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  • Title: Pulmonary hypertension and hepatic encephalopathy: lethal complications of Rendu-Osler-Weber disease.
    Author: Ford TJ, Fong MW, Cheah BC, Alexopolous C.
    Journal: J R Coll Physicians Edinb; 2014; 44(2):126-9. PubMed ID: 24999773.
    Abstract:
    Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterised by epistaxis, mucocutaneous telangiectasia with systemic manifestations due to visceral telangiectasia and arterio-venous malformations (AVMs). We describe unusual complications of HHT in a 68-year-old male who developed high-output cardiac failure with pulmonary hypertension in combination with hepatic encephalopathy due to hepatic AVMs. This case shows the importance of a multi-system approach to HHT and considers the treatment of its hepatic complications.
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