Perioperative management of living‐donor liver transplantation for methylmalonic acidemia

Paediatric Anaesthesia - Tập 26 Số 7 - Trang 694-702 - 2016
Chiaki Baba1, Mureo Kasahara2, Yasuhiro Kogure1, Shugo Kasuya1, Sukeyuki Ito1, Takako Tamura1, Akinari Fukuda2, Reiko Horikawa3, Yasuyuki Suzuki1
1Department of Anesthesia and Intensive Care, National Center for Child Health and Development, Tokyo, Japan
2Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan
3Department of Endocrinology and Metabolism, National Center for Child Health and Development, Tokyo, Japan

Tóm tắt

SummaryMethymalonic acidemia (MMA) is a hereditary metabolic disorder characterized by a defect of the methylmalonyl‐CoA mutase that breaks down propionate. The efficacy of liver transplantation for MMA was recently reported. However, the anesthetic management of liver transplant for MMA is not clear. The aim of this article is to describe an anesthetic management algorithm of liver transplant for MMA by reviewing our cases of liver transplant for MMA. Fourteen patients received a liver transplant; three cases showed metabolic decompensation during the transplant and two of the patients died. In the two patients who expired, propofol was used for maintenance anesthesia and preoperative continuous hemodiafiltration was used to reduce plasma methylmalonic acid level in one case, and to control severe metabolic decompensation before transplant for the other case. Their renal function was also worse than others and they were already experiencing metabolic decompensation before induction of anesthesia. Based on our experience of these 14 cases, we have established an anesthetic algorithm for patients with MMA undergoing liver transplant or other procedures. There are three important points in our experience: propofol should be avoided, dextrose infusion therapy should be continued to prevent metabolic decompensation, and liver transplant or other procedures should be avoided during metabolic decompensation.

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Thông tin xuất bản

Paediatric Anaesthesia

Tập 26 Số 7

694-702

Thông tin tác giả