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Rendu-Osler Syndrome

definition

Autosomal dominant disorder characterized by multiple telangiectases on the skin and mucous membranes. Internal organs may also be affected. The dark red, knob-shaped vascular dilatations may be as large as 2 - 5 mm, often with star-like angiectases. There is marked tendency to cutaneous and mucosal bleeding. The initial sign is often strikingly frequent epistaxis in children or youth, which may be associated with hematuria and intestinal bleeding. Arteriovenous pulmonary fistulas have also been reported.

synonyms

Rendu-Osler Syndrome, Osler's Disease, Rendu-Osler Syndrome, Hereditary Hemorrhagic Telangiectasia

UMLS

Disease, Osler-Rendu, Disease, Rendu-Osler-Weber, Epistaxis, hereditary, Hematemesis, Goldstein's, Hemorrhagic Telangiectasia, Hereditary, Hemorrhagic Telangiectasias, Hereditary, Hered haemorrhagic telangiect, Hered hemorrhagic telangiect, Hereditary haemorrhagic telangiectasia, Hereditary hemorrhagic telangiectasia, Hereditary Hemorrhagic Telangiectasias, HHT - Hered haem telangiect, HHT - Hereditary haemorrhagic telangiectasia, HHT - Hereditary hemorrhagic telangiectasia, Osler - Rendu - Weber disease, Osler - Rendu - Weber syndrome, Osler hemorrhagic telangiectasia syndrome, Osler Rendu Disease, Osler-Rendu Disease, Osler's disease, Osler's hemorrhagic telangiectasia syndrome, Osler-Weber-Rendu disease, OSLER-WEBER-RENDU SYNDROME, RENDU OSLER WEBER SYNDROME, Syndrome, Osler-Weber-Rendu, Syndrome, Rendu-Osler-Weber, Syndrome, Weber-Osler, Telangiectasia, hemorrhagic, hereditary, Telangiectasia, Hereditary Hemorrhagic, TELANGIECTASIA, HEREDITARY HEMORRHAGIC, OF RENDU, OSLER AND WEBER, Telangiectasias, Hereditary Hemorrhagic, Telangiectasis, hemorrhagic, hereditary, Telangiectasis, hereditary hemorrhagic, Weber-Osler syndrome

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