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Record W4403612001 · doi:10.11124/jbies-24-00091

Barriers and facilitators to designing, maintaining, and utilizing rare disease patient registries: a scoping review protocol

2024· review· en· W4403612001 on OpenAlexaffabout
Catherine Stratton, Andrew Taylor, M. Konstantinidis, Vanda McNiven, Pekka Kannus, Peter Gill, Ian Stedman, Areti Angeliki Veroniki, Martin Offringa, Beth K. Potter, Durhane Wong‐Rieger, J Adams, Kathy Hodgkinson, Alison M. Elliott, Alexandra Neville, Marie E. Faughnan, Sarah Dyack, Pavel Zhelnov, Jennifer Daly-Cyr, Jessie McGowan, Sharon E. Straus, Maureen A. Smith, Laura C. Rosella, Andrea C. Tricco

Bibliographic record

VenueJBI Evidence Synthesis · 2024
Typereview
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicGenomics and Rare Diseases
Canadian institutionsDalhousie UniversityUniversity of British ColumbiaMemorial University of NewfoundlandUniversity of OttawaInstitute for Clinical Evaluative SciencesUniversity of AlbertaHealth CanadaMcMaster UniversitySickKids FoundationYork UniversityHospital for Sick ChildrenPublic Health OntarioUniversity of TorontoSt. Michael's Hospital
Fundersnot available
KeywordsPsycINFOGrey literatureMEDLINECochrane LibrarySystematic reviewInclusion (mineral)Psychological interventionProtocol (science)Agency (philosophy)MedicineMedical educationComputer sciencePsychologyAlternative medicineNursingPathology

Abstract

fetched live from OpenAlex

OBJECTIVE: The objectives of this review are to identify barriers and facilitators to designing, maintaining, and utilizing rare disease patient registries; determine whether and how these differ among patient partners, other knowledge users, and researchers; and chart definitions of rare diseases and rare disease patient registries. INTRODUCTION: Rare disease patient registries are vital to improving the understanding of the natural histories and predictors of outcomes of rare diseases, assessing interventions, and identifying potential participants for clinical trials. Currently, however, the functionality of rare disease patient registries is not fully optimized. To improve the quality and functionality of rare disease patient registries, it is important to understand the barriers and/or facilitators involved in their design, maintenance, and utilization; how these might differ among patient partners, other knowledge users, and researchers; and to delineate the range of definitions for rare diseases and rare disease patient registries. INCLUSION CRITERIA: Evidence from any study design or format (including empirical studies, books, manuals, commentaries, editorials, guidance documents, conference abstracts, review documents, and gray literature) that references barriers or facilitators for designing, maintaining, or utilizing rare disease patient registries will be considered for inclusion. METHODS: The review will follow the JBI methodology for scoping reviews. We will search health science databases, including the Cochrane Library, Embase, MEDLINE, the JBI EBP Database, and PsycINFO, from inception onwards. Multiple sources will be searched for theses and gray literature, including the Canadian Agency for Drugs and Technologies in Health (CADTH) Grey Matters. Two independent reviewers will screen titles and abstracts and full-text documents, as well as abstract data. Disagreements will be resolved through discussion or with a third reviewer. Evidence will be synthesized descriptively and reported using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews (PRIMSA-ScR). REVIEW REGISTRATION: Open Science Framework https://osf.io/mvf9r.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame distilled prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.

metaresearch head score (Codex)0.001
metaresearch head score (Gemma)0.006
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesMeta-epidemiology (narrow)
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Systematic review · Consensus signal: none
GenreCandidate signal: Review · Consensus signal: Review
Teacher disagreement score0.549
Threshold uncertainty score1.000

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0010.006
Meta-epidemiology (narrow)0.0010.000
Meta-epidemiology (broad)0.0010.000
Bibliometrics0.0000.000
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.001
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0000.000

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.024
GPT teacher head0.357
Teacher spread0.333 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one teacher head, not a consensus.

Study designSystematic review
Domainnot available
GenreReview

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations1
Published2024
Admission routes2
Has abstractyes

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