메뉴 건너뛰기




Volumn 15, Issue 2, 2016, Pages 147-157

Cystic fibrosis in young children: A review of disease manifestation, progression, and response to early treatment

Author keywords

Child; Comprehensive survey; Data collection; Disease progression; Health status; Infant; Preschool

Indexed keywords

CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR;

EID: 84951064471     PISSN: 15691993     EISSN: 18735010     Source Type: Journal    
DOI: 10.1016/j.jcf.2015.09.008     Document Type: Review
Times cited : (100)

References (143)
  • 1
  • 2
    • 84930200398 scopus 로고    scopus 로고
    • Cystic Fibrosis Trust
    • UK Cystic Fibrosis Registry Annual Data Report 2013 2014, Cystic Fibrosis Trust.
    • (2014) Annual Data Report 2013
  • 3
    • 0024453308 scopus 로고
    • Identification of the cystic fibrosis gene: chromosome walking and jumping
    • Rommens J.M., Iannuzzi M.C., Kerem B., Drumm M.L., Melmer G., Dean M., et al. Identification of the cystic fibrosis gene: chromosome walking and jumping. Science 1989, 245(4922):1059-1065.
    • (1989) Science , vol.245 , Issue.4922 , pp. 1059-1065
    • Rommens, J.M.1    Iannuzzi, M.C.2    Kerem, B.3    Drumm, M.L.4    Melmer, G.5    Dean, M.6
  • 4
    • 0024424270 scopus 로고
    • Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA
    • Riordan J.R., Rommens J.M., Kerem B., Alon N., Rozmahel R., Grzelczak Z., et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 1989, 245(4922):1066-1073.
    • (1989) Science , vol.245 , Issue.4922 , pp. 1066-1073
    • Riordan, J.R.1    Rommens, J.M.2    Kerem, B.3    Alon, N.4    Rozmahel, R.5    Grzelczak, Z.6
  • 5
    • 0026532895 scopus 로고
    • Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR)
    • Bear C.E., Li C.H., Kartner N., Bridges R.J., Jensen T.J., Ramjeesingh M., et al. Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR). Cell 1992, 68(4):809-818.
    • (1992) Cell , vol.68 , Issue.4 , pp. 809-818
    • Bear, C.E.1    Li, C.H.2    Kartner, N.3    Bridges, R.J.4    Jensen, T.J.5    Ramjeesingh, M.6
  • 6
    • 84867649989 scopus 로고    scopus 로고
    • The impact of newborn screening and earlier intervention on the clinical course of cystic fibrosis
    • Dijk F.N., Fitzgerald D.A. The impact of newborn screening and earlier intervention on the clinical course of cystic fibrosis. Paediatr Respir Rev 2012, 13(4):220-225.
    • (2012) Paediatr Respir Rev , vol.13 , Issue.4 , pp. 220-225
    • Dijk, F.N.1    Fitzgerald, D.A.2
  • 7
    • 84859742024 scopus 로고    scopus 로고
    • A review of pathophysiology and management of fetuses and neonates with meconium ileus for the pediatric surgeon
    • Carlyle B.E., Borowitz D.S., Glick P.L. A review of pathophysiology and management of fetuses and neonates with meconium ileus for the pediatric surgeon. J Pediatr Surg 2012, 47(4):772-781.
    • (2012) J Pediatr Surg , vol.47 , Issue.4 , pp. 772-781
    • Carlyle, B.E.1    Borowitz, D.S.2    Glick, P.L.3
  • 9
    • 84880046935 scopus 로고    scopus 로고
    • Early lung disease in cystic fibrosis
    • Grasemann H., Ratjen F. Early lung disease in cystic fibrosis. Lancet Respir Med 2013, 1(2):148-157.
    • (2013) Lancet Respir Med , vol.1 , Issue.2 , pp. 148-157
    • Grasemann, H.1    Ratjen, F.2
  • 10
    • 84879893299 scopus 로고    scopus 로고
    • Cystic fibrosis in the era of genomic medicine
    • Milla C.E. Cystic fibrosis in the era of genomic medicine. Curr Opin Pediatr 2013, 25(3):323-328.
    • (2013) Curr Opin Pediatr , vol.25 , Issue.3 , pp. 323-328
    • Milla, C.E.1
  • 11
    • 84880040035 scopus 로고    scopus 로고
    • Early intervention studies in infants and preschool children with cystic fibrosis: are we ready?
    • Stick S., Tiddens H., Aurora P., Gustafsson P., Ranganathan S., Robinson P., et al. Early intervention studies in infants and preschool children with cystic fibrosis: are we ready?. Eur Respir J 2013, 42(2):527-538.
    • (2013) Eur Respir J , vol.42 , Issue.2 , pp. 527-538
    • Stick, S.1    Tiddens, H.2    Aurora, P.3    Gustafsson, P.4    Ranganathan, S.5    Robinson, P.6
  • 12
    • 84899046260 scopus 로고    scopus 로고
    • Cystic fibrosis: NHLBI workshop on the primary prevention of chronic lung diseases
    • Pittman J.E., Cutting G., Davis S.D., Ferkol T., Boucher R. Cystic fibrosis: NHLBI workshop on the primary prevention of chronic lung diseases. Ann Am Thorac Soc 2014, 11 Suppl. 3:S161-S168.
    • (2014) Ann Am Thorac Soc , pp. S161-S168
    • Pittman, J.E.1    Cutting, G.2    Davis, S.D.3    Ferkol, T.4    Boucher, R.5
  • 13
  • 15
    • 0027195534 scopus 로고
    • Cell-specific localization of CFTR mRNA shows developmentally regulated expression in human fetal tissues
    • Tizzano E.F., Chitayat D., Buchwald M. Cell-specific localization of CFTR mRNA shows developmentally regulated expression in human fetal tissues. Hum Mol Genet 1993, 2(3):219-224.
    • (1993) Hum Mol Genet , vol.2 , Issue.3 , pp. 219-224
    • Tizzano, E.F.1    Chitayat, D.2    Buchwald, M.3
  • 17
    • 0028227498 scopus 로고
    • Immunohistochemical localization of cystic fibrosis transmembrane conductance regulator in human fetal airway and digestive mucosa
    • Gaillard D., Ruocco S., Lallemand A., Dalemans W., Hinnrasky J., Puchelle E. Immunohistochemical localization of cystic fibrosis transmembrane conductance regulator in human fetal airway and digestive mucosa. Pediatr Res 1994, 36(2):137-143.
    • (1994) Pediatr Res , vol.36 , Issue.2 , pp. 137-143
    • Gaillard, D.1    Ruocco, S.2    Lallemand, A.3    Dalemans, W.4    Hinnrasky, J.5    Puchelle, E.6
  • 18
    • 84874117123 scopus 로고    scopus 로고
    • Evaluation of the intestinal current measurement method as a diagnostic test for cystic fibrosis
    • Cohen-Cymberknoh M., Yaakov Y., Shoseyov D., Shteyer E., Schachar E., Rivlin J., et al. Evaluation of the intestinal current measurement method as a diagnostic test for cystic fibrosis. Pediatr Pulmonol 2013, 48(3):229-235.
    • (2013) Pediatr Pulmonol , vol.48 , Issue.3 , pp. 229-235
    • Cohen-Cymberknoh, M.1    Yaakov, Y.2    Shoseyov, D.3    Shteyer, E.4    Schachar, E.5    Rivlin, J.6
  • 19
    • 77953705905 scopus 로고    scopus 로고
    • Measurement of nasal potential difference in young children with an equivocal sweat test following newborn screening for cystic fibrosis
    • Sermet-Gaudelus I., Girodon E., Roussel D., Deneuville E., Bui S., Huet F., et al. Measurement of nasal potential difference in young children with an equivocal sweat test following newborn screening for cystic fibrosis. Thorax 2010, 65(6):539-544.
