Executive summary of the 11th HHT international scientific conference

Angiogenesis. 2015 Oct;18(4):511-24. doi: 10.1007/s10456-015-9482-5.

Abstract

Hereditary hemorrhagic telangiectasia (HHT) is a hereditary condition that results in vascular malformations throughout the body, which have a proclivity to rupture and bleed. HHT has a worldwide incidence of about 1:5000 and approximately 80 % of cases are due to mutations in ENG, ALK1 (aka activin receptor-like kinase 1 or ACVRL1) and SMAD4. Over 200 international clinicians and scientists met at Captiva Island, Florida from June 11-June 14, 2015 to present and discuss the latest research on HHT. 156 abstracts were accepted to the meeting and 60 were selected for oral presentations. The first two sections of this article present summaries of the basic science and clinical talks. Here we have summarized talks covering key themes, focusing on areas of agreement, disagreement, and unanswered questions. The final four sections summarize discussions in the Workshops, which were theme-based topical discussions led by two moderators. We hope this overview will educate as well as inspire those within the field and from outside, who have an interest in the science and treatment of HHT.

Keywords: ALK1; Arteriovenous malformation; Endoglin; Epistaxis; HHT; Hereditary hemorrhagic telangiectasia.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Activin Receptors, Type II / genetics
  • Activin Receptors, Type II / metabolism
  • Antigens, CD / genetics
  • Antigens, CD / metabolism
  • Congresses as Topic
  • Endoglin
  • Humans
  • Receptors, Cell Surface / genetics
  • Receptors, Cell Surface / metabolism
  • Smad4 Protein / genetics
  • Smad4 Protein / metabolism
  • Telangiectasia, Hereditary Hemorrhagic* / genetics
  • Telangiectasia, Hereditary Hemorrhagic* / metabolism
  • Telangiectasia, Hereditary Hemorrhagic* / pathology
  • Telangiectasia, Hereditary Hemorrhagic* / therapy

Substances

  • Antigens, CD
  • ENG protein, human
  • Endoglin
  • Receptors, Cell Surface
  • SMAD4 protein, human
  • Smad4 Protein
  • ACVRL1 protein, human
  • Activin Receptors, Type II