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Volumn 50, Issue 3, 2015, Pages 302-315

Novel outcome measures for clinical trials in cystic fibrosis

Author keywords

CFTR activity; Cystic fibrosis; Endpoints; Imaging; Outcome measures; Sputum biomarkers

Indexed keywords

BIOLOGICAL MARKER; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR;

EID: 84923201553     PISSN: 87556863     EISSN: 10990496     Source Type: Journal    
DOI: 10.1002/ppul.23146     Document Type: Article
Times cited : (36)

References (149)
  • 1
    • 77956891816 scopus 로고    scopus 로고
    • Bethesda, Matyland. Last accessed 14 August 2013
    • Cystic Fibrosis Foundation. Cystic Fibrosis Foundation Patient Registry. 2008 annual data report. Bethesda, Matyland. Last accessed 14 August 2013. http://www.cff.org/treatments/Care- CenterNetwork/PatientRegistryReport/.
    • Cystic Fibrosis Foundation Patient Registry. 2008 Annual Data Report
  • 2
    • 34548246514 scopus 로고    scopus 로고
    • Use of lung imaging studies as outcome measures for development of new therapies in cystic fibrosis
    • Ramsey BW. Use of lung imaging studies as outcome measures for development of new therapies in cystic fibrosis. Proc Am Thorac Soc 2007;4:359-363.
    • (2007) Proc Am Thorac Soc , vol.4 , pp. 359-363
    • Ramsey, B.W.1
  • 3
    • 84855242865 scopus 로고    scopus 로고
    • A new model for cystic fibrosis management: Control concept
    • Yuksel H, Yilmaz O. A new model for cystic fibrosis management: control concept. Pneumologia 2011;60:150-154.
    • (2011) Pneumologia , vol.60 , pp. 150-154
    • Yuksel, H.1    Yilmaz, O.2
  • 10
    • 84880887459 scopus 로고    scopus 로고
    • An official American Thoracic Society workshop report: Optimal lung function tests for monitoring cystic fibrosis, bronchopulmonary dysplasia, and recurrent wheezing in children less than 6 years of age
    • Rosenfeld M, Allen J, Arets BH, Aurora P, Beydon N, Calogero C, Castile RG, Davis SD, Fuchs S, Gappa M, et al. An official American Thoracic Society workshop report: optimal lung function tests for monitoring cystic fibrosis, bronchopulmonary dysplasia, and recurrent wheezing in children less than 6 years of age. Ann Am Thorac Soc 2013;10:S1-S11.
    • (2013) Ann Am Thorac Soc , vol.10 , pp. S1-S11
    • Rosenfeld, M.1    Allen, J.2    Arets, B.H.3    Aurora, P.4    Beydon, N.5    Calogero, C.6    Castile, R.G.7    Davis, S.D.8    Fuchs, S.9    Gappa, M.10
  • 12
    • 30344465311 scopus 로고    scopus 로고
    • ATS/ERS statement: Raised volume forced expirations in infants: Guidelines for current practice
    • ATS/ERS statement: raised volume forced expirations in infants: guidelines for current practice. Am J Respir Crit Care Med 2005;172:1463-1471.
    • (2005) Am J Respir Crit Care Med , vol.172 , pp. 1463-1471
  • 16
    • 84859823106 scopus 로고    scopus 로고
    • Improvement in pulmonary function following antibiotics in infants with cystic fibrosis
    • Pittman JE, Johnson RC, Davis SD. Improvement in pulmonary function following antibiotics in infants with cystic fibrosis. Pediatr Pulmonol 2012;47:441-446.
    • (2012) Pediatr Pulmonol , vol.47 , pp. 441-446
    • Pittman, J.E.1    Johnson, R.C.2    Davis, S.D.3
  • 17
    • 84861892411 scopus 로고    scopus 로고
    • Inhaled hypertonic saline in infants and children younger than 6 years with cystic fibrosis: The ISIS randomized controlled trial
    • Rosenfeld M, Ratjen F, Brumback L, Daniel S, Rowbotham R, McNamara S, Johnson R, Kronmal R, Davis SD. Inhaled hypertonic saline in infants and children younger than 6 years with cystic fibrosis: the ISIS randomized controlled trial. JAMA 2012;307:2269-2277.
