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January 25, 1947


JAMA. 1947;133(4):247-248. doi:10.1001/jama.1947.02880040033011

Hereditary hemorrhagic telangiectasia, or Rendu-Osler-Weber disease, is an uncommon but well established clinical entity characterized by multiple telangiectases, hemorrhages and hereditary transmission. Goldstein1 in 1931 presented a comprehensive review of the literature on the subject, according to which there were some 90 to 95 families and about 500 to 550 persons affected with this condition. Epistaxis is the most frequent symptom, but bleeding from the skin or oral mucous membranes occurs in the majority of patients. The telangiectases usually do not appear before the age of puberty. In the third and fourth decades of life they become more numerous and more subject to hemorrhage. Anemia severe enough to be incapacitating often results from the long continued loss of blood, and a number of fatal hemorrhages have been reported. The frequency of internal bleeding in patients with hereditary hemorrhagic telangiectasia with hematemesis, hemoptysis, melena, hematuria and even cerebral hemorrhages has