Fig. 2
Negative ion FAB-MS spectra of the urine from the proband and her brother. a The mass spectrum of first urine sample collected from the proband after cessation of oral ursodeoxycholic acid; b the mass spectrum of the second urine sample was collected from the proband after chenodeoxycholic acid treatment for nearly 4 months; c the mass spectrum of the urine collected from the brother of the proband. Indicated are the specific ions that characterize the presence of elevated levels of unsaturated 3β-hydroxy-Δ5 bile acids associated with CYP7B1 deficiency