    • (2012) JAMA , vol.307 , pp. 2269-2277
    • Rosenfeld, M.1    Ratjen, F.2    Brumback, L.3    Daniel, S.4    Rowbotham, R.5    McNamara, S.6    Johnson, R.7    Kronmal, R.8    Davis, S.D.9
  • 18
    • 0034071721 scopus 로고    scopus 로고
    • The raised volume rapid thoracoabdominal compression technique. The Joint American Thoracic Society/European Respiratory Society Working Group on Infant Lung Function
    • The raised volume rapid thoracoabdominal compression technique. The Joint American Thoracic Society/European Respiratory Society Working Group on Infant Lung Function. Am J Respir Crit Care Med 2000;161:1760-1762.
    • (2000) Am J Respir Crit Care Med , vol.161 , pp. 1760-1762
  • 19
    • 67349175293 scopus 로고    scopus 로고
    • Lung clearance index in the assessment of airways disease
    • Horsley A. Lung clearance index in the assessment of airways disease. Respir Med 2009;103:793-799.
    • (2009) Respir Med , vol.103 , pp. 793-799
    • Horsley, A.1
  • 20
    • 0346121620 scopus 로고    scopus 로고
    • Evaluation of ventilation maldistribution as an early indicator of lung disease in children with cystic fibrosis
    • Gustafsson PM, Aurora P, Lindblad A. Evaluation of ventilation maldistribution as an early indicator of lung disease in children with cystic fibrosis. Eur Respir J 2003;22:972-979.
    • (2003) Eur Respir J , vol.22 , pp. 972-979
    • Gustafsson, P.M.1    Aurora, P.2    Lindblad, A.3
  • 21
    • 39049084491 scopus 로고    scopus 로고
    • Multiple-breath inert gas washout and spirometry versus structural lung disease in cystic fibrosis
    • Gustafsson PM, De Jong PA, Tiddens HA, Lindblad A. Multiplebreath inert gas washout and spirometry versus structural lung disease in cystic fibrosis. Thorax 2008;63:129-134.
    • (2008) Thorax , vol.63 , pp. 129-134
    • Gustafsson, P.M.1    De Jong, P.A.2    Tiddens, H.A.3    Lindblad, A.4
  • 26
    • 84890309789 scopus 로고    scopus 로고
    • Lung clearance index predicts pulmonary exacerbations in young patients with cystic fibrosis
    • Vermeulen F, Proesmans M, Boon M, Havermans T, De Boeck K. Lung clearance index predicts pulmonary exacerbations in young patients with cystic fibrosis. Thorax 2014;69:39-45.
    • (2014) Thorax , vol.69 , pp. 39-45
    • Vermeulen, F.1    Proesmans, M.2    Boon, M.3    Havermans, T.4    De Boeck, K.5
  • 29
    • 84885071273 scopus 로고    scopus 로고
    • Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D-CFTR mutation and preserved spirometry: A randomised controlled trial
    • Davies J, Sheridan H, Bell N, Cunningham S, Davis SD, Elborn JS, Milla CE, Starner TD,Weiner DJ, Lee PS, Ratjen F. Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D-CFTR mutation and preserved spirometry: a randomised controlled trial. Lancet Respir Med 2013;1:630-638.
    • (2013) Lancet Respir Med , vol.1 , pp. 630-638
    • Davies, J.1    Sheridan, H.2    Bell, N.3    Cunningham, S.4    Davis, S.D.5    Elborn, J.S.6    Milla, C.E.7    Starner, T.D.8    Weiner, D.J.9    Lee, P.S.10    Ratjen, F.11
  • 30
    • 73449102215 scopus 로고    scopus 로고
    • Lung clearance index: Normal values, repeatability, and reproducibility in healthy children and adolescents
    • Fuchs SI, Eder J, Ellemunter H, Gappa M. Lung clearance index: normal values, repeatability, and reproducibility in healthy children and adolescents. Pediatr Pulmonol 2009;44:1180-1185.
