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Hereditary haemorrhagic telangiectasia
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> Also known as Osler-Weber-Rendu syndrome, autosomal dominant condition characterised by multiple telangiectasia over skin and mucous membranes. 20% occur spontaneously without prior family history. ''4 diagnostic criteria'' if 2 then possible diagnosis of HHT, if 3+ definite diagnosis of HHT: epistaxis : spontaneous, recurrent nosebleeds telangiectases: multiple at characteristic sites (lips, oral cavity, fingers, nose) visceral lesions: for example gastrointestinal telangiectasia (with or without bleeding), pulmonary arteriovenous malformations (AVM), hepatic AVM, cerebral AVM, spinal AVM family history: a first-degree relative with HHT
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Haematology