{"id":"W2123714825","doi":"10.1203/01.pdr.0000190579.24054.39","title":"Cardiac Functional and Histopathologic Findings in Humans and Mice with Mucopolysaccharidosis Type I: Implications for Assessment of Therapeutic Interventions in Hurler Syndrome","year":2005,"lang":"en","type":"article","venue":"Pediatric Research","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":51,"is_retracted":false,"has_abstract":false,"ca_institutions":"University of British Columbia","funders":"National Heart, Lung, and Blood Institute","keywords":"Hurler syndrome; Mucopolysaccharidosis type I; Hunter syndrome; Mucopolysaccharidosis; Medicine; Mucopolysaccharidosis I; Psychological intervention; Mucopolysaccharidosis type II; Pathology; Intensive care medicine; Internal medicine; Disease; Enzyme replacement therapy; Psychiatry","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":"codex-gemma-dda1882f352a","candidate_categories":[],"consensus_categories":[],"category_scores_codex":[0.001352643,0.00008955345,0.0002592199,0.0009009908,0.00009817383,0.00001998017,0.00008721123,0.0000766212,0.00006220597],"category_scores_gemma":[0.0001754692,0.00007515201,0.00004982414,0.0009974637,0.0001537727,0.00009487497,0.00008482642,0.000370777,0.000002499068],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.00025108,"about_ca_system_score_gemma":0.0002405652,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0001756591,"about_ca_topic_score_gemma":0.0001616567,"domain_scores_codex":[0.9986042,0.0001277541,0.0002732154,0.0003227143,0.0003422974,0.0003297979],"domain_scores_gemma":[0.9990162,0.0003976798,0.00003577725,0.0001759015,0.0002884897,0.00008600766],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"observational","study_design_gemma":"observational","study_design_scores_codex":[0.000114319,0.0003483527,0.9947255,0.0003358166,0.00001853821,0.000004981244,0.0002054178,0.000008552379,0.001764377,0.001155925,0.0001410259,0.001177176],"study_design_scores_gemma":[0.001161045,0.0007213372,0.9970653,0.00003423702,0.00003744938,0.00001795289,0.0002074696,0.0001395791,0.00001669496,0.0004212432,0.0001095573,0.00006811323],"study_design_candidate":"observational","study_design_consensus":"observational","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9916481,0.004595197,0.00026588,0.002140342,0.00001748686,0.001020578,0.00001755586,0.000008722226,0.0002861184],"genre_scores_gemma":[0.9968045,0.001418465,0.000894062,0.00001776958,0.00004342513,0.0003582873,0.00001879957,0.00001561641,0.0004290897],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.005156366,"threshold_uncertainty_score":0.306461,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.1575384698769147,"score_gpt":0.4344349063480268,"score_spread":0.2768964364711122,"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."}}