    • (2010) Thorax , vol.65 , Issue.6 , pp. 539-544
    • Sermet-Gaudelus, I.1    Girodon, E.2    Roussel, D.3    Deneuville, E.4    Bui, S.5    Huet, F.6
  • 20
    • 0025619380 scopus 로고
    • Pathological confirmation of foetal cystic fibrosis following prenatal diagnosis
    • Damjanovich L., Szeifert G.T., Szabo M., Papp Z. Pathological confirmation of foetal cystic fibrosis following prenatal diagnosis. Acta Morphol Hung 1990, 38(2):141-148.
    • (1990) Acta Morphol Hung , vol.38 , Issue.2 , pp. 141-148
    • Damjanovich, L.1    Szeifert, G.T.2    Szabo, M.3    Papp, Z.4
  • 22
    • 78349237748 scopus 로고    scopus 로고
    • Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children
    • Meyerholz D.K., Stoltz D.A., Namati E., Ramachandran S., Pezzulo A.A., Smith A.R., et al. Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children. Am J Respir Crit Care Med 2010, 182(10):1251-1261.
    • (2010) Am J Respir Crit Care Med , vol.182 , Issue.10 , pp. 1251-1261
    • Meyerholz, D.K.1    Stoltz, D.A.2    Namati, E.3    Ramachandran, S.4    Pezzulo, A.A.5    Smith, A.R.6
  • 23
    • 33745128407 scopus 로고    scopus 로고
    • Inducible NO synthase expression is low in airway epithelium from young children with cystic fibrosis
    • Moeller A., Horak F., Lane C., Knight D., Kicic A., Brennan S., et al. Inducible NO synthase expression is low in airway epithelium from young children with cystic fibrosis. Thorax 2006, 61(6):514-520.
    • (2006) Thorax , vol.61 , Issue.6 , pp. 514-520
    • Moeller, A.1    Horak, F.2    Lane, C.3    Knight, D.4    Kicic, A.5    Brennan, S.6
  • 24
    • 2442581515 scopus 로고    scopus 로고
    • Quantitative cytokine gene expression in CF airway
    • Muhlebach M.S., Reed W., Noah T.L. Quantitative cytokine gene expression in CF airway. Pediatr Pulmonol 2004, 37(5):393-399.
    • (2004) Pediatr Pulmonol , vol.37 , Issue.5 , pp. 393-399
    • Muhlebach, M.S.1    Reed, W.2    Noah, T.L.3
  • 25
    • 0034774914 scopus 로고    scopus 로고
    • Distinct pattern of immune cell population in the lung of human fetuses with cystic fibrosis
    • Hubeau C., Puchelle E., Gaillard D. Distinct pattern of immune cell population in the lung of human fetuses with cystic fibrosis. J Allergy Clin Immunol 2001, 108(4):524-529.
    • (2001) J Allergy Clin Immunol , vol.108 , Issue.4 , pp. 524-529
    • Hubeau, C.1    Puchelle, E.2    Gaillard, D.3
  • 29
    • 0032770489 scopus 로고    scopus 로고
    • Quantitation of inflammatory responses to bacteria in young cystic fibrosis and control patients
    • Muhlebach M.S., Stewart P.W., Leigh M.W., Noah T.L. Quantitation of inflammatory responses to bacteria in young cystic fibrosis and control patients. Am J Respir Crit Care Med 1999, 160(1):186-191.
    • (1999) Am J Respir Crit Care Med , vol.160 , Issue.1 , pp. 186-191
    • Muhlebach, M.S.1    Stewart, P.W.2    Leigh, M.W.3    Noah, T.L.4
  • 30
    • 67650712233 scopus 로고    scopus 로고
    • Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening
    • Sly P.D., Brennan S., Gangell C., de Klerk N., Murray C., Mott L., et al. Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening. Am J Respir Crit Care Med 2009, 180(2):146-152.
    • (2009) Am J Respir Crit Care Med , vol.180 , Issue.2 , pp. 146-152
    • Sly, P.D.1    Brennan, S.2    Gangell, C.3    de Klerk, N.4    Murray, C.5    Mott, L.6
  • 32
    • 34848901700 scopus 로고    scopus 로고
    • Novel neutrophil-derived proteins in bronchoalveolar lavage fluid indicate an exaggerated inflammatory response in pediatric cystic fibrosis patients
    • McMorran B.J., Patat S.A., Carlin J.B., Grimwood K., Jones A., Armstrong D.S., et al. Novel neutrophil-derived proteins in bronchoalveolar lavage fluid indicate an exaggerated inflammatory response in pediatric cystic fibrosis patients. Clin Chem 2007, 53(10):1782-1791.
    • (2007) Clin Chem , vol.53 , Issue.10 , pp. 1782-1791
    • McMorran, B.J.1    Patat, S.A.2    Carlin, J.B.3    Grimwood, K.4    Jones, A.5    Armstrong, D.S.6
  • 33
    • 70349095833 scopus 로고    scopus 로고
    • Alveolar macrophages and CC chemokines are increased in children with cystic fibrosis
    • Brennan S., Sly P.D., Gangell C.L., Sturges N., Winfield K., Wikstrom M., et al. Alveolar macrophages and CC chemokines are increased in children with cystic fibrosis. Eur Respir J 2009, 34(3):655-661.
    • (2009) Eur Respir J , vol.34 , Issue.3 , pp. 655-661
    • Brennan, S.1    Sly, P.D.2    Gangell, C.L.3    Sturges, N.4    Winfield, K.5    Wikstrom, M.6
  • 34
    • 0034764166 scopus 로고    scopus 로고
    • Early pulmonary infection, inflammation, and clinical outcomes in infants with cystic fibrosis
    • Rosenfeld M., Gibson R.L., McNamara S., Emerson J., Burns J.L., Castile R., et al. Early pulmonary infection, inflammation, and clinical outcomes in infants with cystic fibrosis. Pediatr Pulmonol 2001, 32(5):356-366.
    • (2001) Pediatr Pulmonol , vol.32 , Issue.5 , pp. 356-366
    • Rosenfeld, M.1    Gibson, R.L.2    McNamara, S.3    Emerson, J.4    Burns, J.L.5    Castile, R.6
  • 35
    • 0041353720 scopus 로고    scopus 로고
    • Longitudinal evaluation of bronchopulmonary disease in children with cystic fibrosis
    • Farrell P.M., Li Z., Kosorok M.R., Laxova A., Green C.G., Collins J., et al. Longitudinal evaluation of bronchopulmonary disease in children with cystic fibrosis. Pediatr Pulmonol 2003, 36(3):230-240.
    • (2003) Pediatr Pulmonol , vol.36 , Issue.3 , pp. 230-240
    • Farrell, P.M.1    Li, Z.2    Kosorok, M.R.3    Laxova, A.4    Green, C.G.5    Collins, J.6
  • 36
    • 0037067169 scopus 로고    scopus 로고
    • Respiratory infections with Pseudomonas aeruginosa in children with cystic fibrosis: early detection by serology and assessment of risk factors
    • West S.E., Zeng L., Lee B.L., Kosorok M.R., Laxova A., Rock M.J., et al. Respiratory infections with Pseudomonas aeruginosa in children with cystic fibrosis: early detection by serology and assessment of risk factors. JAMA 2002, 287(22):2958-2967.
    • (2002) JAMA , vol.287 , Issue.22 , pp. 2958-2967
    • West, S.E.1    Zeng, L.2    Lee, B.L.3    Kosorok, M.R.4    Laxova, A.5    Rock, M.J.6
  • 37
    • 84879410271 scopus 로고    scopus 로고
    • Distribution of early structural lung changes due to cystic fibrosis detected with chest computed tomography
    • (e241-243)
    • Mott L.S., Park J., Gangell C.L., de Klerk N.H., Sly P.D., Murray C.P., et al. Distribution of early structural lung changes due to cystic fibrosis detected with chest computed tomography. J Pediatr 2013, 163(1):243-248. (e241-243).
