Localized brain proton NMR spectroscopy in young adult phenylketonuria patients
作者:
K. Johannik,
P. van Hecke,
B. François,
G. Marchal,
M‐H. Smet,
J. Jaeken,
L. Breysem,
G. Wilms,
A. L. Baert,
期刊:
Magnetic Resonance in Medicine
(WILEY Available online 1994)
卷期:
Volume 31,
issue 1
页码: 53-57
ISSN:0740-3194
年代: 1994
DOI:10.1002/mrm.1910310108
出版商: Wiley Subscription Services, Inc., A Wiley Company
关键词: brain;phenylketonuria;(PKU);proton;NMR;spectroscopy
数据来源: WILEY
摘要:
AbstractLocalized proton magnetic resonance spectroscopy with short echo time (TE= 20 ms) was used to investigate biochemical changes in the cerebral white matter of 20 young adult patients (median 19 years) with phenylketonuria (PKU). Results were compared with those of a group of 12 age‐matched healthy volunteers (median 25 years). Concentrations of Nacetyl‐aspartate (NAA) and choline (Cho) relative to creatine (Cr) were unchanged. However, concentrations of inositol (Ins) relative to creatine were found to be significantly lower (P<0.001) in the PKU patients (0.30 ± 0.09 versus 0.57 ± 0.17). Individual inositol concentrations did not correlate with age, diet, serum phenylalanine (Phe) levels or extent of pathological regions in the T,‐weighted images. The lack of correlation with individual data suggests that the decreased inositol concentration could be related to a metabolic deficiency during fetal development. No signal from the phenyl ring protons of phenylalanine was detected in the PKU patients (phenylalanine serum concentration ⩽ 1.27 mM), which suggests that concentration of phenylalanine may be lower in brain than
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