{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T08:15:25Z","timestamp":1788336925154,"version":"build-2803163510"},"reference-count":51,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","license":[{"start":{"date-parts":[[2022,11,3]],"date-time":"2022-11-03T00:00:00Z","timestamp":1667433600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2022,11,3]],"date-time":"2022-11-03T00:00:00Z","timestamp":1667433600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,2]]},"abstract":"\n Background and Aims:<\/jats:title>\n Detailed investigation of the biological pathways leading to hepatic fibrosis and identification of liver fibrosis biomarkers may facilitate early interventions for pediatric cholestasis.<\/jats:p>\n <\/jats:sec>\n \n Approach and Results:<\/jats:title>\n A targeted enzyme\u2010linked immunosorbent assay\u2013based panel of nine biomarkers (lysyl oxidase, tissue inhibitor matrix metalloproteinase (MMP) 1, connective tissue growth factor [CTGF], IL\u20108, endoglin, periostin, Mac\u20102\u2013binding protein, MMP\u20103, and MMP\u20107) was examined in children with biliary atresia (BA; n<\/jats:italic> =\u2009187), alpha\u20101 antitrypsin deficiency (A1AT; n<\/jats:italic> =\u200978), and Alagille syndrome (ALGS; n<\/jats:italic> =\u200965) and correlated with liver stiffness (LSM) and biochemical measures of liver disease. Median age and LSM were 9\u2009years and 9.5\u2009kPa. After adjusting for covariates, there were positive correlations among LSM and endoglin (p<\/jats:italic> =\u20090.04) and IL\u20108 (p<\/jats:italic> <\u20090.001) and MMP\u20107 (p<\/jats:italic> <\u20090.001) in participants with BA. The best prediction model for LSM in BA using clinical and lab measurements had an R<\/jats:italic>\n 2<\/jats:sup> =\u20090.437; adding IL\u20108 and MMP\u20107 improved R<\/jats:italic>\n 2<\/jats:sup> to 0.523 and 0.526 (both p<\/jats:italic> <\u20090.0001). In participants with A1AT, CTGF and LSM were negatively correlated (p<\/jats:italic> =\u20090.004); adding CTGF to an LSM prediction model improved R<\/jats:italic>\n 2<\/jats:sup> from 0.524 to 0.577 (p<\/jats:italic> =\u20090.0033). Biomarkers did not correlate with LSM in ALGS. A significant number of biomarker\/lab correlations were found in participants with BA but not those with A1AT or ALGS.<\/jats:p>\n <\/jats:sec>\n \n Conclusions:<\/jats:title>\n Endoglin, IL\u20108, and MMP\u20107 significantly correlate with increased LSM in children with BA, whereas CTGF inversely correlates with LSM in participants with A1AT; these biomarkers appear to enhance prediction of LSM beyond clinical tests. Future disease\u2010specific investigations of change in these biomarkers over time and as predictors of clinical outcomes will be important.<\/jats:p>\n <\/jats:sec>","DOI":"10.1002\/hep.32777","type":"journal-article","created":{"date-parts":[[2022,9,7]],"date-time":"2022-09-07T08:59:00Z","timestamp":1662541140000},"page":"530-545","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":29,"title":["Serum biomarkers correlated with liver stiffness assessed in a multicenter study of pediatric cholestatic liver disease"],"prefix":"10.1097","volume":"77","author":[{"given":"Daniel H.","family":"Leung","sequence":"first","affiliation":[{"name":"Division of Gastroenterology, Hepatology, and Nutrition, Texas Children's Hospital, Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sridevi","family":"Devaraj","sequence":"additional","affiliation":[{"name":"Department of Pathology and Immunology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathan P.","family":"Goodrich","sequence":"additional","affiliation":[{"name":"Arbor Research Collaborative for Health, Ann Arbor, Michigan, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xinpu","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Pathology and Immunology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deepthi","family":"Rajapakshe","sequence":"additional","affiliation":[{"name":"Department of Pathology and Immunology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wen","family":"Ye","sequence":"additional","affiliation":[{"name":"Department of Biostatistics, University of Michigan, Ann Arbor, Michigan, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victor","family":"Andreev","sequence":"additional","affiliation":[{"name":"Arbor Research Collaborative for Health, Ann Arbor, Michigan, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles G.","family":"Minard","sequence":"additional","affiliation":[{"name":"Institute for Clinical and Translational Research, Baylor College of Medicine, Houston, Texas, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danielle","family":"Guffey","sequence":"additional","affiliation":[{"name":"Institute for Clinical and Translational Research, Baylor College of Medicine, Houston, Texas, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean P.","family":"Molleston","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Riley Hospital for Children, Indiana University, Indianapolis, Indiana, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lee M.","family":"Bass","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Ann & Robert H. 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