Коллектив авторов - Долгая жизнь с муковисцидозом

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Simmonds NJ, Cullinan R Hodson ME. Growing old with cystic fibrosis – the characteristics of long-term survivors of cystic fibrosis. Respir Med 2009; 103: 629-35.

Simmonds NJ, Cullinan R Hodson ME. Growing old with cystic fibrosis – the characteristics of long-term survivors of cystic fibrosis. Respir Med 2009; 103: 629-35.

Rodman DM, Polls JM, Heltshe SL, et al. Late diagnosis defines a unique population of long-term survivors of cystic fibrosis. Am J Respir Crit Care Med 2005; 171: 621-6.

Hodson ME, Simmonds NJ, Warwick WJ, et al. An international/ multicentre report on patients with cystic fibrosis (CF) over the age of 40 years. J Cyst Fibros 2008; 7: 537-42.

Hodson ME, Simmonds NJ, Warwick WJ, et al. An international/ multicentre report on patients with cystic fibrosis (CF) over the age of 40 years. J Cyst Fibros 2008; 7: 537-42.

Simmonds NJ, MacNeill SJ, Cullinan P, et al. Cystic fibrosis and survival to 40 years: a case-control study. Eur Respir J 2010; 36:1277-83.

Ramsey BW, Pepe MS, QuanJM, et al. Intermittent administration of inhaled tobramycin in patients with cystic fibrosis. Cystic Fibrosis Inhaled Tobramycin Study Group. N Engl J Med 1999; 340: 23–30.

Elkins MR, Robinson M, Rose BR, et al. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 2006; 354: 229-10.

OermannCM, Retsch BogartGZ, Quittner AL, et al. An 18 month study of the safety and efficacy of repeated courses of inhaled aztreonam lysine in cystic fibrosis. Pediatr Pulmonol 2010; 45: 1121-34.

Daniels T, Goodacre L, Sutton C, et al. Accurate assessment of adherence: self-report and clinician report vs electronic monitoring of nebulizers. Chest 2011; 140: 425-32.

Briesacher BA, Quittner AL, Saiman L, et al. Adherence with tobramycin inhaled solution and health care utilization. BMC Pulm Med 2011; 11: 5.

Sawicki GS, Sellers DE, Robinson WM. High treatment burden in adults with cystic fibrosis: challenges to disease self-management. J Cyst Fibros 2009; 8: 91-6.

OermannCM, Retsch BogartGZ, Quittner AL, et al. An 18 month study of the safety and efficacy of repeated courses of inhaled aztreonam lysine in cystic fibrosis. Pediatr Pulmonol 2010; 45: 1121-34.

Schuster A, Haliburn C, Doring G, et al. Safety, efficacy and convenience of colistimethate sodium dry powder for inhalation (Colobreathe DPI) in patients with cystic fibrosis: a randomised study. Thorax 2013; 68: 344-50.

Konstan MW, Flume PA, Kappler M, et al. Safety, efficacy and convenience of tobramycin inhalation powder in cystic fibrosis patients: The EAGER trial. J Cyst Fibros 2011: 10:54–61.

Harrison M, McCarthy M, Fleming C, et al. Inhaled versus nebulised tobramycin: a real world comparison in adult cystic fibrosis (CF). J Cyst Fibros 2014; 13: 692-8.

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Ferrari S, Geddes DM, Alton EW. Barriers to and new approaches for gene therapy and gene delivery in cystic fibrosis. Adv Drug Deliv Rev 2002; 54: 1373-93.

Ramsey BW, Davies J, McElvaney NG, et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med 2011; 365: 1663-72.

DaviesJC, Wainwright CE, Canny GJ, et al. 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 2013; 187: 1219-25.

Boyle MP, Bell SC, Konstan MW, et al. A CFTR corrector (luma-caftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a 508del CFTR mutation: a phase 2 randomised controlled trial. Lancet Respir Med 2014; 2: 527-38.

Ramsey BW, Davies J, McElvaney NG, et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med 2011; 365: 1663-72.

DaviesJC, Wainwright CE, Canny GJ, et al. 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 2013; 187: 1219-25.

De Boeck K, Munck A, Walker S, et al. The effect of ivacaftor, a CFTR potentiator, in patients with cystic fibrosis and a non-G551D-CFTR gating mutation, the KONNECTION study. J Cyst Fibros 2014; 13: S1.

Ramsey BW, Davies J, McElvaney NG, et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med 2011; 365: 1663-72.

DaviesJC, Wainwright CE, Canny GJ, et al. 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 2013; 187: 1219-25.

De Boeck K, Munck A, Walker S, et al. The effect of ivacaftor, a CFTR potentiator, in patients with cystic fibrosis and a non-G551D-CFTR gating mutation, the KONNECTION study. J Cyst Fibros 2014; 13: S1.

McKone E, Borowitz D, Drevinek R et al. Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp-CFTR mutation: a phase 3, open-label extension study (PERSIST). Lancet Respir Med 2014; 2: 902-10.

Rowe SM, Heltshe SL, Gonska X et al. Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G557D-mediated cystic fibrosis. Am J Respir Crit Care Med 2014; 190: 175-84.

Barry PJ, Plant BJ, Nair A, et al. Effects of ivacaftor in cystic fibrosis patients who carry the G551D mutation and have severe lung disease. Chest 2014; 146: 152-8.

Harrison MJ, Murphy DM, Plant BJ. Ivacaftor in a G557D homozygote with cystic fibrosis. N Engl J Med 2013; 369: 1280-2.

EMA. Kalydeco (Ivacaftor) assessment report. Secondary Kalydeco (Ivacaftor) assessment report, http://www.ema.europa.eu/docs/en_GB/document_library/EPAR_-_Public_assessment_report/human/002494/WC500130766.pdf – 28 July 2014.

Boyle MP, Bell SC, Konstan MW, et al. A CFTR corrector (luma-caftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a 508del CFTR mutation: a phase 2 randomised controlled trial. Lancet Respir Med 2014; 2: 527-38.