{"id":"W4320036799","doi":"10.51731/cjht.2023.572","title":"Design, Rationale, and Preliminary Results of the Canadian Homozygous Familial Hypercholesterolemia Registry: 2008 to 2022 Update","year":2023,"lang":"en","type":"article","venue":"Canadian Journal of Health Technologies","topic":"Lipoproteins and Cardiovascular Health","field":"Medicine","cited_by":5,"is_retracted":false,"has_abstract":true,"ca_institutions":"","funders":"Canadian Institutes of Health Research","keywords":"Familial hypercholesterolemia; Medicine; Patient registry; Orphan drug; Disease; Atherosclerotic cardiovascular disease; Disease registry; Apheresis; Pediatrics; Intensive care medicine; Cholesterol; Family medicine; Internal medicine; Bioinformatics","routes":{"ca_aff":false,"ca_fund":true,"ca_venue":false,"about_ca":true,"invisible_to_affiliation_only":true},"retraction":null,"screen":null,"direct_labels":[],"prediction":{"model_version":"codex-gemma-dda1882f352a","candidate_categories":[],"consensus_categories":[],"category_scores_codex":[0.001945201,0.0001325086,0.0004342441,0.0007845656,0.0004438713,0.00002353175,0.0003224041,0.0001899361,0.000003561726],"category_scores_gemma":[0.001162816,0.00009741192,0.00009868429,0.0008685022,0.0002326503,0.00005819968,0.0000408361,0.0005496012,0.000005470491],"about_ca_system_candidate":true,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.000612821,"about_ca_system_score_gemma":0.01193241,"about_ca_topic_candidate":true,"about_ca_topic_consensus":true,"about_ca_topic_score_codex":0.2730078,"about_ca_topic_score_gemma":0.4299745,"domain_scores_codex":[0.9981692,0.0001389176,0.0006192164,0.0001879452,0.0003562798,0.0005284817],"domain_scores_gemma":[0.9984536,0.00004435839,0.0002789799,0.0005787059,0.0002618596,0.0003824629],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"design_other","study_design_gemma":"not_applicable","study_design_scores_codex":[0.0005236384,0.00001968392,0.003639427,0.0005682957,0.0002106778,0.001487726,0.001405889,0.0005424531,0.0001897597,0.0002726541,0.1940092,0.7971305],"study_design_scores_gemma":[0.00296624,0.002890639,0.212596,0.001223056,0.00008549373,0.004816581,0.004000911,0.00007692321,0.001001113,0.00105876,0.7689798,0.0003045452],"study_design_candidate":"not_applicable","study_design_consensus":null,"genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.5802014,0.03609777,0.0002454075,0.3792358,0.0008473968,0.00259085,0.0004520695,0.0001122203,0.0002169905],"genre_scores_gemma":[0.9957426,0.001259336,0.0008441369,0.001795303,0.00004139294,0.00001950129,0.000009879282,0.00001964388,0.0002682682],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.796826,"threshold_uncertainty_score":0.993669,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.0310804090823588,"score_gpt":0.2634483542726371,"score_spread":0.2323679451902783,"validation_status":"score_only:v0-immature-baseline","note":"Baseline scores from an immature model (maturity gate not passed). Scores rank; they never assert a category."}}