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Given its role in adult cholestasis, we evaluated human beta-defensin-1 (hBD1) in murine and human BA for associations with disease progression and outcome. This study analyzed hepatic expression of\n                    <jats:italic>hBD1<\/jats:italic>\n                    and\n                    <jats:italic>TGF-\u00df<\/jats:italic>\n                    by qRT-PCR in BA at KPE (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200936) and liver transplantation (LT,\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200944), and compared with normal (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200915) and cholestatic disease controls (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200936). Serum hBD1 was measured by ELISA in BA (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200923) and healthy infants (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200911). Murine\n                    <jats:italic>BD1<\/jats:italic>\n                    was assessed in a Rhesus rotavirus (RRV) BA model.\n                    <jats:italic>BD1<\/jats:italic>\n                    was found to be upregulated in murine and human BA, with higher expression at LT than at KPE. Hepatic\n                    <jats:italic>hBD1<\/jats:italic>\n                    correlated with\n                    <jats:italic>TGF-\u00df<\/jats:italic>\n                    (R\n                    <jats:sup>2<\/jats:sup>\n                    = 0.21), Ishak fibrosis score (R\n                    <jats:sup>2<\/jats:sup>\n                    = 0.36), and serum bile acids (R\n                    <jats:sup>2<\/jats:sup>\n                    = 0.23). Serum hBD1 was elevated in BA versus controls. Elevated hBD1 at KPE predicted persistent jaundice and reduced native liver survival (X\n                    <jats:sup>2<\/jats:sup>\n                    = 9.5), with ROC analysis showing good discrimination for failure of jaundice clearance at 3 months post-KPE (AUC 0.81 for liver and 0.87 for serum). Thus, hBD1 may serve as a negative predictor of jaundice clearance and native liver survival at the time of KPE.\n                  <\/jats:p>","DOI":"10.1038\/s41598-026-43602-9","type":"journal-article","created":{"date-parts":[[2026,3,26]],"date-time":"2026-03-26T10:30:14Z","timestamp":1774521014000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Upregulated beta-defensin-1 in murine and human biliary atresia associates with human native liver survival"],"prefix":"10.1038","volume":"16","author":[{"given":"Christoph","family":"Slavetinsky","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jule","family":"Basenach","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pascal","family":"Damm","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Caterina","family":"Bertolini","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maximilian","family":"Holweg","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Steffen","family":"Hartleif","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Johannes","family":"Hilberath","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephan","family":"Singer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claus","family":"Petersen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J\u00f6rg","family":"Fuchs","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ekkehard","family":"Sturm","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2026,3,26]]},"reference":[{"key":"43602_CR1","doi-asserted-by":"publisher","first-page":"631","DOI":"10.1016\/j.jhep.2016.04.032","volume":"65","author":"HJ Verkade","year":"2016","unstructured":"Verkade, H. 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