title: [Hereditary haemorrhagic telangiectasia: importance of a multidisciplinary approach].
abstract: The Rendu-Osler-Weber disease, also known as hereditary haemorrhagic telangiectasia, is an autosomal dominant inherited disease. Its main manifestations are nosebleeds and digestive tract bleeding due to angiodysplasia. The presence of arteriovenous malformations in organs such as lung, liver, brain, etc. can cause serious complications (haemorrhage, stroke, brain abscess, hypoxemia, increased cardiac output, pulmonary arterial hypertension). Diagnosis is based on clinical criteria and can be confirmed by genetic analysis. The prevalence of this rare disease is 1/5,000 to 1/10,000 and its expression varies widely, even in the same family. The management must be multidisciplinary and based on prevention and treatment of bleeding complications as well as screening and treatment of arteriovenous malformations.
authors.forename: Françoise
authors.initials: F
authors.lastname: Boehlen
authors.forename: Basile N
authors.initials: BN
authors.lastname: Landis
authors.forename: Laurent
authors.initials: L
authors.lastname: Spahr
authors.forename: Anne-Lise
authors.initials: AL
authors.lastname: Hachulla
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Ranza
authors.forename: Thierry
authors.initials: T
authors.lastname: Nouspikel
authors.forename: Frédéric
authors.initials: F
authors.lastname: Lador
authors.forename: Kaveh
authors.initials: K
authors.lastname: Samii
authors.forename: Stéphane
authors.initials: S
authors.lastname: Noble
mesh_terms: D001165:Arteriovenous Malformations / Q000209:etiology / Q000628:therapy*; D006470:Hemorrhage / Q000209:etiology / Q000628:therapy*; D006801:Humans; D033183:Interdisciplinary Communication; D015995:Prevalence; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*