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Mol Genet Metab. 2017 May;121(1):9-15. doi: 10.1016/j.ymgme.2017.03.003. Epub 2017 Mar 10.

Brain MRS glutamine as a biomarker to guide therapy of hyperammonemic coma.

Author information

1
Division of Genetics and Genomics, Department of Medicine, Boston Children's Hospital, Harvard Medical School, Boston, MA 02115, USA.
2
Department of Radiology, Brigham and Women's Hospital, Boston, MA 02115, USA.
3
Department of Medicine, Brigham and Women's Hospital, Boston, MA 02115, USA.
4
Department of Neurology, Brigham and Women's Hospital, Boston, MA 02115, USA.
5
Division of Genetics and Genomics, Department of Medicine, Boston Children's Hospital, Harvard Medical School, Boston, MA 02115, USA. Electronic address: gerard.berry@childrens.harvard.edu.
6
Division of Genetics, Department of Medicine, Brigham and Women's Hospital, Boston, MA 02115, USA; Department of Medicine, VA Boston Healthcare System, Boston, MA 02115, USA. Electronic address: nfrank@partners.org.

Abstract

Acute idiopathic hyperammonemia in an adult patient is a life-threatening condition often resulting in a rapid progression to irreversible cerebral edema and death. While ammonia-scavenging therapies lower blood ammonia levels, in comparison, clearance of waste nitrogen from the brain may be delayed. Therefore, we used magnetic resonance spectroscopy (MRS) to monitor cerebral glutamine levels, the major reservoir of ammonia, in a gastric bypass patient with hyperammonemic coma undergoing therapy with N-carbamoyl glutamate and the ammonia-scavenging agents, sodium phenylacetate and sodium benzoate. Improvement in mental status mirrored brain glutamine levels, as coma persisted for 48h after plasma ammonia normalized. We hypothesize that the slower clearance for brain glutamine levels accounts for the delay in improvement following initiation of treatment in cases of chronic hyperammonemia. We propose MRS to monitor brain glutamine as a noninvasive approach to be utilized for diagnostic and therapeutic monitoring purposes in adult patients presenting with idiopathic hyperammonemia.

PMID:
28408159
DOI:
10.1016/j.ymgme.2017.03.003
[Indexed for MEDLINE]

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