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Hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber disease)

Authors :
Hirotoshi Miyamori
Shuichi Kaneko
Uichiro Fuchizaki
Kenichi Kobayashi
Shunsuke Kitagawa
Source :
The Lancet. 362:1490-1494
Publication Year :
2003
Publisher :
Elsevier BV, 2003.

Abstract

Correspondence to: Dr Uichiro Fuchizaki, Keiju Medical Center, 94 Tomioka-cho, Nanao, Ishikawa 926-8605, Japan (e-mail: fuchi@p1.coralnet.or.jp) Hereditary haemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber disease, is an autosomal dominant disorder characterised by epistaxis, cutaneous telangiectasia (figure 3), and visceral arteriovenous malformations (AVMs). The characteristic lesions in this disorder are telangiectases that consist of focal dilatations of postcapillary venules and AVMs. Recurrent epistaxis often restricts daily life, as in our patient (case presentation). AVMs can cause serious complications if located in the lungs, liver, or brain. Hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber disease)

Details

ISSN :
01406736
Volume :
362
Database :
OpenAIRE
Journal :
The Lancet
Accession number :
edsair.doi.dedup.....8f519c024fae9d1afd1767dea98718f7
Full Text :
https://doi.org/10.1016/s0140-6736(03)14696-x