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KMID : 0880420010020040239
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Korean Journal of Radiology
2001 Volume.2 No. 4 p.239 ~ p.242
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Localized Proton MR Spectroscopic Detection of Nonketotic Hyperglycinemia in an Infant
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Choong Gon Choi/Choong Gon Choi
Ho Kyu Lee/Jong Hyun Yoon
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Abstract
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Nonketotic hyperglycinemia (NKH) is a rare metabolic brain disease caused by deficient activity of the glycine cleveage system. Localized proton MR spectroscopy (echo-time 166 msec), performed in an infant with the typical clinical and biochemical features of neonatal NKH, showed a markedly increased peak intensity at 3.55 ppm, which was assigned to glycine. Serial proton MR spectrosocpic studies indicated that glycine/choline and glycine/total creatine ratios correlated closely with the patient's clinical course. Proton MR spectroscopy was useful for the non-invasive detection and monitoring of cerebral glycine levels in this infant with NKH.
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KEYWORD
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Brain, MR spectroscopy, Brain, metabolism,
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