Survival of propionic acidemia patients with liver transplant

Abstract

Propionic acidemia (PA) is a rare metabolic disorder affecting amino acid metabolism. Liver transplantation improves some outcomes, but the impact on long-term survival remains unclear. A systematic literature review and survival analysis, identifying 94 PA patients who underwent transplantation, revealed a survival probability of 62% at age 33; while median survival was estimated at 40 years. These findings highlight a substantial survival deficit of PA patients compared to the general population despite liver transplantation.

Keywords

• Propionic acidemia
• Liver transplantation
• Survival
• Life expectancy
• Systematic literature review
• Modeling