Disease of Returned-Osler
The disease of Returned-Osler or disease of Return-Osler-Weber or family hemorrhagic telangiectasy is a Angiomatose of dominant autosomic Transmission, belonging to the Phacomatoses.
It associates demonstrations cutanéo-mucous membranes (telangiectasy S) and malformations vascular resulting from the absence of capillaries between the veins and the arteries. These malformations are located everywhere with risks of rupture making the gravity of this disease.
The most common demonstration is spontaneous and frequent nosebleed beginning the majority of time around 12 years. Digestive hemorrhages occur in a quarter of the cases after 50 years. Malformations locating itself in the brain, the digestive system or pulmonary are responsible for the mortality of this disease.
It owes its name to the doctors Henri Rendu and William Osler.
Diagnosis
The diagnosis of disease of Returned-Osler is based on the presence of cutaneous or visceral arteriovenous malformations. The diagnostic criteria are:- spontaneous and recurring Nosebleed.
- Télangiectasies multiples and typically localized on the level of the lips, the mouth, the nose and the fingers.
- Malformations vascular pulmonary, cerebral, hepatic, vertebral and intestinal.
- a relative of the first degree reached of the disease.
Sources
- Alan E Guttmacher, Jamie McDonald, Hereditary Hemorrhagic Telangiectasia in GeneTests: Medical Genetics Information Resource (database online). Copyright, University off Washington, Seattle. 1993-2006 ===Références===
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