Evidence map›Paper›PMID 30337492›Full record

ArticleArchives of disease in childhood2019

Surveillance for variant CJD: should more children with neurodegenerative diseases have autopsies?

Christopher Verity, Anne Marie Winstone, Robert Will, Alison Powell, Peter Baxter, Carlos de Sousa, Paul Gissen, Manju Kurian, John Livingston, Robert McFarland and 5 more

Open access · hybridAbstract read
In one paragraph

Article in Archives of disease in childhood, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
0.3field-weighted citation impact, top 42% 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

4 citing papers in PubMed, 9 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

15 authors at 11 institutions in 1 country.

Christopher VerityPIND Research Group, Addenbrooke's Hospital, Cambridge, UK.
Anne Marie WinstonePIND Research Group, Addenbrooke's Hospital, Cambridge, UK.
Robert WillThe National Creutzfeldt-Jakob Disease Research and Surveillance Unit, Western General Hospital, Edinburgh, UK.
Alison PowellPIND Research Group, Addenbrooke's Hospital, Cambridge, UK.
Peter BaxterPaediatric Neurology Department, Sheffield Children's NHS Foundation Trust, Sheffield, UK.
Carlos de SousaNeurology Department, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Paul GissenNeurology Department, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Manju KurianInstitute of Child Health, Great Ormond Street Hospital for Children, London, UK.
John LivingstonPaediatric Neurology Department, Leeds General Infirmary, Leeds, UK.
Robert McFarlandWellcome Trust Centre for Mitochondrial Research, Institute of Neuroscience, The Medical School, Newcastle upon Tyne, UK.
Suvankar PalThe National Creutzfeldt-Jakob Disease Research and Surveillance Unit, Western General Hospital, Edinburgh, UK.
Michael PikePaediatric Neurology Department, Oxford Children's Hospital, John Radcliffe Hospital site, Oxford, UK.
Richard RobinsonPaediatric Neurology Department, Guy's Hospital, London, UK.
Evangeline WassmerPaediatric Neurology Department, Birmingham Children's Hospital, Birmingham, UK.
Sameer ZuberiPaediatric Neurosciences Research Group, Royal Hospital for Children and School of Medicine, University of Glasgow, Glasgow, UK.
Addenbrooke's Hospital · GBGreat Ormond Street Hospital for Children NHS Foundation Trust · GBWestern General Hospital · GBBirmingham Children's Hospital · GBGreat Ormond Street Hospital · GBGuy's Hospital · GBJohn Radcliffe Hospital · GBLeeds General Infirmary · GBSheffield Children's NHS Foundation Trust · GBUniversity of Glasgow · GBWellcome Centre for Mitochondrial Research · GB

Funding

Department of Health RP-2016-07-019
6 · The paper itself

Abstract

objectivesTo report investigations performed in children with progressive neurodegenerative diseases reported to this UK study.

designSince 1997 paediatric surveillance for variant Creutzfeldt-Jakob disease (vCJD) has been performed by identifying children aged less than 16 years with progressive intellectual and neurological deterioration (PIND) and searching for vCJD among them.

settingThe PIND Study obtains case details from paediatricians who notify via the British Paediatric Surveillance Unit.

participantsBetween May 1997 and October 2017, a total of 2050 cases meeting PIND criteria had been notified and investigated.

resultsSix children had vCJD. 1819 children had other diagnoses, made in 12 cases by antemortem brain biopsy and in 15 by postmortem investigations. 225 children were undiagnosed: only 3 had antemortem brain biopsies and only 14 of the 108 who died were known to have had autopsies; postmortem neuropathological studies were carried out in just 10% (11/108) and only two had prion protein staining of brain tissue. Of the undiagnosed cases 43% were known to come from Asian British families.

conclusionsMost of the notified children had a diagnosis other than vCJD to explain their neurological deterioration. None of the undiagnosed cases had the clinical phenotype of vCJD but brain tissue was rarely studied to exclude vCJD. Clinical surveillance via the PIND Study remains the only practical means of searching for vCJD in UK children.

Indexed as

AdolescentAutopsyChildChild, PreschoolConsanguinityCreutzfeldt-Jakob SyndromeDisease ProgressionFemaleHumansInfantInfant, NewbornMagnetic Resonance ImagingMaleNeurodegenerative DiseasesUnited Kingdomepidemiologyneurologyneuropathology

Identifiers

PMID30337492
PMCPMC6530073
OpenAlexW2897612127

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC
Read underepoch 390

Registered trials

None linked

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.