{"id":"W2617335676","doi":"10.1002/ajh.24801","title":"Transformation in pretreatment manifestations of Gaucher disease type 1 during two decades of alglucerase/imiglucerase enzyme replacement therapy in the International Collaborative Gaucher Group (ICGG) Gaucher Registry","year":2017,"lang":"en","type":"article","venue":"American Journal of Hematology","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":56,"is_retracted":false,"has_abstract":true,"ca_institutions":"University of Calgary","funders":"National Center for Advancing Translational Sciences; National Institute of Arthritis and Musculoskeletal and Skin Diseases","keywords":"Enzyme replacement therapy; Glucocerebrosidase; Medicine; Disease; Gaucher's disease; Pediatrics; Internal medicine","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":[0.0005316593,0.0001489205,0.0004863836,0.0003028603,0.00006392839,0.00002045954,0.0003716,0.00004228561,0.0001562774],"category_scores_gemma":[0.0003617382,0.0001058034,0.0001017513,0.0002791252,0.0004255605,0.0002166076,0.00002780197,0.0002828214,0.000001742806],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0002039833,"about_ca_system_score_gemma":0.0003359503,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0004914181,"about_ca_topic_score_gemma":0.0002884573,"domain_scores_codex":[0.998098,0.0002670845,0.0007482442,0.0001618534,0.0005158699,0.0002089211],"domain_scores_gemma":[0.9980729,0.0001674386,0.0008889615,0.0003730338,0.00040279,0.00009485557],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"observational","study_design_gemma":"observational","study_design_scores_codex":[0.01861702,0.002832683,0.9364647,0.0001951962,0.0006868181,0.002305585,0.0175483,0.0001833948,0.01431705,0.001276192,0.0003454939,0.005227578],"study_design_scores_gemma":[0.01816852,0.00365675,0.9240676,0.000650418,0.0001478042,0.001613045,0.02365457,0.0004032961,0.02641584,0.0005370742,0.0004166126,0.0002684726],"study_design_candidate":"observational","study_design_consensus":"observational","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9936578,0.0009736149,0.00008387066,0.004120682,0.00005819897,0.0004658715,0.00001451101,0.000002880017,0.0006225794],"genre_scores_gemma":[0.9982133,0.001026712,0.0005164894,0.0001116453,0.00002962029,0.00003161748,0.00001441024,0.00001534173,0.0000408762],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.0123971,"threshold_uncertainty_score":0.4314537,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.02437694954253163,"score_gpt":0.3534491133295444,"score_spread":0.3290721637870128,"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."}}