Propionic acidemia (PA) is an uncommon inborn metabolic mistake caused by a lack of the enzyme required for catabolism of branched-chain amino acids, certain odd chain fatty acids, and cholesterol. Despite the best medical care, PA frequently results in immediate and gradual brain damage. There have been few and diverse reports on liver transplantation (LT) as a cellular treatment. The goal of this study was to look at the biggest group of patients who had LT for PA. Researchers conducted a multicenter, retrospective analysis of LT over a 16-year period using the Scientific Registry of Transplant Recipients and the Pediatric Health Information System administrative billing databases. During this time, 4849 paediatric LT procedures were performed at 10 different facilities, with 23 of them being for PA. The majority of patients were 5 years old or less at the time of transplant and received status 1b exemption points. PA LT recipients had 84.6 percent graft survival after one, three, and five years, and 89.5 percent patient survival after one, three, and five years. There was no statistically significant difference in graft or patient survival between PA LT patients and non-PA LT recipients. Despite previous research, we found no higher risk of hepatic arterial thrombosis in individuals having LT for PA. Patients in the PA LT group, on the other hand, had a substantially greater postoperative readmission rate when compared to the non-PA LT group.LT is a feasible therapy option for children with PA, with acceptable results.

Reference:https://journals.lww.com/jpgn/Fulltext/2020/02000/Liver_Transplantation_for_Propionic_Acidemia__A.8.aspx

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