    • (2013) J Pediatr , vol.163 , Issue.1 , pp. 243-248
    • Mott, L.S.1    Park, J.2    Gangell, C.L.3    de Klerk, N.H.4    Sly, P.D.5    Murray, C.P.6
  • 38
    • 84898994097 scopus 로고    scopus 로고
    • Magnetic resonance imaging detects changes in structure and perfusion, and response to therapy in early cystic fibrosis lung disease
    • Wielputz M.O., Puderbach M., Kopp-Schneider A., Stahl M., Fritzsching E., Sommerburg O., et al. Magnetic resonance imaging detects changes in structure and perfusion, and response to therapy in early cystic fibrosis lung disease. Am J Respir Crit Care Med 2014, 189(8):956-965.
    • (2014) Am J Respir Crit Care Med , vol.189 , Issue.8 , pp. 956-965
    • Wielputz, M.O.1    Puderbach, M.2    Kopp-Schneider, A.3    Stahl, M.4    Fritzsching, E.5    Sommerburg, O.6
  • 39
    • 80051850244 scopus 로고    scopus 로고
    • Air trapping on chest CT is associated with worse ventilation distribution in infants with cystic fibrosis diagnosed following newborn screening
    • Hall G.L., Logie K.M., Parsons F., Schulzke S.M., Nolan G., Murray C., et al. Air trapping on chest CT is associated with worse ventilation distribution in infants with cystic fibrosis diagnosed following newborn screening. PLoS ONE 2011, 6(8).
    • (2011) PLoS ONE , vol.6 , Issue.8
    • Hall, G.L.1    Logie, K.M.2    Parsons, F.3    Schulzke, S.M.4    Nolan, G.5    Murray, C.6
  • 41
    • 27644531001 scopus 로고    scopus 로고
    • Comparison of quiet breathing and controlled ventilation in the high-resolution CT assessment of airway disease in infants with cystic fibrosis
    • Long F.R., Williams R.S., Adler B.H., Castile R.G. Comparison of quiet breathing and controlled ventilation in the high-resolution CT assessment of airway disease in infants with cystic fibrosis. Pediatr Radiol 2005, 35(11):1075-1080.
    • (2005) Pediatr Radiol , vol.35 , Issue.11 , pp. 1075-1080
    • Long, F.R.1    Williams, R.S.2    Adler, B.H.3    Castile, R.G.4
  • 42
    • 84878000440 scopus 로고    scopus 로고
    • Risk factors for bronchiectasis in children with cystic fibrosis
    • Sly P.D., Gangell C.L., Chen L., Ware R.S., Ranganathan S., Mott L.S., et al. Risk factors for bronchiectasis in children with cystic fibrosis. N Engl J Med 2013, 368(21):1963-1970.
    • (2013) N Engl J Med , vol.368 , Issue.21 , pp. 1963-1970
    • Sly, P.D.1    Gangell, C.L.2    Chen, L.3    Ware, R.S.4    Ranganathan, S.5    Mott, L.S.6
  • 43
    • 1542287428 scopus 로고    scopus 로고
    • Structural airway abnormalities in infants and young children with cystic fibrosis
    • Long F.R., Williams R.S., Castile R.G. Structural airway abnormalities in infants and young children with cystic fibrosis. J Pediatr 2004, 144(2):154-161.
    • (2004) J Pediatr , vol.144 , Issue.2 , pp. 154-161
    • Long, F.R.1    Williams, R.S.2    Castile, R.G.3
  • 45
    • 0033769001 scopus 로고    scopus 로고
    • Early detection of lung disease and its association with the nutritional status, genetic background and life events in patients with cystic fibrosis
    • Kraemer R., Aebi C., Casaulta Aebischer C., Gallati S. Early detection of lung disease and its association with the nutritional status, genetic background and life events in patients with cystic fibrosis. Respiration 2000, 67(5):477-490.
    • (2000) Respiration , vol.67 , Issue.5 , pp. 477-490
    • Kraemer, R.1    Aebi, C.2    Casaulta Aebischer, C.3    Gallati, S.4
  • 46
    • 0027410084 scopus 로고
    • Relationship of genotype to early pulmonary function in infants with cystic fibrosis identified through neonatal screening
    • Mohon R.T., Wagener J.S., Abman S.H., Seltzer W.K., Accurso F.J. Relationship of genotype to early pulmonary function in infants with cystic fibrosis identified through neonatal screening. J Pediatr 1993, 122(4):550-555.
    • (1993) J Pediatr , vol.122 , Issue.4 , pp. 550-555
    • Mohon, R.T.1    Wagener, J.S.2    Abman, S.H.3    Seltzer, W.K.4    Accurso, F.J.5
  • 47
    • 12744277962 scopus 로고    scopus 로고
    • Multiple-breath washout as a marker of lung disease in preschool children with cystic fibrosis
    • Aurora P., Bush A., Gustafsson P., Oliver C., Wallis C., Price J., et al. Multiple-breath washout as a marker of lung disease in preschool children with cystic fibrosis. Am J Respir Crit Care Med 2005, 171(3):249-256.
    • (2005) Am J Respir Crit Care Med , vol.171 , Issue.3 , pp. 249-256
    • Aurora, P.1    Bush, A.2    Gustafsson, P.3    Oliver, C.4    Wallis, C.5    Price, J.6
  • 48
    • 2542496991 scopus 로고    scopus 로고
    • Serial lung function and responsiveness in cystic fibrosis during early childhood
    • Nielsen K.G., Pressler T., Klug B., Koch C., Bisgaard H. Serial lung function and responsiveness in cystic fibrosis during early childhood. Am J Respir Crit Care Med 2004, 169(11):1209-1216.
    • (2004) Am J Respir Crit Care Med , vol.169 , Issue.11 , pp. 1209-1216
    • Nielsen, K.G.1    Pressler, T.2    Klug, B.3    Koch, C.4    Bisgaard, H.5
  • 49
    • 84887660050 scopus 로고    scopus 로고
    • Opening interrupter technique in pre-school children with chronic respiratory diseases: a perspective case-control study in the diagnosis of airway hyperesponsiveness
    • Vitaliti G., Leonardi S., La Rosa M. Opening interrupter technique in pre-school children with chronic respiratory diseases: a perspective case-control study in the diagnosis of airway hyperesponsiveness. J Asthma 2013, 50(10):1045-1048.
    • (2013) J Asthma , vol.50 , Issue.10 , pp. 1045-1048
    • Vitaliti, G.1    Leonardi, S.2    La Rosa, M.3
  • 50
    • 84866070431 scopus 로고    scopus 로고
    • Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening
    • Hoo A.F., Thia L.P., Nguyen T.T., Bush A., Chudleigh J., Lum S., et al. Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening. Thorax 2012, 67(10):874-881.
    • (2012) Thorax , vol.67 , Issue.10 , pp. 874-881
    • Hoo, A.F.1    Thia, L.P.2    Nguyen, T.T.3    Bush, A.4    Chudleigh, J.5    Lum, S.6
  • 51
    • 0031754675 scopus 로고    scopus 로고
    • Genotype-phenotype association in infants with cystic fibrosis at the time of diagnosis
    • Kraemer R., Birrer P., Liechti-Gallati S. Genotype-phenotype association in infants with cystic fibrosis at the time of diagnosis. Pediatr Res 1998, 44(6):920-926.
    • (1998) Pediatr Res , vol.44 , Issue.6 , pp. 920-926
    • Kraemer, R.1    Birrer, P.2    Liechti-Gallati, S.3
  • 52
    • 84884683771 scopus 로고    scopus 로고
    • Evolution of lung function during the first year of life in newborn screened cystic fibrosis infants
    • Nguyen T.T-D., Thia L.P., Hoo A-F., Bush A., Aurora P., Wade A., et al. Evolution of lung function during the first year of life in newborn screened cystic fibrosis infants. Thorax 2014, 69(10):910-917.
    • (2014) Thorax , vol.69 , Issue.10 , pp. 910-917
    • Nguyen, T.T.-D.1    Thia, L.P.2    Hoo, A.-F.3    Bush, A.4    Aurora, P.5    Wade, A.6
  • 53
    • 84895927063 scopus 로고    scopus 로고
    • Is chest CT useful in newborn screened infants with cystic fibrosis at 1 year of age?
