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Osler-Weber-Rendu disease: a six generation family.

Authors: L H, Cheng; W J, Tsai;

Osler-Weber-Rendu disease: a six generation family.

Abstract

A Taiwanese family, with at least nine members in six generations has been affected with Osler-Weber-Rendu disease, also known as hereditary hemorrhagic telangiectasia. It is an autosomal dominant familial disease which involves systemic subepithelial fibrovascular dysplasia and manifests with telangiectasia or angiomas of the face, the palms, or the mucosa of the aerodigestive tract (such as the nose, lips, oral cavity, or stomach, etc.). The most common clinical symptom is recurrent epistaxis with/without upper gastrointestinal bleeding which may be resistant to the otolaryngologist's best efforts for control. Repeated bleeding marks the typical course of the disease. Epistaxis from hereditary hemorrhagic telangiectasias often requires a combination of therapeutic approaches. Carbon dioxide laser surgery was highly successful in decreasing postoperative morbidity and epistaxis in the four patients we treated.

Related Organizations
Keywords

Adult, Diagnosis, Differential, Male, Humans, Female, Telangiectasia, Hereditary Hemorrhagic, Middle Aged, Pedigree

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citations
The total number of citations received by all articles of the researcher of interest.
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
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