{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T23:47:24Z","timestamp":1791935244727,"version":"4.3.4"},"reference-count":29,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2018,2,19]],"date-time":"2018-02-19T00:00:00Z","timestamp":1518998400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Autonomous Region of Sardinia, Department of Public Education, 09123 Cagliari, ITALY"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hepatology Research"],"published-print":{"date-parts":[[2018,7]]},"abstract":"<jats:sec>\n                    <jats:title>Aim<\/jats:title>\n                    <jats:p>The etiopathogenesis of non\u2010syndromic biliary atresia (BA) is obscure. The primary aim was to investigate intrahepatic bile duct cilia (IHBC) in BA at diagnosis and its correlation with clinical outcome. The secondary aim was to analyze IHBC in routine paraffin\u2010embedded liver biopsies using conventional scanning electron microscopy (SEM).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Surgical liver biopsies taken at diagnosis from 22 BA infants (age range, 39\u2013116\u00a0days) and from eight children with non\u2010BA chronic cholestasis (age range, 162\u00a0days \u221216.8\u00a0years) were evaluated for IHBC by immunofluorescence (IF) and SEM. A minimum 18\u2010month follow\u2010up after surgery was available for all patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      By IF, cilia were present in 6\/8 (75%) non\u2010BA but only in 3\/22 (14%) BA cases, and cilia were reduced or absent in 19\/22 (86%) BA and 2\/8 (25%) non\u2010BA livers (\n                      <jats:italic>P<\/jats:italic>\n                      \u2009&lt;\u20090.01). In BA, cilia presence was found to be associated with clearance of jaundice at 6\u2010month follow\u2010up (\n                      <jats:italic>P<\/jats:italic>\n                      \u2009&lt;\u20090.05). However, high overall survival rates with native liver, &gt;90% at 12\u00a0months, and &gt;70% at 24\u00a0months post\u2010surgery, were recorded regardless of cilia presence\/absence at diagnosis. Electron microscopy was able to detect bile ducts and cilia in routine liver biopsies, revealing significant abnormalities in 100% BA livers.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The presence of IHBC in BA livers at the diagnosis was associated with resolution of cholestasis, although was not predictive of short\u2010term survival with native liver. Scanning electron microscopy represents a powerful new tool to study routine liver biopsies in biliary disorders. Cilia dysfunction in BA pathogenesis and\/or disease progression warrants further investigation.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/hepr.13060","type":"journal-article","created":{"date-parts":[[2018,1,13]],"date-time":"2018-01-13T08:43:11Z","timestamp":1515832991000},"page":"664-674","source":"Crossref","is-referenced-by-count":29,"title":["Intrahepatic bile duct primary cilia in biliary atresia"],"prefix":"10.1111","volume":"48","author":[{"given":"Roberta","family":"Frassetto","sequence":"first","affiliation":[{"name":"Pediatric Clinic, Department of Clinical and Experimental Medicine University of Sassari  Sassari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Filippo","family":"Parolini","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery \u201cSpedali Civili\u201d Children's Hospital  Brescia 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