{"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":"metacan-v3-hybrid-931329e0061c","candidate_categories":[],"consensus_categories":[],"category_scores_codex":[0.0003104805,0.0004258675,0.0008728831,0.0004773324,0.0006363749,0.0005221502,0.0004614508,0.0008763908,0.003886389],"category_scores_gemma":[0.0004779144,0.00006544161,0.0003222048,0.0006665158,0.0002233763,0.000278223,0.0005660076,0.0007006377,0.001228279],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0005486965,"about_ca_system_score_gemma":0.0006928579,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.001711733,"about_ca_topic_score_gemma":0.001425017,"domain_scores_codex":[0.9997635,0.0000441718,0.00001667703,0.00003655112,0.00006894747,0.00007003317],"domain_scores_gemma":[0.9998682,0.0000182497,0.00003257657,0.000007637032,0.00002598579,0.00004735792],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"design_other","study_design_gemma":"not_applicable","study_design_scores_codex":[0.0008918391,0.0008290798,0.03939544,0.004131175,0.0003911055,0.0409964,0.0003893642,0.0005291087,0.01888173,0.007057787,0.1015997,0.7849073],"study_design_scores_gemma":[0.0003811705,0.001162574,0.09448082,0.004165809,0.0008416871,0.2286647,0.0005734339,0.001372146,0.006292833,0.009661253,0.6523194,0.00008414109],"study_design_candidate":"not_applicable","study_design_consensus":null,"genre_codex":"review","genre_gemma":"review","genre_scores_codex":[0.1633215,0.7446303,0.01261429,0.01831937,0.001648799,0.0003190869,0.001844109,0.001123663,0.05617876],"genre_scores_gemma":[0.7480804,0.2126041,0.005326959,0.007783416,0.002553519,0.0001876975,0.002598771,0.00008129725,0.02078388],"genre_candidate":"review","genre_consensus":"review","teacher_disagreement_score":0.003886389,"threshold_uncertainty_score":0.01300132,"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."}}