    • (2009) Pediatr Pulmonol , vol.44 , pp. 1180-1185
    • Fuchs, S.I.1    Eder, J.2    Ellemunter, H.3    Gappa, M.4
  • 31
    • 80955131827 scopus 로고    scopus 로고
    • Lung clearance index: Clinical and research applications in children
    • Fuchs SI, Gappa M. Lung clearance index: clinical and research applications in children. Paediatr Respir Rev 2011;12: 264-270.
    • (2011) Paediatr Respir Rev , vol.12 , pp. 264-270
    • Fuchs, S.I.1    Gappa, M.2
  • 32
    • 33845385898 scopus 로고    scopus 로고
    • Multiple breath washout with a sidestream ultrasonic flow sensor and mass spectrometry: A comparative study
    • Fuchs SI, Buess C, Lum S, Kozlowska W, Stocks J, Gappa M. Multiple breath washout with a sidestream ultrasonic flow sensor and mass spectrometry: a comparative study. Pediatr Pulmonol 2006;41:1218-1225.
    • (2006) Pediatr Pulmonol , vol.41 , pp. 1218-1225
    • Fuchs, S.I.1    Buess, C.2    Lum, S.3    Kozlowska, W.4    Stocks, J.5    Gappa, M.6
  • 36
    • 80053411033 scopus 로고    scopus 로고
    • The importance of imaging in cystic fibrosis
    • Daines C, Morgan W. The importance of imaging in cystic fibrosis. Am J Respir Crit Care Med 2011;184:751-752.
    • (2011) Am J Respir Crit Care Med , vol.184 , pp. 751-752
    • Daines, C.1    Morgan, W.2
  • 37
    • 2142771098 scopus 로고    scopus 로고
    • Pulmonary disease assessment in cystic fibrosis: Comparison of CT scoring systems and value of bronchial and arterial dimension measurements
    • De Jong PA, Ottink MD, Robben SG, Lequin MH, Hop WC, Hendriks JJ, Pare PD, Tiddens HA. Pulmonary disease assessment in cystic fibrosis: comparison of CT scoring systems and value of bronchial and arterial dimension measurements. Radiology 2004;231:434-439.
    • (2004) Radiology , vol.231 , pp. 434-439
    • De Jong, P.A.1    Ottink, M.D.2    Robben, S.G.3    Lequin, M.H.4    Hop, W.C.5    Hendriks, J.J.6    Pare, P.D.7    Tiddens, H.A.8
  • 44
    • 1642386147 scopus 로고    scopus 로고
    • Positron emission tomography with [18F] fluorodeoxyglucose to evaluate neutrophil kinetics during acute lung injury
    • Chen DL, Schuster DP. Positron emission tomography with [18F] fluorodeoxyglucose to evaluate neutrophil kinetics during acute lung injury. Am J Physiol Lung Cell Mol Physiol 2004;286:L834-L840.
    • (2004) Am J Physiol Lung Cell Mol Physiol , vol.286 , pp. L834-L840
    • Chen, D.L.1    Schuster, D.P.2
  • 45
    • 30844473744 scopus 로고    scopus 로고
    • Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis
    • De Jong PA, Lindblad A, Rubin L, Hop WC, de Jongste JC, Brink M, Tiddens HA. Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis. Thorax 2006;61:80-85.
    • (2006) Thorax , vol.61 , pp. 80-85
    • De Jong, P.A.1    Lindblad, A.2    Rubin, L.3    Hop, W.C.4    De Jongste, J.C.5    Brink, M.6    Tiddens, H.A.7
  • 46
    • 79955655587 scopus 로고    scopus 로고
    • Randomised controlled trials in cystic fibrosis: What, when and how?
    • Sly PD, Ware RS, de Klerk N, Stick SM. Randomised controlled trials in cystic fibrosis: what, when and how?. Eur Respir J 2011;37:991-993.
    • (2011) Eur Respir J , vol.37 , pp. 991-993
    • Sly, P.D.1    Ware, R.S.2    De Klerk, N.3    Stick, S.M.4
  • 48
  • 51
    • 79960412250 scopus 로고    scopus 로고
    • Bronchiectasis and pulmonary exacerbations in children and young adults with cystic fibrosis
    • Loeve M, Gerbrands K, Hop WC, Rosenfeld M, Hartmann IC, Tiddens HA. Bronchiectasis and pulmonary exacerbations in children and young adults with cystic fibrosis. Chest 2011;140:178-185.
