{
  "abstract": "Biliary Atresia (BA) is the leading cause of pediatric liver transplantation. Given the heterogeneity of the disease, outcomes after diagnosis remain unpredictable. A multiscale atlas of BA hepatic tissue established early lymphatic dysplasia in infants that later proceed toward liver failure and transplantation ( severe BA). An architecturally distorted, immature and CCL21high lymphatic vasculature marked severe BA, along with an expanding cholangiocyte compartment pivoting toward immune-reactive MMP7/SOX9high states. Hepatocyte-to-biliary trans differentiation emerged broadly across all BA- and non-BA-cholestatic injuries, while dedifferentiation and acute-phase-reactions were specific to severe BA. Functionally, lymphatic endothelial tissue, isolated from severe BA livers, induced an analogous LGR5- and SOX9-positive epithelial progenitor response when assembled with cholangiocytes as organoids. Further experiments revealed that lymphatic endothelium signals ectopically on liver epithelium via CCL21-CCR7-axis after BA-specific inflammatory priming. CCL21-depletion strategies mitigated the BA-specific MMP7-program. In sum, a maladaptive lymphatic endothelial dysplasia perturbs intrahepatic epithelium in severe BA and offers CCL21 as a possible target for epithelial stability.",
  "abstract_shown": true,
  "affiliations": [
    "Charite Universitaetsmedizin Berlin"
  ],
  "article_type": "PUBLISHAHEADOFPRINT",
  "arxiv": null,
  "author_total": 27,
  "authors": [
    {
      "name": "L. Schumm"
    },
    {
      "name": "R. L. Chua"
    },
    {
      "name": "M. Z. Kuzinska"
    },
    {
      "name": "M. Meindl"
    },
    {
      "name": "S. Quach"
    },
    {
      "name": "J. Weihs"
    },
    {
      "name": "D. Gogol"
    },
    {
      "name": "M. Zschiesche"
    },
    {
      "name": "R. Duennebacke"
    },
    {
      "name": "M. Uecker"
    },
    {
      "name": "R. Y. Behncke"
    },
    {
      "name": "A. Sudy"
    },
    {
      "name": "J. Liebig"
    },
    {
      "name": "J.-C. Kamp"
    },
    {
      "name": "M. Kucklick"
    },
    {
      "name": "B. Globke"
    },
    {
      "name": "R. Oellinger"
    },
    {
      "name": "J. Kuebler"
    },
    {
      "name": "S. Henning"
    },
    {
      "name": "C. Slavetinsky"
    },
    {
      "name": "R. Haegerling"
    },
    {
      "name": "O. Madadi-Sanjani"
    },
    {
      "name": "R. Eils"
    },
    {
      "name": "A. Guillot"
    },
    {
      "name": "P. Bufler"
    },
    {
      "name": "C. Conrad"
    },
    {
      "affs": [
        0
      ],
      "name": "M. Rezvani"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/9bs8wqa78nnc1.bib",
  "cited_by": 0,
  "doi": "10.64898/2026.10.12.26365253",
  "fulltext_url": "https://www.medrxiv.org/content/10.64898/2026.10.12.26365253v1.full",
  "id": "9bs8wqa78nnc1",
  "issns": [],
  "landing_url": "https://www.medrxiv.org/content/10.64898/2026.10.12.26365253v1",
  "language": null,
  "licence": "CC-BY-NC-ND",
  "licence_label": "CC BY-NC-ND",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by-nc-nd/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "medrxiv",
      "pediatrics"
    ],
    [
      "openalex",
      "2746|Pediatric Hepatobiliary Diseases and Treatments"
    ]
  ],
  "openalex": "W7221653375",
  "pdf_url": "https://www.medrxiv.org/content/10.64898/2026.10.12.26365253v1.full.pdf",
  "pmcid": null,
  "pmid": null,
  "published": "2026-10-12",
  "publisher": null,
  "ris": "https://research.draconitic.com/paper/9bs8wqa78nnc1.ris",
  "sources": [
    {
      "first_seen": "2026-10-30T12:20:49Z",
      "native_id": "10.64898/2026.10.12.26365253",
      "source": "medrxiv",
      "url": "https://www.medrxiv.org/content/10.64898/2026.10.12.26365253v1"
    }
  ],
  "subjects": [
    {
      "field": "medicine",
      "origin": "medrxiv:pediatrics",
      "subfield": "reproductive-child"
    }
  ],
  "title": "Lymphatic Dysplasia Shapes Liver Epithelial Outcomes in Biliary Atresia",
  "title_text": "Lymphatic Dysplasia Shapes Liver Epithelial Outcomes in Biliary Atresia",
  "type": "preprint",
  "updated_at": "2026-10-30T12:20:49Z",
  "url": "https://research.draconitic.com/paper/9bs8wqa78nnc1",
  "venue": "medRxiv",
  "versions": []
}