Literature DB >> 1640294

Plasma glutamine concentration: a guide in the management of urea cycle disorders.

N E Maestri1, K D McGowan, S W Brusilow.   

Abstract

Because increases in plasma glutamine concentrations are almost always associated with hyperammonemia in patients with urea cycle disorders, we determined the correlation between these two variables for 2 years in a child with ornithine transcarbamylase deficiency. A correlation coefficient of 0.77 (p less than 0.0001) was found. Hyperammonemia was rarely observed when plasma glutamine levels were near normal. These data suggest that one goal of therapy is the maintenance of plasma glutamine levels at or near normal values.

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Year:  1992        PMID： 1640294     DOI： 10.1016/s0022-3476(05)81200-4

Source DB:  PubMed          Journal:  J Pediatr        ISSN： 0022-3476            Impact factor:   4.406


  18 in total

Review 1.  Evidence of a vicious cycle in glutamine synthesis and breakdown in pathogenesis of hepatic encephalopathy-therapeutic perspectives.

Authors:  Milan Holecek
Journal:  Metab Brain Dis       Date:  2013-08-31       Impact factor: 3.584

2.  Plasma glutamine and ammonia concentrations in ornithine carbamoyltransferase deficiency and citrullinaemia.

Authors:  C J Wilson; P J Lee; J V Leonard
Journal:  J Inherit Metab Dis       Date:  2001-12       Impact factor: 4.982

3.  New insights in nutritional management and amino acid supplementation in urea cycle disorders.

Authors:  Fernando Scaglia
Journal:  Mol Genet Metab       Date:  2010-03-01       Impact factor: 4.797

Review 4.  Early recognition of metabolic decompensation.

Authors:  A A Morris; J V Leonard
Journal:  Arch Dis Child       Date:  1997-06       Impact factor: 3.791

5.  Oncogenic Kaposi's Sarcoma-Associated Herpesvirus Upregulates Argininosuccinate Synthase 1, a Rate-Limiting Enzyme of the Citrulline-Nitric Oxide Cycle, To Activate the STAT3 Pathway and Promote Growth Transformation.

Authors:  Tingting Li; Ying Zhu; Fan Cheng; Chun Lu; Jae U Jung; Shou-Jiang Gao
Journal:  J Virol       Date:  2019-02-05       Impact factor: 5.103

Review 6.  Urea cycle disorders-update.

Authors:  Shirou Matsumoto; Johannes Häberle; Jun Kido; Hiroshi Mitsubuchi; Fumio Endo; Kimitoshi Nakamura
Journal:  J Hum Genet       Date:  2019-05-20       Impact factor: 3.172

7.  Pregnancy and argininosuccinic aciduria.

Authors:  S Worthington; J Christodoulou; B Wilcken; B Peat
Journal:  J Inherit Metab Dis       Date:  1996       Impact factor: 4.982

8.  Rapidly fatal hyperammonemic coma in adults. Urea cycle enzyme deficiency.

Authors:  B E Wilson; W N Hobbs; J J Newmark; S J Farrow
Journal:  West J Med       Date:  1994-08

9.  Ammonia control and neurocognitive outcome among urea cycle disorder patients treated with glycerol phenylbutyrate.

Authors:  George A Diaz; Lauren S Krivitzky; Masoud Mokhtarani; William Rhead; James Bartley; Annette Feigenbaum; Nicola Longo; William Berquist; Susan A Berry; Renata Gallagher; Uta Lichter-Konecki; Dennis Bartholomew; Cary O Harding; Stephen Cederbaum; Shawn E McCandless; Wendy Smith; Gerald Vockley; Stephen A Bart; Mark S Korson; David Kronn; Roberto Zori; J Lawrence Merritt; Sandesh C S Nagamani; Joseph Mauney; Cynthia Lemons; Klara Dickinson; Tristen L Moors; Dion F Coakley; Bruce F Scharschmidt; Brendan Lee
Journal:  Hepatology       Date:  2013-01-03       Impact factor: 17.425

Review 10.  Alternative pathway therapy for urea cycle disorders.

Authors:  F Feillet; J V Leonard
Journal:  J Inherit Metab Dis       Date:  1998       Impact factor: 4.982
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