title: Perioperative management of living-donor liver transplantation for methylmalonic acidemia.
abstract: Methymalonic 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.
authors.affiliation: Department of Anesthesia and Intensive care, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Chiaki
authors.initials: C
authors.lastname: Baba
authors.affiliation: Organ transplantation Center, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Mureo
authors.initials: M
authors.lastname: Kasahara
authors.affiliation: Department of Anesthesia and Intensive care, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Kogure
authors.affiliation: Department of Anesthesia and Intensive care, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Shugo
authors.initials: S
authors.lastname: Kasuya
authors.affiliation: Department of Anesthesia and Intensive care, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Sukeyuki
authors.initials: S
authors.lastname: Ito
authors.affiliation: Department of Anesthesia and Intensive care, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Takako
authors.initials: T
authors.lastname: Tamura
authors.affiliation: Organ transplantation Center, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Akinari
authors.initials: A
authors.lastname: Fukuda
authors.affiliation: Department of Endocrinology and Metabolism, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Reiko
authors.initials: R
authors.lastname: Horikawa
authors.affiliation: Department of Anesthesia and Intensive care, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Suzuki
mesh_terms: D000592:Amino Acid Metabolism, Inborn Errors / Q000601:surgery*; D000758:Anesthesia / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D008297:Male; D019990:Perioperative Care / Q000379:methods*
keywords: anesthetic management; liver transplantation; methymalonic acidemia