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Rendu-Osler Syndrome


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.




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