    • (2011) Chest , vol.140 , pp. 178-185
    • Loeve, M.1    Gerbrands, K.2    Hop, W.C.3    Rosenfeld, M.4    Hartmann, I.C.5    Tiddens, H.A.6
  • 52
    • 84923136209 scopus 로고    scopus 로고
    • Reversibility of trapped air on CT scans of CF patients, an automated approach
    • Loeve M, Tiddens H, Gorbunova V, Hop WC, de Bruijne M. Reversibility of trapped air on CT scans of CF patients, an automated approach. Pediatr Pulmonol 2011;46:-359. Abstract 349.
    • (2011) Pediatr Pulmonol , vol.46 , pp. 359
    • Loeve, M.1    Tiddens, H.2    Gorbunova, V.3    Hop, W.C.4    De Bruijne, M.5
  • 56
    • 84923123433 scopus 로고    scopus 로고
    • Bronchiectasis on chest CT predicts future lung disease severity in children with CF
    • Sanders DB, Li Z, Brody A, Farrell PM. Bronchiectasis on chest CT predicts future lung disease severity in children with CF. Pediatr Pulmonol 2011;46(Suppl 34):330. Abstract 327.
    • (2011) Pediatr Pulmonol , vol.46 , pp. 330
    • Sanders, D.B.1    Li, Z.2    Brody, A.3    Farrell, P.M.4
  • 58
    • 36549007486 scopus 로고    scopus 로고
    • Computed tomography-an increasing source of radiation exposure
    • Brenner DJ, Hall EJ. Computed tomography-an increasing source of radiation exposure. N Engl J Med 2007;357:2277-2284.
    • (2007) N Engl J Med , vol.357 , pp. 2277-2284
    • Brenner, D.J.1    Hall, E.J.2
  • 59
    • 84861233270 scopus 로고    scopus 로고
    • The sensitivity of lung disease surrogates in detecting chest CT abnormalities in children with cystic fibrosis
    • Sanders DB, Li Z, Rock MJ, Brody AS, Farrell PM. The sensitivity of lung disease surrogates in detecting chest CT abnormalities in children with cystic fibrosis. Pediatr Pulmonol 2012;47:567-573.
    • (2012) Pediatr Pulmonol , vol.47 , pp. 567-573
    • Sanders, D.B.1    Li, Z.2    Rock, M.J.3    Brody, A.S.4    Farrell, P.M.5
  • 60
    • 84883526598 scopus 로고    scopus 로고
    • Pediatric chest computed tomography at a radiation dose approaching a chest radiograph
    • Willemink MJ, De Jong PA. Pediatric chest computed tomography at a radiation dose approaching a chest radiograph. Am J Respir Crit Care Med 2013;188:626-627.
    • (2013) Am J Respir Crit Care Med , vol.188 , pp. 626-627
    • Willemink, M.J.1    De Jong, P.A.2
  • 65
    • 84904040133 scopus 로고    scopus 로고
    • Don't let radiation scare trump patient care: 10 ways you can harm your patients by fear of radiation-induced cancer from diagnostic imaging
    • Brody AS, Guillerman RP. Don't let radiation scare trump patient care: 10 ways you can harm your patients by fear of radiation-induced cancer from diagnostic imaging. Thorax 2014;69:782-784.
    • (2014) Thorax , vol.69 , pp. 782-784
    • Brody, A.S.1    Guillerman, R.P.2
  • 69
    • 60749128436 scopus 로고    scopus 로고
    • Lung morphology assessment using MRI: A robust ultra-short TR/TE 2D steady state free precession sequence used in cystic fibrosis patients
    • Failo R, Wielopolski PA, Tiddens HA, Hop WC, Mucelli RP, Lequin MH. Lung morphology assessment using MRI: a robust ultra-short TR/TE 2D steady state free precession sequence used in cystic fibrosis patients. Magn Reson Med 2009;61:299-306.
