TY - JOUR
T1 - Current role of liver transplantation for methylmalonic acidemia
T2 - A review of the literature
AU - Kasahara, Mureo
AU - Horikawa, Reiko
AU - Tagawa, Manabu
AU - Uemoto, Shinji
AU - Yokoyama, Satoshi
AU - Shibata, Yumiko
AU - Kawano, Takafumi
AU - Kuroda, Tatsuo
AU - Honna, Toshiro
AU - Tanaka, Koichi
AU - Saeki, Morihiro
PY - 2006/12
Y1 - 2006/12
N2 - 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.
AB - 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.
KW - Liver transplantation
KW - Living donor
KW - Metabolic
KW - Organic acidemia
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U2 - 10.1111/j.1399-3046.2006.00585.x
DO - 10.1111/j.1399-3046.2006.00585.x
M3 - Article
C2 - 17096763
AN - SCOPUS:33750600921
VL - 10
SP - 943
EP - 947
JO - Pediatric Transplantation
JF - Pediatric Transplantation
SN - 1397-3142
IS - 8
ER -