    • Thia L.P., Calder A., Stocks J., Bush A., Owens C.M., Wallis C., et al. Is chest CT useful in newborn screened infants with cystic fibrosis at 1 year of age?. Thorax 2014, 69(4):320-327.
    • (2014) Thorax , vol.69 , Issue.4 , pp. 320-327
    • Thia, L.P.1    Calder, A.2    Stocks, J.3    Bush, A.4    Owens, C.M.5    Wallis, C.6
  • 54
    • 0026404346 scopus 로고
    • Assessment of airway responsiveness in infants with cystic fibrosis
    • Ackerman V., Montogomery G., Eigen H., Tepper R.S. Assessment of airway responsiveness in infants with cystic fibrosis. Am Rev Respir Dis 1991, 144(2):344-346.
    • (1991) Am Rev Respir Dis , vol.144 , Issue.2 , pp. 344-346
    • Ackerman, V.1    Montogomery, G.2    Eigen, H.3    Tepper, R.S.4
  • 55
    • 84860622874 scopus 로고    scopus 로고
    • Determining presence of lung disease in young children with cystic fibrosis: lung clearance index, oxygen saturation and cough frequency
    • Bakker E.M., van der Meijden J.C., Nieuwhof E.M., Hop W.C., Tiddens H.A. Determining presence of lung disease in young children with cystic fibrosis: lung clearance index, oxygen saturation and cough frequency. J Cyst Fibros 2012, 11(3):223-230.
    • (2012) J Cyst Fibros , vol.11 , Issue.3 , pp. 223-230
    • Bakker, E.M.1    van der Meijden, J.C.2    Nieuwhof, E.M.3    Hop, W.C.4    Tiddens, H.A.5
  • 56
    • 84860110054 scopus 로고    scopus 로고
    • Early cystic fibrosis lung disease detected by bronchoalveolar lavage and lung clearance index
    • Belessis Y., Dixon B., Hawkins G., Pereira J., Peat J., MacDonald R., et al. Early cystic fibrosis lung disease detected by bronchoalveolar lavage and lung clearance index. Am J Respir Crit Care Med 2012, 185(8):862-873.
    • (2012) Am J Respir Crit Care Med , vol.185 , Issue.8 , pp. 862-873
    • Belessis, Y.1    Dixon, B.2    Hawkins, G.3    Pereira, J.4    Peat, J.5    MacDonald, R.6
  • 57
    • 81455154503 scopus 로고    scopus 로고
    • Long-term course of lung clearance index between infancy and school-age in cystic fibrosis subjects
    • Kieninger E., Singer F., Fuchs O., Abbas C., Frey U., Regamey N., et al. Long-term course of lung clearance index between infancy and school-age in cystic fibrosis subjects. J Cyst Fibros 2011, 10(6):487-490.
    • (2011) J Cyst Fibros , vol.10 , Issue.6 , pp. 487-490
    • Kieninger, E.1    Singer, F.2    Fuchs, O.3    Abbas, C.4    Frey, U.5    Regamey, N.6
  • 59
    • 34247167236 scopus 로고    scopus 로고
    • Early detection of cystic fibrosis lung disease: multiple-breath washout versus raised volume tests
    • Lum S., Gustafsson P., Ljungberg H., Hulskamp G., Bush A., Carr S.B., et al. Early detection of cystic fibrosis lung disease: multiple-breath washout versus raised volume tests. Thorax 2007, 62(4):341-347.
    • (2007) Thorax , vol.62 , Issue.4 , pp. 341-347
    • Lum, S.1    Gustafsson, P.2    Ljungberg, H.3    Hulskamp, G.4    Bush, A.5    Carr, S.B.6
  • 60
    • 0037111312 scopus 로고    scopus 로고
    • Relative ability of full and partial forced expiratory maneuvers to identify diminished airway function in infants with cystic fibrosis
    • Ranganathan S.C., Bush A., Dezateux C., Carr S.B., Hoo A.F., Lum S., et al. Relative ability of full and partial forced expiratory maneuvers to identify diminished airway function in infants with cystic fibrosis. Am J Respir Crit Care Med 2002, 166(10):1350-1357.
    • (2002) Am J Respir Crit Care Med , vol.166 , Issue.10 , pp. 1350-1357
    • Ranganathan, S.C.1    Bush, A.2    Dezateux, C.3    Carr, S.B.4    Hoo, A.F.5    Lum, S.6
  • 61
    • 11144355490 scopus 로고    scopus 로고
    • The evolution of airway function in early childhood following clinical diagnosis of cystic fibrosis
    • Ranganathan S.C., Stocks J., Dezateux C., Bush A., Wade A., Carr S., et al. The evolution of airway function in early childhood following clinical diagnosis of cystic fibrosis. Am J Respir Crit Care Med 2004, 169(8):928-933.
    • (2004) Am J Respir Crit Care Med , vol.169 , Issue.8 , pp. 928-933
    • Ranganathan, S.C.1    Stocks, J.2    Dezateux, C.3    Bush, A.4    Wade, A.5    Carr, S.6
  • 62
    • 84899898309 scopus 로고    scopus 로고
    • Multiple breath washout is feasible in the clinical setting and detects abnormal lung function in infants and young children with cystic fibrosis
    • Stahl M., Joachim C., Blessing K., Hammerling S., Sommerburg O., Latzin P., et al. Multiple breath washout is feasible in the clinical setting and detects abnormal lung function in infants and young children with cystic fibrosis. Respiration 2014, 87(5):357-363.
    • (2014) Respiration , vol.87 , Issue.5 , pp. 357-363
    • Stahl, M.1    Joachim, C.2    Blessing, K.3    Hammerling, S.4    Sommerburg, O.5    Latzin, P.6
  • 63
    • 84861226870 scopus 로고    scopus 로고
    • Lung function distinguishes preschool children with CF from healthy controls in a multi-center setting
    • Kerby G.S., Rosenfeld M., Ren C.L., Mayer O.H., Brumback L., Castile R., et al. Lung function distinguishes preschool children with CF from healthy controls in a multi-center setting. Pediatr Pulmonol 2012, 47(6):597-605.
    • (2012) Pediatr Pulmonol , vol.47 , Issue.6 , pp. 597-605
    • Kerby, G.S.1    Rosenfeld, M.2    Ren, C.L.3    Mayer, O.H.4    Brumback, L.5    Castile, R.6
  • 67
    • 33847119061 scopus 로고    scopus 로고
    • Spirometry in early childhood in cystic fibrosis patients
    • Vilozni D., Bentur L., Efrati O., Minuskin T., Barak A., Szeinberg A., et al. Spirometry in early childhood in cystic fibrosis patients. Chest 2007, 131(2):356-361.
    • (2007) Chest , vol.131 , Issue.2 , pp. 356-361
    • Vilozni, D.1    Bentur, L.2    Efrati, O.3    Minuskin, T.4    Barak, A.5    Szeinberg, A.6
  • 68
    • 84888074505 scopus 로고    scopus 로고
    • Evidence of diminished FEV1 and FVC in 6-year-olds followed in the European cystic fibrosis patient registry, 2007-2009
    • VanDevanter D.R., Pasta D.J. Evidence of diminished FEV1 and FVC in 6-year-olds followed in the European cystic fibrosis patient registry, 2007-2009. J Cyst Fibros 2013, 12(6):786-789.
    • (2013) J Cyst Fibros , vol.12 , Issue.6 , pp. 786-789
    • VanDevanter, D.R.1    Pasta, D.J.2
  • 69
    • 56849107024 scopus 로고    scopus 로고
    • Lung function in 3-5-year-old children with cystic fibrosis
    • Mayer O.H., Jawad A.F., McDonough J., Allen J. Lung function in 3-5-year-old children with cystic fibrosis. Pediatr Pulmonol 2008, 43(12):1214-1223.
