Evidence map›Paper›PMID 28056875›Full record

ArticleBMC nephrology2017

Turkish pediatric atypical hemolytic uremic syndrome registry: initial analysis of 146 patients.

Nesrin Besbas, Bora Gulhan, Oguz Soylemezoglu, Z Birsin Ozcakar, Emine Korkmaz, Mutlu Hayran, Fatih Ozaltin

Abstract read
In one paragraph

Article in BMC nephrology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 4 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
25citing papers in PubMed, 4 pooled it
–field-weighted citation impact
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

25 citing papers in PubMed, 4 syntheses or guidelines pooled it.

  1. Pooled it
  2. Guideline
  3. Pooled it
  4. Pooled it
  5. Review
  6. Review
  7. Article
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  15. Review
  16. Hemolytic Uremic Syndrome in Children.Turkish archives of pediatrics · 2021
    Article
  17. Article
  18. Article
  19. Article
  20. Extra-renal manifestations of atypical hemolytic uremic syndrome.Pediatric nephrology (Berlin, Germany) · 2019
    Review
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.

Nesrin BesbasDepartment of Pediatric Nephrology, Nephrogenetics Laboratory, Hacettepe University Faculty of Medicine, Sihhiye, 06100, Ankara, Turkey.
Bora GulhanDepartment of Pediatric Nephrology, Nephrogenetics Laboratory, Hacettepe University Faculty of Medicine, Sihhiye, 06100, Ankara, Turkey.
Oguz SoylemezogluDepartment of Pediatric Nephrology, Gazi University Faculty of Medicine, Ankara, Turkey.
Z Birsin OzcakarDepartment of Pediatric Nephrology, Ankara University Faculty of Medicine, Ankara, Turkey.
Emine KorkmazDepartment of Pediatric Nephrology, Nephrogenetics Laboratory, Hacettepe University Faculty of Medicine, Sihhiye, 06100, Ankara, Turkey.
Mutlu HayranDepartment of Preventive Oncology, Hacettepe University, Ankara, Turkey.
Fatih OzaltinDepartment of Pediatric Nephrology, Nephrogenetics Laboratory, Hacettepe University Faculty of Medicine, Sihhiye, 06100, Ankara, Turkey. fozaltin@hacettepe.edu.tr.ORCID http://orcid.org/0000-0003-1194-0164

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundAtypical hemolytic uremic syndrome (aHUS) is a devastating disease with significant morbidity and mortality. Its genetic heterogeneity impacts its clinical presentation, progress, and outcome, and there is no consensus on its clinical management.

methodsTo identify the characteristics of aHUS in Turkish children, an industry-independent registry was established for data collection that includes both retrospective and prospective patients.

resultsIn total, 146 patients (62 boys, 84 girls) were enrolled; 53 patients (36.3%) were less than 2 years old at initial presentation. Among the 42 patients (37.1%) whose mutation screening was complete for CFH, CFI, MCP, CFB, C3, DGKE, and CHFR5 genes, underlying genetic abnormalities were uncovered in 34 patients (80.9%). Sixty-one patients (41.7%) had extrarenal involvement. During the acute stage, 33 patients (22.6%) received plasma therapy alone, among them 17 patients (51.5%) required dialysis, and 4 patients (12.1%) were still on dialysis at the time of discharge. In total, 103 patients (70.5%) received eculizumab therapy, 16 of whom (15.5%) received eculizumab as a first-line therapy. Plasma therapy was administered to 84.5% of the patients prior to eculizumab. In this group, renal replacement therapy was administered to 80 patients (77.7%) during the acute period. A total of 3 patients died during the acute stage. A total of 101 patients (77.7%) had a glomerular filtration rate >90 mL/min/1.73 m

conclusionsThe Turkish aHUS registry will increase our knowledge of patients with aHUS who have different genetic backgrounds and will enable evaluation of the different treatment options and outcomes.

Indexed as

RegistriesAdolescentAtypical Hemolytic Uremic SyndromeBlood TransfusionChildChild, PreschoolComorbidityFemaleGenetic Predisposition to DiseaseHumansImmunosuppressive AgentsInfantMalePilot ProjectsPrevalenceRenal DialysisImmunosuppressive AgentsAtypical hemolytic uremic syndromeOutcomePrognosisTreatmentTurkish registry

Identifiers

PMID28056875
PMCPMC5217238

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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.