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枫糖尿症一例的脑电图:其与血清支链氨基酸水平的关系。

Electroencephalograms in a case of maple syrup urine disease: their relation to serum levels of branched-chain amino acids.

作者信息

Iinuma K, Saito T, Wada Y, Onuma A, Takamatsu N

出版信息

Tohoku J Exp Med. 1976 Oct;120(2):191-5. doi: 10.1620/tjem.120.191.

DOI:10.1620/tjem.120.191
PMID:982436
Abstract

During dietary treatment of a case of maple syrup urine disease, it was found that abnormal EEGs were observed when serum levels of leucine were abnormally high while those of valine and isoleucine were normal, and also when serum levels of valine and isoleucine were abnormally high while serum leucine levels were normal.

摘要

在对一例枫糖尿症患者进行饮食治疗期间,发现当血清亮氨酸水平异常升高而缬氨酸和异亮氨酸水平正常时,以及当血清缬氨酸和异亮氨酸水平异常升高而血清亮氨酸水平正常时,均观察到异常脑电图。

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Electroencephalograms in a case of maple syrup urine disease: their relation to serum levels of branched-chain amino acids.枫糖尿症一例的脑电图:其与血清支链氨基酸水平的关系。
Tohoku J Exp Med. 1976 Oct;120(2):191-5. doi: 10.1620/tjem.120.191.
2
Plasma amino acid analyses in two cases of maple syrup urine disease.两例枫糖尿症的血浆氨基酸分析
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Prospective study of maple-syrup-urine disease for the first four days of life.对出生后前四天的枫糖尿症进行前瞻性研究。
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Semiquantitation of leucine, isoleucine, and valine by thin-layer chromatography in management of maple-syrup urine diseases.通过薄层色谱法对亮氨酸、异亮氨酸和缬氨酸进行半定量分析在枫糖尿症管理中的应用
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Evaluation of branched-chain amino acid intake in children with maple syrup urine disease and methylmalonic aciduria.枫糖尿症和甲基丙二酸尿症患儿支链氨基酸摄入量的评估。
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Diagnosis of maple syrup urine disease by determination of L-valine, L-isoleucine, L-leucine and L-phenylalanine in neonatal blood spots by gas chromatography-mass spectrometry.通过气相色谱-质谱法测定新生儿血斑中的L-缬氨酸、L-异亮氨酸、L-亮氨酸和L-苯丙氨酸来诊断枫糖尿症。
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[Metabolic study of the classic form of leucinosis during exchange transfusion therapy].
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