{"id":"W1997440200","doi":"10.1016/j.ymgme.2009.10.178","title":"A validated disease severity scoring system for Fabry disease","year":2009,"lang":"en","type":"article","venue":"Molecular Genetics and Metabolism","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":72,"is_retracted":false,"has_abstract":false,"ca_institutions":"Hospital for Sick Children; University of Toronto; SickKids Foundation; Dalhousie University; Université de Sherbrooke; Université de Montréal; Hôpital du Sacré-Cœur de Montréal","funders":"Ministerul Cercetării, Inovării şi Digitalizării","keywords":"Fabry disease; Concordance; Convergent validity; Construct validity; Disease; Medicine; Severity of illness; Reliability (semiconductor); Internal medicine; Psychometrics; Clinical psychology; Internal consistency","routes":{"ca_aff":true,"ca_fund":false,"ca_venue":false,"about_ca":false,"invisible_to_affiliation_only":false},"retraction":null,"screen":null,"direct_labels":[],"prediction":{"model_version":"metacan-v3-hybrid-931329e0061c","candidate_categories":[],"consensus_categories":[],"category_scores_codex":[0.004033516,0.0009178896,0.001198538,0.003171694,0.0005124396,0.0009643046,0.0008507059,0.0006874281,0.002482284],"category_scores_gemma":[0.006667147,0.0002159877,0.001214424,0.001477696,0.0004265678,0.0007912412,0.001113942,0.0007888336,0.0008273366],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.001020025,"about_ca_system_score_gemma":0.001445993,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.002114441,"about_ca_topic_score_gemma":0.003434982,"domain_scores_codex":[0.9977605,0.0005988436,0.000517629,0.0002514184,0.0006982986,0.0001732044],"domain_scores_gemma":[0.9967526,0.0007663367,0.0007441366,0.0001896227,0.001356198,0.0001909738],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"observational","study_design_gemma":"observational","study_design_scores_codex":[0.002963936,0.0006804556,0.7023588,0.0008980344,0.001349665,0.0006582945,0.000494593,0.006256924,0.02834328,0.002412125,0.02335723,0.2302266],"study_design_scores_gemma":[0.0007925074,0.002595178,0.9412368,0.0002771251,0.0007055317,0.004254272,0.0002608701,0.0204742,0.01051744,0.001913033,0.01681215,0.0001609505],"study_design_candidate":"observational","study_design_consensus":"observational","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.888337,0.004462751,0.06668606,0.0007524707,0.0004822784,0.00387353,0.01430512,0.001120863,0.0199799],"genre_scores_gemma":[0.8647206,0.001130595,0.1067936,0.0003690598,0.0001232987,0.002596471,0.01902139,0.0001556935,0.005089333],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.004033516,"threshold_uncertainty_score":0.02133155,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.01627455212714097,"score_gpt":0.2864332693373287,"score_spread":0.2701587172101877,"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."}}