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Record W4409277231 · doi:10.1002/pd.6786

Brain Abnormalities in Prenatally Diagnosed Rubinstein‐Taybi Syndrome

2025· review· en· W4409277231 on OpenAlexaff
Laurence Carmant, Elka Miller, Susan Blaser, Patrick Shannon, Karen Chong, David Chitayat, Shiri Shinar

Bibliographic record

VenuePrenatal Diagnosis · 2025
Typereview
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicCongenital limb and hand anomalies
Canadian institutionsHospital for Sick ChildrenUniversity of TorontoSickKids FoundationMount Sinai Hospital
Fundersnot available
KeywordsRubinstein–Taybi syndromeExome sequencingCorpus callosumCraniofacialIntellectual disabilityPrenatal diagnosisMedicineHypoplasiaPediatricsPathologyMutationFetusBiologyPregnancyAnatomyGeneticsGeneDermatologyPsychiatry

Abstract

fetched live from OpenAlex

Rubinstein-Taybi syndrome (RSTS; OMIM 180849) is a rare autosomal dominant disorder characterized by craniofacial dysmorphism, broad halluces and thumbs, variable structural abnormalities and intellectual disability. It is caused by a pathogenic variant in the CREBBP or EP300 genes. Although distinctive features are described, prenatal diagnosis is rare. We report five prenatally diagnosed cases of RSTS, all with brain abnormalities. On neurosonography, corpus callosum abnormalities were found in 3/5 cases. The remaining two cases had abnormal posterior fossas, one with vermian hypoplasia and the other with Dandy-Walker malformation (DWM). A CREBBP gene mutation was identified in all cases-two on microarray (CMA) and three on Trio-whole-exome-sequencing (Trio-WES). This report will allow for a better understanding of the fetal brain findings associated with RSTS.

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)0.000
metaresearch head score (Gemma)0.000
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Not applicable · Consensus signal: none
GenreCandidate signal: Review · Consensus signal: Review
Teacher disagreement score0.003
Threshold uncertainty score0.007

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0000.000
Meta-epidemiology (narrow)0.0010.000
Meta-epidemiology (broad)0.0010.000
Bibliometrics0.0030.002
Science and technology studies0.0000.000
Scholarly communication0.0000.001
Open science0.0010.000
Research integrity0.0010.001
Insufficient payload (model declined to judge)0.0020.001

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 head0.014
GPT teacher head0.281
Teacher spread0.267 · 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 designNot applicable
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
Published2025
Admission routes1
Has abstractyes

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