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February 1957

Hereditary Hemorrhagic Telangiectasia in Three Families

Author Affiliations

U. S. Army

AMA Arch Intern Med. 1957;99(2):301-306. doi:10.1001/archinte.1957.00260020137021

Hemorrhagic telangiectasia, or Rendu-Weber-Osler's disease, is a hereditary primary vascular entity, characterized by mucosal and visceral telangiectases and angiomata which give rise to profuse hemorrhage either spontaneously or as a result of trauma. We have recently had the opportunity to study three different families, all of which showed several members similarly affected. All suffered from repeated, severe epistaxes, the most frequent complication of the disease. One patient also had multiple pulmonary arteriovenous fistulae, which is another frequent complication.

Family 1.  —A 28-year-old, white unipara in the last trimester of an uncomplicated pregnancy was admitted to the U. S. Army Hospital, Landstuhl, Germany, on Jan. 22, 1955. Her admitting diagnosis was congenital heart disease, cyanotic type. She recalled being slightly cyanotic since early childhood, but since the age of 12, the cyanosis had not been as apparent. She denied ankle edema, chronic cough, and hemoptyses, but as long as she could

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