[Rendu-Osler-Weber disease].
Rendu-Osler-Weber disease or hereditary hemorrhagic telangiectasia (HHT) is a multisystem autosomal dominant hereditary disorder. The disorder is manifested by multiple dysplasia of blood vessels of the skin and mucous membranes. This results in recurrent and sometimes severe bleeding, of which epistaxis is the most common. Cardial, pulmonary and cerebral manifestations can be responsible for complications. A patient is presented with Rendu-Osler-Weber disease followed by a review of literature.
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Uit de afdeling Keel-, Neusen Oorheelkunde en Hoofd-Halschirurgie van het Universitair Medisch Centrum Sint Radboud in Nijmegen. liensys@tiscali.be
van den Hoogen
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FJ
D000368:Aged; D000369:Aged, 80 and over; D004844:Epistaxis / Q000209:etiology; D005260:Female; D006801:Humans; D009154:Mutation; D010348:Patient Care Team; D011379:Prognosis; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy