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Fig. 2 | Orphanet Journal of Rare Diseases

Fig. 2

From: Cholbam® and Zellweger spectrum disorders: treatment implementation and management

Fig. 2

Klouwer et al. [20]: impact of cholic acid therapy on bile acid levels, and liver chemistry. A, B Tukey box plots showing the effect of oral cholic acid (CA) on plasma 3α,7α- dihydroxycholestanoic acid (DHCA) and 3α,7α,12α-trihydroxycholestanoic acid (THCA) after 1, 3, 9, 15 and 21 months of treatment. The control reference range for THCA is < 0.05–0.1 μmol/L and levels of DHCA are undetectable (< 0.05 μmol/L) in control individuals. C, D Tukey box plots showing the levels of plasma DHCA and THCA at baseline, study end (after 9 or 21 months of CA treatment) and at follow-up (6–12 months after discontinuation of CA). Only the levels of patients for which follow-up values were available are shown (n = 16). E, F Graphs showing the individual courses of alanine transaminase (ALT) and aspartate transaminase (AST) levels during oral CA treatment (n = 22). The patients with liver cirrhosis are depicted in red. The upper control reference ranges of ALT (40 U/L) and AST (45 U/L) are indicated by the dotted lines. *P<0.05, **P<0.01, ***P<0.005, ****P<0.001, ns, not significant. Reprinted from Klouwer et al. [20]; with permission from John Wiley and Sons. This is an open access article distributed under the terms of the Creative Commons CC BY license, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited

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