A Rare Presentation of Osler-Weber-Rendu Disease as Severe Anemia.
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Date
2013-05
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Abstract
Hereditary hemorrhagic telangiectasia (HHT) or Osler-Weber-Rendu disease is a rare fibrovascular dysplasia that makes vascular
walls vulnerable to trauma and rupture causing skin and mucosal bleeding. It is of autosomal dominant inheritance characterized
by recurrent epistaxis and telangiectasia on the face, hands and oral cavity; visceral arteriovenous malformations (AVMs) and
positive family history. Epistaxis is often the foremost manifestation. It is associated with AVMs in several organs. There are
possible hematologic, neurologic, pulmonary, dermatologic and gastrointestinal complications. Treatment is supportive and
helps prevent complications. We report herein a patient with this syndrome who came to Medicine Unit first at our hospital.
Description
Keywords
Hereditary hemorrhagic telangiectasia, arteriovenous malformations, epistaxis
Citation
Nayak Kailash Chandra, Gupta Ashish, Varun K, Vyas Ashwani K, Kumar Surendra, Prakash Parul. A Rare Presentation of Osler-Weber-Rendu Disease as Severe Anemia. Indian Journal of Clinical Practice. 2013 May; 23(12): 833-834.