{"id":"W2106564695","doi":"10.1007/s12519-009-0062-x","title":"Progression of organ manifestations upon enzyme replacement therapy in a patient with mucopolysaccharidosis type I/Hurler","year":2009,"lang":"en","type":"article","venue":"World Journal of Pediatrics","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":13,"is_retracted":false,"has_abstract":false,"ca_institutions":"BC Children's Hospital","funders":"","keywords":"Enzyme replacement therapy; Medicine; Hurler syndrome; Mucopolysaccharidosis; Mucopolysaccharidosis type I; Mucopolysaccharidosis I; Pediatric surgery; Pediatrics; Pathology; Dermatology; Surgery; Disease","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":[0.0002592237,0.0007428008,0.001075211,0.001253274,0.001054322,0.0007079398,0.0008079046,0.001659014,0.001627403],"category_scores_gemma":[0.001983609,0.0004636463,0.0008313488,0.000806731,0.0007594089,0.0006374427,0.000453683,0.001970987,0.0005335748],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0007226659,"about_ca_system_score_gemma":0.0004740599,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.003889225,"about_ca_topic_score_gemma":0.00298572,"domain_scores_codex":[0.9997645,0.00003512523,0.00003413733,0.00005260054,0.00002883261,0.00008483716],"domain_scores_gemma":[0.9985846,0.00055348,0.0002114052,0.0000964258,0.0000918046,0.0004623444],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"case_report","study_design_gemma":"case_report","study_design_scores_codex":[0.0009573632,0.0001678899,0.02775218,0.000062143,0.00002578443,0.9609601,0.0004762884,0.0001726167,0.004790709,0.00009578043,0.0002865336,0.004252601],"study_design_scores_gemma":[0.0001954855,0.002489045,0.1501506,0.00003463693,0.0002126095,0.8358402,0.0004878023,0.001319305,0.007620052,0.0002475185,0.001322998,0.00007968617],"study_design_candidate":"case_report","study_design_consensus":"case_report","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9895303,0.001301995,0.0006371862,0.0007997468,0.00007040924,0.00007060417,0.00035814,0.0001432567,0.007088379],"genre_scores_gemma":[0.998453,0.0003381971,0.0002273833,0.0002001505,0.00006828274,0.000006123577,0.00008216668,0.00001459597,0.0006099879],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.003889225,"threshold_uncertainty_score":0.007733166,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.01841437951471032,"score_gpt":0.3123884275485492,"score_spread":0.2939740480338389,"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."}}