    • (2008) Pediatr Pulmonol , vol.43 , Issue.12 , pp. 1214-1223
    • Mayer, O.H.1    Jawad, A.F.2    McDonough, J.3    Allen, J.4
  • 70
    • 0036311232 scopus 로고    scopus 로고
    • Relationship between nutritional status and lung function in cystic fibrosis: cross sectional and longitudinal analyses from the German CF quality assurance (CFQA) project
    • Steinkamp G., Wiedemann B. Relationship between nutritional status and lung function in cystic fibrosis: cross sectional and longitudinal analyses from the German CF quality assurance (CFQA) project. Thorax 2002, 57(7):596-601.
    • (2002) Thorax , vol.57 , Issue.7 , pp. 596-601
    • Steinkamp, G.1    Wiedemann, B.2
  • 72
    • 33749078004 scopus 로고    scopus 로고
    • Neonatal screening for cystic fibrosis does not affect time to first infection with Pseudomonas aeruginosa
    • Baussano I., Tardivo I., Bellezza-Fontana R., Forneris M.P., Lezo A., Anfossi L., et al. Neonatal screening for cystic fibrosis does not affect time to first infection with Pseudomonas aeruginosa. Pediatrics 2006, 118(3):888-895.
    • (2006) Pediatrics , vol.118 , Issue.3 , pp. 888-895
    • Baussano, I.1    Tardivo, I.2    Bellezza-Fontana, R.3    Forneris, M.P.4    Lezo, A.5    Anfossi, L.6
  • 73
    • 0035253464 scopus 로고    scopus 로고
    • Longitudinal assessment of Pseudomonas aeruginosa in young children with cystic fibrosis
    • Burns J.L., Gibson R.L., McNamara S., Yim D., Emerson J., Rosenfeld M., et al. Longitudinal assessment of Pseudomonas aeruginosa in young children with cystic fibrosis. J Infect Dis 2001, 183(3):444-452.
    • (2001) J Infect Dis , vol.183 , Issue.3 , pp. 444-452
    • Burns, J.L.1    Gibson, R.L.2    McNamara, S.3    Yim, D.4    Emerson, J.5    Rosenfeld, M.6
  • 74
    • 0036529046 scopus 로고    scopus 로고
    • Inflammation, infection, and pulmonary function in infants and young children with cystic fibrosis
    • Dakin C.J., Numa A.H., Wang H., Morton J.R., Vertzyas C.C., Henry R.L. Inflammation, infection, and pulmonary function in infants and young children with cystic fibrosis. Am J Respir Crit Care Med 2002, 165(7):904-910.
    • (2002) Am J Respir Crit Care Med , vol.165 , Issue.7 , pp. 904-910
    • Dakin, C.J.1    Numa, A.H.2    Wang, H.3    Morton, J.R.4    Vertzyas, C.C.5    Henry, R.L.6
  • 75
    • 59649123905 scopus 로고    scopus 로고
    • Acquisition and eradication of P. aeruginosa in young children with cystic fibrosis
    • Douglas T.A., Brennan S., Gard S., Berry L., Gangell C., Stick S.M., et al. Acquisition and eradication of P. aeruginosa in young children with cystic fibrosis. Eur Respir J 2009, 33(2):305-311.
    • (2009) Eur Respir J , vol.33 , Issue.2 , pp. 305-311
    • Douglas, T.A.1    Brennan, S.2    Gard, S.3    Berry, L.4    Gangell, C.5    Stick, S.M.6
  • 76
    • 0033064891 scopus 로고    scopus 로고
    • Effects of viral lower respiratory tract infection on lung function in infants with cystic fibrosis
    • Hiatt P.W., Grace S.C., Kozinetz C.A., Raboudi S.H., Treece D.G., Taber L.H., et al. Effects of viral lower respiratory tract infection on lung function in infants with cystic fibrosis. Pediatrics 1999, 103(3):619-626.
    • (1999) Pediatrics , vol.103 , Issue.3 , pp. 619-626
    • Hiatt, P.W.1    Grace, S.C.2    Kozinetz, C.A.3    Raboudi, S.H.4    Treece, D.G.5    Taber, L.H.6
  • 77
    • 0025770657 scopus 로고
    • Early bacteriologic, immunologic, and clinical courses of young infants with cystic fibrosis identified by neonatal screening
    • Abman S.H., Ogle J.W., Harbeck R.J., Butler-Simon N., Hammond K.B., Accurso F.J. Early bacteriologic, immunologic, and clinical courses of young infants with cystic fibrosis identified by neonatal screening. J Pediatr 1991, 119(2):211-217.
    • (1991) J Pediatr , vol.119 , Issue.2 , pp. 211-217
    • Abman, S.H.1    Ogle, J.W.2    Harbeck, R.J.3    Butler-Simon, N.4    Hammond, K.B.5    Accurso, F.J.6
  • 78
    • 13544260495 scopus 로고    scopus 로고
    • Correlation of forced oscillation technique in preschool children with cystic fibrosis with pulmonary inflammation
    • Brennan S., Hall G.L., Horak F., Moeller A., Pitrez P.M., Franzmann A., et al. Correlation of forced oscillation technique in preschool children with cystic fibrosis with pulmonary inflammation. Thorax 2005, 60(2):159-163.
    • (2005) Thorax , vol.60 , Issue.2 , pp. 159-163
    • Brennan, S.1    Hall, G.L.2    Horak, F.3    Moeller, A.4    Pitrez, P.M.5    Franzmann, A.6
  • 79
    • 0030788625 scopus 로고    scopus 로고
    • Surfactant composition in infants and young children with cystic fibrosis
    • Hull J., South M., Phelan P., Grimwood K. Surfactant composition in infants and young children with cystic fibrosis. Am J Respir Crit Care Med 1997, 156(1):161-165.
    • (1997) Am J Respir Crit Care Med , vol.156 , Issue.1 , pp. 161-165
    • Hull, J.1    South, M.2    Phelan, P.3    Grimwood, K.4
  • 80
    • 79955959572 scopus 로고    scopus 로고
    • Prevalence and histopathology of chronic polypoid sinusitis in pediatric patients with cystic fibrosis
    • Schraven S.P., Wehrmann M., Wagner W., Blumenstock G., Koitschev A. Prevalence and histopathology of chronic polypoid sinusitis in pediatric patients with cystic fibrosis. J Cyst Fibros 2011, 10(3):181-186.
    • (2011) J Cyst Fibros , vol.10 , Issue.3 , pp. 181-186
    • Schraven, S.P.1    Wehrmann, M.2    Wagner, W.3    Blumenstock, G.4    Koitschev, A.5
  • 82
    • 80053359109 scopus 로고    scopus 로고
    • Socioeconomic status and the likelihood of antibiotic treatment for signs and symptoms of pulmonary exacerbation in children with cystic fibrosis
    • Schechter M.S., McColley S.A., Regelmann W., Millar S.J., Pasta D.J., Wagener J.S., et al. Socioeconomic status and the likelihood of antibiotic treatment for signs and symptoms of pulmonary exacerbation in children with cystic fibrosis. J Pediatr 2011, 159(5):819-824. e811.
    • (2011) J Pediatr , vol.159 , Issue.5 , pp. 819-824
    • Schechter, M.S.1    McColley, S.A.2    Regelmann, W.3    Millar, S.J.4    Pasta, D.J.5    Wagener, J.S.6
  • 83
    • 84878346897 scopus 로고    scopus 로고
    • Changing thresholds and incidence of antibiotic treatment of cystic fibrosis pulmonary exacerbations, 1995-2005
    • VanDevanter D.R., Elkin E.P., Pasta D.J., Morgan W.J., Konstan M.W. Changing thresholds and incidence of antibiotic treatment of cystic fibrosis pulmonary exacerbations, 1995-2005. J Cyst Fibros 2013, 12(4):332-337.
