{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T09:12:22Z","timestamp":1782983542441,"version":"3.54.5"},"reference-count":43,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","license":[{"start":{"date-parts":[[2017,6,26]],"date-time":"2017-06-26T00:00:00Z","timestamp":1498435200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"},{"start":{"date-parts":[[2017,6,26]],"date-time":"2017-06-26T00:00:00Z","timestamp":1498435200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":["Hepatology"],"published-print":{"date-parts":[[2017,8]]},"abstract":"<jats:p>Cystic fibrosis (CF) liver disease (CFLD), a leading cause of death in CF, is mostly described in pediatric populations. Adult\u2010onset CFLD lacks sufficient characterization and diagnostic tools. A cohort of CF patients without CFLD during childhood were followed for up to 38 years with serologic testing, imaging, and noninvasive fibrosis markers. Historical CFLD diagnostic criteria were compared with newly proposed CFLD criteria. Thirty\u2010six CF patients were followed for a median of 24.5 years (interquartile range 15.6\u201032.9). By the last follow\u2010up, 11 (31%) had died. With conventional criteria, 8 (22%) patients had CFLD; and by the new criteria, 17 (47%) had CFLD at a median age of 36.6 years (interquartile range 26.5\u201043.2). By the new criteria, those with CFLD had higher median alanine aminotransferase (42 versus 27, <jats:italic toggle=\"yes\">P<\/jats:italic> = 0.005), aspartate aminotransferase (AST; 26 versus 21, <jats:italic toggle=\"yes\">P<\/jats:italic> = 0.01), direct bilirubin (0.13 versus 0.1, <jats:italic toggle=\"yes\">P<\/jats:italic> = 0.01), prothrombin time (14.4 versus 12.4, <jats:italic toggle=\"yes\">P<\/jats:italic> = 0.002), and AST\u2010to\u2010platelet ratio index (0.31 versus 0.23, <jats:italic toggle=\"yes\">P<\/jats:italic> = 0.003) over the last 2 years of follow\u2010up. Subjects with a FibroScan &gt;6.8 kPa had higher alanine aminotransferase (42 versus 28U\/L, <jats:italic toggle=\"yes\">P<\/jats:italic> = 0.02), AST (35 versus 25U\/L, <jats:italic toggle=\"yes\">P<\/jats:italic> = 0.02), AST\u2010to\u2010platelet ratio index (0.77 versus 0.25, <jats:italic toggle=\"yes\">P<\/jats:italic> = 0.0004), and Fibrosis\u20104 index (2.14 versus 0.74, <jats:italic toggle=\"yes\">P<\/jats:italic> = 0.0003) and lower platelet counts (205 versus 293, <jats:italic toggle=\"yes\">P<\/jats:italic> = 0.02). One CFLD patient had nodular regenerative hyperplasia. Longitudinally, mean platelet counts significantly declined in the CFLD group (from 310 to 230 U\/L, <jats:italic toggle=\"yes\">P<\/jats:italic> = 0.0005). Deceased CFLD patients had lower platelet counts than those alive with CFLD (143 versus 258 U\/L, <jats:italic toggle=\"yes\">P<\/jats:italic> = 0.004) or those deceased with no CFLD (143 versus 327U\/L, <jats:italic toggle=\"yes\">P<\/jats:italic> = 0.006). <jats:italic toggle=\"yes\">Conclusion<\/jats:italic>: Adult\u2010onset CFLD may be more prevalent than previously described, which suggests a later wave of CFLD that impacts morbidity; routine liver tests, radiologic imaging, noninvasive fibrosis markers, and FibroScan can be used algorithmically to identify adult CFLD; and further evaluation in other CF cohorts should be performed for validation. 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