{"id":"W4411266312","doi":"10.1164/rccm.202502-0512oc","title":"Long-Term Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor in Children ≥6 Years with Cystic Fibrosis and at Least One <i>F508del</i> Allele: A 192-Week, Phase 3, Open-Label Extension Study","year":2025,"lang":"en","type":"article","venue":"American Journal of Respiratory and Critical Care Medicine","topic":"Cystic Fibrosis Research Advances","field":"Medicine","cited_by":10,"is_retracted":false,"has_abstract":true,"ca_institutions":"St. Michael's Hospital; BC Children's Hospital; University of Toronto","funders":"Vertex Pharmaceuticals","keywords":"Ivacaftor; Medicine; Cystic fibrosis; Open label; Internal medicine; Pediatrics; Adverse effect; Cystic fibrosis transmembrane conductance regulator","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":[0.002005834,0.0006838161,0.001374985,0.0002535552,0.0003848113,0.000565924,0.0005817794,0.000913741,0.001922144],"category_scores_gemma":[0.001254437,0.0003542931,0.001179549,0.0002365225,0.0006131039,0.0007049973,0.0003380245,0.002291981,0.0005090034],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0004800055,"about_ca_system_score_gemma":0.0009303892,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.001074823,"about_ca_topic_score_gemma":0.001651805,"domain_scores_codex":[0.9993497,0.0002808152,0.00004926141,0.00009399558,0.0001107238,0.0001154765],"domain_scores_gemma":[0.9986932,0.0002057704,0.0004114514,0.00009641357,0.00009633056,0.0004969313],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"randomized_trial","study_design_gemma":"nonrandomized_trial","study_design_scores_codex":[0.7479377,0.05539893,0.03798643,0.0007686224,0.001119716,0.00066975,0.0004683211,0.001303952,0.06394733,0.0001280794,0.002055177,0.088216],"study_design_scores_gemma":[0.2797382,0.6196213,0.08955041,0.00009500211,0.0006209477,0.0006886161,0.0001373348,0.0006549709,0.006601038,0.00006550861,0.002186552,0.00004011632],"study_design_candidate":"nonrandomized_trial","study_design_consensus":null,"genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9980217,0.0007331273,0.000132045,0.00006480033,0.00001888947,0.0003254699,0.0004064279,0.00001665067,0.0002808406],"genre_scores_gemma":[0.9963102,0.0006322694,0.0006143614,0.0002138264,0.00005447413,0.0006879595,0.0007205894,0.000008843403,0.0007573895],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.002005834,"threshold_uncertainty_score":0.01060796,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.02336011345914958,"score_gpt":0.3666972141712782,"score_spread":0.3433371007121286,"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."}}