Ivacaftor in severe cystic fibrosis lung disease and a G551D mutation

Respirology Case Reports

Volumn 1, Issue 2, 2013, Pages 52-54

  Wood, Michelle E ^a   Smith, Daniel J ^a,b,d   Reid, David W ^a,b   Masel, Philip J ^a   France, Megan W ^a   Bell, Scott C ^a,c,d  

^a THE PRINCE CHARLES HOSPITAL   (Australia)

^b QIMR BERGHOFER MEDICAL RESEARCH INSTITUTE   (Australia)

^c ROYAL CHILDREN'S HOSPITAL   (Australia)

^d UNIVERSITY OF QUEENSLAND   (Australia)

Author keywords

CFTR; Cystic fibrosis; G551D mutation; Ivacaftor

Indexed keywords

ANTIBIOTIC AGENT; C REACTIVE PROTEIN; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; IVACAFTOR; MEROPENEM;

ADULT; ARTICLE; CASE REPORT; COMPARATIVE STUDY; CYSTIC FIBROSIS; CYSTIC FIBROSIS QUESTIONNAIRE REVISED; ELECTROLYTE BLOOD LEVEL; FEMALE; FORCED EXPIRATORY VOLUME; GENE MUTATION; HUMAN; HYPERTRANSAMINASEMIA; INFLAMMATION; LUNG DISEASE; MALE; OUTCOME ASSESSMENT; QUESTIONNAIRE; RASH; SPIROMETRY; TRAFFIC ACCIDENT; VIRUS INFECTION; WEIGHT GAIN;

EID: 85002291943     PISSN: None     EISSN: 20513380     Source Type: Journal    
DOI: 10.1002/rcr2.27     Document Type: Article

References (4)

1. Bell SC, Bye PTB, Cooper P, et al. 2011. Cystic fibrosis in Australia: results from a data registry. Med. J. Aust. 195:396-400.
2. Ramsey BW, Davies J, McElvaney NG, et al. 2011. A CFTR potentiator in patients with cystic fibrosis and the G551D Mutation. N. Engl. J. Med. 365:1663-1672.
3. Davies JC, Wainwright CE, Canny GJ, et al. 2013. Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation. Am. J. Respir. Crit. Care Med. 187:1219-1225.
4. Hebestrait H, Sauer-Heilborn A, Fischer R, et al. 2013. Effects of ivacfator on severely ill patients with cystic fibrosis carrying the G551D mutation. J. Cyst. Fibros., in press. published online http://dx.doi.org/10.1016/j.jcf.2012.05.006.