biostudies-literatureShneider BLNCATS NIH HHSNIDDK NIH HHSNIEHS NIH HHSNIMHD NIH HHSNCI NIH HHSe0796https://www.ebi.ac.uk/biostudies/studies/S-EPMC12483069biostudies-literatureUnknown9(10)<h4>Background</h4>Pediatric cholestatic liver diseases can be characterized by rapidly progressive fibrosis. A multicenter cross-sectional analysis of vibration-controlled elastography in biliary atresia (BA), alpha-1 antitrypsin deficiency (A1AT), and Alagille syndrome (ALGS) was leveraged to interrogate the plasma proteome relative to liver stiffness measurements (LSM).<h4>Methods</h4>Slow off-rate modified aptamer scanning profiling of >7000 proteins in plasma from 187 children with BA (n=93), A1AT (n=31), ALGS (n=46), and healthy pediatric controls (n=17) was performed, and correlations with LSM were undertaken.<h4>Results</h4>There was an abundance of LSM correlated proteins (BA n=2720, A1AT n=694, ALGS n=5968). Interestingly, a distinct plasma proteome was found in ALGS relative to Hepatology communicationsPlasma proteome correlations with liver stiffness in pediatric cholestasis implicate epithelial to mesenchymal transition.PMC12483069U01 DK062497U01 DK062453U01 DK062470U01 DK062481U01 DK103149U24 DK062456P30 ES030285U01 DK062436U01 DK084575U01 DK084538U01 DK062456U01 DK062500UM1 TR004399P30 CA125123U01 DK084536U01 DK062466P50 MD015496P42 ES027725U01 DK103140K08 CA245220Emamaullee JGrimm SLHsu EHorslen SPWang KSCoarfa CShneider BLKriegmeier AChildhood Liver Disease Research NetworkBezerra JASchraw JLupo PJKanchi RSMagee JCBarbetta ASokol RJMolleston JPLoomes KMRosenthal PKohli RJensen MKDevaraj SKarpen SJfalsePlasma proteome correlations with liver stiffness in pediatric cholestasis implicate epithelial to mesenchymal transition.<h4>Background</h4>Pediatric cholestatic liver diseases can be characterized by rapidly progressive fibrosis. A multicenter cross-sectional analysis of vibration-controlled elastography in biliary atresia (BA), alpha-1 antitrypsin deficiency (A1AT), and Alagille syndrome (ALGS) was leveraged to interrogate the plasma proteome relative to liver stiffness measurements (LSM).<h4>Methods</h4>Slow off-rate modified aptamer scanning profiling of >7000 proteins in plasma from 187 children with BA (n=93), A1AT (n=31), ALGS (n=46), and healthy pediatric controls (n=17) was performed, and correlations with LSM were undertaken.<h4>Results</h4>There was an abundance of LSM correlated proteins (BA n=2720, A1AT n=694, ALGS n=5968). Interestingly, a distinct plasma proteome was found in ALGS relative to 2025-01-01T00:00:00Z2025 Oct2026-06-15T06:09:19.173Z2026-06-15T03:08:35.509ZS-EPMC124830694102127710.1097/HC9.0000000000000796