{"id":"W4387728974","doi":"10.1017/s1047951123003347","title":"Natural history of cardiac findings in mucopolysaccharidosis type I: report from an international registry","year":2023,"lang":"en","type":"article","venue":"Cardiology in the Young","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":0,"is_retracted":false,"has_abstract":true,"ca_institutions":"BC Children's Hospital; University of British Columbia","funders":"Sanofi","keywords":"Medicine; Mucopolysaccharidosis type I; Cardiology; Internal medicine; Ejection fraction; Diastole; Cardiomyopathy; Mucopolysaccharidosis; Natural history; Mitral valve; Hurler syndrome; Mitral regurgitation; Regurgitation (circulation); Heart failure; Disease; Enzyme replacement therapy; Blood pressure","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":[0.0009532833,0.0002040891,0.0003625024,0.001225171,0.000311665,0.0006288862,0.0003779862,0.000276444,0.0009203421],"category_scores_gemma":[0.002478658,0.0001871901,0.0003257459,0.00135112,0.0002724859,0.0005482388,0.0005076119,0.0004390156,0.0002516581],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0002080754,"about_ca_system_score_gemma":0.0003374144,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.003052711,"about_ca_topic_score_gemma":0.002003499,"domain_scores_codex":[0.9991546,0.0002352051,0.0001096959,0.0002674587,0.0001426116,0.00009051227],"domain_scores_gemma":[0.9957762,0.000448055,0.002403326,0.0004590111,0.0004210631,0.0004923987],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"observational","study_design_gemma":"observational","study_design_scores_codex":[0.0000308828,0.00001245127,0.9989619,0.000004176006,0.00001586584,0.00004164916,0.00004383691,0.00001490317,0.00008881459,0.000007493749,0.00008036611,0.0006976661],"study_design_scores_gemma":[0.000002347023,0.00004034195,0.9993112,0.000003133678,0.00001404259,0.0003103521,0.00009278306,0.00004468181,0.00003702638,0.000004686704,0.0001362611,0.000003121806],"study_design_candidate":"observational","study_design_consensus":"observational","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9972766,0.000265543,0.00009593998,0.00003489809,0.000003786568,0.00001189374,0.001537801,0.00001137637,0.0007621329],"genre_scores_gemma":[0.9979084,0.0002367855,0.00008134667,0.00002202866,0.00000915288,0.0000167311,0.001622489,0.000004761339,0.00009841794],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.003052711,"threshold_uncertainty_score":0.006069899,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.03441312207536541,"score_gpt":0.3299752119498775,"score_spread":0.2955620898745121,"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."}}