MétaCan
Menu
Back to cohort
Record W4402838373 · doi:10.1016/j.gim.2024.101282

Genomic insights from a deeply phenotyped highly consanguineous neurodevelopmental disorders cohort

2024· article· en· W4402838373 on OpenAlexaff
Hosneara Akter, Atikur Rahaman, Tamannyat Binte Eshaque, Nesrin Mohamed, Amirul Islam, Md. Adnan Morshed, Zaha Shahin, Al Muhaimin, Arif Md Foyzullah, Rabeya Akter Mim, Farjana Binta Omar, Md. Nahid Hasan, Dharana Satsangi, Nahid Ahmed, Abdullah Al Saba, Nargis Jahan, Md. Arif Hossen, MA Mondol, Ahammad Sharif Sakib, Rezwana Kabir, Mohammod Shah Jahan Chowdhury, Nusrat Shams, Shireen Afroz, Shayla Imam Kanta, Sarwar Jahan Bhuiyan, Rabi Biswas, Shehzad Hanif, Richa Tambi, Nasna Nassir, Muhammad Mizanur Rahman, Jinjie Duan, Anders D. Børglum, Md Robed Amin, Mohammed Basiruzzaman, Md Kamruzzaman, Shaoli Sarker, Marc Woodbury‐Smith, K. M. Furkan Uddin, AHM Nurun Nabi, Mohammed Uddin

Bibliographic record

VenueGenetics in Medicine · 2024
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicGenomics and Rare Diseases
Canadian institutionsGenome Canada
FundersCollege of Medicine, Drexel UniversityMohammed Bin Rashid University of Medicine and Health SciencesAl Jalila Foundation
KeywordsMedicineGeneticsCohortIntellectual disabilityPediatricsBiologyInternal medicine

Abstract

fetched live from OpenAlex

PURPOSE: The genetic underpinning of neurodevelopmental disorders (NDDs) in diverse ethnic populations, especially those with high rates of consanguinity, remains largely unexplored. Here, we aim to elucidate genomic insight from 576 well-phenotyped and highly consanguineous (16%) NDD cohort. METHODS: We used chromosomal microarray (CMA; N:247), exome sequencing (ES; N:127), combined CMA and ES (N:202), and long-read genome sequencing to identify genetic etiology. Deep clinical multivariate data were coupled with genomic variants for stratification analysis. RESULTS: Genetic diagnosis rates were 17% with CMA, 29.92% with ES, and 37.13% with combined CMA and ES. Notably, children of consanguineous parents showed a significantly higher diagnostic yield (P < .01) compared to those from nonconsanguineous parents. Among the ES-identified pathogenic variants, 36.19% (38/105) were novel, implicating 35 unique genes. Long-read sequencing of seizure participants unresolved by combined test identified expanded FMR1 trinucleotide repeats. Additionally, we identified 2 recurrent X-linked variants in the G6PD in 3.65% (12/329) of NDD participants. These variants were absent in large-population control cohorts and cohort comprising neurodevelopmental and neuropsychiatric populations of European descendants, indicating a possible associated risk factor potentially resulting from ancient genetic drift. CONCLUSION: This study unveils unique clinical and genomic insights from a consanguinity rich Bangladeshi NDD cohort.

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)0.001
Version: metacan-v3-hybrid-931329e0061cValidation 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 score0.005
Threshold uncertainty score0.010

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0000.001
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0010.001
Science and technology studies0.0010.000
Scholarly communication0.0010.000
Open science0.0000.001
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0010.000

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 head0.006
GPT teacher head0.233
Teacher spread0.227 · 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 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

Citations8
Published2024
Admission routes1
Has abstractyes

Explore more

Same venueGenetics in MedicineSame topicGenomics and Rare DiseasesFrench-language works237,207