[Rendu-Osler-Weber syndrome--a complex systemic disease].
Hereditary hemorrhagic teleangiectasia (HHT or Rendu-Osler-Weber Syndrome) is an inherited autosomal dominant disorder of the vascular connective tissue. The resulting vascular malformations can occur in virtually any organ. Nosebleeds can massively impact on the quality of life of those afflicted. However, visceral manifestations are likely to be more serious, and may be comparable with a "ticking time bomb". Most commonly affected are the vascular systems of the lungs, liver, brain and gastrointestinal tract. Screening is recommended--at least with regard to the lungs. Difficult constellations of this complex condition may be successfully managed by an interdisciplinary approach.
Geisthoff
U W
UW
Klinik und Poliklinik für HNO, Universitätskliniken des Saarlandes, Homburg/Saar. urban.geisthoff@uniklinik-saarland.de
Koester
M
M
Fischinger
J
J
Schneider
G
G
D000328:Adult; D000368:Aged; D001165:Arteriovenous Malformations / Q000175:diagnosis; D001922:Brain Abscess / Q000175:diagnosis / Q000209:etiology; D003937:Diagnosis, Differential; D004844:Epistaxis / Q000209:etiology; D005260:Female; D006801:Humans; D002538:Intracranial Arteriovenous Malformations / Q000175:diagnosis; D008099:Liver / Q000098:blood supply; D008168:Lung / Q000098:blood supply; D018810:Magnetic Resonance Angiography; D008297:Male; D010348:Patient Care Team; D015995:Prevalence; D011788:Quality of Life; D011859:Radiography; D012657:Self-Help Groups; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000235:genetics