    • (2013) J Cyst Fibros , vol.12 , Issue.4 , pp. 332-337
    • VanDevanter, D.R.1    Elkin, E.P.2    Pasta, D.J.3    Morgan, W.J.4    Konstan, M.W.5
  • 84
    • 77957154655 scopus 로고    scopus 로고
    • Notable contribution of large CFTR gene rearrangements to the diagnosis of cystic fibrosis in fetuses with bowel anomalies
    • de Becdelievre A., Costa C., LeFloch A., Legendre M., Jouannic J.M., Vigneron J., et al. Notable contribution of large CFTR gene rearrangements to the diagnosis of cystic fibrosis in fetuses with bowel anomalies. Eur J Hum Genet 2010, 18(10):1166-1169.
    • (2010) Eur J Hum Genet , vol.18 , Issue.10 , pp. 1166-1169
    • de Becdelievre, A.1    Costa, C.2    LeFloch, A.3    Legendre, M.4    Jouannic, J.M.5    Vigneron, J.6
  • 85
    • 0026486282 scopus 로고
    • Sonographically detected hyperechoic fetal bowel: significance and implications for pregnancy management
    • Dicke J.M., Crane J.P. Sonographically detected hyperechoic fetal bowel: significance and implications for pregnancy management. Obstet Gynecol 1992, 80(5):778-782.
    • (1992) Obstet Gynecol , vol.80 , Issue.5 , pp. 778-782
    • Dicke, J.M.1    Crane, J.P.2
  • 86
    • 0025609226 scopus 로고
    • Abnormal fucosylation of ileal mucus in cystic fibrosis: I. A histochemical study using peroxidase labelled lectins
    • Thiru S., Devereux G., King A. Abnormal fucosylation of ileal mucus in cystic fibrosis: I. A histochemical study using peroxidase labelled lectins. J Clin Pathol 1990, 43(12):1014-1018.
    • (1990) J Clin Pathol , vol.43 , Issue.12 , pp. 1014-1018
    • Thiru, S.1    Devereux, G.2    King, A.3
  • 87
    • 84906833163 scopus 로고    scopus 로고
    • Disrupted intestinal microbiota and intestinal inflammation in children with cystic fibrosis and its restoration with Lactobacillus GG: a randomised clinical trial
    • Bruzzese E., Callegari M.L., Raia V., Viscovo S., Scotto R., Ferrari S., et al. Disrupted intestinal microbiota and intestinal inflammation in children with cystic fibrosis and its restoration with Lactobacillus GG: a randomised clinical trial. PLoS ONE 2014, 9(2).
    • (2014) PLoS ONE , vol.9 , Issue.2
    • Bruzzese, E.1    Callegari, M.L.2    Raia, V.3    Viscovo, S.4    Scotto, R.5    Ferrari, S.6
  • 88
    • 0026600469 scopus 로고
    • Pancreatic insufficiency, growth, and nutrition in infants identified by newborn screening as having cystic fibrosis
    • Bronstein M.N., Sokol R.J., Abman S.H., Chatfield B.A., Hammond K.B., Hambidge K.M., et al. Pancreatic insufficiency, growth, and nutrition in infants identified by newborn screening as having cystic fibrosis. J Pediatr 1992, 120(4 Pt 1):533-540.
    • (1992) J Pediatr , vol.120 , Issue.4 , pp. 533-540
    • Bronstein, M.N.1    Sokol, R.J.2    Abman, S.H.3    Chatfield, B.A.4    Hammond, K.B.5    Hambidge, K.M.6
  • 89
    • 0030665216 scopus 로고    scopus 로고
    • Failure to thrive: the earliest feature of cystic fibrosis in infants diagnosed by neonatal screening
    • Giglio L., Candusso M., D'Orazio C., Mastella G., Faraguna D. Failure to thrive: the earliest feature of cystic fibrosis in infants diagnosed by neonatal screening. Acta Paediatr 1997, 86(11):1162-1165.
    • (1997) Acta Paediatr , vol.86 , Issue.11 , pp. 1162-1165
    • Giglio, L.1    Candusso, M.2    D'Orazio, C.3    Mastella, G.4    Faraguna, D.5
  • 90
    • 0025820987 scopus 로고
    • Evaluation of growth and changes in body composition following neonatal diagnosis of cystic fibrosis
    • Greer R., Shepherd R., Cleghorn G., Bowling F.G., Holt T. Evaluation of growth and changes in body composition following neonatal diagnosis of cystic fibrosis. J Pediatr Gastroenterol Nutr 1991, 13(1):52-58.
    • (1991) J Pediatr Gastroenterol Nutr , vol.13 , Issue.1 , pp. 52-58
    • Greer, R.1    Shepherd, R.2    Cleghorn, G.3    Bowling, F.G.4    Holt, T.5
  • 91
    • 0027432656 scopus 로고
    • Measurement of fat digestion in early life using a stable isotope breath test
    • McClean P., Harding M., Coward W.A., Green M.R., Weaver L.T. Measurement of fat digestion in early life using a stable isotope breath test. Arch Dis Child 1993, 69(3):366-370.
    • (1993) Arch Dis Child , vol.69 , Issue.3 , pp. 366-370
    • McClean, P.1    Harding, M.2    Coward, W.A.3    Green, M.R.4    Weaver, L.T.5
  • 92
    • 84884211531 scopus 로고    scopus 로고
    • Evolution of pancreatic function during the first year in infants with cystic fibrosis
    • O'Sullivan B.P., Baker D., Leung K.G., Reed G., Baker S.S., Borowitz D. Evolution of pancreatic function during the first year in infants with cystic fibrosis. J Pediatr 2013, 162(4):808-812. (e801).
    • (2013) J Pediatr , vol.162 , Issue.4 , pp. 808-812
    • O'Sullivan, B.P.1    Baker, D.2    Leung, K.G.3    Reed, G.4    Baker, S.S.5    Borowitz, D.6
  • 94
    • 0025181586 scopus 로고
    • Pancreatic function in infants identified as having cystic fibrosis in a neonatal screening program
    • Waters D.L., Dorney S.F., Gaskin K.J., Gruca M.A., O'Halloran M., Wilcken B. Pancreatic function in infants identified as having cystic fibrosis in a neonatal screening program. N Engl J Med 1990, 322(5):303-308.
    • (1990) N Engl J Med , vol.322 , Issue.5 , pp. 303-308
    • Waters, D.L.1    Dorney, S.F.2    Gaskin, K.J.3    Gruca, M.A.4    O'Halloran, M.5    Wilcken, B.6
  • 96
    • 70349586105 scopus 로고    scopus 로고
    • Protein-energy malnutrition as the first manifestation of cystic fibrosis in infancy
    • Fustik S., Jacovska T., Spirevska L., Koceva S. Protein-energy malnutrition as the first manifestation of cystic fibrosis in infancy. Pediatr Int 2009, 51(5):678-683.
    • (2009) Pediatr Int , vol.51 , Issue.5 , pp. 678-683
    • Fustik, S.1    Jacovska, T.2    Spirevska, L.3    Koceva, S.4
  • 98
    • 70449687890 scopus 로고    scopus 로고
    • Evaluation of salt supplementation in CF infants
    • Coates A.J., Crofton P.M., Marshall T. Evaluation of salt supplementation in CF infants. J Cyst Fibros 2009, 8(6):382-385.
    • (2009) J Cyst Fibros , vol.8 , Issue.6 , pp. 382-385
    • Coates, A.J.1    Crofton, P.M.2    Marshall, T.3
  • 99
    • 84864277119 scopus 로고    scopus 로고
    • Prevalence of hyponatremia at diagnosis and factors associated with the longitudinal variation in serum sodium levels in infants with cystic fibrosis
    • Guimarães E.V., Schettino G.C., Camargos P.A., Penna F.J. Prevalence of hyponatremia at diagnosis and factors associated with the longitudinal variation in serum sodium levels in infants with cystic fibrosis. J Pediatr 2012, 161(2):285-289.
