Evidence map›Paper›PMID 21160462›Full record

ArticleKidney international2011

Progressive podocyte injury and globotriaosylceramide (GL-3) accumulation in young patients with Fabry disease.

Behzad Najafian, Einar Svarstad, Leif Bostad, Marie-Claire Gubler, Camilla Tøndel, Chester Whitley, Michael Mauer

Registry-linked trialOpen access · greenAbstract readMulticenter Study
In one paragraph

Article in Kidney international, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT02994303 (Podocyturia, a Non-Invasive Predictor of Renal Dysfunction in Fabry Nephropathy), which is not on this map. Cited by 96 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
96citing papers in PubMed, 1 pooled it
3.9field-weighted citation impact, top 6% 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.

NCT02994303 unknown statusnot on this mapstarted 2014, after this paper: background citation

Podocyturia, a Non-Invasive Predictor of Renal Dysfunction in Fabry Nephropathy

TypeobservationalSponsorUniversity of WashingtonRan2014 to 2019Enrolled58ConditionsFabry Disease
3 · Its place in the literature

Who cites it

96 citing papers in PubMed, 1 synthesis or guideline pooled it, 173 citations in OpenAlex.

  1. Fabry disease in infancy and early childhood: a systematic literature review.Genetics in medicine : official journal of the American College of Medical Genetics · 2015
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  12. Pathogenic mechanisms in Fabry disease.Frontiers in medicine · 2026
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36 more citing papers are in PubMed but not listed here.

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

7 authors at 5 institutions in 3 countries.

Behzad NajafianDepartment of Pathology, University of Washington, Seattle, Washington, USA. Electronic address: najafian@u.washington.edu.
Einar SvarstadDepartment of Medicine, Haukeland University Hospital, Bergen, Norway; Institute of Medicine, University of Bergen, Bergen, Norway.
Leif BostadDepartment of Pathology, University of Bergen, Bergen, Norway; Institute of Gade, University of Bergen, Bergen, Norway.
Marie-Claire GublerINSERM, U983, Université René Descartes, Hôpital Necker-Enfants Malades AP-HP, Paris, France.
Camilla TøndelInstitute of Medicine, University of Bergen, Bergen, Norway; Department of Pediatrics, Haukeland University Hospital, Bergen, Norway.
Chester WhitleyDepartment of Pediatrics, University of Minnesota, Minneapolis, Minnesota, USA.
Michael MauerDepartment of Pediatrics, University of Minnesota, Minneapolis, Minnesota, USA; Department of Medicine, University of Minnesota, Minneapolis, Minnesota, USA.
Haukeland University Hospital · NOUniversity of Minnesota · USHôpital Necker-Enfants Malades · FRUniversity of Bergen · NOUniversity of Washington · US

Funding

Training & EducationU54NS065768 · NINDS · UNIVERSITY OF MINNESOTA · PI WHITLEY, CHESTER B. · 2009 to 2024
$17.7M
NINDS NIH HHS U54 NS065768
6 · The paper itself

Abstract

Progressive renal failure often complicates Fabry disease, the pathogenesis of which is not well understood. To further explore this we applied unbiased stereological quantitative methods to electron microscopic changes of Fabry nephropathy and the relationship between parameters of glomerular structure and renal function in 14 young Fabry patients (median age 12 years). Renal biopsies were obtained shortly before enzyme replacement therapy from these patients and from nine normal living kidney donors as controls. Podocyte globotriaosylceramide (GL-3) inclusion volume density increased progressively with age; however, there were no significant relationships between age and endothelial or mesangial inclusion volume densities. Foot process width, greater in male Fabry patients, also progressively increased with age compared with the controls, and correlated directly with proteinuria. In comparison to the biopsies of the controls, endothelial fenestration was reduced in Fabry patients. Thus, our study found relationships between quantitative parameters of glomerular structure in Fabry nephropathy and age, as well as urinary protein excretion. Hence, podocyte injury may play a pivotal role in the development and progression of Fabry nephropathy.

Indexed as

AdolescentAge FactorsBiomarkersBiopsyCase-Control StudiesChildChild, PreschoolCreatinineDisease ProgressionEndothelial CellsEuropeFabry DiseaseFemaleGlomerular Filtration RateHumansKidney GlomerulusBiomarkersCreatinineglobotriaosylceramideTrihexosylceramides

Identifiers

PMID21160462
PMCPMC3640823
OpenAlexW2151748014

What OpenQuestion holds

Textmetadata
LicenceTDM
Read underepoch 390

Registered trials

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.