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Record W2111572489 · doi:10.1002/ajmg.a.31732

Absence of <i>PITX2</i>, <i>BARX1</i>, and <i>FOXC1</i> mutations in De Hauwere syndrome (Axenfeld–Rieger anomaly, hydrocephaly, hearing loss): A 25‐year follow up

2007· article· en· W2111572489 on OpenAlexaff
R. Brian Lowry, Douglas B. Gould, Michael A. Walter, Paul R. Savage

Bibliographic record

VenueAmerican Journal of Medical Genetics Part A · 2007
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicBone Metabolism and Diseases
Canadian institutionsAlberta Health ServicesSouth Health CampusAlberta Children's HospitalUniversity of AlbertaUniversity of Calgary
Fundersnot available
KeywordsPITX2Anomaly (physics)Hearing lossMedicinePediatricsAudiologyBiologyGeneticsPhysicsGeneTranscription factor

Abstract

fetched live from OpenAlex

This study reports a 25-year follow-up of a patient with De Hauwere syndrome (Axenfeld-Rieger anomaly, hydrocephalus, and hearing loss) whose intelligence is normal. Short stature and hyperlaxity of joints later leading to severe joint pain were noted. Mutation analysis of candidate genes known or suspected to be associated with Axenfeld-Rieger eye malformations was performed. This included complete sequencing for PITX2, BARX1 and the forkhead domain of FOXC1. The results of these analyses were negative and suggest that De Hauwere syndrome is caused by a different gene.

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)0.000
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.056
Threshold uncertainty score0.686

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0010.000
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0000.000
Science and technology studies0.0000.001
Scholarly communication0.0000.000
Open science0.0000.000
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 head0.008
GPT teacher head0.267
Teacher spread0.259 · 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.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
Domainnot available
GenreEmpirical

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

Citations14
Published2007
Admission routes1
Has abstractyes

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