Nunes da Silva, Inês and Matos, Clara and Correia, Fábio and Carola, Sofia and João Gomes, Maria and Branco, Teresa (2020) Osler-Weber-Rendu Syndrome with Severe Hepatic Manifestations: A Rare Clinical Case. European Journal of Case Reports in Internal Medicine, 7 (11). pp. 1-3. ISSN ISSN: 2284-2594
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Abstract
Rendu-Osler-Weber syndrome is a rare inherited syndrome with autosomal dominant transmission characterized by systemic arteriovenous malformations (AVMs) with multi-organ involvement. Its incidence is 1–2/100,000 and it is predominant in females (the male/female ratio varies from 1:2 to 1:4.5).Clinical manifestations and complications are related to recurrent bleeding and, in some cases, the development of end-organ failure. Management is mostly supportive care and it is essential to promote control of the disease as much as possible and screen eventual complications. We describe the case of a 67-year-old male patient with Rendu-Osler-Weber syndrome admitted to the emergency department with decompensated heart failure due to acute anaemia because of severe epistaxis. During hospitalization, the patient progressed to acute-on-chronic liver failure with hepatic encephalopathy and an abdominal computed tomography scan showed multiple hepatic AVMs considered to be the cause of the chronic liver disease.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | Rendu-Osler-Weber syndrome, arteriovenous malformations, epistaxis, congestive heart failure, chronic hepatic disease |
| Subjects: | 600 Tecnologia - Scienze applicate 600 Tecnologia - Scienze applicate > 610 Medicina e salute (Classificare qui la tecnologia dei servizi medici) 600 Tecnologia - Scienze applicate > 610 Medicina e salute (Classificare qui la tecnologia dei servizi medici) > 616 Malattie (classificare qui la Clinica medica, la medicina basata sull'evidenza, la Medicina interna, la Medicina sperimentale) |
| Depositing User: | Marina Spanti |
| Date Deposited: | 15 Dec 2021 14:25 |
| Last Modified: | 15 Dec 2021 14:25 |
| URI: | http://eprints.bice.rm.cnr.it/id/eprint/21055 |
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