Liver Transplantation for Propionic Acidemia in Children

The retrospective review from 1987 to 2008 included five children with propionic acidemia (PA) who underwent liver transplantation (LT) at a median age of 1.5 years. The study found that after a median follow-up of 7.3 years, all children had normal graft function and an improved quality of life, with unrestricted protein diets and no further metabolic decompensations. One child required retransplantation due to hepatic artery thrombosis, and complications included acute cellular rejection, hyperammonemia, worsening renal function in one patient, and posttransplant lymphoproliferative disorder in two patients. Despite these issues, the study concluded that LT is a viable treatment option for PA, reducing the risk of metabolic decompensation and improving quality of life, although it does not eliminate the risk of metabolic sequelae and the long-term neurological outcomes remain uncertain.