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Record W4283330635 · doi:10.1002/pbc.29869

Defining vascular anomaly phenotypes in children based on a systematic literature search: A critical step in developing a single severity score for interventional clinical trials

2022· review· en· W4283330635 on OpenAlexaff
Laurence Gariépy‐Assal, Josée Dubois, Kelley Zwicker, Alix Pincivy, Julie Powell, Yang Zhang, Vicky R. Breakey, Victoria Price, Leonardo R. Brandão, Manuel Carção, Niina Kleiber

Bibliographic record

VenuePediatric Blood & Cancer · 2022
Typereview
Languageen
FieldMedicine
TopicVascular Malformations and Hemangiomas
Canadian institutionsPublic Health OntarioHospital for Sick ChildrenUniversity of TorontoDalhousie UniversityBank of CanadaChildren's Hospital of Eastern OntarioUniversity of OttawaUniversité de MontréalMcMaster UniversityCentre Hospitalier Universitaire Sainte-Justine
Fundersnot available
KeywordsMedicinePhenotypeClinical trialClinical phenotypeBioinformaticsIntensive care medicineInternal medicineGeneGenetics

Abstract

fetched live from OpenAlex

INTRODUCTION: Genetically targeted drugs in vascular anomalies (VA) are used despite the absence of a validated severity score. The aim of this study was to evaluate the feasibility of grouping phenotypic VA clinical characteristics into a single severity score. METHODS: A systematic literature review including children treated with sirolimus accompanied by a detailed description of phenotype and management was conducted. Demographic data and clinical features were extracted to define distinct categories of phenotypes. RESULTS: Children with VA display two main phenotypes regardless of VA subtype, which may overlap. A systemic phenotype results from direct invasion and compression of vital structures generally leading to hospitalization and aggressive management in infancy. A functional phenotype is associated with chronic pain and disability manifesting mainly during early adolescence and managed in the outpatient setting. CONCLUSION: The two distinct phenotypes described could be the basis for developing a unified scoring system for VA severity assessment.

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 machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.025
metaresearch head score (Gemma)0.058
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Systematic review · Consensus signal: Systematic review
GenreCandidate signal: Review · Consensus signal: Review
Teacher disagreement score0.025
Threshold uncertainty score0.133

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0250.058
Meta-epidemiology (narrow)0.0010.001
Meta-epidemiology (broad)0.0100.006
Bibliometrics0.0140.010
Science and technology studies0.0010.001
Scholarly communication0.0030.003
Open science0.0020.002
Research integrity0.0010.001
Insufficient payload (model declined to judge)0.0040.000

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 head0.174
GPT teacher head0.439
Teacher spread0.265 · 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 source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
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

Citations2
Published2022
Admission routes1
Has abstractyes

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Same venuePediatric Blood & CancerSame topicVascular Malformations and HemangiomasFrench-language works237,207