{"id":"W4400570519","doi":"10.20517/rdodj.2023.61","title":"Fabry nephropathy: a treatable cause of chronic kidney disease","year":2024,"lang":"en","type":"article","venue":"Rare Disease and Orphan Drugs Journal","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":6,"is_retracted":false,"has_abstract":true,"ca_institutions":"Université de Montréal; Queen Elizabeth II Health Sciences Centre; Dalhousie University","funders":"","keywords":"Globotriaosylceramide; Medicine; Fabry disease; Enzyme replacement therapy; Proteinuria; Albuminuria; Nephropathy; Kidney disease; Alpha-galactosidase; Internal medicine; Podocyte; Cardiomyopathy; Dialysis; Glomerular basement membrane; Kidney; Disease; Heart failure; Pathology; Urology; Endocrinology; Diabetes mellitus","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":["insufficient_payload"],"consensus_categories":[],"category_scores_codex":[0.000208496,0.0002195613,0.0003099228,0.0003117915,0.0001764163,0.0001671132,0.0001292906,0.00005045669,0.001568296],"category_scores_gemma":[0.0002391162,0.0001694703,0.0002476144,0.0003636058,0.000157313,0.0003559516,0.00008773889,0.0003744623,0.0000677276],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0001986962,"about_ca_system_score_gemma":0.002560889,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.00002437968,"about_ca_topic_score_gemma":0.000001852374,"domain_scores_codex":[0.9981812,0.00008495777,0.0003231471,0.0003241251,0.0006431494,0.0004434042],"domain_scores_gemma":[0.9969355,0.00005396432,0.00005804991,0.0002913055,0.0001558919,0.002505263],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"not_applicable","study_design_gemma":"not_applicable","study_design_scores_codex":[0.01266468,0.00367899,0.1957039,0.01793583,0.003820597,0.111699,0.005062534,0.00004907107,0.007104594,0.002319932,0.3464399,0.293521],"study_design_scores_gemma":[0.04184481,0.004488619,0.2614822,0.02868198,0.01623488,0.00645782,0.00540369,0.05452045,0.004122951,0.01345416,0.559285,0.004023341],"study_design_candidate":"not_applicable","study_design_consensus":"not_applicable","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.8670782,0.125309,0.0003270765,0.005099413,0.0004060248,0.000531094,0.0004166758,0.0001017919,0.0007307422],"genre_scores_gemma":[0.9906084,0.004571995,0.00002654822,0.0004562751,0.0007628144,0.0000194718,0.00009631762,0.00005227332,0.003405895],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.2894976,"threshold_uncertainty_score":0.9993444,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.01343904179696902,"score_gpt":0.2903369501416745,"score_spread":0.2768979083447055,"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."}}