Correlation Engine 2.0
Clear Search sequence regions


  • ACER (1)
  • celiprolol (5)
  • cohort (1)
  • collagen (1)
  • humans (1)
  • vascular diseases (1)
  • Sizes of these terms reflect their relevance to your search.

    Vascular Ehlers-Danlos syndrome (OMIM 130050, 1/150,000 birth) is caused by mutations in collagen 3A1 gene. It is associated with severe phenotype associating early arterial dissection and rupture, digestive and uterine perforations, and skin and joints fragility. Until recently, no treatment was available. Celiprolol, a beta1 antagonist with beta2 partial antagonist properties betablocker was tested in a randomized, controlled trial. We could show that this compound was associated with a 3-fold decrease in major events related to the disease. This effect was similar in molecular-proven patients. Administration of celiprolol in a cohort of patients followed routinely in France was accompanied to similar benefit. Celiprolol is unavailable in the USA. The ACER Therapeutics company applied for new drug application (NDA) to the Food and Drug Administration. Copyright © 2020 Société française de pharmacologie et de thérapeutique. Published by Elsevier Masson SAS. All rights reserved.

    Citation

    Pierre Boutouyrie. Therapie. 2020 Apr;75(2):175-181

    Expand section icon Mesh Tags

    Expand section icon Substances


    PMID: 32127191

    View Full Text