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Jornal Português de Gastrenterologia
versión impresa ISSN 0872-8178
Resumen
SOARES, Vítor; CARVALHO, Amanda; LIMA, Murilo y PARENTE, José. Liver disease and gastrointestinal bleeding in hereditary haemorrhagic telangiectasia - case report. J Port Gastrenterol. [online]. 2010, vol.17, n.5, pp.213-216. ISSN 0872-8178.
Hereditary hemorrhagic telangectasia (HHT) or Rendu-Osler-Weber syndrome is characterized for fibromuscular dysplasia of the terminals vessels that propitiate the development of vascular ectasias and arteriovenous malformations. The mucosae, skin, lung and brain are the most commonly affected organs. This article describes a case of HHT an adult patient associated with multiple angiodysplasic injuries in the skin, nasal and oral mucosa, gastrointestinal tract, liver and thyroid, and who presents continuous blood loss in result of the severe gastric mucosal damage, demanding the replacement of erythrocytes concentrated in very short time intervals.
Palabras clave : Hereditary haemorrhagic telangiectasia; Rendu-Osler-Weber syndrome; Arteriovenous malformation; Gastrointestinal bleeding; Liver disease.