    • (2009) Magn Reson Med , vol.61 , pp. 299-306
    • Failo, R.1    Wielopolski, P.A.2    Tiddens, H.A.3    Hop, W.C.4    Mucelli, R.P.5    Lequin, M.H.6
  • 71
    • 77954430944 scopus 로고    scopus 로고
    • The role of advanced imaging techniques in cystic fibrosis follow-up: Is there a place for MRI?
    • Puderbach M, Eichinger M. The role of advanced imaging techniques in cystic fibrosis follow-up: is there a place for MRI?. Pediatr Radiol 2010;40:844-849.
    • (2010) Pediatr Radiol , vol.40 , pp. 844-849
    • Puderbach, M.1    Eichinger, M.2
  • 72
    • 0032436357 scopus 로고    scopus 로고
    • Oral ciprofloxacin in the treatment of pseudomonas exacerbations of paediatric cystic fibrosis: Clinical efficacy and safety evaluation using magnetic resonance image scanning
    • Redmond A, Sweeney L, MacFarland M, Mitchell M, Daggett S, Kubin R. Oral ciprofloxacin in the treatment of pseudomonas exacerbations of paediatric cystic fibrosis: clinical efficacy and safety evaluation using magnetic resonance image scanning. J Int Med Res 1998;26:304-312.
    • (1998) J Int Med Res , vol.26 , pp. 304-312
    • Redmond, A.1    Sweeney, L.2    MacFarland, M.3    Mitchell, M.4    Daggett, S.5    Kubin, R.6
  • 79
    • 78649646574 scopus 로고    scopus 로고
    • Multimodality molecular imaging of the lung
    • Chen DL, Kinahan PE. Multimodality molecular imaging of the lung. J Magn Reson Imaging 2010;32:1409-1420.
    • (2010) J Magn Reson Imaging , vol.32 , pp. 1409-1420
    • Chen, D.L.1    Kinahan, P.E.2
  • 81
    • 34548245070 scopus 로고    scopus 로고
    • Positron emission tomography and computed tomography versus positron emission tomography computed tomography: Tools for imaging the lung
    • Dolovich MB, Schuster DP. Positron emission tomography and computed tomography versus positron emission tomography computed tomography: tools for imaging the lung. Proc Am Thorac Soc 2007;4:328-333.
    • (2007) Proc Am Thorac Soc , vol.4 , pp. 328-333
    • Dolovich, M.B.1    Schuster, D.P.2
  • 82
    • 0038732627 scopus 로고    scopus 로고
    • Uptake of 18fluorodeoxyglucose in the cystic fibrosis lung: A measure of lung inflammation?
    • Labiris NR, Nahmias C, Freitag AP, Thompson ML, Dolovich MB. Uptake of 18fluorodeoxyglucose in the cystic fibrosis lung: a measure of lung inflammation?. Eur Respir J 2003;21:848-854.
    • (2003) Eur Respir J , vol.21 , pp. 848-854
    • Labiris, N.R.1    Nahmias, C.2    Freitag, A.P.3    Thompson, M.L.4    Dolovich, M.B.5
  • 84
    • 33745255722 scopus 로고    scopus 로고
    • Positron emission tomography imaging of regional lung function
    • Musch G, Venegas JG. Positron emission tomography imaging of regional lung function. Minerva Anestesiol 2006;72:363-367.
    • (2006) Minerva Anestesiol , vol.72 , pp. 363-367
    • Musch, G.1    Venegas, J.G.2
  • 87
    • 80052574268 scopus 로고    scopus 로고
    • Acute inhalation of hypertonic saline does not improve mucociliary clearance in all children with cystic fibrosis
    • Laube BL, Sharpless G, Carson KA, Kelly A, Mogayzel PJ, Jr. Acute inhalation of hypertonic saline does not improve mucociliary clearance in all children with cystic fibrosis. BMC Pulm Med 2011;11:45.
    • (2011) BMC Pulm Med , vol.11 , pp. 45
    • Laube, B.L.1    Sharpless, G.2    Carson, K.A.3    Kelly, A.4    Mogayzel, P.J.5
  • 91
    • 34548215173 scopus 로고    scopus 로고
    • Mucociliary clearance as an outcome measure for cystic fibrosis clinical research
    • Donaldson SH, Corcoran TE, Laube BL, Bennett WD. Mucociliary clearance as an outcome measure for cystic fibrosis clinical research. Proc Am Thorac Soc 2007;4:399-405.
    • (2007) Proc Am Thorac Soc , vol.4 , pp. 399-405
    • Donaldson, S.H.1    Corcoran, T.E.2    Laube, B.L.3    Bennett, W.D.4
  • 93
    • 0034095691 scopus 로고    scopus 로고
    • Effect of a short course of rhDNase on cough and mucociliary clearance in patients with cystic fibrosis
    • Robinson M, Hemming AL, Moriarty C, Eberl S, Bye PT. Effect of a short course of rhDNase on cough and mucociliary clearance in patients with cystic fibrosis. Pediatr Pulmonol 2000;30: 16-24.
    • (2000) Pediatr Pulmonol , vol.30 , pp. 16-24
    • Robinson, M.1    Hemming, A.L.2    Moriarty, C.3    Eberl, S.4    Bye, P.T.5
  • 97
    • 34548282581 scopus 로고    scopus 로고
    • Sputum biomarkers of inflammation in cystic fibrosis lung disease
    • Sagel SD, Chmiel JF, Konstan MW. Sputum biomarkers of inflammation in cystic fibrosis lung disease. Proc Am Thorac Soc 2007;4:406-417.
    • (2007) Proc Am Thorac Soc , vol.4 , pp. 406-417
    • Sagel, S.D.1    Chmiel, J.F.2    Konstan, M.W.3
  • 100
    • 7444237658 scopus 로고    scopus 로고
    • Variability of markers of inflammation and infection in induced sputum in children with cystic fibrosis
    • Ordoñez CL, Kartashov AI, Wohl ME. Variability of markers of inflammation and infection in induced sputum in children with cystic fibrosis. J Pediatr 2004;145:689-692.
    • (2004) J Pediatr , vol.145 , pp. 689-692
    • Ordoñez, C.L.1    Kartashov, A.I.2    Wohl, M.E.3
  • 102
    • 6344231963 scopus 로고    scopus 로고
    • Induced sputum in cystic fibrosis: Within-week reproducibility of inflammatory markers
    • Smountas AA, Lands LC, Mohammed SR, Grey V. Induced sputum in cystic fibrosis: within-week reproducibility of inflammatory markers. Clin Biochem 2004;37:1031-1036.
    • (2004) Clin Biochem , vol.37 , pp. 1031-1036
    • Smountas, A.A.1    Lands, L.C.2    Mohammed, S.R.3    Grey, V.4
  • 112
    • 84896715802 scopus 로고    scopus 로고
    • Airway, but not serum or urinary, levels of YKL-40 reflect inflammation in early cystic fibrosis lung disease
    • Fantino E, Gangell CL, Hartl D, Sly PD. Airway, but not serum or urinary, levels of YKL-40 reflect inflammation in early cystic fibrosis lung disease. BMC Pulm Med 2014;14:28.
    • (2014) BMC Pulm Med , vol.14 , pp. 28
    • Fantino, E.1    Gangell, C.L.2    Hartl, D.3    Sly, P.D.4
  • 114
    • 84895896211 scopus 로고    scopus 로고
    • Plasma sCD14 as a biomarker to predict pulmonary exacerbations in cystic fibrosis
    • Quon BS, Ngan DA,Wilcox PG, Man SF, Sin DD. Plasma sCD14 as a biomarker to predict pulmonary exacerbations in cystic fibrosis. PLoS ONE 2014;9:e89341.
    • (2014) PLoS ONE , vol.9 , pp. e89341
    • Quon, B.S.1    Ngan, D.A.2    Wilcox, P.G.3    Man, S.F.4    Sin, D.D.5
  • 116
    • 84887447853 scopus 로고    scopus 로고
    • Systematic review of blood biomarkers in cystic fibrosis pulmonary exacerbations
    • Shoki AH, Mayer-Hamblett N, Wilcox PG, Sin DD, Quon BS. Systematic review of blood biomarkers in cystic fibrosis pulmonary exacerbations. Chest 2013;144:1659-1670.
