Literature DB >> 19744364

Osler-Weber-Rendu syndrome--dental implications.

Paulo Sérgio da Silva Santos1, Karin Sá Fernandes, Marina Helena Magalhães.   

Abstract

Osler-Weber-Rendu syndrome (OWRS) is a rare hereditary, autosomal dominant disease characterized by a local angiodysplasia. Its clinical characteristics are vascular hamartomas of the skin and oral mucosa, arteriovenous malformations in the lungs, liver, kidney and brain, and episodes of epistaxis. The oral lesions, which become apparent through hemorrhagic telangiectasia, may be the first sign of the disease. This is a case report of a 74-year-old woman whose diagnosis of OWRS was established by her dentist based on the presence of telangiectasia in the skin and oral mucosa, reports of frequent nosebleeds of unknown etiology and a family history of telangiectasia. Amputation of a lower limb and comorbidities, such as cardiopathy, nephropathy and rheumatic disorders, completed the profile. OWRS causes major vascular changes that can be diagnosed initially by a dentist. In this article, we describe the skills and knowledge that dentists need to monitor patients with OWRS properly.

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Year:  2009        PMID: 19744364

Source DB:  PubMed          Journal:  J Can Dent Assoc        ISSN: 0709-8936            Impact factor:   1.316


  2 in total

Review 1.  Differential diagnosis of hepatopulmonary syndrome (HPS): Portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT).

Authors:  Inna Krynytska; Mariya Marushchak; Anna Mikolenko; Anzhela Bob; Iryna Smachylo; Ludmyla Radetska; Olga Sopel
Journal:  Bosn J Basic Med Sci       Date:  2017-11-20       Impact factor: 3.363

Review 2.  An update on the ophthalmic features in hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber syndrome).

Authors:  Solmaz Abdolrahimzadeh; Martina Formisano; Carla Marani; Siavash Rahimi
Journal:  Int Ophthalmol       Date:  2022-01-16       Impact factor: 2.029

  2 in total

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