{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,11]],"date-time":"2026-08-11T00:52:31Z","timestamp":1786409551058,"version":"build-2736575974"},"reference-count":25,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2020,1,22]],"date-time":"2020-01-22T00:00:00Z","timestamp":1579651200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100004837","name":"Ministerio de Ciencia e Innovaci\u00f3n","doi-asserted-by":"publisher","award":["SAF 2017\u201086343\u2010R"],"award-info":[{"award-number":["SAF 2017\u201086343\u2010R"]}],"id":[{"id":"10.13039\/501100004837","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2020,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and aims<\/jats:title><jats:p>Protein\u2010losing enteropathy (PLE) after Fontan surgery carries significant morbimortality. Its pathophysiology and association with other Fontan complications are poorly understood. Our aims were to examine whether Fontan\u2010PLE is associated with greater liver damage and to assess the presence of systemic and intestinal inflammation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Fontan patients with PLE and Fontan controls without PLE matched for age and Fontan surgery procedure were included. Data were prospectively compiled on blood and stool tests, liver imaging, elastography, cardiac\u2010MRI and cardiac catheterization.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Twenty\u2010nine Fontan patients were enrolled (14 with PLE and 15 controls without PLE). Patients with PLE had more advanced liver disease estimated by non\u2010invasive methods: blunt liver margins on ultrasonography (71.4% vs 26.7%, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.027), greater median liver stiffness (25.4 vs 14.5\u00a0kPa, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.003) and higher FIB\u20104 (<jats:italic>P<\/jats:italic>\u00a0=\u00a0.016). Portal hypertension\u2010related signs were more common in patients with PLE including ascites (<jats:italic>P<\/jats:italic>\u00a0=\u00a0.035), larger spleen size (<jats:italic>P<\/jats:italic>\u00a0=\u00a0.005), oesophageal varices\/splanchnic collateral shunts (<jats:italic>P<\/jats:italic>\u00a0=\u00a0.03), higher liver stiffness\u2010spleen size\u2010to\u2010platelet ratio risk score (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a0.001) and lower platelet count (<jats:italic>P<\/jats:italic>\u00a0=\u00a0.01). Systemic proinflammatory cytokines (TNF\u2010\u03b1, interleukin\u20106), biomarkers of intestinal permeability (intestinal fatty\u2010acid binding protein) and faecal calprotectin concentrations were also significantly increased in Fontan\u2010PLE (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a0.05). Faecal calprotectin directly correlated with alpha\u20101 antitrypsin clearance and inversely with cardiac index, total serum proteins and body mass index.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Fontan\u2010PLE is associated with advanced liver disease and increased markers of systemic inflammation and intestinal permeability. Faecal calprotectin is elevated and correlates with Fontan\u2010PLE severity. Liver assessment is mandatory in all Fontan patients, and especially in those with PLE.<\/jats:p><\/jats:sec>","DOI":"10.1111\/liv.14375","type":"journal-article","created":{"date-parts":[[2020,1,8]],"date-time":"2020-01-08T13:26:13Z","timestamp":1578489973000},"page":"638-645","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Fontan protein\u2010losing enteropathy is associated with advanced liver disease and a proinflammatory intestinal and systemic state"],"prefix":"10.1111","volume":"40","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2852-6042","authenticated-orcid":false,"given":"Enrique","family":"Rodr\u00edguez de Santiago","sequence":"first","affiliation":[{"name":"Department of Gastroenterology and Hepatology Hospital Universitario Ram\u00f3n y Cajal University of Alcala Madrid Spain"},{"name":"Instituto Ram\u00f3n y Cajal de Investigaci\u00f3n Biosanitaria IRYCIS Madrid 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