CONCLUSIONS: MMA accumulation in MA patients causes acute and life-threatening complications, such as metabolic decompensations, and long-term complications such as CKD, eventually leading to renal replacement therapy (RRT). Data reported in the literature show that, overall, all dialytic treatments (intermittent HD, continuous HD, peritoneal dialysis) are effective in MMA removal. HD, in particular, can be useful in the emergency setting to control metabolic crises, even with GFR > 15 mL/min. Kidney and/or liver transplantations are often needed in MA patients. While a solitary transplanted kidney can be rapidly affected by MMA exposure, with a decline in renal function even in the first year of follow-up, the combined liver-kidney transplantation showed better long-term results due to a combination of reduced MMA production along with increased urinary excretion.
CONCLUSIONS: Early diagnosis, multidisciplinary management and preventive measures are pivotal in MA patients to avoid recurrent AKI episodes and, consequently, to slow down CKD progression.
结论:MA患者的MMA积累会导致急性和危及生命的并发症,比如代谢失代偿,和长期并发症,如CKD,最终导致肾脏替代疗法(RRT)。文献报道的数据表明,总的来说,所有透析治疗(间歇性HD,连续HD,腹膜透析)可有效去除MMA。HD,特别是,在紧急情况下可以控制代谢危机,即使GFR>15mL/min。MA患者通常需要进行肾脏和/或肝脏移植。虽然单独移植的肾脏可以迅速受到MMA暴露的影响,即使在随访的第一年,肾功能也会下降,由于MMA产生减少和尿排泄增加,肝肾联合移植显示出更好的长期结果.
结论:早期诊断,多学科管理和预防措施对于MA患者避免复发性AKI发作至关重要,因此,减缓CKD进展。