Which patient groups are at highest risk for Osler-Weber-Rendu disease (OWRD) (hereditary hemorrhagic telangiectasia [HHT])?

Updated: Oct 06, 2020
  • Author: Klaus-Dieter Lessnau, MD, FCCP; Chief Editor: Vincent Lopez Rowe, MD  more...
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Answer

HHT may occur in children, in whom a tendency to bleed may be the first symptom. [38] However, it is far more common during puberty or adulthood. The syndrome most often manifests by the second or third decade of life, though it may also be clinically silent. Pulmonary AVMs may be congenital and therefore may present within the first year of life. The risk of GI tract bleeding increases in patients older than 50 years.

HHT occurs with equal frequency and severity in males and females. [39] Although it most commonly occurs in whites, it has a wide geographic distribution and has been described in people of Asian, African, and Arabic descent.


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