-10-31
• Study Completion Date: 2011-04-30

Brief Summary

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

Detailed 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.

Conditions

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

Study Design

• Allocation Method: RANDOMIZED
• Intervention Model: PARALLEL
• Primary Study Purpose: TREATMENT
• Blinding Strategy: NONE

Study Groups

Treatment

Celiprolol

Group Type: EXPERIMENTAL

celiprolol

Intervention Type: DRUG

celiprolol

Control

Intervention Type: DRUG

Untreated controls excluding betablockers

Interventions

celiprolol

celiprolol

Intervention Type: DRUG

Control

Untreated controls excluding betablockers

Intervention Type: DRUG

Other Intervention Names

celiprolol? Dose ranging 100 to 400 mg, dose adaptation; every 6 months by increment of 100 mg, based on tolerance

Eligibility Criteria

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.

• Minimum Eligible Age: 15 Years
• Maximum Eligible Age: 65 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Aventis Pharmaceuticals

INDUSTRY

Sponsor Role: collaborator

Assistance Publique - Hôpitaux de Paris

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

Pierre BOUTOUYRIE, MD,PhD

Role: PRINCIPAL_INVESTIGATOR

Assistance Publique - Hôpitaux de Paris

References

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.

Reference Type: DERIVED

PMID: 20825986 (View on PubMed)

Other Identifiers

P010309

Identifier Type: -

Identifier Source: org_study_id