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Osler-Weber-Rendu disease or Hereditary Haemorrhagic Telangiectasia (HHT)

17 July 2012, by DUPUIS-GIROD S. & COLLET E.

Osler-Weber-Rendu disease or hereditary familial telangiectasia (HHT) is an inherited form of angiodysplasia of autosomal dominant transmission with variable expression. Its prevalence globally is estimated to be approximately 1 in 6000 (although prevalence is higher in some regions, such as in the department of Ain in France (1/2350) and the Danish island of Funen (1/3500) [1]. Diagnosis is valuable because of the current potential to screen for possible complications. This means it is essential for these patients to be managed at the leading centre or in a centre with expertise in Osler-Weber-Rendu disease.

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