    • (2013) Chest , vol.144 , pp. 1659-1670
    • Shoki, A.H.1    Mayer-Hamblett, N.2    Wilcox, P.G.3    Sin, D.D.4    Quon, B.S.5
  • 118
    • 84883601416 scopus 로고    scopus 로고
    • Biomarkers in exhaled breath condensate: A review of collection, processing and analysis
    • Grob NM, Aytekin M, Dweik RA. Biomarkers in exhaled breath condensate: a review of collection, processing and analysis. J Breath Res 2008;2:037004.
    • (2008) J Breath Res , vol.2 , pp. 037004
    • Grob, N.M.1    Aytekin, M.2    Dweik, R.A.3
  • 122
    • 84855202429 scopus 로고    scopus 로고
    • Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation
    • Clancy JP, Rowe SM, Accurso FJ, Aitken ML, Amin RS, Ashlock MA, Ballmann M, Boyle MP, Bronsveld I, Campbell PW, et al. Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation. Thorax 2012;67:12-18.
    • (2012) Thorax , vol.67 , pp. 12-18
    • Clancy, J.P.1    Rowe, S.M.2    Accurso, F.J.3    Aitken, M.L.4    Amin, R.S.5    Ashlock, M.A.6    Ballmann, M.7    Boyle, M.P.8    Bronsveld, I.9    Campbell, P.W.10
  • 125
    • 57649155710 scopus 로고    scopus 로고
    • A European consensus for the evaluation and management of infants with an equivocal diagnosis following newborn screening for cystic fibrosis
    • Mayell SJ, Munck A, Craig JV, Sermet I, Brownlee KG, Schwarz MJ, Castellani C, Southern KW. A European consensus for the evaluation and management of infants with an equivocal diagnosis following newborn screening for cystic fibrosis. J Cyst Fibros 2009;8:71-78.
    • (2009) J Cyst Fibros , vol.8 , pp. 71-78
    • Mayell, S.J.1    Munck, A.2    Craig, J.V.3    Sermet, I.4    Brownlee, K.G.5    Schwarz, M.J.6    Castellani, C.7    Southern, K.W.8
  • 126
    • 0031900652 scopus 로고    scopus 로고
    • The diagnosis of cystic fibrosis: A consensus statement. Cystic Fibrosis Foundation Consensus Panel
    • Rosenstein BJ, Cutting GR. The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus Panel. J Pediatr 1998;132:589-595.
    • (1998) J Pediatr , vol.132 , pp. 589-595
    • Rosenstein, B.J.1    Cutting, G.R.2
  • 129
    • 80052340084 scopus 로고    scopus 로고
    • Nasal potential difference measurements to assess CFTR ion channel activity
    • Rowe SM, Clancy JP, Wilschanski M. Nasal potential difference measurements to assess CFTR ion channel activity. Methods Mol Biol 2011;741:69-86.
    • (2011) Methods Mol Biol , vol.741 , pp. 69-86
    • Rowe, S.M.1    Clancy, J.P.2    Wilschanski, M.3
  • 132
    • 84880774140 scopus 로고    scopus 로고
    • Optimizing nasal potential difference analysis for CFTR modulator development: Assessment of ivacaftor in CF subjects with the G551DCFTR mutation
    • Rowe SM, Liu B, Hill A, Hathorne H, Cohen M, Beamer JR, Accurso FJ, Dong Q, Ordo~nez CL, Stone AJ, et al. Optimizing nasal potential difference analysis for CFTR modulator development: assessment of ivacaftor in CF subjects with the G551DCFTR mutation. PLoS ONE 2013;8:e66955.
    • (2013) PLoS ONE , vol.8 , pp. e66955
    • Rowe, S.M.1    Liu, B.2    Hill, A.3    Hathorne, H.4    Cohen, M.5    Beamer, J.R.6    Accurso, F.J.7    Dong, Q.8    Ordonez, C.L.9    Stone, A.J.10
  • 137
    • 77955298789 scopus 로고    scopus 로고
    • Intestinal current measurement for diagnostic classification of patients with questionable cystic fibrosis: Validation and reference data
    • Derichs N, Sanz J, Von Kanel T, Stolpe C, Zapf A, Tummler B, Gallati S, Ballmann M. Intestinal current measurement for diagnostic classification of patients with questionable cystic fibrosis: validation and reference data. Thorax 2010;65:594-599.
