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Record W2623299408 · doi:10.14740/jmc.v8i6.2832

Joubert Syndrome: A Rare Case Report From Saudi Arabia

2017· article· en· W2623299408 on OpenAlexvenueno aff
Khulood M. Alharbi

Bibliographic record

VenueJournal of Medical Cases · 2017
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicGenetic and Kidney Cyst Diseases
Canadian institutionsnot available
Fundersnot available
KeywordsMedicineHypotoniaFailure to thriveJoubert syndromePediatricsNystagmusGlobal developmental delayRespiratory distressFourth ventricleMagnetic resonance imagingHypoplasiaBulbar palsyRare diseaseSurgeryRadiologyDiseasePathology

Abstract

fetched live from OpenAlex

Joubert syndrome (JS) is a rare genetic disorder. We report a 15-month-old girl who presented with developmental delay, decrease vision, failure to thrive and history of two hospital admissions with respiratory distress and recurrent aspiration pneumonia due to gastroesophageal reflux disease. On examination, she had facial dysmorphism, nystagmus, retinopathy and hypotonia. Brain magnetic resonance imaging revealed cerebellar vermis hypoplasia, abnormally thick superior cerebellar peduncles, a “batwing” configuration of the fourth ventricle, and the characteristic molar tooth sign of mid-brain suggestive of JS. Patient was referred to the genetic department outside our hospital where the diagnosis of JS was confirmed. In conclusion, awareness of the characteristic clinical and radiological findings in JS will help in early diagnosis, appropriate counseling and proper rehabilitation. J Med Cases. 2017;8(6):180-182 doi: https://doi.org/10.14740/jmc2832w

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)0.002
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Case report · Consensus signal: Case report
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.004
Threshold uncertainty score0.009

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0000.002
Meta-epidemiology (narrow)0.0020.001
Meta-epidemiology (broad)0.0010.001
Bibliometrics0.0030.001
Science and technology studies0.0020.001
Scholarly communication0.0020.002
Open science0.0010.002
Research integrity0.0030.001
Insufficient payload (model declined to judge)0.0030.001

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 head0.018
GPT teacher head0.300
Teacher spread0.282 · 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 designCase report
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

Citations1
Published2017
Admission routes1
Has abstractyes

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