Osler-Weber-Rendu disease: a six generation family.
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.
Cheng
L H
LH
Department of Otolaryngology, Sin-Lau Christian Hospital, Tainan, Taiwan, Republic of China.
Tsai
W J
WJ
D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy