Literature DB >> 11337324

Localized proton MR spectroscopy in infants with urea cycle defect.

C G Choi1, H W Yoo.   

Abstract

SUMMARY: Urea cycle defect is an inborn error of ammonium metabolism caused by a deficient activity of the enzymes involved in urea synthesis. Localized short-TE proton MR spectroscopy, performed in two infants who had citrullinemia and ornithine transcarbamylase deficiency, respectively, showed a prominent increase of glutamine/glutamate and lipid/lactate complex in both cases. N-acetylaspartate, total creatine, and myo-inositol were decreased in the infant with citrullinemia. Proton MR spectroscopy provided useful information for the diagnosis and understanding of the pathophysiology of urea cycle enzyme defect.

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Year:  2001        PMID： 11337324      PMCID： PMC8174930     

Source DB:  PubMed          Journal:  AJNR Am J Neuroradiol        ISSN： 0195-6108            Impact factor:   3.825


  9 in total

1.  Cerebral MRS in infant with suspected Reye's syndrome.

Authors:  T Ernst; B D Ross; R Flores
Journal:  Lancet       Date:  1992-08-22       Impact factor: 79.321

Review 2.  Nitrogen metabolism and ornithine cycle function.

Authors:  A J Meijer; W H Lamers; R A Chamuleau
Journal:  Physiol Rev       Date:  1990-07       Impact factor: 37.312

3.  In vivo proton spectroscopy in presence of eddy currents.

Authors:  U Klose
Journal:  Magn Reson Med       Date:  1990-04       Impact factor: 4.668

Review 4.  New aspects of brain physiology.

Authors:  B D Ross; S Blüml
Journal:  NMR Biomed       Date:  1996-10       Impact factor: 4.044

5.  On the identification of cerebral metabolites in localized 1H NMR spectra of human brain in vivo.

Authors:  T Michaelis; K D Merboldt; W Hänicke; M L Gyngell; H Bruhn; J Frahm
Journal:  NMR Biomed       Date:  1991-04       Impact factor: 4.044

6.  Magnetic resonance spectroscopy shows increased brain glutamine in ornithine carbamoyl transferase deficiency.

Authors:  A Connelly; J H Cross; D G Gadian; J V Hunter; F J Kirkham; J V Leonard
Journal:  Pediatr Res       Date:  1993-01       Impact factor: 3.756

7.  Effect of reducing brain glutamine synthesis on metabolic symptoms of hepatic encephalopathy.

Authors:  R A Hawkins; J Jessy; A M Mans; M R De Joseph
Journal:  J Neurochem       Date:  1993-03       Impact factor: 5.372

8.  Metabolic disorders of the brain in chronic hepatic encephalopathy detected with H-1 MR spectroscopy.

Authors:  R Kreis; B D Ross; N A Farrow; Z Ackerman
Journal:  Radiology       Date:  1992-01       Impact factor: 11.105

9.  Changes in brain metabolism during hyperammonemia and acute liver failure: results of a comparative 1H-NMR spectroscopy and biochemical investigation.

Authors:  D K Bosman; N E Deutz; A A De Graaf; R W vd Hulst; H M Van Eijk; W M Bovée; M A Maas; G G Jörning; R A Chamuleau
Journal:  Hepatology       Date:  1990-08       Impact factor: 17.425
  9 in total
  12 in total

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Journal:  Curr Neurol Neurosci Rep       Date:  2004-03       Impact factor: 5.081

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Authors:  J M U-King-Im; E Yu; E Bartlett; R Soobrah; W Kucharczyk
Journal:  AJNR Am J Neuroradiol       Date:  2010-11-18       Impact factor: 3.825

Review 6.  In Vivo NMR Studies of the Brain with Hereditary or Acquired Metabolic Disorders.

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7.  Magnetic resonance spectroscopy in pediatric neuroradiology: clinical and research applications.

Authors:  Ashok Panigrahy; Marvin D Nelson; Stefan Blüml
Journal:  Pediatr Radiol       Date:  2009-11-24

8.  Brain MR imaging in neonatal hyperammonemic encephalopathy resulting from proximal urea cycle disorders.

Authors:  Jun-ichi Takanashi; A James Barkovich; Sabrina F Cheng; Kara Weisiger; Carol O Zlatunich; Christine Mudge; Philip Rosenthal; Mendel Tuchman; Seymour Packman
Journal:  AJNR Am J Neuroradiol       Date:  2003 Jun-Jul       Impact factor: 3.825

Review 9.  MR spectroscopy in pediatric neuroradiology.

Authors:  Roberto Liserre; Lorenzo Pinelli; Roberto Gasparotti
Journal:  Transl Pediatr       Date:  2021-04

Review 10.  Suggested guidelines for the diagnosis and management of urea cycle disorders.

Authors:  Johannes Häberle; Nathalie Boddaert; Alberto Burlina; Anupam Chakrapani; Marjorie Dixon; Martina Huemer; Daniela Karall; Diego Martinelli; Pablo Sanjurjo Crespo; René Santer; Aude Servais; Vassili Valayannopoulos; Martin Lindner; Vicente Rubio; Carlo Dionisi-Vici
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