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<p>Biliary atresia (BA) is a neonatal cholangiopathy that often progresses to cirrhosis despite timely Kasai portoenterostomy (KPE), and prognostic biomarkers remain undefined. 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 <i>hBD1</i> and <i>TGF-ß</i> by qRT-PCR in BA at KPE (<i>n</i> = 36) and liver transplantation (LT, <i>n</i> = 44), and compared with normal (<i>n</i> = 15) and cholestatic disease controls (<i>n</i> = 36). Serum hBD1 was measured by ELISA in BA (<i>n</i> = 23) and healthy infants (<i>n</i> = 11). Murine <i>BD1</i> was assessed in a Rhesus rotavirus (RRV) BA model. <i>BD1</i> was found to be upregulated in murine and human BA, with higher expression at LT than at KPE. Hepatic <i>hBD1</i> correlated with <i>TGF-ß</i> (R<sup>2</sup> = 0.21), Ishak fibrosis score (R<sup>2</sup> = 0.36), and serum bile acids (R<sup>2</sup> = 0.23). Serum hBD1 was elevated in BA versus controls. Elevated hBD1 at KPE predicted persistent jaundice and reduced native liver survival (X<sup>2</sup> = 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.</p>

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Upregulated beta-defensin-1 in murine and human biliary atresia associates with human native liver survival

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年3月26日

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  • Christoph Slavetinsky,
  • Jule Basenach,
  • Pascal Damm,
  • Caterina Bertolini,
  • Maximilian Holweg,
  • Steffen Hartleif,
  • Johannes Hilberath,
  • Stephan Singer,
  • Claus Petersen,
  • Jörg Fuchs,
  • Ekkehard Sturm

摘要

Biliary atresia (BA) is a neonatal cholangiopathy that often progresses to cirrhosis despite timely Kasai portoenterostomy (KPE), and prognostic biomarkers remain undefined. 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 hBD1 and TGF-ß by qRT-PCR in BA at KPE (n = 36) and liver transplantation (LT, n = 44), and compared with normal (n = 15) and cholestatic disease controls (n = 36). Serum hBD1 was measured by ELISA in BA (n = 23) and healthy infants (n = 11). Murine BD1 was assessed in a Rhesus rotavirus (RRV) BA model. BD1 was found to be upregulated in murine and human BA, with higher expression at LT than at KPE. Hepatic hBD1 correlated with TGF-ß (R2 = 0.21), Ishak fibrosis score (R2 = 0.36), and serum bile acids (R2 = 0.23). Serum hBD1 was elevated in BA versus controls. Elevated hBD1 at KPE predicted persistent jaundice and reduced native liver survival (X2 = 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.