Evidence map›Paper›PMID 42703703›Full record

ArticleLiver international : official journal of the International Association for the Study of the Liver2026

Ductular Reaction Underlies Development of Portal Hypertension in Biliary Atresia.

Petriina Luokkala, Maria Hukkinen, Iiris Nyholm, Nelli Sjöblom, Mikko P Pakarinen

Abstract read
In one paragraph

Article in Liver international : official journal of the International Association for the Study of the Liver, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

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0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

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2 · The registry

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Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Ductular Reaction Underlies Development of Portal Hypertension in Biliary Atresia.Liver international : official journal of the International Association for the Study of the Liver · 2026
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4 · The record

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5 · Who and what money

Authors and funding

5 authors.

Petriina LuokkalaDepartment of Children and Adolescent, Paediatric Liver and Gut Research Group, Section of Paediatric Surgery, New Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.ORCID https://orcid.org/0009-0006-5929-5933
Maria HukkinenDepartment of Children and Adolescent, Paediatric Liver and Gut Research Group, Section of Paediatric Surgery, New Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.ORCID https://orcid.org/0000-0002-2622-8391
Iiris NyholmDepartment of Children and Adolescent, Paediatric Liver and Gut Research Group, Section of Paediatric Surgery, New Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.ORCID https://orcid.org/0000-0003-4999-4280
Nelli SjöblomDepartment of Pathology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.ORCID https://orcid.org/0000-0002-3360-6645
Mikko P PakarinenDepartment of Children and Adolescent, Paediatric Liver and Gut Research Group, Section of Paediatric Surgery, New Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.ORCID https://orcid.org/0000-0001-6016-6334

Funding

Helsinki University Hospital FundLastentautien TutkimussäätiöSigrid Juséliuksen Säätiö
6 · The paper itself

Abstract

BACKGROUND &

aimsAs better understanding of pathophysiology and reliable predictors for portal hypertension (PH) are needed, we investigated how histological ductular reaction (DR) and liver fibrosis predict PH in biliary atresia (BA).

methods60 BA patients treated between 1987 and 2024 at Helsinki University Hospital were enrolled. Patients underwent liver biopsies at Kasai portoenterostomy and 1 year postoperatively. PH was defined as endoscopic detection of oesophageal varices or thrombocytopenia (< 150*109) and splenomegaly. A neural network model was used to quantify proportional DR (DR%) relative to entire liver tissue, composed of cytokeratin-7-positive portal biliary epithelium (BE%) and parenchymal intermediate hepatocytes (PIH%). Fibrosis was measured by Metavir staging and computerized quantification of Sirius-Red-positive area (SRP%). Predictors of PH were studied with AUROC and survival analyses, and Cox regression models.

resultsAfter portoenterostomy, PH was detected in 70% (n = 42) of patients at median age 1.4 years (IQR 0.7-2.6). At portoenterostomy, DR% ≥ 6.9% (p = 0.016) and PIH% ≥ 1.6% (p < 0.001), but not liver fibrosis apart from cirrhosis (n = 5) predicted PH (p = 0.032). At 1 year, DR% ≥ 3.5% (p < 0.001), PIH% ≥ 0.7% (p < 0.001), BE% ≥ 3.2% (p = 0.006) as well as Metavir 3-4 vs. 1-2 (p = 0.037) and SRP% ≥ 28.4% (p = 0.006) predicted PH. Patients with PH showed progression of PIH% (p < 0.001) from portoenterostomy to follow-up biopsy, accompanied by more rapid progression of fibrosis. In univariable regression models, different DR components, but not fibrosis, were associated with PH both at portoenterostomy and follow-up also after successful portoenterostomy.

conclusionDR predicted development of PH at and one year after portoenterostomy, associating with PH more consistently than fibrosis.

Indexed as

Biliary AtresiaHypertension, PortalLiver CirrhosisBiopsyChild, PreschoolFemaleFinlandHumansInfantKeratin-7LiverMalePortoenterostomy, HepaticProportional Hazards ModelsKeratin-7biliary atresiacytokeratin‐7ductular reactionportal hypertensionsirius red for collagen

Identifiers

PMID42703703
PMCPMC13548071

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.