{"id":"W4318773662","doi":"10.1016/j.jaip.2023.01.032","title":"Updated Management Guidelines for Adenosine Deaminase Deficiency","year":2023,"lang":"en","type":"article","venue":"The Journal of Allergy and Clinical Immunology In Practice","topic":"Virus-based gene therapy research","field":"Biochemistry, Genetics and Molecular Biology","cited_by":25,"is_retracted":false,"has_abstract":false,"ca_institutions":"University of Manitoba; SickKids Foundation; Hospital for Sick Children; CancerCare Manitoba; University of Toronto","funders":"","keywords":"Adenosine deaminase deficiency; Adenosine deaminase; Enzyme replacement therapy; Medicine; Asymptomatic; Hematopoietic stem cell transplantation; Genetic enhancement; Sibling; Severe combined immunodeficiency; Immunodeficiency; Transplantation; Immunology; Primary immunodeficiency; Pediatrics; Internal medicine; Disease; Immune system; Adenosine; Biology; Gene; Psychology; Genetics","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":[0.004243949,0.00009563628,0.0001695788,0.00008625748,0.00009421237,0.00001185763,0.0003715099,0.0001486775,0.00001284047],"category_scores_gemma":[0.001914255,0.00006556636,0.00007903152,0.0001876746,0.0002078724,0.00001313417,0.0001753141,0.0002976609,0.00001095693],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.00000810238,"about_ca_system_score_gemma":0.00007274363,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.000006140012,"about_ca_topic_score_gemma":0.0000576224,"domain_scores_codex":[0.9982925,0.0005411269,0.0006721523,0.0001432923,0.0001207788,0.0002301287],"domain_scores_gemma":[0.9984087,0.0005381177,0.0003121354,0.000253356,0.0004471516,0.0000404853],"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":[0.2533448,0.003692573,0.001392215,0.00005219142,0.02501716,0.0006561484,0.000597007,0.003826601,0.2530514,0.003157466,0.1626666,0.2925458],"study_design_scores_gemma":[0.02548676,0.06248617,0.03385592,0.0001831911,0.001779835,0.001625703,0.01259189,0.001237877,0.03330176,0.004401678,0.8220567,0.0009925778],"study_design_candidate":"not_applicable","study_design_consensus":null,"genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9754605,0.008022241,0.000325901,0.01540252,0.0003065569,0.0001779559,0.000002644767,0.000005477196,0.0002962153],"genre_scores_gemma":[0.5913435,0.3958363,0.003772399,0.00584335,0.0006336086,0.00002528916,0.00004901838,0.00005462844,0.00244194],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.65939,"threshold_uncertainty_score":0.2673719,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.07363850833706767,"score_gpt":0.4389759467121602,"score_spread":0.3653374383750925,"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."}}