Organ transplantation is discussed in methylmalonic aciduria (MMA) for renal failure, and poor quality of life and neurological outcome. We retrospectively evaluated 23 French MMA patients after kidney (KT), liver-kidney (LKT), and liver transplantation (LT). Two patients died, one after LKT, one of hepatoblastoma after KT. One graft was lost early after KT. Of 18 evaluable patients, 12 previously on dialysis, 8 underwent KT (mean 12.5 years), 8 LKT (mean 7 years), and 2 LT (7 and 2.5 years). At a median follow-up of 7.3 (KT), 2.3 (LKT), and 1.0 years (LT), no metabolic decompensation occurred except in 1 KT. Plasma and urine MMA levels dramatically decreased, more after LKT. Protein intake was increased more significantly after LKT than KT. Enteral nutrition was stopped in 7/8 LKT, 1/8 KT. Early complications were frequent after LKT. Neurological disorders occurred in four LKT, reversible in one. Five years after KT, four patients had renal failure. The metabolic outcomes were much better after LKT than KT. LKT in MMA is difficult but improves the quality of life. KT will be rarely indicated. We need more long-term data to indicate early LT, in the hope to delay renal failure and prevent neurodevelopmental complications.

中文翻译：

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肾脏，肝脏或肝肾联合移植术后甲基丙二酸尿症的长期结果：法国经验。

在甲基丙二酸尿症（MMA）中讨论了器官移植的肾功能衰竭，生活质量和神经系统预后不良。我们回顾性评估了23名法国MMA患者的肾脏（KT），肝肾（LKT）和肝移植（LT）后的情况。2例患者死亡，1例在LKT后死亡，1例在KT后肝母细胞瘤死亡。KT早期丢失了一个移植物。在18例可评估的患者中，有12例曾接受透析，其中8例接受了KT（平均12.5年），8例接受LKT（平均7年）和2例LT（7和2.5年）。中位随访时间为7.3（KT），2.3（LKT）和1.0年（LT），除1 KT外，未发生代谢失代偿。LKT后，血浆和尿液中的MMA水平急剧下降。LKT后的蛋白质摄入量比KT显着增加。肠内营养停止在7/8 LKT，1/8 KT中。LKT术后早期并发症多发。四个LKT中发生神经系统疾病，其中一个可逆。KT五年后，四名患者出现肾衰竭。LKT后的代谢结果比KT好得多。MMA中的LKT很难，但可以改善生活质量。KT很少显示。我们需要更多的长期数据来指示早期LT，以期延迟肾衰竭并预防神经发育并发症。