Effects of dichloroaeetate in three patients with MELAS

Effects of dichloroaeetate in three patients with MELAS
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二氯乙酸对三名 MELAS 患者的影响

DOI:
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发表时间:
1998
期刊:
影响因子:
9.9
通讯作者:
M. Imai
M. Imai
中科院分区:
医学1区
文献类型:
--
作者:
S. Saitoh;M. Momoi;T. Yamagata;Y. Mori;M. Imai

文献摘要

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我们介绍了二氯醋酸盐(DCA)对三名患有线粒体肌病、脑病、乳酸酸中毒和卒中样发作(MELAS)的儿童的临床和实验室影响,这些儿童对其他药物无反应。DCA的施用降低了血清和CSF中升高的乳酸和丙酮酸水平。在研究期间,DCA改善了三名患者的腹痛、头痛和中风样发作,并改善了认知功能和疲劳度。观察到一些一过性肝功能障碍、低钙血症和周围神经病变。DCA在MELAS中的应用值得进一步研究。
We present the clinical and laboratory effects of dichloroacetate (DCA) in three children with mitochondrial myopathy, encephalopa-thy, lactic acidosis, and strokelike episodes (MELAS) who had not responded to other medications. Administration of DCA lowered the elevated levels of lactate and pyruvate in the serum and CSF. DCA ameliorated abdominal pain, headache, and strokelike episodes, and improved cognitive function and fatigability in the three patients during the study period. Some transient liver dysfunction, hypocalcemia, and peripheral neuropathy were observed. The use of DCA in MELAS merits further study.