메뉴 건너뛰기




Volumn 74, Issue 1, 2017, Pages 129-140

Cystic fibrosis: a clinical view

Author keywords

CFTR; Cystic fibrosis; Genotype; Phenotype; Precision medicine

Indexed keywords

4 PHENYLBUTYRIC ACID; AMINOGLYCOSIDE ANTIBIOTIC AGENT; AMITRIPTYLINE; ANTIBIOTIC AGENT; ATALUREN; CORTICOSTEROID; CURCUMIN; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DENUFOSOL; GENISTEIN; GLUTATHIONE; IBUPROFEN; IVACAFTOR; MACROLIDE; MUCOLYTIC AGENT; PANCREAS ENZYME; RESVERATROL; ROSCOVITINE; TOBRAMYCIN;

EID: 84990855968     PISSN: 1420682X     EISSN: 14209071     Source Type: Journal    
DOI: 10.1007/s00018-016-2393-9     Document Type: Review
Times cited : (169)

References (126)
  • 1
    • 0000471085 scopus 로고
    • Cystic fibrosis of the pancreas and its relation to celiac disease
    • Andersen DH (1938) Cystic fibrosis of the pancreas and its relation to celiac disease. Am J Dis Child 56:344–399
    • (1938) Am J Dis Child , vol.56 , pp. 344-399
    • Andersen, D.H.1
  • 2
    • 85008399707 scopus 로고    scopus 로고
    • Accessed 1 Aug 2016
    • http://www.cysticfibrosis.ca/. Accessed 1 Aug 2016
  • 3
    • 84902314504 scopus 로고    scopus 로고
    • Rate of improvement of CF life expectancy exceeds that of general population–observational death registration study
    • PID: 24418187
    • Hurley MN, McKeever TM, Prayle AP, Fogarty AW, Smyth AR (2014) Rate of improvement of CF life expectancy exceeds that of general population–observational death registration study. J Cyst Fibros 13:410–415
    • (2014) J Cyst Fibros , vol.13 , pp. 410-415
    • Hurley, M.N.1    McKeever, T.M.2    Prayle, A.P.3    Fogarty, A.W.4    Smyth, A.R.5
  • 5
    • 84880030599 scopus 로고    scopus 로고
    • Management of comorbidities in older patients with cystic fibrosis
    • PID: 24429097
    • Plant BJ, Goss CH, Plant WD, Bell SC (2013) Management of comorbidities in older patients with cystic fibrosis. Lancet Respir Med 1:164–174
    • (2013) Lancet Respir Med , vol.1 , pp. 164-174
    • Plant, B.J.1    Goss, C.H.2    Plant, W.D.3    Bell, S.C.4
  • 6
    • 33645307384 scopus 로고    scopus 로고
    • The ABC protein turned chloride channel whose failure causes cystic fibrosis
    • COI: 1:CAS:528:DC%2BD28Xis1OlsLk%3D, PID: 16554808
    • Gadsby DC, Vergani P, Csanády L (2006) The ABC protein turned chloride channel whose failure causes cystic fibrosis. Nature 440:477–483
    • (2006) Nature , vol.440 , pp. 477-483
    • Gadsby, D.C.1    Vergani, P.2    Csanády, L.3
  • 7
    • 80053368675 scopus 로고    scopus 로고
    • New animal models of cystic fibrosis: what are they teaching us?
    • PID: 21857224
    • Keiser NW, Engelhardt JF (2011) New animal models of cystic fibrosis: what are they teaching us? Curr Opin Pulm Med 17:478–483
    • (2011) Curr Opin Pulm Med , vol.17 , pp. 478-483
    • Keiser, N.W.1    Engelhardt, J.F.2
  • 8
    • 84878020008 scopus 로고    scopus 로고
    • Growth retardation and reduced growth hormone secretion in cystic fibrosis. Clinical observations from three CF centers
    • COI: 1:CAS:528:DC%2BC3sXisVGnt7c%3D, PID: 22981294
    • Ciro D, Padoan R, Blau H, Marostica A, Fuoti M, Volpi S, Pilotta A, Meyerovitch J, Sher D, Assael BM (2013) Growth retardation and reduced growth hormone secretion in cystic fibrosis. Clinical observations from three CF centers. J Cyst Fibros 12:165–169
    • (2013) J Cyst Fibros , vol.12 , pp. 165-169
    • Ciro, D.1    Padoan, R.2    Blau, H.3    Marostica, A.4    Fuoti, M.5    Volpi, S.6    Pilotta, A.7    Meyerovitch, J.8    Sher, D.9    Assael, B.M.10
  • 9
    • 33745485883 scopus 로고    scopus 로고
    • The cystic fibrosis transmembrane conductance regulator (CFTR) modulates the timing of puberty in mice
    • COI: 1:STN:280:DC%2BD28zkt1ahsA%3D%3D, PID: 16740913
    • Jin R, Hodges CA, Drumm ML, Palmert MR (2006) The cystic fibrosis transmembrane conductance regulator (CFTR) modulates the timing of puberty in mice. J Med Genet 43:e29
    • (2006) J Med Genet , vol.43
    • Jin, R.1    Hodges, C.A.2    Drumm, M.L.3    Palmert, M.R.4
  • 11
    • 0038724737 scopus 로고    scopus 로고
    • The association of nephrolithiasis with cystic fibrosis
    • COI: 1:CAS:528:DC%2BD3sXmtVajt7g%3D, PID: 12830451
    • Gibney EM, Goldfarb DS (2003) The association of nephrolithiasis with cystic fibrosis. Am J Kidney Dis 42:1–11
    • (2003) Am J Kidney Dis , vol.42 , pp. 1-11
    • Gibney, E.M.1    Goldfarb, D.S.2
  • 12
