case_images: PMC11799544_01
case_images: Flow diagram of key clinical milestones for patient with HLHS and severe cholestatic pruritus treated with odevixibat. ALT, alanine aminotransferase; GGT, gamma-glutamyl transferase; HLHS, hypoplastic left heart syndrome; QoL, quality of life.
case_images: fped-12-1443338-g001.jpg
case_images: PMC11799544_01_fped-12-1443338-g001.jpg
case_images: F1
case_images: Overall, the patient's mother reported his quality of life was greatly improved; he was happy, laughing, and better able to focus on learning and playing (Figure 1).
case_images: Changes in serum bile acids, bilirubin, ALT, and GGT with odevixibat treatment. All available laboratory values on a per-month basis are plotted. In May 2023, three laboratory assessments were taken within 4 days of each other; the last available value is plotted for ALT and GGT; for total bilirubin, all 3 May 2023 laboratory assessments are plotted. The bilirubin panel shows total bilirubin values with the solid line and direct bilirubin values with the dashed line; values for total bilirubin are labeled. ALT, alanine aminotransferase; GGT, gamma-glutamyl transferase.
case_images: fped-12-1443338-g002.jpg
case_images: PMC11799544_01_fped-12-1443338-g002.jpg
case_images: F2
case_images: Serum bile acids decreased from a pretreatment level of 111 mumol/L to 24 mumol/L within 1 month of initiating odevixibat; this reduction in serum bile acids was maintained at the last available assessment in April 2023 (36 mumol/L) (Figure 2).