Bibliographic record
Abstract
Deze database bevat gegevens van alle patiënten met de ziekte van Fabry die in het Academisch Medisch Centrum (Amsterdam), Royal Free London NHS Foundation Trust (Londen, Groot-Brittannië) en het University Hospital Würzburg (Würzburg, Duitsland) gecontroleerd en behandeld worden, en tevens van patiënten uit Canada die geïncludeerd zijn in de Canadian Fabry Disease Initiative.\nDe database bestaat uit vier onderdelen:\n\nPatiëntgegevens: gegevens m.b.t. leeftijd, diagnose, mutatie en enzymactiviteit zijn gecodeerd opgeslagen.\nAnamnese: voorgeschiedenis, aanwezigheid van angiokeratomen, an-/hypohydrosis, acroparesthesiëen en cornea verticillata. Verder; fenotype (klassieke/niet klassieke ziekte of onzekere diagnose), medicatie en het wel of niet krijgen van enzymtherapie.\nAanvullende onderzoeken: biochemie, lab- en urine uitslagen, aanvullend onderzoek (MRI hersenen/MRI hart/echo hart/echo nier/gehooronderzoek/ECG/ev. biopt).\nVragenlijsten: BPI, EQ-5D en SF-36.\n\nDe resultaten van de aanvullende onderzoeken en vragenlijsten zijn tot 2017 ingevuld in de database.\nNb: deze database is wel operationeel, maar er worden niet actief meer gegevens aan de database toegevoegd.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot 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.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.002 | 0.016 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.008 | 0.008 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.004 | 0.002 |
| Open science | 0.002 | 0.003 |
| Research integrity | 0.002 | 0.001 |
| Insufficient payload (model declined to judge) | 0.275 | 0.195 |
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.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
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".