Osler-Weber-Rendu syndrome


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Osler-Weber-Rendu syndrome

[ōs′lər web′ər rando̅o̅′]
Etymology: William Osler; Frederick P. Weber, British physician, 1863-1962; Henri J.L.M. Rendu, French physician, 1844-1902
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Osler-Weber-Rendu syndrome

Osler-Weber-Rendu syndrome

Hereditary hemorrhagic telangiectasia, hereditary multiple aneurysmal telangiectasia Genetics An AD vasculopathy, which may lead to massive episodic hemorrhage Clinical Affected children develop reddish telangiectasias on the lips, tongue, nasal mucosa, face and ears; vascular defects also occur in the throat, larynx, GI tract, liver, bladder, vagina, and brain, where it may result in hemorrhage, causing seizures or death; early signs include frequent nosebleeds in children; the characteristic telangiectasias on the tongue and lips may be delayed until puberty. See Vasculopathy.
References in periodicals archive ?
Based on the above findings (fulfills three criteria of HHT), the man is a definite case of Osler-Weber-Rendu syndrome (HHT) with PAVM treated by wedge resection.
The diagnostis criteria in Osler-Weber-Rendu syndrome include spontaneous recurring epistaxis, mucocutaneous telengiectasies, AVM's in the internal organs and presence of Osler-Weber-Rendu syndrome in the first-degree relatives.
AVM should be investigated using contrast-enhanced radiologic studies in internal organs, if necessary and the diagnosis of Osler-Weber-Rendu syndrome should be excluded.
A diagnosis of Osler-Weber-Rendu syndrome was made with pulmonary AVM, mucocutenaous telangiectases and recurring epistaxis episodes.
Osler-Weber-Rendu syndrome is an autosomal dominant vascular dysplasia defined in 1864 for the first time and shown to be familial, to be characterized by telangiectases and to lead mucosal bleeding by Rendu, Osler and Weber, respectively (1-3).