Current role of liver transplantation for methylmalonic acidemia: a review of the literature.
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.
Kasahara
Mureo
M
Department of Transplant Surgery, National Center for Child Health and Development, Tokyo, Japan. kasahara-m@ncchd.go.jp
Horikawa
Reiko
R
Tagawa
Manabu
M
Uemoto
Shinji
S
Yokoyama
Satoshi
S
Shibata
Yumiko
Y
Kawano
Takafumi
T
Kuroda
Tatsuo
T
Honna
Toshiro
T
Tanaka
Koichi
K
Saeki
Morihiro
M
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
D008764:Methylmalonic Acid; D008765:Methylmalonyl-CoA Mutase