{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,27]],"date-time":"2025-11-27T10:40:32Z","timestamp":1764240032520},"reference-count":29,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2016,11,19]],"date-time":"2016-11-19T00:00:00Z","timestamp":1479513600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Geneva Faculty of Medicine"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2016,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background &amp; Aims<\/jats:title><jats:p>Hereditary haemorrhagic telangiectasia is characterized by arterio\u2010venous malformations (<jats:styled-content style=\"fixed-case\">AVM<\/jats:styled-content>). It frequently involves the liver without clinical symptoms, but may lead to biliary ischaemia, portal hypertension, or fatal high\u2010output heart failure. The indication of liver transplantation is controversial.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Herein, we report the case of a 65\u2010year\u2010old female patient with a \u2018double Osler syndrome\u2019 consisting of hereditary haemorrhagic telangiectasia (<jats:styled-content style=\"fixed-case\">HHT<\/jats:styled-content>) and type I hereditary angioedema diagnosed at the age of 25 and 22\u00a0years respectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Hereditary angioedema was treated with danazol for several decades until multiple hypoechogenic liver masses were detected. Albeit danazol treatment was replaced by C1 esterase inhibitor infusions, hepatocellular carcinoma was diagnosed at the age of 64 and the patient was listed for liver transplantation. <jats:styled-content style=\"fixed-case\">HHT<\/jats:styled-content> was marked by recurrent epistaxis until the age of 63 when severe intestinal bleeding occurred. At the age of 65, severe dyspnoea (<jats:styled-content style=\"fixed-case\">NYHA<\/jats:styled-content> class <jats:styled-content style=\"fixed-case\">IV<\/jats:styled-content>) developed and rapidly progressive high\u2010output cardiac failure was diagnosed. Despite argon plasma coagulation to control bleeding from intestinal angiodysplasia, and treatment with bevacizumab to inhibit angiogenesis, the patient died from severe gastrointestinal bleeding associated with cardiogenic shock at the age of 66 before being transplanted.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The indication to list this patient for liver transplantation was debated several times before the diagnosis of hepatocellular carcinoma because of good general condition and low <jats:styled-content style=\"fixed-case\">MELD<\/jats:styled-content> score. Precise guidelines for screening and management of patients with hepatic <jats:styled-content style=\"fixed-case\">HHT<\/jats:styled-content> need to be better defined.<\/jats:p><\/jats:sec>","DOI":"10.1111\/liv.13187","type":"journal-article","created":{"date-parts":[[2016,11,19]],"date-time":"2016-11-19T09:20:47Z","timestamp":1479547247000},"page":"1735-1740","source":"Crossref","is-referenced-by-count":12,"title":["Hereditary haemorrhagic telangiectasia: to transplant or not to transplant \u2013 is there a right time for liver transplantation?"],"prefix":"10.1111","volume":"36","author":[{"given":"Yannick D.","family":"Muller","sequence":"first","affiliation":[{"name":"Division of Clinical Immunology and Allergology Department of Medical Specialties University Hospitals and Medical Faculty Geneva Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roland","family":"Oppliger","sequence":"additional","affiliation":[{"name":"Private Practice Nyon 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