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PROTON MAGNETIC-RESONANCE SPECTROSCOPY REFLECTS METABOLIC DECOMPENSATION IN MAPLE-SYRUP-URINE-DISEASE

被引：16
作者：
HEINDEL, W
KUGEL, H
WENDEL, U
ROTH, B
BENZBOHM, G
机构：
[1] UNIV DUSSELDORF, CHILDRENS HOSP, W-4000 DUSSELDORF, GERMANY
[2] UNIV COLOGNE, CHILDRENS HOSP, W-5000 COLOGNE, GERMANY
来源：
PEDIATRIC RADIOLOGY | 1995年 / 25卷 / 04期
关键词：
D O I：
10.1007/BF02011106
中图分类号：
R72 [儿科学];
学科分类号：
100202 ;
摘要：
Using localized proton magnetic resonance spectroscopy (H-1-MRS), accumulation of branched-chain amino acids (BCAA) and their corresponding 2-oxo acids (BCOA) could be non-invasively demonstrated in the brain of a 9-year-old girl suffering from classical maple syrup urine disease. During acute metabolic decompensation, the compounds caused a signal at a chemical shift of 0.9 ppm which was assigned by in vitro experiments. The brain tissue concentration of the sum of BCAA and BCOA could be estimated as 0.9 mmol/l. Localized H-1-MRS of the brain appears to be suitable for examining patients suffering from maple syrup urine disease in different metabolic states.
引用
收藏
页码：296 / 299
页数：4
相关论文
共 12 条
  • [11] Uziel G., Savoiardo M., Nardocci N., CT and MRI in maple syrup urine disease, Neurology, 38, pp. 486-488, (1988)
  • [12] Felber S., Sperl W., Chemelli A., Murr C., Wendel U., Maple syrup urine disease: metabolic decompensation monitored by proton magnetic resonance imaging and spectroscopy, Ann Neurol, 33, pp. 396-401, (1993)
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