Preoperative Dialysis for Liver Transplantation in Methylmalonic Acidemia

Therapeutic Apheresis and Dialysis - Tập 15 Số 5 - Trang 488-492 - 2011
Koichi Kamei1, Shuichi Ito1, Takanobu Shigeta2, Seisuke Sakamoto2, Akinari Fukuda2, Reiko Horikawa3, Osamu Saitô4, Takashi Muguruma4, Satoshi Nakagawa4, Kazumoto Iijima5, Mureo Kasahara2
1Departments of Nephrology and Rheumatology
2Transplant Surgery
3Endocrinology and Metabolism
4Pediatric Intensive Care Unit, National Center for Child Health and Development, Tokyo
5Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan

Tóm tắt

AbstractDialysis 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.

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Thông tin
Thông tin xuất bản

Therapeutic Apheresis and Dialysis

Tập 15 Số 5

488-492

Thông tin tác giả