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Review
. 2006 Dec;10(8):943-7.
doi: 10.1111/j.1399-3046.2006.00585.x.

Current role of liver transplantation for methylmalonic acidemia: a review of the literature

Mureo Kasahara  1 , Reiko Horikawa, Manabu Tagawa, Shinji Uemoto, Satoshi Yokoyama, Yumiko Shibata, Takafumi Kawano, Tatsuo Kuroda, Toshiro Honna, Koichi Tanaka, Morihiro Saeki
Affiliations
Review

Current role of liver transplantation for methylmalonic acidemia: a review of the literature

Mureo Kasahara et al. Pediatr Transplant. 2006 Dec.

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.

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