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Maple syrup disease; isolation and identification of organic acids in the urine.

作者信息

MENKES J H

出版信息

Pediatrics. 1959 Feb;23(2):348-53.

PMID:13633350
Abstract
摘要

相似文献

1
Maple syrup disease; isolation and identification of organic acids in the urine.枫糖尿症;尿液中有机酸的分离与鉴定
Pediatrics. 1959 Feb;23(2):348-53.
2
Intermediate maple syrup urine disease: neuroimaging observations in 3 patients from South India.
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Maple syrup urine disease in Thai infants.泰国婴儿中的枫糖尿症
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Maple syrup urine disease: diffusion MRI, and proton MR spectroscopy findings.枫糖尿症:扩散加权磁共振成像及氢质子磁共振波谱成像结果
Comput Med Imaging Graph. 2007 Mar;31(2):106-10. doi: 10.1016/j.compmedimag.2006.11.005. Epub 2007 Jan 4.
5
Simultaneous retention index analysis of urinary amino acids and carboxylic acids for graphic recognition of abnormal state.
J Chromatogr B Analyt Technol Biomed Life Sci. 2005 Jul 5;821(1):94-104. doi: 10.1016/j.jchromb.2005.04.011.
6
[Diagnosis of inborn errors of metabolism using tandem mass spectrometry and gas chromatography mass spectrometry].[运用串联质谱法和气相色谱 - 质谱法诊断先天性代谢缺陷]
Zhonghua Yi Xue Za Zhi. 2008 Aug 5;88(30):2122-6.
7
THE PREVENTION OF CEREBRAL LESIONS DUE TO ENZYMOPATHIES.酶病所致脑损伤的预防
Bibl Paediatr. 1963;81:63-75.
8
A rapid ultra performance liquid chromatography tandem mass spectrometric method for measuring amino acids associated with maple syrup urine disease, tyrosinaemia and phenylketonuria.一种用于测定与枫糖尿症、酪氨酸血症和苯丙酮尿症相关氨基酸的快速超高效液相色谱串联质谱法。
Ann Clin Biochem. 2007 Sep;44(Pt 5):474-81. doi: 10.1258/000456307781646012.
9
Maple syrup urine disease-treatment and outcome in patients of Turkish descent in Germany.德国土耳其裔患者的枫糖尿症——治疗与预后
Turk J Pediatr. 2005 Jan-Mar;47(1):8-13.
10
Restoration of brain stem auditory-evoked potential in maple syrup urine disease.
Otol Neurotol. 2007 Jun;28(4):566-9. doi: 10.1097/MAO.0b013e31802fbaa9.

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Amino acid profiles: exploring their diagnostic and pathophysiological significance in hypertension.氨基酸谱:探索其在高血压中的诊断和病理生理意义。
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2
Acute effects of intracerebroventricular administration of α-ketoisocaproic acid in young rats on inflammatory parameters.脑室内注射α-酮异己酸对幼鼠炎症参数的急性影响
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3
Alzheimer's disease: targeting the glutamatergic system.
阿尔茨海默病:靶向谷氨酸能系统。
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Branched Chain Amino Acids.支链氨基酸。
Annu Rev Physiol. 2019 Feb 10;81:139-164. doi: 10.1146/annurev-physiol-020518-114455. Epub 2018 Nov 28.
5
Evaluation of plasma biomarkers of inflammation in patients with maple syrup urine disease.枫糖尿症患者炎症血浆生物标志物的评估
J Inherit Metab Dis. 2018 May 8. doi: 10.1007/s10545-018-0188-x.
6
Acute and long-term effects of intracerebroventricular administration of α-ketoisocaproic acid on oxidative stress parameters and cognitive and noncognitive behaviors.脑室注射 α-酮异己酸对氧化应激参数及认知和非认知行为的急性和长期影响。
Metab Brain Dis. 2017 Oct;32(5):1507-1518. doi: 10.1007/s11011-017-0035-z. Epub 2017 May 27.
7
Branched-chain amino acid metabolism: from rare Mendelian diseases to more common disorders.支链氨基酸代谢:从罕见的孟德尔疾病到更常见的病症
Hum Mol Genet. 2014 Sep 15;23(R1):R1-8. doi: 10.1093/hmg/ddu123. Epub 2014 Mar 20.
8
Effect of leucine administration to female rats during pregnancy and lactation on oxidative stress and enzymes activities of phosphoryltransfer network in cerebral cortex and hippocampus of the offspring.孕期和哺乳期给雌性大鼠补充亮氨酸对后代大脑皮质和海马磷酸转移网络的氧化应激和酶活性的影响。
Neurochem Res. 2013 Mar;38(3):632-43. doi: 10.1007/s11064-012-0961-4. Epub 2013 Jan 1.
9
Interrupting the mechanisms of brain injury in a model of maple syrup urine disease encephalopathy.中断枫糖尿症脑病模型中脑损伤的机制。
J Inherit Metab Dis. 2012 Jan;35(1):71-9. doi: 10.1007/s10545-011-9333-5. Epub 2011 May 4.
10
Aminoaciduria and lens opacity formation.氨基酸尿症与晶状体混浊形成。
Br J Ophthalmol. 1968 Jan;52(1):40-2. doi: 10.1136/bjo.52.1.40.