    • (2010) Thorax , vol.65 , pp. 594-599
    • Derichs, N.1    Sanz, J.2    Von Kanel, T.3    Stolpe, C.4    Zapf, A.5    Tummler, B.6    Gallati, S.7    Ballmann, M.8
  • 138
    • 11044220951 scopus 로고    scopus 로고
    • Intestinal current measurements to diagnose cystic fibrosis
    • Hug MJ, Tümmler B. Intestinal current measurements to diagnose cystic fibrosis. J Cyst Fibros 2004;3:157-158.
    • (2004) J Cyst Fibros , vol.3 , pp. 157-158
    • Hug, M.J.1    Tümmler, B.2
  • 139
    • 11044231602 scopus 로고    scopus 로고
    • Assessment of CFTR function in rectal biopsies for the diagnosis of cystic fibrosis
    • Mall M, Hirtz S, Gonska T, Kunzelmann K. Assessment of CFTR function in rectal biopsies for the diagnosis of cystic fibrosis. J Cyst Fibros 2004;3:165-169.
    • (2004) J Cyst Fibros , vol.3 , pp. 165-169
    • Mall, M.1    Hirtz, S.2    Gonska, T.3    Kunzelmann, K.4
  • 143
    • 34548286919 scopus 로고    scopus 로고
    • Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials
    • Rowe SM, Accurso F, Clancy JP. Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials. Proc Am Thorac Soc 2007;4:387-398.
    • (2007) Proc Am Thorac Soc , vol.4 , pp. 387-398
    • Rowe, S.M.1    Accurso, F.2    Clancy, J.P.3
  • 144
    • 84871981271 scopus 로고    scopus 로고
    • Change in sweat chloride as a clinical end point in cystic fibrosis clinical trials: The ivacaftor experience
    • Durmowicz AG,Witzmann KA, Rosebraugh CJ, Chowdhury BA. Change in sweat chloride as a clinical end point in cystic fibrosis clinical trials: the ivacaftor experience. Chest 2013;143:14-18.
    • (2013) Chest , vol.143 , pp. 14-18
    • Durmowicz, A.G.1    Witzmann, K.A.2    Rosebraugh, C.J.3    Chowdhury, B.A.4
  • 146
    • 84926005391 scopus 로고    scopus 로고
    • Sweat conductivity: An accurate diagnostic test for cystic fibrosis?
    • Mattar AC, Leone C, Rodrigues JC, Adde FV. Sweat conductivity: an accurate diagnostic test for cystic fibrosis?. J Cyst Fibros 2014;13:528-533.
    • (2014) J Cyst Fibros , vol.13 , pp. 528-533
    • Mattar, A.C.1    Leone, C.2    Rodrigues, J.C.3    Adde, F.V.4
  • 147
    • 84865853894 scopus 로고    scopus 로고
    • Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation
    • Flume PA, Liou TG, Borowitz DS, Li H, Yen K, Ordoñez CL, Geller DE. Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation. Chest 2012;142: 718-724.
    • (2012) Chest , vol.142 , pp. 718-724
    • Flume, P.A.1    Liou, T.G.2    Borowitz, D.S.3    Li, H.4    Yen, K.5    Ordoñez, C.L.6    Geller, D.E.7
  • 149
    • 84886248186 scopus 로고    scopus 로고
    • In vivo readout of CFTR function: Ratiometric measurement of CFTR-dependent secretion by individual, identifiable human sweat glands
    • Wine JJ, Char JE, Chen J, Cho HJ, Dunn C, Frisbee E, Joo NS, Milla C, Modlin SE, Park IH, et al. In vivo readout of CFTR function: ratiometric measurement of CFTR-dependent secretion by individual, identifiable human sweat glands. PLoS ONE 2013;8:e77114.
    • (2013) PLoS ONE , vol.8 , pp. e77114
    • Wine, J.J.1    Char, J.E.2    Chen, J.3    Cho, H.J.4    Dunn, C.5    Frisbee, E.6    Joo, N.S.7    Milla, C.8    Modlin, S.E.9    Park, I.H.10


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.