Rhabdomyolysis in organic acidemia patients manifesting with metabolic decompensation.
Rhabdomyolysis in organic acidemia patients manifesting with metabolic decompensation.
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有机酸血症患者的横纹肌溶解症表现为代谢失代偿。
DOI：
10.1111/hdi.12778
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发表时间：
2019
期刊：
影响因子：
--
通讯作者：
Nakamura K.
中科院分区：
文献类型：
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作者：
Kido J;Matsumoto S;Sawada T;Endo F;Nakamura K.
一些代谢紊乱与横纹肌溶解有关，但它们与甲基丙二酸血症(MMA)和丙酸血症(PA)的关系尚不清楚。2009年1月至2018年12月期间，熊本大学医院对11例MMA和4例PA患者进行了治疗和/或随访。3名MMA患者和1名PA患者在代谢危象发生后1-2 出现横纹肌溶解。例1和4在停用连续性血液透析滤过(CHDF)后出现横纹肌溶解，例2和3在起病时出现横纹肌溶解，停用CHDF后恢复期反复出现横纹肌溶解。线粒体功能障碍与横纹肌溶解有关。MMA和PA患者的横纹肌溶解可能归因于继发性线粒体紊乱导致的能量产生缺陷。因此，医生应密切关注MMA和PA患者，特别是在停止血液透析治疗后，并为他们的线粒体功能提供支持性护理。
Several metabolic disorders are related to rhabdomyolysis, but their association with methylmalonic acidemia (MMA) and propionic acidemia (PA) is unclear. Eleven patients with MMA and four patients with PA were treated and/or followed up in Kumamoto University Hospital between January 2009 and December 2018. Three patients with MMA and one patient with PA developed rhabdomyolysis at 1–2 weeks after onset of metabolic crisis. Cases 1 and 4 initially developed rhabdomyolysis after withdrawal from continuous hemodiafiltration (CHDF), and cases 2 and 3 developed rhabdomyolysis at the time of onset and had recurrent rhabdomyolysis during the recovery phase after withdrawal from CHDF. Mitochondrial dysfunction is associated with rhabdomyolysis. The rhabdomyolysis in patients with MMA and PA may have been attributed to a defect in energy production because of a secondary mitochondrial disorder. Therefore, physicians should closely follow patients with MMA and PA, especially after withdrawal of hemodialysis therapy, and provide supportive care for their mitochondrial function.