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This page was exported from International Journal Of Pathology
[ http://jpathology.com ] Export date: Thu Feb 25 19:48:25 2021 / +0000 GMT |
Osler-Weber-Rendu Syndrome in association with port wine spotAbdul Khalid Awan, Mohammad Mohammad Tariq Baqai, Murtaza Bukhari Department of Medicine Azad Jammu & Kashmir Medical College Muzaffarabad AJK . Abstract Osler-Rendu-Weber syndrome also known as hereditary hemorrhagic telangectasiea (HHT) is an autosomal dominant vascular disorder. The pathogenesis of this disorder involves dilated postcapillary venules in the mucus membranes of different organs and large arteriovenous malformations. Gastrointestinal hemorrhages contribute to iron deficiency anemia in one third of these patients. Port wine stains belong to the family of arteriovenous disorders. These are due to abnormally dilated capillaries and produce reddish or pink discoloration of the skin. These are present at birth and increase in size during the growth period. No association has so far been reported between port wine stains and hereditary hemorrhagic telangectasiea. We report a case of 41 years old lady presenting with co- existence of these two lesions. Key words: Osler-Rendu-Weber syndrome, Port wine stain, GI endoscopy |
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