Odevixibat treatment in a child with hypoplastic left heart syndrome and severe cholestatic pruritus: a case report.

ABSTRACT: Liver-related abnormalities are commonly observed in patients with congenital heart disease, and these may lead to secondary manifestations such as pruritus. Odevixibat is an ileal bile acid transporter inhibitor under investigation for the treatment of cholestatic liver diseases. Here, we describe the effects of odevixibat treatment in a pediatric patient with congenital heart disease and severe cholestatic pruritus. A 2-year-old male with Kleefstra syndrome, hypoplastic left heart syndrome, and a history of Giessen procedure and biventricular correction surgery presented to the pediatric cardiology and hepatology outpatient clinics at University Children's Hospital Bonn. Portal hypertension was evident on imaging, and the patient was experiencing severe itching attacks that did not respo

SUBMITTER: Ganschow R 

PROVIDER: S-EPMC11799544 | biostudies-literature | 2024

REPOSITORIES: biostudies-literature