Evidence map›Paper›PMID 26586473›Full record

Trial reportMolecular genetics and metabolism2016

Glutamine and hyperammonemic crises in patients with urea cycle disorders.

B Lee, G A Diaz, W Rhead, U Lichter-Konecki, A Feigenbaum, S A Berry, C Le Mons, J Bartley, N Longo, S C Nagamani and 11 more

Open access · hybridAbstract readClinical Trial
PubMed Full text at PMC Publisher
In one paragraph

Trial report in Molecular genetics and metabolism, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.

0numbers the graph read from it
0cells of the map it votes in
11citing papers in PubMed
1.5field-weighted citation impact, top 19% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

11 citing papers in PubMed, 39 citations in OpenAlex.

  1. Article
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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

21 authors at 16 institutions in 2 countries.

B LeeBaylor College of Medicine, Houston, TX, USA. Electronic address: blee@bcm.edu.
G A DiazIcahn School of Medicine at Mount Sinai, New York, NY, USA.
W RheadThe Medical College of Wisconsin, Milwaukee, WI, USA.
U Lichter-KoneckiDrexel University College of Medicine, Philadelphia, PA, USA.
A FeigenbaumUniv. of CA, San Diego, CA, USA.
S A BerryUniv. of Minnesota, Minneapolis, MN, USA.
C Le MonsNational Urea Cycle Disorders Foundation, Pasadena, CA, USA.
J BartleyMiller Children's Hospital, Long Beach, CA, USA.
N LongoUniv. of UT, Salt Lake City, UT, USA.
S C NagamaniBaylor College of Medicine, Houston, TX, USA.
W BerquistStanford Univ., Palo Alto, CA, USA.
R C GallagherUniv. of CA, San Francisco, USA.
C O HardingOregon Health Sciences, Portland, OR, USA.
S E McCandlessCase Western Reserve Univ. Medical Center, Cleveland, OH, USA.
W SmithMaine Medical Ctr., Portland, ME, USA.
A SchulzeThe Hospital for Sick Children, Univ. of Toronto, Canada.
M MarinoOregon Health Sciences, Portland, OR, USA.
R RowellMED Technical Consulting, Inc., Union City, CA, USA.
D F CoakleyHorizon Therapeutics, Brisbane, CA, USA.
M MokhtaraniHorizon Therapeutics, Brisbane, CA, USA.
B F ScharschmidtHorizon Therapeutics, Brisbane, CA, USA.
Horizon Therapeutics (United States) · USBaylor College of Medicine · USOregon Health & Science University · USCase Western Reserve University · USDrexel University · USIcahn School of Medicine at Mount Sinai · USMaine Medical Center · USMedical College of Wisconsin · USMiller Children's & Women's Hospital · USNational Urea Cycle Disorders Foundation · USPalo Alto University · USUniversity of California, San Diego · USUniversity of Minnesota · USUniversity of San Francisco · USUniversity of Toronto · CAUniversity of Utah · US

