{"id":"W4313388517","doi":"10.21203/rs.3.rs-2206440/v1","title":"Consensus clinical management guidelines for Acid Sphingomyelinase Deficiency (Niemann-Pick disease types A, B and A/B)","year":2022,"lang":"en","type":"preprint","venue":"Research Square","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":3,"is_retracted":false,"has_abstract":false,"ca_institutions":"University of British Columbia","funders":"European Commission","keywords":"Acid sphingomyelinase; Sphingomyelin; Disease; Medicine; Niemann–Pick disease; Intensive care medicine; Internal medicine; Cholesterol","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":[0.02249298,0.001375952,0.002515419,0.004892376,0.0020522,0.004124307,0.005534306,0.008143575,0.006110508],"category_scores_gemma":[0.04075206,0.0007387995,0.004318752,0.003538624,0.001404275,0.002221705,0.003503392,0.007898409,0.003978614],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.003918001,"about_ca_system_score_gemma":0.01589954,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.01194759,"about_ca_topic_score_gemma":0.01235622,"domain_scores_codex":[0.987472,0.004538038,0.004464356,0.0006367026,0.001996243,0.000892717],"domain_scores_gemma":[0.9707803,0.007296626,0.003242637,0.0007625836,0.01521655,0.002701179],"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":[0.0005332833,0.0003540568,0.004276615,0.006171634,0.0004296341,0.001167844,0.0006770891,0.00225648,0.001184413,0.01546513,0.5429565,0.4245273],"study_design_scores_gemma":[0.001337196,0.0005743392,0.01670095,0.0553273,0.001678389,0.004777498,0.002113645,0.004391846,0.002285942,0.02956913,0.8809627,0.000281038],"study_design_candidate":"not_applicable","study_design_consensus":"not_applicable","genre_codex":"commentary","genre_gemma":"methods","genre_scores_codex":[0.01073058,0.3278596,0.099649,0.4314981,0.02749206,0.004815024,0.0061855,0.002700092,0.08907002],"genre_scores_gemma":[0.1029109,0.2132946,0.4871223,0.1118921,0.01150643,0.01309366,0.02539531,0.000810549,0.03397418],"genre_candidate":"methods","genre_consensus":null,"teacher_disagreement_score":0.02249298,"threshold_uncertainty_score":0.1189557,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.2723755194033582,"score_gpt":0.5346124459862932,"score_spread":0.262236926582935,"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."}}