    • (2012) J Pediatr , vol.161 , Issue.2 , pp. 285-289
    • Guimarães, E.V.1    Schettino, G.C.2    Camargos, P.A.3    Penna, F.J.4
  • 101
    • 0026095212 scopus 로고
    • Nutritional status of infants with cystic fibrosis associated with early diagnosis and intervention
    • Marcus M.S., Sondel S.A., Farrell P.M., Laxova A., Carey P.M., Langhough R., et al. Nutritional status of infants with cystic fibrosis associated with early diagnosis and intervention. Am J Clin Nutr 1991, 54(3):578-585.
    • (1991) Am J Clin Nutr , vol.54 , Issue.3 , pp. 578-585
    • Marcus, M.S.1    Sondel, S.A.2    Farrell, P.M.3    Laxova, A.4    Carey, P.M.5    Langhough, R.6
  • 102
    • 58649099835 scopus 로고    scopus 로고
    • Vitamin D in infants with cystic fibrosis diagnosed by newborn screening
    • Neville L.A., Ranganathan S.C. Vitamin D in infants with cystic fibrosis diagnosed by newborn screening. J Paediatr Child Health 2009, 45(1-2):36-41.
    • (2009) J Paediatr Child Health , vol.45 , Issue.1-2 , pp. 36-41
    • Neville, L.A.1    Ranganathan, S.C.2
  • 103
  • 104
    • 25844486482 scopus 로고    scopus 로고
    • Complications associated with symptomatic diagnosis in infants with cystic fibrosis
    • Accurso F.J., Sontag M.K., Wagener J.S. Complications associated with symptomatic diagnosis in infants with cystic fibrosis. J Pediatr 2005, 147(3 Suppl):S37-S41.
    • (2005) J Pediatr , vol.147 , Issue.3 , pp. S37-S41
    • Accurso, F.J.1    Sontag, M.K.2    Wagener, J.S.3
  • 107
    • 25144435122 scopus 로고    scopus 로고
    • Gestational and neonatal characteristics of children with cystic fibrosis: a cohort study
    • Festini F., Taccetti G., Repetto T., Reali M.F., Campana S., Mergni G., et al. Gestational and neonatal characteristics of children with cystic fibrosis: a cohort study. J Pediatr 2005, 147(3):316-320.
    • (2005) J Pediatr , vol.147 , Issue.3 , pp. 316-320
    • Festini, F.1    Taccetti, G.2    Repetto, T.3    Reali, M.F.4    Campana, S.5    Mergni, G.6
  • 109
    • 28344436257 scopus 로고    scopus 로고
    • Cystic fibrosis at a Brazilian center of excellence: clinical and laboratory characteristics of 104 patients and their association with genotype and disease severity
    • Alvarez A.E., Ribeiro AnF, Hesselm G., Bertuzzo C.S., Ribeiro J.D. Cystic fibrosis at a Brazilian center of excellence: clinical and laboratory characteristics of 104 patients and their association with genotype and disease severity. J Pediatr (Rio J) 2004, 80(5):371-379.
    • (2004) J Pediatr (Rio J) , vol.80 , Issue.5 , pp. 371-379
    • Alvarez, A.E.1    Ribeiro, A.2    Hesselm, G.3    Bertuzzo, C.S.4    Ribeiro, J.D.5
  • 110
    • 79952504576 scopus 로고    scopus 로고
    • Lung clearance index at 4 years predicts subsequent lung function in children with cystic fibrosis
    • Aurora P., Stanojevic S., Wade A., Oliver C., Kozlowska W., Lum S., et al. Lung clearance index at 4 years predicts subsequent lung function in children with cystic fibrosis. Am J Respir Crit Care Med 2011, 183(6):752-758.
    • (2011) Am J Respir Crit Care Med , vol.183 , Issue.6 , pp. 752-758
    • Aurora, P.1    Stanojevic, S.2    Wade, A.3    Oliver, C.4    Kozlowska, W.5    Lum, S.6
  • 112
    • 0038309278 scopus 로고    scopus 로고
    • Growth in prepubertal children with cystic fibrosis, homozygous for the Delta F508 mutation
    • Keller B.M., Aebischer C.C., Kraemer R., Schoni M.H. Growth in prepubertal children with cystic fibrosis, homozygous for the Delta F508 mutation. J Cyst Fibros 2003, 2(2):76-83.
    • (2003) J Cyst Fibros , vol.2 , Issue.2 , pp. 76-83
    • Keller, B.M.1    Aebischer, C.C.2    Kraemer, R.3    Schoni, M.H.4
  • 114
    • 0025013154 scopus 로고
    • Early childhood growth in patients with cystic fibrosis
    • Byard P.J. Early childhood growth in patients with cystic fibrosis. Ann Hum Biol 1990, 17(6):483-499.
    • (1990) Ann Hum Biol , vol.17 , Issue.6 , pp. 483-499
    • Byard, P.J.1
  • 115
    • 0029832665 scopus 로고    scopus 로고
    • Head growth in cystic fibrosis following early diagnosis by neonatal screening
    • Ghosal S., Taylor C.J., Pickering M., McGaw J. Head growth in cystic fibrosis following early diagnosis by neonatal screening. Arch Dis Child 1996, 75(3):191-193.
    • (1996) Arch Dis Child , vol.75 , Issue.3 , pp. 191-193
    • Ghosal, S.1    Taylor, C.J.2    Pickering, M.3    McGaw, J.4
  • 117
    • 4444300853 scopus 로고    scopus 로고
    • Elastic properties of the respiratory system in infants with cystic fibrosis
    • Tepper R.S., Weist A., Williams-Nkomo T., Kisling J. Elastic properties of the respiratory system in infants with cystic fibrosis. Am J Respir Crit Care Med 2004, 170(5):505-507.
    • (2004) Am J Respir Crit Care Med , vol.170 , Issue.5 , pp. 505-507
    • Tepper, R.S.1    Weist, A.2    Williams-Nkomo, T.3    Kisling, J.4
  • 118
    • 66149130974 scopus 로고    scopus 로고
    • Longitudinal assessment of lung function from infancy to childhood in patients with cystic fibrosis
    • Harrison A.N., Regelmann W.E., Zirbes J.M., Milla C.E. Longitudinal assessment of lung function from infancy to childhood in patients with cystic fibrosis. Pediatr Pulmonol 2009, 44(4):330-339.
    • (2009) Pediatr Pulmonol , vol.44 , Issue.4 , pp. 330-339
    • Harrison, A.N.1    Regelmann, W.E.2    Zirbes, J.M.3    Milla, C.E.4
  • 119
    • 0037795302 scopus 로고    scopus 로고
    • Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis
    • Konstan M.W., Butler S.M., Wohl M.E., Stoddard M., Matousek R., Wagener J.S., et al. Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis. J Pediatr 2003, 142(6):624-630.
    • (2003) J Pediatr , vol.142 , Issue.6 , pp. 624-630
    • Konstan, M.W.1    Butler, S.M.2    Wohl, M.E.3    Stoddard, M.4    Matousek, R.5    Wagener, J.S.6
  • 120
    • 46349106358 scopus 로고    scopus 로고
    • Lung function from infancy to the preschool years after clinical diagnosis of cystic fibrosis
    • Kozlowska W.J., Bush A., Wade A., Aurora P., Carr S.B., Castle R.A., et al. Lung function from infancy to the preschool years after clinical diagnosis of cystic fibrosis. Am J Respir Crit Care Med 2008, 178(1):42-49.
    • (2008) Am J Respir Crit Care Med , vol.178 , Issue.1 , pp. 42-49
    • Kozlowska, W.J.1    Bush, A.2    Wade, A.3    Aurora, P.4    Carr, S.B.5    Castle, R.A.6
  • 123
    • 73449118203 scopus 로고    scopus 로고
    • Pulmonary outcome differences in U.S. and French cystic fibrosis cohorts diagnosed through newborn screening
    • Walsh A.C., Rault G., Li Z., Scotet V., Dugueperoux I., Ferec C., et al. Pulmonary outcome differences in U.S. and French cystic fibrosis cohorts diagnosed through newborn screening. J Cyst Fibros 2010, 9(1):44-50.
