Article Text

other Versions

PDF
International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia
  1. M E Faughnan (faughnanm{at}smh.toronto.on.ca)
  1. St. Michael's Hospital, University of Toronto, Canada
    1. V A Palda
    1. St. Michael's Hospital, University of Toronto, Canada
      1. G Garcia-Tsao
      1. Yale University School of Medicine, United States
        1. U W Geisthoff
        1. University of the Saarland, Germany
          1. J McDonald
          1. University of Utah, United States
            1. D D Proctor
            1. Yale University School of Medicine, United States
              1. J Spears
              1. St. Michael's Hospital, University of Toronto, Canada
                1. D H Brown
                1. University of Toronto, Canada
                  1. E Buscarini
                  1. Ospedale Maggiore, Crema, Italy
                    1. M S Chesnutt
                    1. Oregon Health and Science University, United States
                      1. V Cottin
                      1. University of Lyon, France
                        1. A Ganguly
                        1. University of Pennsylvania School of Medicine, United States
                          1. J R Gossage
                          1. Medical College of Georgia, United States
                            1. A E Guttmacher
                            1. National Human Genome Research Institute, United States
                              1. R H Hyland
                              1. St. Michael's Hospital, University of Toronto, Canada
                                1. S J Kennedy
                                1. Children's Hospital of Eastern Ontario, Canada
                                  1. J Korzenik
                                  1. Massachussetts General Hospital, United States
                                    1. J J Mager
                                    1. St Antonius Hospital, Netherlands
                                      1. A P Ozanne
                                      1. Hopital de Bicetre, France
                                        1. J F Piccirillo
                                        1. Washington University School of Medicine, United States
                                          1. D Picus
                                          1. Washington University School of Medicine, United States
                                            1. H Plauchu
                                            1. Hospices Civils de Lyon, France
                                              1. M EM Porteous
                                              1. Western General Hospital, United Kingdom
                                                1. R E Pyeritz
                                                1. University of Pennsylvania School of Medicine, United States
                                                  1. D A Ross
                                                  1. St. Vincent's Medical Center, United States
                                                    1. C Sabba
                                                    1. University if Bari, Italy
                                                      1. K Swanson
                                                      1. Mayo Clinic, Rochester, United States
                                                        1. P Terry
                                                        1. Johns Hopkins Unversity, United States
                                                          1. M C Wallace
                                                          1. University Health Network, Canada
                                                            1. C J J Westermann
                                                            1. St Antonius Hospital, Netherlands
                                                              1. R I White
                                                              1. Yale University School of Medicine, United States
                                                                1. L H Young
                                                                1. Yale University School of Medicine, United States
                                                                  1. R Zarrabeitia
                                                                  1. Hospital Sierrallana, Spain

                                                                    Abstract

                                                                    HHT is an autosomal dominant disease with an estimated prevalence of at least 1/5000 which can frequently be complicated by the presence of clinically significant arteriovenous malformations in the brain, lung, gastrointestinal tract and liver. HHT is under-diagnosed and families may be unaware of the available screening and treatment, leading to unnecessary stroke and life-threatening hemorrhage in children and adults. The goal of this international HHT guidelines process was to develop evidence-informed consensus guidelines regarding the diagnosis of HHT and the prevention of HHT-related complications and treatment of symptomatic disease. The overall guidelines process was developed using the AGREE framework, using a systematic search strategy and literature retrieval with incorporation of expert evidence in a structured consensus process where published literature was lacking. The Guidelines Working Group included experts (clinical and genetic) from eleven countries, in all aspects of HHT, guidelines methodologists, health care workers, health care administrators, HHT clinic staff, medical trainees, patient advocacy representatives and patients with HHT. The Working Group determined clinically relevant questions during the pre-conference process. The literature search was conducted using the OVID MEDLINE database, from 1966 to October 2006. The Working Group subsequently convened at the Guidelines Conference to partake in a structured consensus process using the evidence tables generated from the systematic searches. The outcome of the conference was the generation of 33 recommendations for the diagnosis and management of HHT, with at least 80% agreement amongst the expert panel for 30 of the 33 recommendations.

                                                                    Statistics from Altmetric.com

                                                                    Request permissions

                                                                    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.