Keywords

kidney transplant, liver transplant, medical nutrition therapy, methylmalonic acidemia (MMA), propionic acidemia (PA)

 

Authors

  1. Reineking, Bridget A. MS, RD, CD
  2. Maraglino, Antoinette C. MS, RD, LDN
  3. Ueda, Keiko MPH, RD, LDN

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.