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

页码：296 / 299

页数：4

共 12 条

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