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

Ángela María Giraldo M., MD., Rafael Conde Camacho, MD., Fabio Andrés Varón

Resumen


La telangiectasia hemorrágica hereditaria o síndrome de Osler-Weber-Rendu, es una enfermedad infrecuente, cuya prevalencia estimada es de entre 1:5.000 y 1:8.000 personas. Existen tasas más elevadas en ciertas áreas geográficas como Curazao y Bonaire, pero no es posible definir con exactitud las estadísticas debido al desconocimiento de la enfermedad en la mayoría de los pacientes que la padecen. Es un trastorno autosómico dominante con penetrancia variable que se caracteriza por epistaxis, telangiectasias y malformaciones vasculares en distintos territorios, tales como pulmonar, gastrointestinal, hepático y cerebral.


Palabras clave


síndrome de Osler-Weber-Rendu; hipertensión pulmonar; malformaciones vasculares; activina tipo receptor kinasa 1

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Referencias


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DOI: http://dx.doi.org/10.30789/rcneumologia.v26.n3.2014.39

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