{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T17:21:36Z","timestamp":1779211296787,"version":"3.51.4"},"reference-count":38,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2023,9,19]],"date-time":"2023-09-19T00:00:00Z","timestamp":1695081600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2023,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and Aims<\/jats:title><jats:p>Porto\u2010sinusoidal vascular disease (PSVD) has been described as the prominent pathology in liver explants of patients with cystic fibrosis (CF), but data outside the transplant setting are lacking. We aimed to investigate the prevalence of portal hypertension (PH) in CF\u2010associated liver disease (CFLD) and develop an algorithm to classify liver involvement in CF patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This is a cross\u2010sectional study of consecutive paediatric and adult patients in a tertiary centre between 2018 and 2019, who underwent ultrasound, liver (LSM) and spleen stiffness (SSM) measurement. CFLD was defined according to physical examination, liver tests and ultrasound findings. PSVD was likely if there were PH signs in the absence of advanced chronic liver disease (CF\u2010ACLD, LSM &lt;10\u2009kPa). A historical cohort was used to validate the prognostic significance of the new definitions.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Fifty (27.5%) patients met CFLD criteria. At least one sign of PH was found in 47 (26%) patients, but most (81%) had LSM &lt;10\u2009kPa and were likely to have PSVD; only 9 (5%) had CF\u2010ACLD. PSVD and CFLD (LSM &lt;10\u2009kPa) co\u2010existed in most (23\/36) cases. In the historical cohort (<jats:italic>n<\/jats:italic>\u2009=\u2009599 patients), likely PSVD and CFLD+PH were independently associated with a 2\u2010fold and 3.5\u2010fold increase in mortality compared to patients without PH, respectively. In 34 patients with SSM, values &lt;21 and &gt;50\u2009kPa accurately diagnosed specific signs of PH.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>PSVD is the prevailing cause of PH in CF patients. We developed a new diagnostic algorithm based on clinical and elastosonography criteria to classify liver involvement in patients with CF.<\/jats:p><\/jats:sec>","DOI":"10.1111\/liv.15748","type":"journal-article","created":{"date-parts":[[2023,9,19]],"date-time":"2023-09-19T11:53:44Z","timestamp":1695124424000},"page":"2492-2502","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["The non\u2010invasive evaluation of liver involvement in patients with cystic fibrosis: A prospective study"],"prefix":"10.1111","volume":"43","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2905-1146","authenticated-orcid":false,"given":"Elton","family":"Dajti","sequence":"first","affiliation":[{"name":"Unit of Gastroenterology Borgo Trento University Hospital of Verona  Verona Italy"},{"name":"IRCCS Azienda Ospedaliero\u2010Universitaria di Bologna, Policlinico di S. Orsola European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Bologna Italy"},{"name":"Department of Medical and Surgical Sciences (DIMEC) University of Bologna  Bologna Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1142-8585","authenticated-orcid":false,"given":"Federico","family":"Ravaioli","sequence":"additional","affiliation":[{"name":"Department of Medical and Surgical Sciences (DIMEC) University of Bologna  Bologna Italy"},{"name":"Division of Gastroenterology Azienda Ospedaliero\u2010Universitaria di Modena and University of Modena and Reggio Emilia  Modena Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giulia","family":"Paiola","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Center Borgo Trento University Hospital of Verona  Verona Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sonia","family":"Volpi","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Center Borgo Trento University Hospital of Verona  Verona Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luigi","family":"Colecchia","sequence":"additional","affiliation":[{"name":"Department of Medical and Surgical Sciences (DIMEC) University of Bologna  Bologna Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alberto","family":"Ferrarese","sequence":"additional","affiliation":[{"name":"Unit of Gastroenterology Borgo Trento University Hospital of Verona  Verona Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luigina Vanessa","family":"Alemanni","sequence":"additional","affiliation":[{"name":"Department of Medical and Surgical Sciences (DIMEC) University of Bologna  Bologna Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Caterina","family":"Cusumano","sequence":"additional","affiliation":[{"name":"Unit of Gastroenterology Borgo Trento University Hospital of Verona  Verona Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna Rita","family":"Di\u00a0Biase","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Modena  Modena Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7167-8773","authenticated-orcid":false,"given":"Giovanni","family":"Marasco","sequence":"additional","affiliation":[{"name":"IRCCS Azienda Ospedaliero\u2010Universitaria di Bologna, Policlinico di S. Orsola European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Bologna Italy"},{"name":"Department of Medical and Surgical Sciences (DIMEC) University of Bologna  Bologna Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amanda","family":"Vestito","sequence":"additional","affiliation":[{"name":"IRCCS Azienda Ospedaliero\u2010Universitaria di Bologna, Policlinico di S. 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