Distinct Plasma Bile Acid Profiles of Biliary Atresia and Neonatal Hepatitis Syndrome

• DOI: 10.1021/acs.jproteome.5b00676
• Publication Date: 2015-10-09T10:55:24Z
• Authors: Kejun Zhou; Jun Wang; Guoxiang Xie; Ying Zhou; Weihui Yan; Weihua Pan; Yanran Che; Ting Zhang; Linda Wong; Sandi Kwee; Yongtao Xiao; Jie Wen; Wei Cai; Wei Jia
• Keywords: Chenodeoxycholic acid; Farnesoid X receptor; Neonatal cholestasis; Neonatal hepatitis; Hepatoblastoma; Ursodeoxycholic acid

Biliary atresia (BA) is a severe chronic cholestasis disorder of infants that leads to death if not treated on time. Neonatal hepatitis syndrome (NHS) another leading cause neonatal confounding the diagnosis BA. Recent studies indicate altered bile acid metabolism closely associated with liver injury and cholestasis. In this study, we systematically measured metabolome in plasma BA, NHS, healthy controls. Liver acids were also using biopsy samples from 48 BA 16 NHS undergoing operative cholangiography as well 5 normal adjacent nontumor tissues taken hepatoblastoma patients Both had significantly elevated levels compared showed distinct profile characterized by higher taurochenodeoxycholic (TCDCA) level lower chenodeoxycholic (CDCA) than those patients. The ratio TCDCA CDCA was (p < 0.001) or 0.001). area under receiver operating characteristic curve for TCDCA/CDCA differentiate 0.923 (95% CI: 0.862-0.984). These findings supported expression transporters nuclear receptors including farnesoid X receptor (FXR), small heterodimer partner (SHP), salt export pump (BSEP), multidrug resistant protein 3 (MDR3) NHS. Taken together, profiles are infants, differentially distributed among three groups infants.