    • 84872557485 scopus 로고    scopus 로고
    • Cancer risk in cystic Fibrosis: A 20-year nationwide study from the United States
    • COI: 1:CAS:528:DC%2BC3sXhsVKgu7w%3D, PID: 23178438
    • Maisonneuve P, Marshall BC, Knapp EA, Lowenfels AB (2013) Cancer risk in cystic Fibrosis: A 20-year nationwide study from the United States. J Natl Cancer Inst 105:122–129
    • (2013) J Natl Cancer Inst , vol.105 , pp. 122-129
    • Maisonneuve, P.1    Marshall, B.C.2    Knapp, E.A.3    Lowenfels, A.B.4
  • 13
    • 85027934344 scopus 로고    scopus 로고
    • Early colon screening of adult patients with cystic fibrosis reveals high incidence of adenomatous colon polyps
    • PID: 24275715
    • Billings JL, Dunitz JM, McAllister S, Herzog T, Bobr A, Khoruts A (2014) Early colon screening of adult patients with cystic fibrosis reveals high incidence of adenomatous colon polyps. J Clin Gastroenterol 48:e85–e88
    • (2014) J Clin Gastroenterol , vol.48 , pp. e85-e88
    • Billings, J.L.1    Dunitz, J.M.2    McAllister, S.3    Herzog, T.4    Bobr, A.5    Khoruts, A.6
  • 14
    • 84947438167 scopus 로고    scopus 로고
    • Cystic fibrosis could survive cholera, choleraics could survive cystic fibrosis, hypothesis that explores new horizon in treatmnet of cystic fibrosis
    • Azimi A (2015) Cystic fibrosis could survive cholera, choleraics could survive cystic fibrosis, hypothesis that explores new horizon in treatmnet of cystic fibrosis. Med Hypoth 85:715–717
    • (2015) Med Hypoth , vol.85 , pp. 715-717
    • Azimi, A.1
  • 19
    • 0026641782 scopus 로고
    • The spectrum of cystic fibrosis mutations
    • COI: 1:CAS:528:DyaK3sXpvFSqug%3D%3D, PID: 1279852
    • Tsui LC (1992) The spectrum of cystic fibrosis mutations. Trends Genet 8:392–398
    • (1992) Trends Genet , vol.8 , pp. 392-398
    • Tsui, L.C.1
  • 20
    • 0027162649 scopus 로고
    • Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis
    • COI: 1:CAS:528:DyaK3sXkvFant70%3D, PID: 7686820
    • Welsh MJ, Smith AE (1993) Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell 73:1251–1254
    • (1993) Cell , vol.73 , pp. 1251-1254
    • Welsh, M.J.1    Smith, A.E.2
  • 21
    • 0029616734 scopus 로고
    • Cystic fibrosis: genotypic and phenotypic variations
    • COI: 1:CAS:528:DyaK28XlsF2h, PID: 8825494
    • Zielenski J, Tsui LC (1995) Cystic fibrosis: genotypic and phenotypic variations. Annu Rev Genet 29:777–807
    • (1995) Annu Rev Genet , vol.29 , pp. 777-807
    • Zielenski, J.1    Tsui, L.C.2
  • 22
    • 85008405686 scopus 로고    scopus 로고
    • Genotypes and Phenotypes in Cystic Fibrosis and Cystic Fibrosis Transmembrane Regulator-Related DisordersSemin Respir
    • Bombieri C, Seia M, Castellani C (2015) Genotypes and Phenotypes in Cystic Fibrosis and Cystic Fibrosis Transmembrane Regulator-Related DisordersSemin Respir. Crit Care Med 36:1–14
    • (2015) Crit Care Med , vol.36 , pp. 1-14
    • Bombieri, C.1    Seia, M.2    Castellani, C.3
  • 23
    • 0013279518 scopus 로고
    • Cystic fibrosis of the pancreas
    • COI: 1:STN:280:DyaG1c%2FjsVCgsA%3D%3D, PID: 13491678
    • Andersen DH (1958) Cystic fibrosis of the pancreas. J Chronic Dis 7:58–90
    • (1958) J Chronic Dis , vol.7 , pp. 58-90
    • Andersen, D.H.1
  • 26
    • 0020080186 scopus 로고
    • Genetic and environmental influences on pulmonary function in adult twins
    • COI: 1:STN:280:DyaL387otVektg%3D%3D, PID: 7200340
    • Hubert HB, Fabsitz RR, Feinleib M, Gwinn C (1982) Genetic and environmental influences on pulmonary function in adult twins. Am Rev Respir Dis. 125:409–415
    • (1982) Am Rev Respir Dis. , vol.125 , pp. 409-415
    • Hubert, H.B.1    Fabsitz, R.R.2    Feinleib, M.3    Gwinn, C.4
  • 27
    • 0027938319 scopus 로고
    • Genetic and environmental influences on pulmonary function in aging Swedish twins
    • COI: 1:STN:280:DyaK2M%2Flt1entw%3D%3D, PID: 7963289
    • McClearn GE, Svartengren M, Pedersen NL, Heller DA, Plomin R (1994) Genetic and environmental influences on pulmonary function in aging Swedish twins. J Gerontol. 49:264–268
    • (1994) J Gerontol. , vol.49 , pp. 264-268
    • McClearn, G.E.1    Svartengren, M.2    Pedersen, N.L.3    Heller, D.A.4    Plomin, R.5
  • 28
    • 85008347294 scopus 로고    scopus 로고
    • Genetic and environmental modifiers of cystic fibrosis
    • Cystic fibrosis, ERS Monographs
    • Tümmler B., Stanke F. (2014) Genetic and environmental modifiers of cystic fibrosis. In: Mall MA, Elborn JS (eds) Cystic fibrosis. ERS Monographs
