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DIETARY TREATMENT OF A CHILD WITH MAPLE SYRUP URINE DISEASE (BRANCHED-CHAIN KETOACIDURIA).

作者信息

WESTALL R G

出版信息

Arch Dis Child. 1963 Oct;38(201):485-91. doi: 10.1136/adc.38.201.485.

DOI:10.1136/adc.38.201.485
PMID:14065992
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2019098/
Abstract
摘要
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e609/2019098/6cc4b5c67759/archdisch01590-0064-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e609/2019098/6cc4b5c67759/archdisch01590-0064-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e609/2019098/6cc4b5c67759/archdisch01590-0064-a.jpg

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Early dietary management of sugar intolerance in infancy.婴儿期糖不耐受的早期饮食管理。
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The longest-surviving patient with classical maple syrup urine disease.
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[INTOLERANCE TO DIETARY PROTEINS AND SUGARS BASED ON CELLULAR METABOLIC DEFECTS].[基于细胞代谢缺陷的膳食蛋白质和糖类不耐受]
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本文引用的文献

1
An inborn defect in the metabolism of tyrosine in infants on a normal diet.正常饮食的婴儿体内酪氨酸代谢的先天性缺陷。
Biochem J. 1960 Nov;77(2):320-6. doi: 10.1042/bj0770320.
2
A DEFECT IN THE METABOLISM OF TYROSINE AND PHENYLALANINE IN PREMATURE INFANTS. II. SPONTANEOUS OCCURRENCE AND ERADICATION BY VITAMIN C.早产儿酪氨酸和苯丙氨酸代谢缺陷。II. 维生素C的自然发生和消除作用
J Clin Invest. 1941 Mar;20(2):209-19. doi: 10.1172/JCI101213.
3
A new finding in maple-syrup-urine disease.
4
[MAPLE SYRUP DISEASE WITH FAMILIAL INCIDENCE].[家族性发病率的枫糖尿症]
Virchows Arch Pathol Anat Physiol Klin Med. 1964 May 22;337:425-45.
5
The adult and adolescent clinic for inborn errors of metabolism.成人及青少年先天性代谢缺陷诊所。
J Inherit Metab Dis. 2000 May;23(3):215-28. doi: 10.1023/a:1005627911682.
6
Hudson memorial lecture. Neonatal management of organic acidurias. Clinical update.哈德逊纪念讲座。有机酸尿症的新生儿管理。临床进展
J Inherit Metab Dis. 1984;7 Suppl 1:2-9. doi: 10.1007/978-94-009-5612-4_2.
7
Dietary treatment of homocystinuria.同型胱氨酸尿症的饮食治疗。
Arch Dis Child. 1966 Dec;41(220):666-71. doi: 10.1136/adc.41.220.666.
8
Urinary screening tests in the prevention of mental deficiency.预防智力缺陷的尿液筛查试验。
Can Med Assoc J. 1966 Jul 16;95(3):89-95.
9
[On the effect of dietetic prophylaxis on the myelogenesis of leucionsis (maple syrup urine disease)].[关于饮食预防对白血病（枫糖尿症）骨髓生成的影响]
Klin Wochenschr. 1965 Sep 1;43(17):926-30. doi: 10.1007/BF01712059.
10
Diagnosis and treatment of tyrosinosis.酪氨酸血症的诊断与治疗。
Arch Dis Child. 1968 Oct;43(231):540-7. doi: 10.1136/adc.43.231.540.
Lancet. 1962 Jan 6;1(7219):26-7. doi: 10.1016/s0140-6736(62)92646-6.
4
A modified procedure for the automatic analysis of amino acids.一种用于氨基酸自动分析的改进方法。
Anal Biochem. 1960 Nov;1:187-201. doi: 10.1016/0003-2697(60)90045-2.
5
Studies in maple syrup urine disease.枫糖尿症的研究。
Arch Dis Child. 1961 Jun;36(187):259-68. doi: 10.1136/adc.36.187.259.
6
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.
7
Maple syrup urine disease.枫糖尿症
Br Med J. 1959 Jan 10;1(5114):91-3. doi: 10.1136/bmj.1.5114.91.
8
Maple syrup urine disease; an inborn error of the metabolism of valine, leucine, and isoleucine associated with gross mental deficiency.枫糖尿症；一种与严重智力缺陷相关的缬氨酸、亮氨酸和异亮氨酸代谢先天性缺陷。
Br Med J. 1959 Jan 10;1(5114):90-1. doi: 10.1136/bmj.1.5114.90.
9
Studies on the metabolism of amino acids and related compounds in vivo. I. Toxicity of essential amino acids, individually and in mixtures, and the protective effect of L-arginine.体内氨基酸及相关化合物的代谢研究。I. 必需氨基酸单独及混合使用时的毒性以及L-精氨酸的保护作用。
Arch Biochem Biophys. 1956 Oct;64(2):319-32. doi: 10.1016/0003-9861(56)90276-4.
10
Procedures for the chromatographic determination of amino acids on four per cent cross-linked sulfonated polystyrene resins.在4%交联磺化聚苯乙烯树脂上进行氨基酸色谱测定的方法。
J Biol Chem. 1954 Dec;211(2):893-906.