The clinical impact of Lumacaftor-Ivacaftor on structural lung disease and lung function in children aged 6-11 with cystic fibrosis in a real-world setting.

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作者： Paul,McNally ^[1] ; Barry,Linnane ^[2] ; Michael,Williamson ^[3] ; Basil,Elnazir ^[1] ; Christopher,Short ^[1] ; Clare,Saunders ^[4] ; Laura,Kirwan ^[5] ; Rea,David ^[6] ; Mariette P C,Kemner-Van de Corput ^[5] ; Harm A W M,Tiddens ^[6] ; Jane C,Davies ^[7] ; Des W,Cox ^[1]

作者单位： Respiratory Department, Children's Health Ireland, Crumlin, Dublin, Ireland. ^[1] RCSI University of Medicine and Health Sciences, Dublin, Ireland. ^[2] University of Limerick School of Medicine, Limerick, Ireland. ^[3] Trinity College, Dublin, Ireland. ^[4] NHLI, Imperial College, London, UK. ^[5] Royal Brompton and Harefield Hospitals, Guy's and St Thomas' Trust, London, UK. ^[6] Cystic Fibrosis Registry of Ireland, Dublin, Ireland. ^[7] Department of Paediatric Pulmonology and Allergology, Department of Radiology and Nuclear Medicine, Erasmus Medical Centre - Sophia Children's Hospital, Rotterdam, The Netherlands. ^[8] Respiratory Department, Children's Health Ireland, Crumlin, Dublin, Ireland. desmond.cox@ucd.ie. ^[9] University College Dublin, Dublin, Ireland. desmond.cox@ucd.ie. ^[10]

关键词 Children Lung clearance index PRAGMA CF scores Real-world studies