Kasahara Mureo, Horikawa Reiko, Tagawa Manabu, Uemoto Shinji, Yokoyama Satoshi, Shibata Yumiko, Kawano Takafumi, Kuroda Tatsuo, Honna Toshiro, Tanaka Koichi, Saeki Morihiro
Department of Transplant Surgery, National Center for Child Health and Development, Tokyo, Japan.
Pediatr Transplant. 2006 Dec;10(8):943-7. doi: 10.1111/j.1399-3046.2006.00585.x.
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.
为评估肝移植（LT）治疗甲基丙二酸血症（MMA）的当前作用，我们回顾了有关该治疗结局的文献，并描述了我们自己的3例活体肝移植（LDLT）病例。确定的LT病例总数为18例。移植方式为尸体供肝LT 12例，其中包括5例尸体供肝肝肾联合移植（CLKT），活体肝移植6例。记录到3例医院死亡病例，原因分别为代谢失代偿、败血症和曲霉病。尽管术后血清MMA平均水平降至术前水平的13.8%±9.2%（范围1.25 - 26.1%），但4例（22.2%）单纯LT术后出现肾功能不全，3例（16.7%）术后出现神经功能障碍。即使LT成功，肾脏和大脑仍可能持续发生代谢损伤。需要进一步评估以确定这种治疗方式的长期适用性。
