{"doi":"10.1016/j.jcf.2017.05.002","title":"Real-life acute lung function changes after lumacaftor/ivacaftor first administration in pediatric patients with cystic fibrosis","abstract":null,"journal":"Journal of Cystic Fibrosis","year":2017,"id":616720,"datarank":0.4493598410330987,"base_score":2.995732273553991,"endowment":2.995732273553991,"self_citation_contribution":0.4493598410330987,"citation_network_contribution":0.0,"self_endowment_contribution":0.4493598410330987,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":19,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":null,"is_data_producer":false,"deposit_databanks":null,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":null,"fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1590146,"name":"Camille Ohlmann","orcid":null,"position":1,"is_corresponding":false},{"id":1590147,"name":"Catherine Mainguy","orcid":null,"position":2,"is_corresponding":false},{"id":1590148,"name":"Virginie Jubin","orcid":null,"position":3,"is_corresponding":false},{"id":1590149,"name":"Marie Perceval","orcid":null,"position":4,"is_corresponding":false},{"id":1590150,"name":"Laurianne Coutier","orcid":null,"position":5,"is_corresponding":false},{"id":845906,"name":"Philippe Reix","orcid":"0000-0002-9192-8335","position":6,"is_corresponding":false},{"id":1590145,"name":"Aurélie Labaste","orcid":null,"position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"resolved":true,"title":"Real-life acute lung function changes after lumacaftor/ivacaftor first administration in pediatric patients with cystic fibrosis","abstract":"The combination of lumacaftor and ivacaftor (LUM/IVA) has been reported to induce a mean acute absolute drop of -4.1% predicted forced expiratory volume in 1s (FEV<sub>1</sub>) after a unique administration in healthy subjects. The aim of the present study was to assess acute FEV<sub>1</sub> changes after the first dose of LUM/IVA in CF patients. A total of 32 pediatric patients were included. Respiratory manifestations occurred in only 3 patients (9.4%), but FEV<sub>1</sub> consistently decreased (-10.4±4.6%, range: -1.5; -21.8%). FEV<sub>1</sub> only partially resumed after salbutamol inhalation. Patients with previously known significant reversible airway obstruction and low FEV<sub>1</sub> were more at risk of FEV<sub>1</sub> decrease.","is_dataset_classified":null,"base_score":2.995732273553991,"endowment":2.995732273553991,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"28529073","pmcid":null,"openalex_id":"https://openalex.org/W2615423082","authors":[],"funders":[],"total_grants":0,"fwci":2.7556,"citation_percentile":0.90883379,"influential_citations":0,"citation_trend":[{"year":2017,"count":1},{"year":2018,"count":3},{"year":2019,"count":6},{"year":2020,"count":5},{"year":2021,"count":2},{"year":2023,"count":1},{"year":2025,"count":1}],"oa_status":"bronze","license":"http://www.elsevier.com/open-access/userlicense/1.0/","oa_locations":[{"url":"http://www.cysticfibrosisjournal.com/article/S1569199317301534/pdf","host_type":"journal"},{"url":"http://www.cysticfibrosisjournal.com/article/S1569199317301534/pdf","host_type":"publisher"},{"url":"https://api.elsevier.com/content/article/PII:S1569199317301534?httpAccept=text/xml","host_type":"publisher"},{"url":"https://api.elsevier.com/content/article/PII:S1569199317301534?httpAccept=text/plain","host_type":"publisher"},{"url":"https://doi.org/10.1016/j.jcf.2017.05.002","host_type":"journal"},{"url":"https://pubmed.ncbi.nlm.nih.gov/28529073","host_type":"repository"},{"url":"https://hal.science/hal-04753779","host_type":"repository"},{"url":"https://hal.science/hal-04834180","host_type":"repository"}],"fields_of_study":["Cystic Fibrosis Research Advances","Neonatal Respiratory Health Research","Asthma and respiratory diseases","Adolescent","Aminophenols","Aminopyridines","Benzodioxoles","Chloride Channel Agonists","Cystic Fibrosis","Drug Combinations","Drug Monitoring","Female","Forced Expiratory Volume","France","Humans","Male","Quinolones","Respiratory Function Tests","Symptom Assessment","Treatment Outcome"],"mesh_terms":["Adolescent","Aminophenols","Aminopyridines","Cystic Fibrosis","Drug Combinations","Female","Forced Expiratory Volume","France","Humans","Male","Respiratory Function Tests","Quinolones","Treatment Outcome","Drug Monitoring","Benzodioxoles","Symptom Assessment","Chloride Channel Agonists"],"keywords":["Medicine","Ivacaftor","Cystic fibrosis","Salbutamol","Pulmonary function testing","Lung function","Inhalation","Airway","Airway obstruction","Respiratory system","Vital capacity","Anesthesia","Internal medicine","Lung","Asthma","Cystic fibrosis transmembrane conductance regulator","Diffusing capacity","Acute","Pediatric","Bronchoconstriction","Modulators","Lumacaftor"],"sdg_mappings":[{"sdg_number":0,"sdg_label":"Good health and well-being"}],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[],"source":"live","citation_network_status":"fetched"},"created_at":"2026-08-02T23:39:35.404297Z","pmid":null,"pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}