Abstract
Methylmalonic acidemia (MMA) and propionic acidemia (PA) are inborn errors of metabolism, specifically types of organic acidemias. Individuals with MMA and PA are unable to catabolize branched-chain amino acids because of specific enzymatic defects. Nutritionally, patients are managed by intact protein-restricted diets. Despite strict medical and nutritional management, metabolic instability may continue. Liver or liver and kidney transplantation may be a treatment option. Transplant may result in improvement of laboratory values, increased oral intake, and developmental gains. The aim of medical nutrition therapy for MMA/PA-transplanted patients is promotion of metabolic stability peritransplant.