Evidence map›Paper›PMID 23375471›Full record

ArticleMolecular genetics and metabolism2013

Long chain fatty acid (Lcfa) abnormalities in hyper Igd syndrome (Hids) and Familial Mediterranean Fever (Fmf): new insight into heritable periodic fevers.

Anna Simon, Joost P H Drenth, Dietrich Matern, Eric S Goetzman, Elizabeth J Hager, K Michael Gibson

Open access · greenAbstract read
In one paragraph

Article in Molecular genetics and metabolism, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
0.1field-weighted citation impact, top 44% 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

2 citing papers in PubMed, 4 citations in OpenAlex.

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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

6 authors at 5 institutions in 2 countries.

Anna SimonDepartment of General Internal Medicine, Radboud University of Nijmegen Medical Center, The Netherlands.
Joost P H Drenth
Dietrich Matern
Eric S Goetzman
Elizabeth J Hager
K Michael Gibson
Radboud University Nijmegen · NLChildren's Hospital of Pittsburgh · USMayo Clinic · USNational Cancer Institute · USWashington State University Spokane · US

Funding

Sterol and Isoprenoid Diseases Consortium Supplement 2018U54HD061939 · NICHD · UNIVERSITY OF NEBRASKA MEDICAL CENTER · PI BOERKOEL, CORNELIUS F · 2009 to 2018
$6.6M
Regulation of Peroxisomal Metabolism by Lysine AcylationR01DK090242 · NIDDK · UNIVERSITY OF PITTSBURGH AT PITTSBURGH · PI GOETZMAN, ERIC S · 2011 to 2024
$5.4M
Murine Knockout Model of Mevalonic AciduriaR03HD057864 · NICHD · UNIVERSITY OF PITTSBURGH AT PITTSBURGH · PI GIBSON, K MICHAEL · 2008 to 2009
$144k
NICHD NIH HHS 1U54HD061939NICHD NIH HHS HD 57864NICHD NIH HHS R03 HD057864NICHD NIH HHS U54 HD061939NIDDK NIH HHS R01 DK090242
6 · The paper itself

Abstract

objectiveTo examine essential fatty acids (EFAs) in hyper-IgD syndrome (HIDS) and Familial Mediterranean Fever (FMF).

methodsEFAs were determined in sera derived from an archival, cross-sectional group of HIDS/FMF patients, stratified for presence and absence of fever. Control populations included healthy afebrile adults, and individuals with non-periodic fever (septic shock). EFAs were quantified using isotope dilution gas chromatography-mass spectrometry and data analyzed employing a Kruskal-Wallis non-parametric ANOVA with Dunn's post-hoc test.

resultsSera samples derived from HIDS patients showed significantly decreased C20, C26, phytanic and pristanic acids during febrile crises that normalized in the afebrile state, and a significantly increased afebrile C22_4ω6 level that normalized with fever. Samples derived from FMF patients revealed increased ω-oxidized LCFAs as compared to controls, and the trend was for these same species to be increased in comparison to febrile, but not afebrile, HIDS patients. Individuals with non-periodic fever demonstrated global decreases in C10-C24 fatty acids, both saturated and unsaturated, accompanied by an elevated triene/tetraene ratio.

conclusionsOur results suggest that different mechanisms are active in hereditary periodic fever syndromes that appear unrelated to fever, including depletion of very long chain fatty acids (VLCFAs) in febrile HIDS patients and increased ω-oxidized LCFAs in patients with FMF. These findings underscore new roles for EFAs in the potential production of inflammatory species in patients with hereditary periodic fever.

Indexed as

PeriodicityAdolescentAdultAnalysis of VarianceCase-Control StudiesCross-Sectional StudiesFamilial Mediterranean FeverFatty Acids, EssentialFemaleGas Chromatography-Mass SpectrometryHumansImmunoglobulin DInheritance PatternsMaleMevalonate Kinase DeficiencyMiddle AgedFatty Acids, EssentialImmunoglobulin Dmevalonate kinasePhosphotransferases (Alcohol Group Acceptor)

Identifiers

PMID23375471
PMCPMC3654528
OpenAlexW2003886723

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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.