{"id":"W4243623535","doi":"10.1016/j.ymgme.2020.12.170","title":"Comparison of cognitive function in siblings with neuronopathic mucopolysaccharidosis type II: Evaluation of early treatment with intravenous idursulfase and intrathecal idursulfase-IT","year":2021,"lang":"en","type":"article","venue":"Molecular Genetics and Metabolism","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":0,"is_retracted":false,"has_abstract":false,"ca_institutions":"Hospital for Sick Children; University of Toronto","funders":"","keywords":"Hunter syndrome; Mucopolysaccharidosis type II; Intrathecal; Medicine; Cognition; Pediatrics; Anesthesia; Internal medicine; Enzyme replacement therapy; Disease; Psychiatry","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":[0.000482392,0.0005572915,0.0004897985,0.0004173729,0.0003197198,0.0002893661,0.0003474886,0.0005857283,0.0009403864],"category_scores_gemma":[0.002560386,0.0001454268,0.0004126815,0.0002440945,0.0004808522,0.0002475098,0.0002455965,0.0005052348,0.0001636586],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0004879774,"about_ca_system_score_gemma":0.0004160012,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.003374089,"about_ca_topic_score_gemma":0.001368699,"domain_scores_codex":[0.9997332,0.00007146104,0.00003743454,0.00005986217,0.00005106906,0.00004692075],"domain_scores_gemma":[0.9992338,0.0003648532,0.00008394969,0.00003233011,0.00009005706,0.0001949114],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"observational","study_design_gemma":"observational","study_design_scores_codex":[0.01527919,0.002513551,0.8779131,0.0001068247,0.0003613451,0.0564782,0.001259435,0.0008195118,0.02563725,0.0002155041,0.000266287,0.01914987],"study_design_scores_gemma":[0.0002935137,0.007780874,0.9543402,0.00001908432,0.0002445278,0.02970766,0.0008104957,0.0008747937,0.005262563,0.0001597393,0.000479213,0.00002744947],"study_design_candidate":"observational","study_design_consensus":"observational","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9991578,0.0001207239,0.00006814885,0.00003142523,0.000004468076,0.000005602321,0.00005652917,0.00000263862,0.0005524981],"genre_scores_gemma":[0.9996087,0.00008106193,0.00006721905,0.00002394197,0.000005740101,0.000005099255,0.00009092204,0.00000255275,0.0001148234],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.003374089,"threshold_uncertainty_score":0.00670892,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.04084824515031275,"score_gpt":0.3300579981460235,"score_spread":0.2892097529957108,"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."}}