Liver transplantation for propionic acidemia: Survival and metabolic outcomes
Abstract
BACKGROUND: Propionic acidemia (PA) is a severe metabolic disorder that leads to multiorgan damage despite comprehensive management. Liver transplantation (LT), particularly living donor liver transplantation, has been proposed as an effective treatment, but evidence from large-scale studies is limited. METHODS: This retrospective study analyzed clinical outcomes of 39 children with PA who underwent LT at Shanghai Ren Ji Hospital between September 2017 and October 2023. The data included demographics, surgical details, biochemical/metabolic markers, and progression of symptoms. Patients were grouped based on the Diagnosis-to-Transplant Interval (
Fields of Study & Subjects
Metabolism and Genetic Disorders
Liver Disease and Transplantation
Renal function and acid-base balance
Publication details
- Journal / source
- Hepatology Communications
- Publisher
- Ovid Technologies (Wolters Kluwer Health)
- Volume
- 10
- Issue
- 2
- Publication date
- 9 January 2026
- Language
- EN