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Record W4412961724 · doi:10.1016/j.gimo.2025.103446

Pathogenic variation underlying rare diseases in an Arab population: Implications for screening programs

2025· article· en· W4412961724 on OpenAlex
Ruchi Jain, Sami Bizzari, Sathishkumar Ramaswamy, Khaleem F. Hasham, Shruti Sinha, Ikram Chekroun, Fatma Rabea, Eman Abuijlan, Maha El Naofal, Massomeh Sheikh Hassani, Shruti Shenbagam, Alan Taylor, Mohammed Uddin, Mohamed A. Almarri, Omer S. Alkhnbashi, Hamda Khansaheb, Hanan Al Suwaidi, Stefan S. du Plessis, Stephany El‐Hayek, Alawi Alsheikh‐Ali, Ahmad Abou Tayoun

Why this work is in the frame

A frame that forgets how it found something cannot be audited. These are the routes that admitted this work.

affAt least one author lists a Canadian institution in the pinned OpenAlex snapshot.

Bibliographic record

VenueGenetics in Medicine Open · 2025
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicGenomics and Rare Diseases
Canadian institutionsGenome Canada
Fundersnot available
KeywordsVariation (astronomy)PopulationBiologyMedicineEnvironmental health

Abstract

fetched live from OpenAlex

Purpose: Genetic variation underlying rare diseases in Arab populations is poorly understood limiting effective carrier screening for recessive disorders, which are prevalent because of high consanguineous rates. Methods: Using the American College of Medical Genetics and Genomics/Association for Molecular Pathology guidelines, we curated pathogenic (P) and likely pathogenic (LP) variants in 1333 Arab Emirati families (346 internal cohort and 987 from the literature). We also analyzed P/LP variants in 1194 Emirati exomes, calculated allele frequencies, and estimated carrier rates for the associated recessive conditions. Results: (HGNC:34) (4.3%). Using a provisional gene list for carrier screening, based on our analysis, we estimated an at-risk couples rate of 4% to 21%, which varies across different screening panels recommended in other populations. Conclusion: Our findings emphasize the necessity of identifying prevalent diseases in underrepresented populations to develop effective and equitable preventive public health measures, including premarital screening programs.

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.

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)0.000
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 score0.169
Threshold uncertainty score0.458

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.000
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0000.000
Science and technology studies0.0000.000
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 head0.063
GPT teacher head0.384
Teacher spread0.321 · 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