Retrospective observational study of French patients with cystic fibrosis and a Gly551Asp-CFTR mutation after 1 and 2 years of treatment with ivacaftor in a real-world setting

Journal of Cystic Fibrosis

Volumn 17, Issue 1, 2018, Pages 89-95

  Hubert, Dominique ^a,b   Dehillotte, Clémence ^c   Munck, Anne ^d   David, Valérie ^e   Baek, Jinmi ^a   Mely, Laurent ^f   Dominique, Stéphane ^g   Ramel, Sophie ^h   Danner Boucher, Isabelle ⁱ   Lefeuvre, Sylvaine ^j   Reynaud, Quitterie ^k   Colomb Jung, Virginie ^c   Bakouboula, Prissile ^a   Lemonnier, Lydie ^c  

^a AP HP ^*  (France)

^b UNIVERSITÉ PARIS DESCARTES   (France)

^c Vaincre la Mucoviscidose   (France)

^d HÔPITAL ROBERT DEBRÉ   (France)

^e Hopital Mere Enfant CHU de Nantes   (France)

^f Renée Sabran Hospital   (France)

^g HÔPITAL CHARLES NICOLLE   (France)

^h Centre de Perharidy   (France)

ⁱ CHU de Nantes   (France)

^j SERVICE DE RHUMATOLOGIE   (France)

^k CENTRE HOSPITALIER LYON SUD   (France)

more..

Author keywords

CFTR modulators; Cystic fibrosis; Lumacaftor

Indexed keywords

ANTIBIOTIC AGENT; AZITHROMYCIN; DEOXYRIBONUCLEASE; IVACAFTOR; AMINOPHENOL DERIVATIVE; ANTIINFECTIVE AGENT; CHLORIDE CHANNEL STIMULATING AGENT; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; QUINOLONE DERIVATIVE;

ADOLESCENT; ADULT; ARTICLE; BODY MASS; CHEMICAL INDUSTRY; CHILD; CYSTIC FIBROSIS; DIET SUPPLEMENTATION; FEMALE; FORCED EXPIRATORY VOLUME; FRENCHMAN; GENE MUTATION; HUMAN; LUNG DISEASE; MAINTENANCE THERAPY; MAJOR CLINICAL STUDY; MALE; OBSERVATIONAL STUDY; PRESCRIPTION; PSEUDOMONAS AERUGINOSA; RESPIRATORY FUNCTION; RETROSPECTIVE STUDY; STAPHYLOCOCCUS AUREUS; TREATMENT DURATION; TREATMENT RESPONSE; CLINICAL TRIAL; FRANCE; GENETICS; ISOLATION AND PURIFICATION; LUNG FUNCTION TEST; MICROBIOLOGY; MULTICENTER STUDY; PATHOPHYSIOLOGY; RESPIRATORY SYSTEM; TIME;

ADOLESCENT; ADULT; AMINOPHENOLS; ANTI-BACTERIAL AGENTS; CHILD; CHLORIDE CHANNEL AGONISTS; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; FEMALE; FRANCE; HUMANS; MALE; PSEUDOMONAS AERUGINOSA; QUINOLONES; RESPIRATORY FUNCTION TESTS; RESPIRATORY SYSTEM; STAPHYLOCOCCUS AUREUS; TIME;

EID: 85023598529     PISSN: 15691993     EISSN: 18735010     Source Type: Journal    
DOI: 10.1016/j.jcf.2017.07.001     Document Type: Article

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