Celiprolol in Patients With Ehlers-Danlos Syndrome, Vascular Type

Assistance Publique - Hôpitaux de Paris

Status and phase

Completed

Phase 4

Conditions

CHROMOSOME 2q31.2 DELETION SYNDROME

EHLERS-DANLOS SYNDROME, TYPE IV, AUTOSOMAL DOMINANT

Treatments

Drug: Control

Drug: celiprolol

Study type

Interventional

Funder types

Other

Industry

Identifiers

NCT00190411

P010309

Details and patient eligibility

About

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

Full 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.

Enrollment

54 patients

Sex

All

Ages

15 to 65 years old

Volunteers

No Healthy Volunteers

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.