Funding

Transform Dissemination and Implementation Science in CTSA ProgramsUL1TR002319 · NCATS · UNIVERSITY OF WASHINGTON · PI John K. Amory · 2017 to 2026
$100.0M
CTSA INFRASTRUCTURE FOR PEDIATRIC RESEARCHUL1RR025780 · NCRR · UNIVERSITY OF COLORADO DENVER · PI SOKOL, RONALD J. · 2008 to 2011
$61.3M
OREGON CLINICAL AND TRANSLATIONAL SCIENCE INSTITUTEUL1RR024140 · NCRR · OREGON HEALTH & SCIENCE UNIVERSITY · PI ORWOLL, ERIC S. · 2006 to 2011
$61.1M
University of Pittsburgh Clinical and Translational Science InstituteUL1TR000005 · NCATS · UNIVERSITY OF PITTSBURGH AT PITTSBURGH · PI REIS, STEVEN E · 2012 to 2015
$50.8M
Institute of Translational Health SciencesUL1TR000423 · NCATS · UNIVERSITY OF WASHINGTON · PI DISIS, MARY L. · 2012 to 2016
$49.4M
Using social networks to map and evaluate team science across CTSA hubsUL1TR001427 · NCATS · UNIVERSITY OF FLORIDA · PI MITCHELL, DUANE A. · 2015 to 2024
$37.2M
Spectrum Stanford Center for clinical and Translational Research and EducationUL1TR001085 · NCATS · STANFORD UNIVERSITY · PI CULLEN, MARK RICHARD, GREENBERG, HARRY BERNARD · 2013 to 2017
$37.1M
University of Minnesota Clinical and Translational Science Institute (UMN CTSI)UL1TR000114 · NCATS · UNIVERSITY OF MINNESOTA · PI BLAZAR, BRUCE R · 2012 to 2015
$35.0M
ZOLENDRONIC ACID IN CHILDREN W/ SEVERE OSTEOGENESIS IMPERFECTAM01RR000188 · NCRR · BAYLOR COLLEGE OF MEDICINE · PI PLON, SHARON E. · 1985 to 2011
$30.6M
Stanford Center for Clinical and Translational Education and Research (SCCTER)UL1UL1RR025744 · NCRR · STANFORD UNIVERSITY · PI GREENBERG, HARRY BERNARD · 2008 to 2011
$22.4M
UNIVERSITY OF UTAH CENTER FOR CLINICAL AND TRANSLATIONAL SCIENCE-UL1: BPCAUL1RR025764 · NCRR · UNIVERSITY OF UTAH · PI MCCLAIN, DONALD A. · 2008 to 2011
$19.4M
MOUNT SINAI INSTITUTES FOR CLINICAL AND TRANSLATIONAL SCIENCESUL1RR029887 · NCRR · ICAHN SCHOOL OF MEDICINE AT MOUNT SINAI · PI SAMPSON, HUGH A · 2009 to 2011
$19.2M
Doris Duke Charitable Foundation 2013095NCATS NIH HHS NIH UL1TR000005NCATS NIH HHS UL1 TR000005NCATS NIH HHS UL1 TR000114NCATS NIH HHS UL1 TR000423NCATS NIH HHS UL1TR000423NCATS NIH HHS UL1 TR001085NCATS NIH HHS UL1 TR001427NCATS NIH HHS UL1 TR002319NCRR NIH HHS M01 RR000188NCRR NIH HHS M01RR00188NCRR NIH HHS U54 RR019453NCRR NIH HHS UL1 RR024140NCRR NIH HHS UL1 RR025744NCRR NIH HHS UL1 RR025752NCRR NIH HHS UL1 RR025764NCRR NIH HHS UL1 RR025780NCRR NIH HHS UL1 RR029887NCRR NIH HHS UL1 RR029890NCRR NIH HHS UL1 RR031973NCRR NIH HHS UL1 RR031988NCRR NIH HHS UL1 RR033176NCRR NIH HHS UL1 RR033183NCRR NIH HHS UL1RR25744NCRR NIH HHS UL1RR25752NCRR NIH HHS UL1RR25764NCRR NIH HHS UL1RR25780NCRR NIH HHS UL1RR29887NCRR NIH HHS UL1RR29890NCRR NIH HHS UL1RR31973NCRR NIH HHS UL1RR31988NCRR NIH HHS UL1RR33176NCRR NIH HHS UL1RR33183NIDDK NIH HHS 1K08DK074573NIDDK NIH HHS K08 DK074573NIDDK NIH HHS R01 DK092921
6 · The paper itself

Abstract

unlabelledBlood ammonia and glutamine levels are used as biomarkers of control in patients with urea cycle disorders (UCDs). This study was undertaken to evaluate glutamine variability and utility as a predictor of hyperammonemic crises (HACs) in UCD patients.

methodsThe relationships between glutamine and ammonia levels and the incidence and timing of HACs were evaluated in over 100 adult and pediatric UCD patients who participated in clinical trials of glycerol phenylbutyrate.

resultsThe median (range) intra-subject 24-hour coefficient of variation for glutamine was 15% (8-29%) as compared with 56% (28%-154%) for ammonia, and the correlation coefficient between glutamine and concurrent ammonia levels varied from 0.17 to 0.29. Patients with baseline (fasting) glutamine values >900 μmol/L had higher baseline ammonia levels (mean [SD]: 39.6 [26.2]μmol/L) than patients with baseline glutamine ≤ 900 μmol/L (26.6 [18.0]μmol/L). Glutamine values >900 μmol/L during the study were associated with an approximately 2-fold higher HAC risk (odds ratio [OR]=1.98; p=0.173). However, glutamine lost predictive significance (OR=1.47; p=0.439) when concomitant ammonia was taken into account, whereas the predictive value of baseline ammonia ≥ 1.0 upper limit of normal (ULN) was highly statistically significant (OR=4.96; p=0.013). There was no significant effect of glutamine >900 μmol/L on time to first HAC crisis (hazard ratio [HR]=1.14; p=0.813), but there was a significant effect of baseline ammonia ≥ 1.0 ULN (HR=4.62; p=0.0011).

conclusionsThe findings in this UCD population suggest that glutamine is a weaker predictor of HACs than ammonia and that the utility of the predictive value of glutamine will need to take into account concurrent ammonia levels.

Indexed as

AdolescentAdultAmmoniaBiomarkersChildChild, PreschoolFastingFemaleGlutamineGlycerolHumansHyperammonemiaMalePhenylbutyratesPredictive Value of TestsUrea Cycle Disorders, InbornAmmoniaBiomarkersGlutamineGlycerolglycerol phenylbutyratePhenylbutyratesAmmoniaGlycerol phenylbutyrateMetabolic disordersRAVICTI

Identifiers

PMID26586473
PMCPMC4915945
OpenAlexW2158358118

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.