Pulmonary hypertension as manifestation of hereditary hemorrhagic telangiectasia, or Osler-Weber-Rendu syndrome

Hipertensión pulmonar como manifestación de la telangiectasia hemorrágica hereditaria o síndrome de Osler-Weber-Rendu

Published 2016-08-07

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Vol. 26 No. 3 (2014)
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Pulmonary hypertension as manifestation of hereditary hemorrhagic telangiectasia, or Osler-Weber-Rendu syndrome.
rev. colomb. neumol. [Internet]. 2016 Aug. 7 [cited 2024 Nov. 24];26(3). Disponible en: https://doi.org/10.30789/rcneumologia.v26.n3.2014.39
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Ángela María Giraldo M., MD.
Universidad de la Sabana. Fundación Neumológica Colombiana, Bogotá, Colombia.
    Rafael Conde Camacho, MD.
    Fundación Neumológica Colombiana. Bogotá, Colombia.
      Fabio Andrés Varón
      Fundación Neumológica Colombiana, Bogotá, Colombiana.
        Show authors biography

        Ángela María Giraldo M., MD.,

        Fellow de Neumología, Universidad de la Sabana. Fundación Neumológica Colombiana, Bogotá, Colombia.

        Rafael Conde Camacho, MD.,

        Neumólogo, Fundación Neumológica Colombiana. Bogotá, Colombia. 

        Fabio Andrés Varón,

        Neumólogo Intensivista, Fundación Neumológica Colombiana. Bogotá, Colombia.

        Hereditary hemorrhagic telangiectasia, or Osler-Weber-Rendu syndrome, is an uncommon disease whose estimated prevalence ranges from 1:5.000 to 1:8.000. There are higher rates in certain geographic areas, such as Curazao and Bonaire, but it is impossible to accurately define the statistics, due to lack of knowledge of the existence of the disease in most sufferers. It is a dominant autosomal disorder with variable penetrance, characterized by epistaxis, telangiectasia, and vascular malformations in many territories, such as the pulmonary, gastrointestinal, hepatic, and cerebral vascular beds (1).

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