    • (2014) Mall MA
    • Tümmler, B.1    Stanke, F.2    Elborn, J.S.3
  • 31
    • 51249089910 scopus 로고    scopus 로고
    • Modulation of cystic fibrosis lung disease by variants in interleukin-8
    • COI: 1:CAS:528:DC%2BD1cXhtVKrsLzL, PID: 18563170
    • Hillian AD, Londono D, Dunn JM, Goddard KAB, Pace RG, Knowles MR, Drumm ML, CF Gene Modifier Study Group (2008) Modulation of cystic fibrosis lung disease by variants in interleukin-8. Genes Immun 9:501–508
    • (2008) Genes Immun , vol.9 , pp. 501-508
    • Hillian, A.D.1    Londono, D.2    Dunn, J.M.3    Goddard, K.A.B.4    Pace, R.G.5    Knowles, M.R.6    Drumm, M.L.7
  • 36
    • 84927710902 scopus 로고    scopus 로고
    • Early pulmonary inflammation and lung damage in children with cystic fibrosis
    • PID: 25823858
    • Schultz A, Stick S (2015) Early pulmonary inflammation and lung damage in children with cystic fibrosis. Respirology 20:569–578
    • (2015) Respirology , vol.20 , pp. 569-578
    • Schultz, A.1    Stick, S.2
  • 37
    • 84859620802 scopus 로고    scopus 로고
    • Cystic fibrosis: a mucosal immunodeficiency syndrome
    • COI: 1:CAS:528:DC%2BC38XltFOgs78%3D, PID: 22481418
    • Cohen TS, Prince A (2012) Cystic fibrosis: a mucosal immunodeficiency syndrome. Nat Med 18:509–519
    • (2012) Nat Med , vol.18 , pp. 509-519
    • Cohen, T.S.1    Prince, A.2
  • 39
    • 84960128253 scopus 로고    scopus 로고
    • The role of airway macrophages in apoptotic cell clearance following acute and chronic lung inflammation
    • COI: 1:CAS:528:DC%2BC28XktlCjtLc%3D, PID: 26957481
    • Grabiec AM, Hussell T (2016) The role of airway macrophages in apoptotic cell clearance following acute and chronic lung inflammation. Semin Immunopathol. 38:409–423
    • (2016) Semin Immunopathol. , vol.38 , pp. 409-423
    • Grabiec, A.M.1    Hussell, T.2
  • 40
    • 84867758702 scopus 로고    scopus 로고
    • Update of faecal markers of inflammation in children with cystic fibrosis
    • PID: 22988347
    • Lee JM, Leach ST, Katz T, Day AS, Jaffe A, Ooi CY (2012) Update of faecal markers of inflammation in children with cystic fibrosis. Mediators Inflamm 2012:948367
    • (2012) Mediators Inflamm , vol.2012 , pp. 948367
    • Lee, J.M.1    Leach, S.T.2    Katz, T.3    Day, A.S.4    Jaffe, A.5    Ooi, C.Y.6
  • 43
    • 0028991826 scopus 로고
    • In vivo nasal potential techniques and protocols for the assessment of the efficiency of gene transfer in cystic fibrosis
    • COI: 1:STN:280:DyaK2MzjslWrtg%3D%3D, PID: 7542031
    • Knowles MR, Paradiso AM, Boucher RC (1995) In vivo nasal potential techniques and protocols for the assessment of the efficiency of gene transfer in cystic fibrosis. Hum Gene Ther 6:445–455
    • (1995) Hum Gene Ther , vol.6 , pp. 445-455
    • Knowles, M.R.1    Paradiso, A.M.2    Boucher, R.C.3
  • 44
    • 11044232030 scopus 로고    scopus 로고
    • Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambers
    • PID: 15463951
    • De Jonge HR, Ballmann M, Veeze H, Bronsveld I, Stanke F, Tümmler B, Sinaasappel M (2004) Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambers. J Cyst Fibros 3:159–163
    • (2004) J Cyst Fibros , vol.3 , pp. 159-163
    • De Jonge, H.R.1    Ballmann, M.2    Veeze, H.3    Bronsveld, I.4    Stanke, F.5    Tümmler, B.6    Sinaasappel, M.7
  • 45
    • 84892948463 scopus 로고    scopus 로고
    • Hyperechogenic fetal bowel: an ultrasonographic marker for adverse fetal and neonatal outcome?
    • De Oronzo MA (2011) Hyperechogenic fetal bowel: an ultrasonographic marker for adverse fetal and neonatal outcome? J Prenatal Med 5:9–13
    • (2011) J Prenatal Med , vol.5 , pp. 9-13
    • De Oronzo, M.A.1
  • 46
    • 84961864501 scopus 로고    scopus 로고
    • Newborn screening for cystic fibrosis, Lancet Respir Med
    • Castellani C, Massie J, Sontag M, Southern KW (2016) Newborn screening for cystic fibrosis. Lancet Respir Med. doi:10.1016/S2213-2600(16)00053-9
    • (2016) Southern KW
    • Castellani, C.1    Massie, J.2    Sontag, M.3
  • 47
    • 34447523813 scopus 로고    scopus 로고
    • Cystic fibrosis: lessons from the sweat gland
    • Quinton P (2006) Cystic fibrosis: lessons from the sweat gland. Physiology 22:212–225
    • (2006) Physiology , vol.22 , pp. 212-225
    • Quinton, P.1
  • 49
    • 9644303188 scopus 로고    scopus 로고
    • Detecting early lung disease in cystic fibrosis: are current techniques sufficient?
