{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,25]],"date-time":"2026-04-25T10:04:07Z","timestamp":1777111447156,"version":"3.51.4"},"reference-count":7,"publisher":"SMC Media","issue":"12","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:p>Introduction:\u00a0Porto-sinusoidal vascular syndrome is characterised by specific histological changes that do not include cirrhosis, with or without portal hypertension. Patients are usually asymptomatic until development of portal hypertension complications. Case description:\u00a0A 69-year-old female with history of JAK2 positive essential thrombocythemia (ET) was referred to internal medicine consultation due to elevated liver enzymes. The patient had no previous history of liver disease. Seven months earlier, she had an ischaemic stroke and started treatment with atorvastatin. After discontinuing medication, liver enzymes returned to normal and atorvastatin-related drug-induced liver disease (DILI) was presumed. During a follow-up visit, iron deficiency anaemia was detected and an endoscopic study was performed. It revealed a gastric varix actively bleeding, which was successfully treated with cyanoacrylate. Two months later, the patient was admitted due to a new episode of variceal bleeding, and a portal hypertension complementary study was made. Discussion:\u00a0Although the pathogenesis of porto-sinusoidal vascular disease (PSVD) remains poorly understood, vascular changes within the liver have been associated with several predisposing conditions, such as hypercoagulable states. Patients with ET, especially those with JAK2 mutation, are known to be at increased risk of non-cirrhotic vein thrombosis. Concerning PSVD, the association is not clear but it is believed that both PSVD and myeloproliferative neoplasms share a common denominator: a state characterised by hypercoagulability, inflammation, endothelial dysfunction and, in some cases, portal hypertension. Conclusion:\u00a0Portal hypertension without cirrhosis is a rare condition, presenting diagnostic challenges and significant impact on the patient\u2019s prognosis.<\/jats:p>","DOI":"10.12890\/2023_004138","type":"journal-article","created":{"date-parts":[[2023,10,30]],"date-time":"2023-10-30T11:42:13Z","timestamp":1698666133000},"source":"Crossref","is-referenced-by-count":1,"title":["A case of porto-sinusoidal vascular disease"],"prefix":"10.12890","volume":"10","author":[{"given":"Luciana","family":"Silva","sequence":"first","affiliation":[{"name":"Internal Medicine Department, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Raquel","family":"Moura","sequence":"first","affiliation":[{"name":"Internal Medicine Department, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lu\u00eds","family":"Rocha","sequence":"first","affiliation":[{"name":"Internal Medicine Department, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tiago","family":"Costa","sequence":"first","affiliation":[{"name":"Internal Medicine Department, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Filipe","family":"Breda","sequence":"first","affiliation":[{"name":"Internal Medicine Department, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joana","family":"Cochicho","sequence":"first","affiliation":[{"name":"Internal Medicine Department, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"10397","published-online":{"date-parts":[[2023,10,30]]},"reference":[{"key":"10.12890\/2023_004138_ref1","doi-asserted-by":"crossref","unstructured":"<li>Kmeid M, Liu X, Ballentine S, Lee H. Idiopathic non-cirrhotic portal hypertension and porto-sinusoidal vascular disease: review of current data. <em>Gastroenterology Res<\/em> 2021;<strong>14<\/strong>:49\u201365. <\/li>","DOI":"10.14740\/gr1376"},{"key":"10.12890\/2023_004138_ref2","doi-asserted-by":"crossref","unstructured":"<li>De Gottardi A, Sempoux C, Berzigotti A. Porto-sinusoidal vascular disorder. <em>J Hepatol<\/em> 2022;<strong>77<\/strong>:1124\u20131135. <\/li>","DOI":"10.1016\/j.jhep.2022.05.033"},{"key":"10.12890\/2023_004138_ref3","doi-asserted-by":"crossref","unstructured":"<li>De Gottardi A, Rautou P-E, Schouten J, Rubbia-Brandt L, Leebeek F, Trebicka J, et al. Porto-sinusoidal vascular disease: proposal and description of a novel entity. <em>Lancet Gastroenterol Hepatol<\/em> 2019;<strong>4<\/strong>:399\u2013411. <\/li>","DOI":"10.1016\/S2468-1253(19)30047-0"},{"key":"10.12890\/2023_004138_ref4","doi-asserted-by":"crossref","unstructured":"<li>Gioia S, Nardelli S, Ridola L, Riggio O. Causes and management of non-cirrhotic portal hypertension. <em>Curr Gastroenterol Rep<\/em> 2020;<strong>22<\/strong>:56. <\/li>","DOI":"10.1007\/s11894-020-00792-0"},{"key":"10.12890\/2023_004138_ref5","doi-asserted-by":"crossref","unstructured":"<li>Turon F, Silva-Junior G, Hernandez-Gea V, Garcia-Pagan JC. Hipertensi\u00f3n Portal Idiop\u00e1tica no cirr\u00f3tica. [Idiopathic non-cirrhotic portal hypertension] <em>Gastroenterol Hepatol<\/em> 2015;<strong>38<\/strong>:556\u2013562.<\/li>","DOI":"10.1016\/j.gastrohep.2015.07.004"},{"key":"10.12890\/2023_004138_ref6","doi-asserted-by":"crossref","unstructured":"<li>Tefferi A, Barbui T. Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk-stratification and management. <em>Am J Hematol<\/em> 2020;<strong>95<\/strong>:1599\u20131613.<\/li>","DOI":"10.1002\/ajh.26008"},{"key":"10.12890\/2023_004138_ref7","doi-asserted-by":"crossref","unstructured":"<li>Perner F, Perner C, Ernst T, Heidel FH. Roles of JAK2 in aging, inflammation, hematopoiesis and malignant transformation. <em>Cells<\/em> 2019;<strong>8<\/strong>:854. <\/li>","DOI":"10.3390\/cells8080854"}],"container-title":["European Journal of Case Reports in Internal Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/4138\/3701","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/4138\/3702","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/4138\/3702","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,20]],"date-time":"2025-08-20T07:30:40Z","timestamp":1755675040000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/view\/4138"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,10,30]]},"references-count":7,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2023]]}},"URL":"https:\/\/doi.org\/10.12890\/2023_004138","relation":{"has-manifestation":[{"id-type":"other","id":"1-107-4138-g3701","asserted-by":"subject"},{"id-type":"other","id":"1-107-4138-g3702","asserted-by":"subject"}],"is-part-of":[{"id-type":"other","id":"1-107","asserted-by":"subject"}]},"ISSN":["2284-2594"],"issn-type":[{"value":"2284-2594","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,10,30]]}}}