Dataset Information

Assessing Native-Liver Post-Kasai Portoenterostomy for biliary atresia through Stool Proteome Analysis

ABSTRACT: In this prospective study, we analyzed stool proteomes from four patients with well-controlled conditions, four patients with recurrent cholangitis, and three patients with prolonged jaundice but no cholangitis, all of whom had undergone Kasai portoenterostomy for biliary atresia (BA). The study also included 15 healthy controls without BA.

ORGANISM(S): Homo Sapiens (human)

SUBMITTER: Eiichiro Watanabe

PROVIDER: PXD055756 | JPOST Repository | Thu Sep 04 00:00:00 BST 2025

REPOSITORIES: jPOST

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Assessing Native Liver Post-Kasai Portoenterostomy for Biliary Atresia Through Stool Proteome Analysis.

Watanabe Eiichiro E Saito Takeshi T Yoshihara Masahito M Konno Ryo R Fujishiro Jun J Takazawa Shinya S Ichinose Akinori A Miyake Kazue K Kakihara Tomo T Ishimaru Tetsuya T Nishi Akira A Fukuda Akinari A Kasahara Mureo M Ohara Osamu O Kawashima Yusuke Y

Gastro hep advances 20250429 8

<h4>Background and aims</h4>Biliary atresia (BA) is a severe neonatal condition, characterized by jaundice and hyperbilirubinemia, resulting in cholestasis. Although early diagnosis followed by Kasai portoenterostomy (KPE) can rescue patients, they are prone to complications such as cholangitis. Moreover, a comprehensive study assessing intestinal environment is currently lacking. Therefore, in this study, we aimed to elucidate the stool protein profiles of patients with BA following KPE, provid ...[more]

PMID: 40585902

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Project description:Biliary atresia (BA) is a rare cholestatic disease of unknown etiology that affects infants and shows an incidence of 1 out of 18,000 live births in Europe (1). The first therapeutic option is a timely performed portoenterostomy. However, the majority of patients suffer from a progressive inflammatory process, which leads to complete destruction of the extra- and intrahepatic biliary system followed by end-stage liver cirrhosis. Hence, BA is the leading indication for pediatric liver transplantation worldwide (2, 3). To understand the pathogenesis of the disease and improve theoutcome of BA patients, research has focused on the inflammatory process in liver and bile ducts, in which several factors are remarkably elevated, such as activated CD4 and CD8 T-cells, TNF alpha,IFN alpha and other proinflammatory TH1 cytokines (3-8). By the time of diagnosis, however, the disease has already reached an advanced state, characterized by the complete obstruction of the extrahepatic bile ducts with impaired bile flow and fibrosis or cirrhosis of the liver. Therefore, studies in humans focusing on the trigger mechanism of BA are limited due to the paucity of liver and availability of bile duct tissue for research. One infectious animal model has been developed, in which newborn Balb/c mice exclusively show the experimental BA phenotype after infection with rhesus rotavirus (RRV) (9, 10). This model allows the analysis of the inflammatory reactions in liver and bile ducts at early steps in the development of bile duct atresia (11-20). Furthermore, inbred mouse strains have been shown to have a different susceptibility for the development of experimental BA, suggesting that Balb/c mice have an immunological gap responsible for disease progression (10, 12). The aim of this study was to identify key genes responsible for the BA phenotype by comparing the transcriptomes at an early time point after virus infection, i.e. before bile duct atresia, between two mouse strains with different susceptibilities to BA. Differences in the virus titration and the clinical course of infected mice were analyzed, and variations in the hepatic gene response assessed by comparative microarray assays were correlated to variances in the hepatic inflammatory reaction. Balb/c mice and C57Black/6 (Black/6) mice were infected with RRV postpartum and signs of BA and survival were noted. Liver sections of diseased, healthy and control animals were assessed for T-cell expression, and the virus loads were determined. Second, mice were sacrificed after three days, and isolated hepatic RNA was subjected to gene expression analysis using Affymetrix Gene Chip MOE 430 2.0.We compared three individual expression profiles from RRV-infected Balb/c mice against 2 individual expression profiles from RRV-infected C57/BL6 control mice using the Affymetrix GeneChip MOE 430 2.0.

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Dataset Information

Assessing Native-Liver Post-Kasai Portoenterostomy for biliary atresia through Stool Proteome Analysis

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Publications

Assessing Native Liver Post-Kasai Portoenterostomy for Biliary Atresia Through Stool Proteome Analysis.

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