    • COI: 1:STN:280:DC%2BD2crptVKqsg%3D%3D, PID: 15563695
    • Sly PD, Brennan S (2004) Detecting early lung disease in cystic fibrosis: are current techniques sufficient? Thorax 59:1008–1010
    • (2004) Thorax , vol.59 , pp. 1008-1010
    • Sly, P.D.1    Brennan, S.2
  • 50
    • 0036708198 scopus 로고    scopus 로고
    • Detecting early structural lung damage in cystic fibrosis
    • PID: 12203854
    • Tiddens HA (2002) Detecting early structural lung damage in cystic fibrosis. Pediatr Pulmonol 34:228–231
    • (2002) Pediatr Pulmonol , vol.34 , pp. 228-231
    • Tiddens, H.A.1
  • 53
    • 84906850138 scopus 로고    scopus 로고
    • Current concepts: host-pathogen interactions in cystic fibrosis airways disease
    • PID: 25176968
    • Tang AC, Turvey SE, Alves MP, Regamey N, Tümmler B, Hartl D (2014) Current concepts: host-pathogen interactions in cystic fibrosis airways disease. Eur Respir Rev 23:320–332
    • (2014) Eur Respir Rev , vol.23 , pp. 320-332
    • Tang, A.C.1    Turvey, S.E.2    Alves, M.P.3    Regamey, N.4    Tümmler, B.5    Hartl, D.6
  • 55
    • 84959318214 scopus 로고    scopus 로고
    • The Microbiome in Cystic Fibrosis
    • PID: 26857768
    • Huang YJ, LiPuma JJ (2016) The Microbiome in Cystic Fibrosis. Clin Chest Med 37:59–67
    • (2016) Clin Chest Med , vol.37 , pp. 59-67
    • Huang, Y.J.1    LiPuma, J.J.2
  • 56
    • 84924971561 scopus 로고    scopus 로고
    • Management of pulmonary exacerbations in cystic fibrosis: still an unmet medical need in clinical practice
    • COI: 1:CAS:528:DC%2BC2MXktlersrY%3D, PID: 25692532
    • Justicia JL, Solé A, Quintana-Gallego E, Gartner S, de Gracia J, Prados C, Máiz L (2015) Management of pulmonary exacerbations in cystic fibrosis: still an unmet medical need in clinical practice. Expert Rev Respir Med. 9:183–194
    • (2015) Expert Rev Respir Med. , vol.9 , pp. 183-194
    • Justicia, J.L.1    Solé, A.2    Quintana-Gallego, E.3    Gartner, S.4    de Gracia, J.5    Prados, C.6    Máiz, L.7
  • 57
    • 84869120178 scopus 로고    scopus 로고
    • Treatment of lung infection in patients with cystic fibrosis: current and future strategies
    • Döring G, Flume P, Heijerman H, Elborn JS
    • Döring G, Flume P, Heijerman H, Elborn JS; Consensus Study Group (2012) Treatment of lung infection in patients with cystic fibrosis: current and future strategies. J Cyst Fibros 1:461–479
    • (2012) J Cyst Fibros , vol.1 , pp. 461-479
  • 58
    • 80053364019 scopus 로고    scopus 로고
    • Pulmonary exacerbations in cystic fibrosis
    • PID: 21881509
    • Stenbit AE, Flume PA (2011) Pulmonary exacerbations in cystic fibrosis. Curr Opin Pulm Med. 17:442–447
    • (2011) Curr Opin Pulm Med. , vol.17 , pp. 442-447
    • Stenbit, A.E.1    Flume, P.A.2
  • 59
    • 0030974760 scopus 로고    scopus 로고
    • Epidemiology of chronic Pseudomonas aeruginosa infections in the airways of lung transplant recipients with cystic fibrosis
    • COI: 1:STN:280:DyaK2szjvF2kug%3D%3D, PID: 9196512
    • Walter S, Gudowius P, Bosshammer J, Römling U, Weissbrodt H, Schürmann W, von der Hardt H, Tümmler B (1997) Epidemiology of chronic Pseudomonas aeruginosa infections in the airways of lung transplant recipients with cystic fibrosis. Thorax 52:318–321
    • (1997) Thorax , vol.52 , pp. 318-321
    • Walter, S.1    Gudowius, P.2    Bosshammer, J.3    Römling, U.4    Weissbrodt, H.5    Schürmann, W.6    von der Hardt, H.7    Tümmler, B.8
  • 61
    • 84927760300 scopus 로고    scopus 로고
    • Management of the upper airway in cystic fibrosis
    • PID: 25250804
    • Illing EA, Woodworth BA (2014) Management of the upper airway in cystic fibrosis. Curr Opin Pulm Med. 20:623–631
    • (2014) Curr Opin Pulm Med. , vol.20 , pp. 623-631
    • Illing, E.A.1    Woodworth, B.A.2
  • 64
    • 33645965850 scopus 로고    scopus 로고
    • Fecal elastase-1: utility in pancreatic function in cystic fibrosis
    • COI: 1:CAS:528:DC%2BD28XktVyiurw%3D, PID: 16603421
    • Daftary A, Acton J, Heubi J, Amin R (2006) Fecal elastase-1: utility in pancreatic function in cystic fibrosis. J Cyst Fibros 5:71–76
    • (2006) J Cyst Fibros , vol.5 , pp. 71-76
    • Daftary, A.1    Acton, J.2    Heubi, J.3    Amin, R.4
  • 66
    • 0034000370 scopus 로고    scopus 로고
