{"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":"metacan-v3-hybrid-931329e0061c","candidate_categories":[],"consensus_categories":[],"category_scores_codex":[0.001578723,0.0008582032,0.001518643,0.002209637,0.0006983695,0.00170163,0.001220095,0.003652685,0.01149301],"category_scores_gemma":[0.007358117,0.0002902878,0.001630523,0.001004004,0.0004270499,0.00134396,0.0009141798,0.004779158,0.003596416],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0011549,"about_ca_system_score_gemma":0.001632769,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.003848592,"about_ca_topic_score_gemma":0.005694721,"domain_scores_codex":[0.9985739,0.000413825,0.0004286309,0.00009694933,0.0003386431,0.0001480008],"domain_scores_gemma":[0.9976382,0.0008651951,0.0003017347,0.00005650783,0.0008786414,0.0002597472],"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":[0.000205484,0.0002772476,0.002964573,0.001109112,0.0001148203,0.003175004,0.0001414284,0.0009812306,0.0008071621,0.00330493,0.5251622,0.461757],"study_design_scores_gemma":[0.0004656836,0.0002329514,0.009763534,0.01022976,0.0003181464,0.01972575,0.0002394534,0.001274195,0.0006541656,0.008100962,0.9488895,0.0001058375],"study_design_candidate":"not_applicable","study_design_consensus":"not_applicable","genre_codex":"review","genre_gemma":"methods","genre_scores_codex":[0.009291198,0.5719181,0.03162659,0.2301997,0.0250637,0.0008699498,0.003124467,0.002146559,0.1257596],"genre_scores_gemma":[0.0899315,0.4374712,0.139695,0.2072393,0.03696809,0.003125687,0.01412688,0.0007212001,0.07072119],"genre_candidate":"methods","genre_consensus":null,"teacher_disagreement_score":0.01149301,"threshold_uncertainty_score":0.03844804,"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."}}