Leigh 亚急性坏死性脑病：临床与生化研究，特别提及硫辛酸治疗 - Suppr

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Leigh's subacute necrotizing encephalopathy: clinical and biochemical study, with special reference to therapy with lipoate.

作者信息

Clayton B E, Dobbs R H, Patrick A D

出版信息

Arch Dis Child. 1967 Oct;42(225):467-78. doi: 10.1136/adc.42.225.467.

DOI:10.1136/adc.42.225.467

PMID:4862967

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2019798/

Abstract

摘要

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e8ef/2019798/08bbc168aa97/archdisch01566-0014-a.jpg

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Leigh's subacute necrotizing encephalopathy: clinical and biochemical study, with special reference to therapy with lipoate.Leigh 亚急性坏死性脑病：临床与生化研究，特别提及硫辛酸治疗

Arch Dis Child. 1967 Oct;42(225):467-78. doi: 10.1136/adc.42.225.467.

Subacute necrotizing encephalomyelopathy (Leigh's disease): a consideration of clinical features and etiology.亚急性坏死性脑脊髓病（ Leigh 病）：临床特征与病因学探讨

Dev Med Child Neurol. 1972 Feb;14(1):87-101. doi: 10.1111/j.1469-8749.1972.tb02563.x.

Biochemical studies and therapy in subacute necrotizing encephalomyelopathy (Leigh's syndrome).亚急性坏死性脑脊髓病（ Leigh 综合征）的生化研究与治疗

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Hyperalaninemia hyperpyruvicemia and lactic acidosis due to pyruvate carboxylase deficiency of the liver; treatment with thiamine and lipoic acid.由于肝脏丙酮酸羧化酶缺乏导致的高丙氨酸血症、高丙酮酸血症和乳酸酸中毒；硫胺素和硫辛酸治疗。

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Community care study. Leigh's disease.

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[Subacute necrotizing encephalopathy, Leigh's disease. Apropos of a case].[亚急性坏死性脑病， Leigh 病。附 1 例报告]

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Leigh's necrotizing encephalopathy with pyruvate carboxylase deficiency.伴有丙酮酸羧化酶缺乏的 Leigh 坏死性脑病

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CT scan appearances in Leigh's disease (subacute necrotizing encephalomyelopathy).

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引用本文的文献

Alpha-lipoic acid in the treatment of psychiatric and neurological disorders: a systematic review.硫辛酸治疗精神和神经疾病的系统评价。

Metab Brain Dis. 2019 Feb;34(1):39-52. doi: 10.1007/s11011-018-0344-x. Epub 2018 Nov 22.

Lipoic (thioctic) acid increases brain energy availability and skeletal muscle performance as shown by in vivo 31P-MRS in a patient with mitochondrial cytopathy.硫辛酸可提高脑能量供应及骨骼肌功能，线粒体细胞病患者的活体31P磁共振波谱分析结果显示了这一点。

J Neurol. 1995 Jul;242(7):472-7. doi: 10.1007/BF00873552.

Erythrocyte transketolase activity in suspected cases of Leigh's disease, or subacute necrotising encephalomyelopathy.疑似 Leigh 病或亚急性坏死性脑脊髓病病例中的红细胞转酮醇酶活性。

Arch Dis Child. 1980 Oct;55(10):789-94. doi: 10.1136/adc.55.10.789.

Encephalomyelopathy of Leigh.Leigh 脑脊髓病

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[Familial leukoencephalopathy associated with congenital lactic acidosis].

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Alaninuria, associated with microcephaly, dwarfism, enamel hypoplasia, and diabetes mellitus in two sisters.

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An inherited defect affecting the tricarboxylic acid cycle in a patient with congenital lactic acidosis.一名患有先天性乳酸性酸中毒的患者存在影响三羧酸循环的遗传性缺陷。

J Clin Invest. 1972 Jul;51(7):1845-51. doi: 10.1172/JCI106986.

Necrotizing encephalomyelopathy of Leigh. Neuropathological findings in 8 cases.Leigh 坏死性脑脊髓病。8例神经病理学发现。

Arch Dis Child. 1970 Feb;45(239):39-48. doi: 10.1136/adc.45.239.39.

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Arch Dis Child. 1968 Aug;43(230):423-6. doi: 10.1136/adc.43.230.423.

Chronic lactic acidosis in association with myopathy.伴有肌病的慢性乳酸酸中毒

Arch Dis Child. 1973 Jun;48(6):489-92. doi: 10.1136/adc.48.6.489.

本文引用的文献

Enzymatic synthesis of citric acid. II. Crystalline condensing enzyme.柠檬酸的酶促合成。II. 结晶缩合酶。

J Biol Chem. 1951 Dec;193(2):691-702.

Subacute necrotizing encephalomyelopathy in an infant.一名婴儿的亚急性坏死性脑脊髓病

J Neurol Neurosurg Psychiatry. 1951 Aug;14(3):216-21. doi: 10.1136/jnnp.14.3.216.

Studies on experimental thiamine deficiency. Trends of keto acid formtion and detection of glyoxylic acid.实验性硫胺素缺乏症的研究。酮酸形成趋势及乙醛酸的检测。

Biochem J. 1962 Mar;82(3):429-34. doi: 10.1042/bj0820429.

SUBACUTE NECROTIZING ENCEPHALOMYELOPATHY. CASE REPORT WITH SPECIAL EMPHASIS ON ASSOCIATED PATHOLOGY OF PERIPHERAL NERVOUS SYSTEM.亚急性坏死性脑脊髓病。病例报告，特别强调周围神经系统的相关病理学表现。

Arch Neurol. 1965 Jan;12:98-107. doi: 10.1001/archneur.1965.00460250102013.

ERYTHROCYTE AS A BIOPSY TISSUE FOR FUNCTIONAL EVALUATION OF THIAMINE ADEQUACY.红细胞作为用于硫胺素充足性功能评估的活检组织。

JAMA. 1964 Mar 7;187:762-6. doi: 10.1001/jama.1964.03060230090022.

THE GLYOXYLIC ACID CONTENT OF HUMAN BLOOD AND ITS RELATIONSHIP TO THIAMINE DEFICIENCY.人体血液中的乙醛酸含量及其与硫胺素缺乏的关系。

Clin Sci. 1963 Oct;25:207-17.

Infantile chronic necrotizing encephalopathy.

Acta Paediatr (Stockh). 1963 May;52:304-12. doi: 10.1111/j.1651-2227.1963.tb03783.x.

Effect of lipoic acid on the normal rat liver.

J Lab Clin Med. 1962 Oct;60:649-54.

Control of the citric acid cycle by glyoxylate. 2. Mechanism of the inhibition of respiration in liver and kidney particles.乙醛酸对柠檬酸循环的调控。2. 肝脏和肾脏微粒体呼吸抑制机制。

Biochem J. 1962 Dec;85(3):593-600. doi: 10.1042/bj0850593.

Glycolysis in young and mature normal human erythrocytes.

Nature. 1963 Jun 29;198:1312-3. doi: 10.1038/1981312a0.

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