    • Evidence of chronic inflammation in morphologically normal small intestine of cystic fibrosis patients
    • COI: 1:STN:280:DC%2BD3c7nt12ltQ%3D%3D, PID: 10709733
    • Raia V, Maiuri L, de Ritis G, de Vizia B, Vacca L, Conte R, Auricchio S, Londei M (2000) Evidence of chronic inflammation in morphologically normal small intestine of cystic fibrosis patients. Pediatr Res 47:344–350
    • (2000) Pediatr Res , vol.47 , pp. 344-350
    • Raia, V.1    Maiuri, L.2    de Ritis, G.3    de Vizia, B.4    Vacca, L.5    Conte, R.6    Auricchio, S.7    Londei, M.8
  • 68
    • 84901625117 scopus 로고    scopus 로고
    • Cystic fibrosis: evidence for gut inflammation
    • COI: 1:CAS:528:DC%2BC2cXjslKqtbY%3D, PID: 24548777
    • Munck A (2014) Cystic fibrosis: evidence for gut inflammation. Int J Biochem Cell Biol 52:180–183
    • (2014) Int J Biochem Cell Biol , vol.52 , pp. 180-183
    • Munck, A.1
  • 69
    • 84878020984 scopus 로고    scopus 로고
    • Cirrhosis and other liver disease in cystic fibrosis
    • PID: 23266093
    • Flass T, Narkewicz MR (2013) Cirrhosis and other liver disease in cystic fibrosis. J Cyst Fibros 12:116–124
    • (2013) J Cyst Fibros , vol.12 , pp. 116-124
    • Flass, T.1    Narkewicz, M.R.2
  • 70
    • 0035254204 scopus 로고    scopus 로고
    • Liver expression in cystic fibrosis could be modulated by genetic factors different from the cystic fibrosis transmembrane regulator genotype
    • COI: 1:STN:280:DC%2BD3M3mvVOltQ%3D%3D, PID: 11170070
    • Castaldo G, Fuccio A, Salvatore D, Raia V, Santostasi T, Leonardi S, Lizzi N, La Rosa M, Rigillo N, Salvatore F (2001) Liver expression in cystic fibrosis could be modulated by genetic factors different from the cystic fibrosis transmembrane regulator genotype. Am J Med Genet 98:294–297
    • (2001) Am J Med Genet , vol.98 , pp. 294-297
    • Castaldo, G.1    Fuccio, A.2    Salvatore, D.3    Raia, V.4    Santostasi, T.5    Leonardi, S.6    Lizzi, N.7    La Rosa, M.8    Rigillo, N.9    Salvatore, F.10
  • 73
    • 84878350788 scopus 로고    scopus 로고
    • Update on cystic fibrosis-related diabetes
    • PID: 23562217, (Erratum. In: J Cyst Fibros. 2014;13:119)
    • Kelly A, Moran A (2013) Update on cystic fibrosis-related diabetes. J Cyst Fibros 12:318–331. doi:10.1016/j.jcf.2013.02.008 (Erratum. In: J Cyst Fibros. 2014;13:119)
    • (2013) J Cyst Fibros , vol.12 , pp. 318-331
    • Kelly, A.1    Moran, A.2
  • 76
    • 34447325620 scopus 로고    scopus 로고
    • Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in human bone
    • PID: 17600296
    • Shead EF, Haworth CS, Condliffe AM, McKeon DJ, Scott MA, Compston JE (2007) Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in human bone. Thorax 62:650–651
    • (2007) Thorax , vol.62 , pp. 650-651
    • Shead, E.F.1    Haworth, C.S.2    Condliffe, A.M.3    McKeon, D.J.4    Scott, M.A.5    Compston, J.E.6
  • 77
    • 83755185534 scopus 로고    scopus 로고
    • CFTR mutations in men with congenital bilateral absence of the vas deferens (CBAVD): a systemic review and meta-analysis
    • COI: 1:CAS:528:DC%2BC3MXhs1CqtrzJ, PID: 22081250
    • Yu J, Chen Z, Ni Y, Li Z (2012) CFTR mutations in men with congenital bilateral absence of the vas deferens (CBAVD): a systemic review and meta-analysis. Hum Reprod 27:25–35
    • (2012) Hum Reprod , vol.27 , pp. 25-35
    • Yu, J.1    Chen, Z.2    Ni, Y.3    Li, Z.4
  • 78
    • 0033709330 scopus 로고    scopus 로고
    • The cystic fibrosis transmembrane regulator gene and male infertility
    • COI: 1:CAS:528:DC%2BD3cXos1yksLo%3D, PID: 11097434
    • Quinzii C, Castellani C (2000) The cystic fibrosis transmembrane regulator gene and male infertility. J Endocrinol Invest 23:684–689
    • (2000) J Endocrinol Invest , vol.23 , pp. 684-689
    • Quinzii, C.1    Castellani, C.2
  • 79
    • 66749174619 scopus 로고    scopus 로고
    • Growth and long-term lung function in cystic fibrosis: a longitudinal study of patients diagnosed by neonatal screening
    • PID: 19230003
    • Assael BM, Casazza G, Iansa P, Volpi S, Milani S (2009) Growth and long-term lung function in cystic fibrosis: a longitudinal study of patients diagnosed by neonatal screening. Pediatr Pulmonol 44:209–215
    • (2009) Pediatr Pulmonol , vol.44 , pp. 209-215
    • Assael, B.M.1    Casazza, G.2    Iansa, P.3    Volpi, S.4    Milani, S.5
  • 82
    • 0023923679 scopus 로고
