Changes in plasma bile acid profiles after partial internal biliary diversion in PFIC2 patients.

ABSTRACT:

Background

We ask if plasma bile acid profiles can be used to monitor the effectiveness of partial internal biliary diversion (PIBD) for treating uncontrolled cholestasis in progressive familial intrahepatic cholestasis type 2 (PFIC2) patients.

Methods

Plasma bile acids were profiled in 3 cases of ATP-binding cassette, sub-family B member 11 (ABCB11)-mutated PFIC2 children before and after PIBD compared to healthy controls and 8 PFIC2 patients. The quantitation of bile acids was performed by reversed-phase ultrahigh-performance liquid chromatography/multiple-reaction monitoring-mass spectrometry (UPLC/MRM-MS) with negative ion detection.

Results

Before PIBD, all three patients presented with >50-fold higher levels of total plasma bile acids, 2-7 folds hi

SUBMITTER: Liu T 

PROVIDER: S-EPMC7154393 | biostudies-literature | 2020 Mar

REPOSITORIES: biostudies-literature