Preoperative dialysis for liver transplantation in methylmalonic acidemia.

Dialysis immediately before liver transplantation for patients with methylmalonic academia (MMA) with the mut0 mutation is considered to be necessary to reduce plasma methylmalonic acid (MMA) levels and prevent metabolic decompensation for a successful surgical outcome; however, this has not yet been conclusively confirmed. Ten pediatric patients underwent living donor liver transplantation at the National Center for Child Health and Development, Tokyo, Japan. Seven patients received dialysis immediately before surgery, but the three most recent patients did not receive dialysis. We monitored plasma MMA levels and evaluated metabolic status during the perioperative period. Plasma MMA levels of patients who received preoperative dialysis were significantly decreased. However, lactic acidosis developed in two patients during surgery. One of the patients who had decreased renal function suffered from severe lactic acidosis after the transplantation and died on post operative day 44. In the three patients who did not receive preoperative dialysis, high plasma MMA levels persisted, but they did not develop metabolic decompensation. Their plasma MMA levels gradually decreased after transplantation. Our results indicated that reducing MMA with preoperative dialysis does not decrease the risk of metabolic decompensation. We will need to evaluate whether preoperative dialysis is necessary for the success of surgery with more cases in the future. Adequate perioperative glucose infusion and careful lactate monitoring are pivotal for success.