    • A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto
    • COI: 1:STN:280:DyaL1c3lvVGmuw%3D%3D, PID: 3260274
    • Corey M, McLaughlin FJ, Williams M, Levison H (1988) A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol 41:583–591
    • (1988) J Clin Epidemiol , vol.41 , pp. 583-591
    • Corey, M.1    McLaughlin, F.J.2    Williams, M.3    Levison, H.4
  • 84
    • 84869087168 scopus 로고    scopus 로고
    • Relation between dietary fat intake type and serum fatty acid status in children with cystic fibrosis
    • COI: 1:CAS:528:DC%2BC38XhsFymsbnP, PID: 22699835
    • Maqbool A, Schall JI, Gallagher PR, Zemel BS, Strandvik B, Stallings VA (2012) Relation between dietary fat intake type and serum fatty acid status in children with cystic fibrosis. J Pediatr Gastroenterol Nutr 55:605–611
    • (2012) J Pediatr Gastroenterol Nutr , vol.55 , pp. 605-611
    • Maqbool, A.1    Schall, J.I.2    Gallagher, P.R.3    Zemel, B.S.4    Strandvik, B.5    Stallings, V.A.6
  • 85
    • 77956492443 scopus 로고    scopus 로고
    • Fatty acid metabolism in cystic fibrosis
    • COI: 1:CAS:528:DC%2BC3cXhtFGhurzF, PID: 20673710
    • Strandvik B (2010) Fatty acid metabolism in cystic fibrosis. Prostaglandins Leukot Essent Fatty Acids 83:121–129
    • (2010) Prostaglandins Leukot Essent Fatty Acids , vol.83 , pp. 121-129
    • Strandvik, B.1
  • 88
    • 84959317612 scopus 로고    scopus 로고
    • Lung transplantation for cystic fibrosis
    • Morrell MR, Pilewski JM (2016) Lung transplantation for cystic fibrosis. Clin Chest Med 37:127–138
    • (2016) Clin Chest Med , vol.37 , pp. 127-138
    • Morrell, M.R.1    Pilewski, J.M.2
  • 89
    • 84926225425 scopus 로고    scopus 로고
    • Lung transplantation for cystic fibrosis: results, indications, complications, and controversies
    • PID: 25826595
    • Lynch JP 3rd, Sayah DM, Belperio JA, Weigt SS (2015) Lung transplantation for cystic fibrosis: results, indications, complications, and controversies. Semin Respir Crit Care Med. 36:299–320
    • (2015) Semin Respir Crit Care Med. , vol.36 , pp. 299-320
    • Lynch, J.P.1    Sayah, D.M.2    Belperio, J.A.3    Weigt, S.S.4
  • 91
    • 84939774600 scopus 로고    scopus 로고
    • What is the role of endoscopic sinus surgery in adult patients with cystic fibrosis?
    • PID: 25682930
    • Hughes A, Adil EA (2015) What is the role of endoscopic sinus surgery in adult patients with cystic fibrosis? Laryngoscope 125:2018–2020
    • (2015) Laryngoscope , vol.125 , pp. 2018-2020
    • Hughes, A.1    Adil, E.A.2
  • 92
    • 34548038663 scopus 로고    scopus 로고
    • High-dose ibuprofen in cystic fibrosis: Canadian safety and effectiveness trial
    • COI: 1:CAS:528:DC%2BD2sXpsFagu7k%3D, PID: 17719932
    • Lands LC, Milner R, Cantin AM, Manson D, Corey M (2007) High-dose ibuprofen in cystic fibrosis: Canadian safety and effectiveness trial. J Pediatr 151:249–254
    • (2007) J Pediatr , vol.151 , pp. 249-254
    • Lands, L.C.1    Milner, R.2    Cantin, A.M.3    Manson, D.4    Corey, M.5
  • 93
    • 84879524095 scopus 로고    scopus 로고
    • Long-term macrolide treatment for chronic respiratory disease
    • COI: 1:CAS:528:DC%2BC3sXht1Clu7bJ, PID: 23180583
    • Spagnolo P, Fabbri LM, Bush A (2013) Long-term macrolide treatment for chronic respiratory disease. Eur Respir J 42:239–251
    • (2013) Eur Respir J , vol.42 , pp. 239-251
    • Spagnolo, P.1    Fabbri, L.M.2    Bush, A.3
  • 95
  • 96
    • 84861891585 scopus 로고    scopus 로고
    • Advances in cell and gene-based therapies for cystic fi brosis lung disease
    • COI: 1:CAS:528:DC%2BC38XivV2rsLo%3D, PID: 22371844
    • Oakland M, Sinn PL, McCray PB Jr (2012) Advances in cell and gene-based therapies for cystic fi brosis lung disease. Mol Ther 20:1108–1115
    • (2012) Mol Ther , vol.20 , pp. 1108-1115
    • Oakland, M.1    Sinn, P.L.2    McCray, P.B.3
  • 98
    • 0036665609 scopus 로고    scopus 로고
    • Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate
    • COI: 1:CAS:528:DC%2BD38XltVGqsL4%3D, PID: 12095312
    • Zeitlin PL, Diener-West M, Rubenstein RC, Boyle MP, Lee CK, Brass-Ernst L (2002) Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate. Mol Ther 6:119–126
    • (2002) Mol Ther , vol.6 , pp. 119-126
