Pulmonary manifestations of hereditary hemorrhagic telangiectasia

摘要: INTRODUCTION Osler-Weber-Rendu disease (hereditary hemorrhagic telangiectasia) is an autosomal dominant genetic disorder with variable penetrance. It estimated to affect at least one in ten thousand of the population France. The diagnosis clinical and depends on association epistaxis, telangiectasia, visceral manifestations disease, familial occurrence. STATE OF THE ART Pulmonary arterio-venous malformations (AVM) which occur about 15-30% patients this condition represent main complication disease. Infectious ischaemic neurological due paradoxical embolism may be presenting feature. high frequency complications even asymptomatic justifies systematic screening for pulmonary AVMs, using chest radiography, contrast echocardiography, and/or CT. Treatment based percutaneous transcatheter coil vaso-occlusion feeding artery. CONCLUSION arterial hypertension rare. systemic arteriovenous shunting liver increasing cardiac output, or similar idiopathic hypertension.