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Due to the overlap in clinical, biochemical, and histological features with other causes of cholestasis, the diagnosis requires an intraoperative cholangiogram. Thus, we determined whether diseased livers express a gene expression signature unique to BA. Applying stringent statistical analysis to a genome-wide liver expression platform of 64 infants with BA at the time of diagnosis, 14 age-appropriate subjects with intrahepatic cholestasis as diseased controls and seven normal controls, we identified 15 genes uniquely expressed in BA with an accuracy of 92.3%. Among these genes,<\/jats:bold>\n                      <jats:bold>IL8<\/jats:bold>\n                      <jats:bold>and<\/jats:bold>\n                      <jats:bold>LAMC2<\/jats:bold>\n                      <jats:bold>were sufficient to classify subjects with BA distinctly from diseased controls with an area under the curve of 0.934 (95% confidence interval [CI]: 0.84-1.03), sensitivity of 96.9%, and specificity of 85.7% using their combined first principal component. Direct measurement of interleukin (IL)8 protein in the serum, however, was not different between the two groups. To investigate whether the liver-restricted increase in<\/jats:bold>\n                      <jats:bold>IL8<\/jats:bold>\n                      <jats:bold>was relevant to disease pathogenesis, we inactivated the signaling of<\/jats:bold>\n                      <jats:bold>IL8<\/jats:bold>\n                      <jats:bold>homologs by genetic targeting of the<\/jats:bold>\n                      <jats:bold>Cxcr2<\/jats:bold>\n                      <jats:bold>receptor in a murine model of experimental BA. Disruption of<\/jats:bold>\n                      <jats:bold>Cxcr2<\/jats:bold>\n                      <jats:bold>shortened the duration of cholestasis, decreased the incidence of bile duct obstruction, and improved survival above wild-type neonatal mice.<\/jats:bold>\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion :<\/jats:title>\n                    <jats:p>\n                      <jats:bold>The hepatic expression of<\/jats:bold>\n                      <jats:bold>IL8<\/jats:bold>\n                      <jats:bold>and<\/jats:bold>\n                      <jats:bold>LAMC2<\/jats:bold>\n                      <jats:bold>has high sensitivity for BA at diagnosis and may serve as a biomarker of disease, with an important role for the IL8 signaling in experimental BA. (Hepatology 2014;60:211\u2013223)<\/jats:bold>\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/hep.27045","type":"journal-article","created":{"date-parts":[[2014,2,3]],"date-time":"2014-02-03T20:59:22Z","timestamp":1391461162000},"page":"211-223","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":88,"title":["Gene Expression Signature for Biliary Atresia and a Role for Interleukin-8 in Pathogenesis of Experimental Disease"],"prefix":"10.1097","volume":"60","author":[{"given":"Kazuhiko","family":"Bessho","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reena","family":"Mourya","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pranavkumar","family":"Shivakumar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie","family":"Walters","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John C.","family":"Magee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marepalli","family":"Rao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anil G.","family":"Jegga","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorge 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