Here we report a simple, sensitive, and accurate method for detecting urinary sulfated tauro- and glyco-bile acids that uses electrospray ionization mass spectrometry. The sulfated tauro- and glycodihydroxycholic acids mainly generated [M-2H](2-) negative ions at m/z 288.6 and m/z 263.6, respectively. These doubly charged ions appeared primarily in samples prepared from the urine of patients with cholestasis and were detected quantitatively. Cholestatic jaundice is the primary clinical sign of biliary atresia. The measurement of doubly charged negative ions, especially of sulfated taurodihydroxycholic acid (principally taurochenodeoxycholate-3-sulfate), is a useful screening modality for biliary atresia in neonates.
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http://dx.doi.org/10.1016/j.jchromb.2007.04.010 | DOI Listing |
JHEP Rep
August 2024
Department of Molecular and Cellular Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Int J Mol Sci
March 2024
Yuelushan Laboratory, Changsha 410128, China.
Dietary methionine restriction (MetR) offers an integrated set of beneficial health effects, including delaying aging, extending health span, preventing fat accumulation, and reducing oxidative stress. This study aimed to investigate whether MetR exerts entero-protective effects by modulating intestinal flora, and the effect of MetR on plasma metabolites in rats. Rats were fed diets containing 0.
View Article and Find Full Text PDFJ Clin Transl Hepatol
February 2024
Department of Pediatrics, Shanghai Medical College, Fudan University, The Center for Pediatric Liver Diseases, Children's Hospital of Fudan University, Shanghai, China.
Background And Aims: We asked if comprehensive bile acid profiling could provide insights into the physiopathology of -mutated patients and evaluated the prognostic value of taurine-conjugated tetrahydroxylated bile acid (tauro-THBA) in cholestasis.
Methods: Serum bile acid profiles were evaluated in 13 -mutated patients with 65 healthy controls by ultra-high-performance liquid chromatography/multiple-reaction monitoring-mass spectrometry (UPLC/MRM-MS). The concentration of tauro-THBA was compared between -mutated patients with different prognoses.
Sci Rep
January 2024
Department of Pediatric Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan.
Bile acids have received increasing attention as a marker of the long-term prognosis and a potential therapeutic target in patients with biliary atresia, which is a progressive disease of the hepatobiliary system. A detailed analysis of serum and urinary bile acid compositions was conducted to assess the characteristics of bile acid profiles and the correlation between bile acid profiles and liver fibrosis markers in adult patients with biliary atresia who achieved bilirubin normalization. Serum total bile acids and glucuronide-conjugated (glyco- and tauro-) cholic acids (GCA and TCA) and chenodeoxycholic acids (GCDCA and TCDCA) were significantly higher in patients with biliary atresia than in healthy controls, whereas unconjugated CA and CDCA showed no significant difference.
View Article and Find Full Text PDFMol Genet Metab
November 2023
Department of Pediatrics, Juntendo University Graduate School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan.
Objective: To examine whether it is possible to screen for bile acid synthesis disorders (BASDs) including peroxisome biogenesis disorder 1a (PBD1A) and Niemann-Pick type C1 (NPC1) at the time of newborn mass screening by measuring the intermediary metabolites of bile acid (BA) synthesis.
Methods: Patients with 3β-hydroxy-ΔSuchy et al. (2021)-C-steroid dehydrogenase/isomerase (HSD3B7) deficiency (n = 2), 3-oxo-ΔPandak and Kakiyama (n.
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