    • Zeitlin, P.L.1    Diener-West, M.2    Rubenstein, R.C.3    Boyle, M.P.4    Lee, C.K.5    Brass-Ernst, L.6
  • 102
    • 77953251984 scopus 로고    scopus 로고
    • Acid sphingomyelinase inhibitors normalize pulmonary ceramide and inflammation in cystic fibrosis
    • COI: 1:CAS:528:DC%2BC3cXhsVKrtLjI, PID: 19635928
    • Becker KA, Riethmüller J, Lüth A, Döring G, Kleuser B, Gulbins E (2010) Acid sphingomyelinase inhibitors normalize pulmonary ceramide and inflammation in cystic fibrosis. Am J Respir Cell Mol Biol 42:716–724
    • (2010) Am J Respir Cell Mol Biol , vol.42 , pp. 716-724
    • Becker, K.A.1    Riethmüller, J.2    Lüth, A.3    Döring, G.4    Kleuser, B.5    Gulbins, E.6
  • 104
    • 84992067869 scopus 로고    scopus 로고
    • Natural Compounds as Therapeutic Agents in the Treatment Cystic Fibrosis
    • Dey I, Shah K, Bradbury NA (2016) Natural Compounds as Therapeutic Agents in the Treatment Cystic Fibrosis. J Genet Syndr Gene Ther 7pii: 284
    • (2016) J Genet Syndr Gene Ther 7pii , pp. 284
    • Dey, I.1    Shah, K.2    Bradbury, N.A.3
  • 105
    • 84884213627 scopus 로고    scopus 로고
    • Pitfalls of drug development: lessons learned from trials of denufosol in cystic fibrosis
    • PID: 23290508
    • Moss RB (2013) Pitfalls of drug development: lessons learned from trials of denufosol in cystic fibrosis. J Pediatr 162:676–680
    • (2013) J Pediatr , vol.162 , pp. 676-680
    • Moss, R.B.1
  • 106
    • 85008385792 scopus 로고    scopus 로고
    • ClinicalTrials.gov Evaluation of (R)-Roscovitine Safety and Effects in Subjects With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation (ROSCO-CF) Last verified July 2016
    • ClinicalTrials.gov Evaluation of (R)-Roscovitine Safety and Effects in Subjects With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation (ROSCO-CF) Last verified July 2016
  • 107
    • 84893480156 scopus 로고    scopus 로고
    • European Cystic Fibrosis Society Clinical Trial Network Standardisation Committee. Clinimetric properties of bronchoalveolar lavage inflammatory markers in cystic fibrosis
    • PID: 23845715
    • Fayon M, Kent L, Bui S, Dupont L, Sermet I (2014) European Cystic Fibrosis Society Clinical Trial Network Standardisation Committee. Clinimetric properties of bronchoalveolar lavage inflammatory markers in cystic fibrosis. Eur Respir J 43:610–626
    • (2014) Eur Respir J , vol.43 , pp. 610-626
    • Fayon, M.1    Kent, L.2    Bui, S.3    Dupont, L.4    Sermet, I.5
  • 108
    • 0034073736 scopus 로고    scopus 로고
    • A pilot study of the effect of gentamicin on nasal potential difference measurements in cystic fibrosis patients carrying stop mutations
    • COI: 1:STN:280:DC%2BD3c7nsl2isA%3D%3D, PID: 10712334
    • Wilschanski M, Famini C, Blau H, Rivlin J, Augarten A, Avital A, Kerem B, Kerem E (2000) A pilot study of the effect of gentamicin on nasal potential difference measurements in cystic fibrosis patients carrying stop mutations. Am J Respir Crit Care Med 161:860–865
    • (2000) Am J Respir Crit Care Med , vol.161 , pp. 860-865
    • Wilschanski, M.1    Famini, C.2    Blau, H.3    Rivlin, J.4    Augarten, A.5    Avital, A.6    Kerem, B.7    Kerem, E.8
  • 113
    • 84964968279 scopus 로고    scopus 로고
    • Potentiator synergy in rectal organoids carrying S1251N, G551D, or F508del CFTR mutations
    • Dekkers JF, Van Mourik P, Vonk AM, Kruisselbrink E, Berkers G, de Winter-de Groot KM, Janssens HM, Bronsveld I, van der Ent CK, de Beekman JM (2016) Potentiator synergy in rectal organoids carrying S1251N, G551D, or F508del CFTR mutations. J Cyst Fibros. doi:10.1016/j.jcf.2016.04.007
    • (2016) J Cyst Fibros
    • Dekkers, J.F.1    Van Mourik, P.2    Vonk, A.M.3    Kruisselbrink, E.4    Berkers, G.5
  • 114
    • 84980016718 scopus 로고    scopus 로고
    • Optimal correction of distinct CFTR folding mutants in rectal cystic fibrosis organoids
    • Beekman JM
    • Dekkers JF, Gogorza Gondra RA, Kruisselbrink E, Vonk AM, Janssens HM, de Winter-de Groot KM, van der Ent CK, Beekman JM (2016) Optimal correction of distinct CFTR folding mutants in rectal cystic fibrosis organoids. Eur Respir J. doi:10.1183/13993003.01192-2015
    • (2016) Eur Respir J
    • Dekkers, J.F.1    Gogorza Gondra, R.A.2    Kruisselbrink, E.3    Vonk, A.M.4    Janssens, H.M.5
  • 116
    • 84904016376 scopus 로고    scopus 로고
    • Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial
    • Kerem E, Konstan MW, De Boeck K, Accurso FJ, Sermet-Gaudelus I, Wilschanski M, Elborn JS, Melotti P, Bronsveld I, Fajac I, Malfroot A, Rosenbluth DB, Walker PA, McColley SA, Knoop C, Quattrucci S, Rietschel E, Zeitlin PL, Barth J, Elfring GL, Welch EM, Branstrom A, Spiegel RJ, Peltz SW, Ajayi T, Rowe SM;
    • Kerem E, Konstan MW, De Boeck K, Accurso FJ, Sermet-Gaudelus I, Wilschanski M, Elborn JS, Melotti P, Bronsveld I, Fajac I, Malfroot A, Rosenbluth DB, Walker PA, McColley SA, Knoop C, Quattrucci S, Rietschel E, Zeitlin PL, Barth J, Elfring GL, Welch EM, Branstrom A, Spiegel RJ, Peltz SW, Ajayi T, Rowe SM; Cystic Fibrosis Ataluren Study Group (2014) Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial. Lancet Respir Med. 2:539–547
    • (2014) Lancet Respir Med. , vol.2 , pp. 539-547
  • 117
    • 80455162465 scopus 로고    scopus 로고
    • A CFTR potentiator in patients with cystic fibrosis and the G551D mutation
    • Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Dřevínek P, Griese M, McKone EF, Wainwright CE, Konstan MW, Moss R, Ratjen F, Sermet-Gaudelus I, Rowe SM, Dong Q, Rodriguez S, Yen K, Ordoñez C, Elborn JS
    • Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Dřevínek P, Griese M, McKone EF, Wainwright CE, Konstan MW, Moss R, Ratjen F, Sermet-Gaudelus I, Rowe SM, Dong Q, Rodriguez S, Yen K, Ordoñez C, Elborn JS; VX08-770-102 Study Group (2011) A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med 365:1663–1672
    • (2011) N Engl J Med , vol.365 , pp. 1663-1672
  • 119
    • 84943138352 scopus 로고    scopus 로고
    • Efficacy and safety of ivacaftor in patients with cystic fibrosis who have an Arg117His-CFTR mutation: a double-blind, randomised controlled trial
    • Moss RB, Flume PA, Elborn JS, Cooke J, Rowe SM, McColley SA, Rubenstein RC, Higgins M;
    • Moss RB, Flume PA, Elborn JS, Cooke J, Rowe SM, McColley SA, Rubenstein RC, Higgins M; VX11-770-110 (KONDUCT) Study Group (2015) Efficacy and safety of ivacaftor in patients with cystic fibrosis who have an Arg117His-CFTR mutation: a double-blind, randomised controlled trial. Lancet Respir Med. 3:524–533
    • (2015) Lancet Respir Med. , vol.3 , pp. 524-533
  • 120
    • 84911493956 scopus 로고    scopus 로고
    • Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation
    • PID: 25266159
    • De Boeck K, Munck A, Walker S, Faro A, Hiatt P, Gilmartin G, Higgins M (2014) Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation. J Cyst Fibros 13:674–680
    • (2014) J Cyst Fibros , vol.13 , pp. 674-680
    • De Boeck, K.1    Munck, A.2    Walker, S.3    Faro, A.4    Hiatt, P.5    Gilmartin, G.6    Higgins, M.7
  • 121
    • 84937035647 scopus 로고    scopus 로고
    • Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR
    • Wainwright CE, Elborn JS, Ramsey BW, Marigowda G, Huang X, Cipolli M, Colombo C, Davies JC, De Boeck K, Flume PA, Konstan MW, McColley SA, McCoy K, McKone EF, Munck A, Ratjen F, Rowe SM, Waltz D, Boyle MP;
    • Wainwright CE, Elborn JS, Ramsey BW, Marigowda G, Huang X, Cipolli M, Colombo C, Davies JC, De Boeck K, Flume PA, Konstan MW, McColley SA, McCoy K, McKone EF, Munck A, Ratjen F, Rowe SM, Waltz D, Boyle MP; TRAFFIC Study Group; TRANSPORT Study Group (2015) Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR. N Engl J Med 373:220–231
    • (2015) N Engl J Med , vol.373 , pp. 220-231
  • 122
    • 85008333588 scopus 로고    scopus 로고
    • Accessed 1 Aug 2016
    • https://www.cff.org/Trials/pipeline. Accessed 1 Aug 2016
  • 125
    • 84871946692 scopus 로고    scopus 로고
    • Cystic fibrosis therapeutics: the road ahead
    • COI: 1:CAS:528:DC%2BC3sXitl2qsbs%3D, PID: 23276843
    • Hoffman LR, Ramsey BW (2013) Cystic fibrosis therapeutics: the road ahead. Chest 143:207–213
    • (2013) Chest , vol.143 , pp. 207-213
    • Hoffman, L.R.1    Ramsey, B.W.2
  • 126
    • 80455144623 scopus 로고    scopus 로고
    • Therapy for cystic fibrosis—the end of the beginning?
    • COI: 1:CAS:528:DC%2BC3MXhsVWqur%2FL, PID: 22047565
    • Davis PB (2011) Therapy for cystic fibrosis—the end of the beginning? N Engl J Med 365:1734–1735
    • (2011) N Engl J Med , vol.365 , pp. 1734-1735
    • Davis, P.B.1


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.