{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T15:13:56Z","timestamp":1787066036797,"version":"build-2736575974"},"reference-count":20,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2020,10,13]],"date-time":"2020-10-13T00:00:00Z","timestamp":1602547200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["81873543"],"award-info":[{"award-number":["81873543"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2020,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background &amp; Aims<\/jats:title>\n                    <jats:p>\n                      ABCB11 deficiency presenting in infancy is believed generally to manifest as persistent\/progressive cholestasis. We describe a group of patients with biallelic\n                      <jats:italic>ABCB11<\/jats:italic>\n                      variants whose disorder manifested as transient neonatal cholestasis (TNC).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Neonatal intrahepatic cholestasis in 68 children (31 males) with biallelic predictedly pathogenic variants (PPV) in\n                      <jats:italic>ABCB11<\/jats:italic>\n                      was classified as transient (TNC group, n\u00a0=\u00a023, 11 males), intermittent (benign recurrent intrahepatic cholestasis [BRIC] group, n\u00a0=\u00a03, 1 male) or persistent\/ progressive (progressive familial intrahepatic cholestasis [PFIC] group, n\u00a0=\u00a042, 19 males). Clinical, genetic and bile salt export pump (BSEP) expression information was correlated with outcomes.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The median onset age of jaundice was 3\u00a0days (birth to 2\u00a0months) for the TNC group and 10.5\u00a0days (birth to 3\u00a0months) for the PFIC group (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a0.034). The median length of follow\u2010up of TNC patients was 44\u00a0months (12\u00a0months\u2010168\u00a0months). At presentation, hepatobiliary\u2010injury biomarker values were similar between the groups (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0&gt;\u00a0.05). TNC patients (17\/23) more often than PFIC patients (20\/42,\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a0.041) harboured biallelic non\u2010null variants (predicted not to terminate translation prematurely). TNC patient livers (7\/7) more often than PFIC patient livers (5\/16,\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a0.005) expressed immunohistochemically detectable BSEP. Kaplan\u2010Meier analysis showed better prognosis for patients with BSEP expression (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a0.009). Too few BRIC patients were available for statistical study.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      Neonatal cholestasis associated with biallelic PPV in\n                      <jats:italic>ABCB11<\/jats:italic>\n                      can resolve temporarily or persistently in one third of cases. Resolution is more likely in patients with biallelic non\u2010null PPV or with liver BSEP expression.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/liv.14642","type":"journal-article","created":{"date-parts":[[2020,8,27]],"date-time":"2020-08-27T23:13:18Z","timestamp":1598569998000},"page":"2788-2796","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["ABCB11 deficiency presenting as transient neonatal cholestasis: Correlation with genotypes and BSEP expression"],"prefix":"10.1111","volume":"40","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1165-4360","authenticated-orcid":false,"given":"Li\u2010Ting","family":"Li","sequence":"first","affiliation":[{"name":"The Center for Pediatric Liver Diseases Children\u2019s Hospital of Fudan University Shanghai China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhong\u2010Die","family":"Li","sequence":"additional","affiliation":[{"name":"The Center for Pediatric Liver Diseases Children\u2019s Hospital of Fudan University Shanghai China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ye","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Jinshan Hospital Fudan University Shanghai China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yi","family":"Lu","sequence":"additional","affiliation":[{"name":"The Center for Pediatric Liver Diseases Children\u2019s Hospital of Fudan University Shanghai China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xin\u2010Bao","family":"Xie","sequence":"additional","affiliation":[{"name":"The Center for Pediatric Liver Diseases Children\u2019s Hospital of Fudan University Shanghai China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lian","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Pathology Children\u2019s Hospital of Fudan University Shanghai China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jia\u2010Yan","family":"Feng","sequence":"additional","affiliation":[{"name":"Department of Pathology Children\u2019s Hospital of Fudan University Shanghai China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. S.","family":"Knisely","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr Pathologie Medizinische Universit\u00e4t Graz Graz Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jian\u2010She","family":"Wang","sequence":"additional","affiliation":[{"name":"The Center for Pediatric Liver Diseases Children\u2019s Hospital of Fudan University Shanghai China"},{"name":"Shanghai Key Laboratory of Birth Defects Shanghai China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,10,13]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/3034"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0016-5085(99)70287-8"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2004.04.065"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.fpc.0000230418.28091.76"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/gut.2008.159541"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/ped.12049"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2020.02.007"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000001334"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1478-3231.2009.02112.x"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2015.08.037"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0153114"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0164058"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/liv.13714"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/hepr.13055"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41390-019-0548-8"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2008.01.038"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2010.01.034"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.20627"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.23539"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/liv.14518"}],"container-title":["Liver International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/liv.14642","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/liv.14642","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/liv.14642","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,2]],"date-time":"2023-09-02T12:09:41Z","timestamp":1693656581000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/liv.14642"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,10,13]]},"references-count":20,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2020,11]]}},"alternative-id":["10.1111\/liv.14642"],"URL":"https:\/\/doi.org\/10.1111\/liv.14642","archive":["Portico"],"relation":{},"ISSN":["1478-3223","1478-3231"],"issn-type":[{"value":"1478-3223","type":"print"},{"value":"1478-3231","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,10,13]]},"assertion":[{"value":"2020-04-19","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-08-13","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-10-13","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}