{"id":"W4415963050","doi":"10.1111/eci.70142","title":"Long‐term effectiveness and safety outcomes in adults with Fabry disease treated with agalsidase alfa: 20 years of data from the Fabry Outcome Survey","year":2025,"lang":"en","type":"article","venue":"European Journal of Clinical Investigation","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":0,"is_retracted":false,"has_abstract":true,"ca_institutions":"Dalhousie University","funders":"","keywords":"Fabry disease; Enzyme replacement therapy; Alpha-galactosidase; Young adult; Disease","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":[0.005296579,0.0001554406,0.0006601232,0.0001131335,0.00004051832,0.00003072663,0.0003872984,0.00004176936,0.00001268339],"category_scores_gemma":[0.006653425,0.00008747236,0.00007056224,0.0003719281,0.0005807601,0.0002617122,0.0001668332,0.0006083791,0.000003710019],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.00003275041,"about_ca_system_score_gemma":0.0003158215,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0002250732,"about_ca_topic_score_gemma":0.0003573293,"domain_scores_codex":[0.994478,0.003409802,0.001175831,0.0003094668,0.0004761116,0.0001507517],"domain_scores_gemma":[0.9948055,0.003478626,0.0005306849,0.0005674944,0.0003230492,0.0002946113],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"observational","study_design_gemma":"observational","study_design_scores_codex":[0.01122421,0.0002319292,0.968846,0.0001097549,0.000271638,0.0006275113,0.00008629186,0.00001340023,0.00001991227,0.000003931279,0.00007103712,0.01849441],"study_design_scores_gemma":[0.009616396,0.0006428346,0.9874245,0.001793577,0.0001950358,0.00000690728,0.00004441595,0.0001359832,0.00001618624,0.00002304289,0.00001465436,0.00008646629],"study_design_candidate":"observational","study_design_consensus":"observational","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9971383,0.0003592876,0.0007293429,0.001075389,0.000050899,0.00043557,0.0001298164,0.000007715608,0.00007368225],"genre_scores_gemma":[0.9990062,0.0001111234,0.0002230027,0.0003267091,0.00005299383,0.000001256598,0.0001981648,0.00002362161,0.00005696228],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.01857852,"threshold_uncertainty_score":0.7965252,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.1166551607942707,"score_gpt":0.402131579719151,"score_spread":0.2854764189248803,"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."}}