title: Current role of liver transplantation for methylmalonic acidemia: a review of the literature.
abstract: To evaluate the current role of liver transplantation (LT) for methylmalonic acidemia (MMA), we reviewed the literature on outcomes of this treatment, and describe three of our own cases of living-donor liver transplantation (LDLT). The total number of LT cases identified was 18. Transplantation mode was deceased donor LT in 12, including five combined liver-kidney transplantations (CLKT) from deceased donors, and LDLT in six. Three hospital mortalities were noted, because of metabolic decompensation, sepsis and aspergillosis. Although mean postoperative serum MMA level decreased to 13.8% +/- 9.2% (range 1.25-26.1%) of preoperative levels, four patients (22.2%) had renal insufficiency after isolated LT and three (16.7%) had postoperative neurological disability. Continuing metabolic damage to the kidney and brain may occur even after successful LT. Further evaluation is required to determine the long-term suitability of this treatment modality.
authors.affiliation: Department of Transplant Surgery, National Center for Child Health and Development, Tokyo, Japan. kasahara-m@ncchd.go.jp
authors.forename: Mureo
authors.initials: M
authors.lastname: Kasahara
authors.forename: Reiko
authors.initials: R
authors.lastname: Horikawa
authors.forename: Manabu
authors.initials: M
authors.lastname: Tagawa
authors.forename: Shinji
authors.initials: S
authors.lastname: Uemoto
authors.forename: Satoshi
authors.initials: S
authors.lastname: Yokoyama
authors.forename: Yumiko
authors.initials: Y
authors.lastname: Shibata
authors.forename: Takafumi
authors.initials: T
authors.lastname: Kawano
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Kuroda
authors.forename: Toshiro
authors.initials: T
authors.lastname: Honna
authors.forename: Koichi
authors.initials: K
authors.lastname: Tanaka
authors.forename: Morihiro
authors.initials: M
authors.lastname: Saeki
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D008661:Metabolism, Inborn Errors / Q000201:enzymology / Q000601:surgery*; D008764:Methylmalonic Acid / Q000097:blood*; D008765:Methylmalonyl-CoA Mutase; D016896:Treatment Outcome
chemical_list: D008764:Methylmalonic Acid; D008765:Methylmalonyl-CoA Mutase