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Record W2093495067 · doi:10.1159/000143406

Estimating Disease Risk Associated with Mutated Genes in Family-Based Designs

2008· article· en· W2093495067 on OpenAlex

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.
fundA Canadian funder is recorded on the work.
aboutThe title or abstract carries a Canadian signal from the geographic lexicon.

Bibliographic record

VenueHuman Heredity · 2008
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicGenetic Associations and Epidemiology
Canadian institutionsAlberta Cancer FoundationLunenfeld-Tanenbaum Research InstituteMount Sinai Hospital
FundersInstitute of GeneticsCanadian Institutes of Health Research
KeywordsPedigree chartPenetranceProbandGeneticsMutationPopulationFamily historyBiologyComputer scienceStatisticsEconometricsGeneMathematicsMedicineInternal medicineEnvironmental health

Abstract

fetched live from OpenAlex

OBJECTIVE: Many clinical decisions require accurate estimates of disease risk associated with inherited gene mutations. While several family-based designs have been proposed, their relative advantages remain unclear. METHODS: We considered four commonly-used family-based designs and evaluated their performance in terms of accuracy and efficiency under several genetic models via simulation studies. We also derived and assessed several ascertainment-corrected likelihood methods for analyzing the simulated data and real data from 12 HNPCC pedigrees from Newfoundland. RESULTS: We found that the design efficiency depends on the question of interest: the clinic-based family design with random probands yields the most efficient estimate of genetic relative risks, whereas the population-based family design with mutation carrier probands provides the most efficient penetrance estimates. For a particular question, an ascertainment correction seems possible using regular likelihood methods but the presence of genetic heterogeneity due to a strong second gene effect can lead to some bias in the risk estimation. CONCLUSIONS: This work gives a general methodological framework for analyzing family-based designs in gene characterization studies and provides more rationale for the choice of an efficient design and an appropriate likelihood method to estimate the risk associated with an inherited gene mutation.

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)0.001
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 score0.020
Threshold uncertainty score0.589

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.001
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 head0.050
GPT teacher head0.282
Teacher spread0.232 · 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