Localized Proton MR Spectroscopic Detection of Nonketotic Hyperglycinemia in an Infant

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 increase...

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Detalles Bibliográficos
Autores principales: Choi, Choong-Gon, Lee, Ho Kyu, Yoon, Jong-Hyun
Formato: Texto
Lenguaje:English
Publicado: The Korean Radiological Society 2001
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2718128/
https://www.ncbi.nlm.nih.gov/pubmed/11754333
http://dx.doi.org/10.3348/kjr.2001.2.4.239
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author Choi, Choong-Gon
Lee, Ho Kyu
Yoon, Jong-Hyun
author_facet Choi, Choong-Gon
Lee, Ho Kyu
Yoon, Jong-Hyun
author_sort Choi, Choong-Gon
collection PubMed
description 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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spelling pubmed-27181282009-07-30 Localized Proton MR Spectroscopic Detection of Nonketotic Hyperglycinemia in an Infant Choi, Choong-Gon Lee, Ho Kyu Yoon, Jong-Hyun Korean J Radiol Case Report 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. The Korean Radiological Society 2001 2001-12-31 /pmc/articles/PMC2718128/ /pubmed/11754333 http://dx.doi.org/10.3348/kjr.2001.2.4.239 Text en Copyright © 2001 The Korean Radiological Society http://creativecommons.org/licenses/by-nc/3.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Choi, Choong-Gon
Lee, Ho Kyu
Yoon, Jong-Hyun
Localized Proton MR Spectroscopic Detection of Nonketotic Hyperglycinemia in an Infant
title Localized Proton MR Spectroscopic Detection of Nonketotic Hyperglycinemia in an Infant
title_full Localized Proton MR Spectroscopic Detection of Nonketotic Hyperglycinemia in an Infant
title_fullStr Localized Proton MR Spectroscopic Detection of Nonketotic Hyperglycinemia in an Infant
title_full_unstemmed Localized Proton MR Spectroscopic Detection of Nonketotic Hyperglycinemia in an Infant
title_short Localized Proton MR Spectroscopic Detection of Nonketotic Hyperglycinemia in an Infant
title_sort localized proton mr spectroscopic detection of nonketotic hyperglycinemia in an infant
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2718128/
https://www.ncbi.nlm.nih.gov/pubmed/11754333
http://dx.doi.org/10.3348/kjr.2001.2.4.239
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AT leehokyu localizedprotonmrspectroscopicdetectionofnonketotichyperglycinemiainaninfant
AT yoonjonghyun localizedprotonmrspectroscopicdetectionofnonketotichyperglycinemiainaninfant

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