    • (2010) J Cyst Fibros , vol.9 , Issue.1 , pp. 44-50
    • Walsh, A.C.1    Rault, G.2    Li, Z.3    Scotet, V.4    Dugueperoux, I.5    Ferec, C.6
  • 124
    • 84862776813 scopus 로고    scopus 로고
    • Progression of early structural lung disease in young children with cystic fibrosis assessed using CT
    • Mott L.S., Park J., Murray C.P., Gangell C.L., de Klerk N.H., Robinson P.J., et al. Progression of early structural lung disease in young children with cystic fibrosis assessed using CT. Thorax 2012, 67(6):509-516.
    • (2012) Thorax , vol.67 , Issue.6 , pp. 509-516
    • Mott, L.S.1    Park, J.2    Murray, C.P.3    Gangell, C.L.4    de Klerk, N.H.5    Robinson, P.J.6
  • 125
    • 84890063721 scopus 로고    scopus 로고
    • Association of lung function, chest radiographs and clinical features in infants with cystic fibrosis
    • Rosenfeld M., Farrell P.M., Kloster M., Swanson J.O., Vu T., Brumback L., et al. Association of lung function, chest radiographs and clinical features in infants with cystic fibrosis. Eur Respir J 2013, 42(6):1545-1552.
    • (2013) Eur Respir J , vol.42 , Issue.6 , pp. 1545-1552
    • Rosenfeld, M.1    Farrell, P.M.2    Kloster, M.3    Swanson, J.O.4    Vu, T.5    Brumback, L.6
  • 126
    • 34547587593 scopus 로고    scopus 로고
    • Radiological and functional changes over 3 years in young children with cystic fibrosis
    • Terheggen-Lagro S.W., Arets H.G., van der Laag J., van der Ent C.K. Radiological and functional changes over 3 years in young children with cystic fibrosis. Eur Respir J 2007, 30(2):279-285.
    • (2007) Eur Respir J , vol.30 , Issue.2 , pp. 279-285
    • Terheggen-Lagro, S.W.1    Arets, H.G.2    van der Laag, J.3    van der Ent, C.K.4
  • 127
    • 0033510667 scopus 로고    scopus 로고
    • Prospective, long-term study of fat-soluble vitamin status in children with cystic fibrosis identified by newborn screen
    • Feranchak A.P., Sontag M.K., Wagener J.S., Hammond K.B., Accurso F.J., Sokol R.J. Prospective, long-term study of fat-soluble vitamin status in children with cystic fibrosis identified by newborn screen. J Pediatr 1999, 135(5):601-610.
    • (1999) J Pediatr , vol.135 , Issue.5 , pp. 601-610
    • Feranchak, A.P.1    Sontag, M.K.2    Wagener, J.S.3    Hammond, K.B.4    Accurso, F.J.5    Sokol, R.J.6
  • 128
    • 0031646977 scopus 로고    scopus 로고
    • Low plasma zinc concentrations in young infants with cystic fibrosis
    • Krebs N.F., Sontag M., Accurso F.J., Hambidge K.M. Low plasma zinc concentrations in young infants with cystic fibrosis. J Pediatr 1998, 133(6):761-764.
    • (1998) J Pediatr , vol.133 , Issue.6 , pp. 761-764
    • Krebs, N.F.1    Sontag, M.2    Accurso, F.J.3    Hambidge, K.M.4
  • 130
    • 84879325565 scopus 로고    scopus 로고
    • Prospective evaluation of respiratory exacerbations in children with cystic fibrosis from newborn screening to 5 years of age
    • Byrnes C.A., Vidmar S., Cheney J.L., Carlin J.B., Armstrong D.S., Cooper P.J., et al. Prospective evaluation of respiratory exacerbations in children with cystic fibrosis from newborn screening to 5 years of age. Thorax 2013, 68(7):643-651.
    • (2013) Thorax , vol.68 , Issue.7 , pp. 643-651
    • Byrnes, C.A.1    Vidmar, S.2    Cheney, J.L.3    Carlin, J.B.4    Armstrong, D.S.5    Cooper, P.J.6
  • 131
    • 0027652118 scopus 로고
    • Longitudinal evaluation of pulmonary function in infants and very young children with cystic fibrosis
    • Tepper R.S., Montgomery G.L., Ackerman V., Eigen H. Longitudinal evaluation of pulmonary function in infants and very young children with cystic fibrosis. Pediatr Pulmonol 1993, 16(2):96-100.
    • (1993) Pediatr Pulmonol , vol.16 , Issue.2 , pp. 96-100
    • Tepper, R.S.1    Montgomery, G.L.2    Ackerman, V.3    Eigen, H.4
  • 132
    • 0036320272 scopus 로고    scopus 로고
    • Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis
    • Emerson J., Rosenfeld M., McNamara S., Ramsey B., Gibson R.L. Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis. Pediatr Pulmonol 2002, 34(2):91-100.
    • (2002) Pediatr Pulmonol , vol.34 , Issue.2 , pp. 91-100
    • Emerson, J.1    Rosenfeld, M.2    McNamara, S.3    Ramsey, B.4    Gibson, R.L.5
  • 133
    • 0029077443 scopus 로고
    • Long term prognosis of patients with cystic fibrosis in relation to early detection by neonatal screening and treatment in a cystic fibrosis centre
    • Dankert-Roelse J.E., te Meerman G.J. Long term prognosis of patients with cystic fibrosis in relation to early detection by neonatal screening and treatment in a cystic fibrosis centre. Thorax 1995, 50(7):712-718.
    • (1995) Thorax , vol.50 , Issue.7 , pp. 712-718
    • Dankert-Roelse, J.E.1    te Meerman, G.J.2
  • 134
    • 25144469405 scopus 로고    scopus 로고
    • Neonatal screening for cystic fibrosis is beneficial even in the context of modern treatment
    • Sims E.J., McCormick J., Mehta G., Mehta A. Neonatal screening for cystic fibrosis is beneficial even in the context of modern treatment. J Pediatr 2005, 147(3 Suppl):S42-S46.
    • (2005) J Pediatr , vol.147 , Issue.3 , pp. S42-S46
    • Sims, E.J.1    McCormick, J.2    Mehta, G.3    Mehta, A.4
  • 135
    • 84856541302 scopus 로고    scopus 로고
    • Comparison of the US and Australian cystic fibrosis registries: the impact of newborn screening
    • Martin B., Schechter M.S., Jaffe A., Cooper P., Bell S.C., Ranganathan S. Comparison of the US and Australian cystic fibrosis registries: the impact of newborn screening. Pediatrics 2012, 129(2):e348-e355.
    • (2012) Pediatrics , vol.129 , Issue.2 , pp. e348-e355
    • Martin, B.1    Schechter, M.S.2    Jaffe, A.3    Cooper, P.4    Bell, S.C.5    Ranganathan, S.6
  • 137
    • 17744372496 scopus 로고    scopus 로고
    • Early diagnosis of cystic fibrosis through neonatal screening prevents severe malnutrition and improves long-term growth
    • Farrell P.M., Kosorok M.R., Rock M.J., Laxova A., Zeng L., Lai H.C., et al. Early diagnosis of cystic fibrosis through neonatal screening prevents severe malnutrition and improves long-term growth. Pediatrics 2001, 107(1):1-13.
    • (2001) Pediatrics , vol.107 , Issue.1 , pp. 1-13
    • Farrell, P.M.1    Kosorok, M.R.2    Rock, M.J.3    Laxova, A.4    Zeng, L.5    Lai, H.C.6
  • 138
    • 0037730320 scopus 로고    scopus 로고
    • Comparing the clinical evolution of cystic fibrosis screened neonatally to that of cystic fibrosis diagnosed from clinical symptoms: a 10-year retrospective study in a French region (Brittany)
    • Siret D., Bretaudeau G., Branger B., Dabadie A., Dagorne M., David V., et al. Comparing the clinical evolution of cystic fibrosis screened neonatally to that of cystic fibrosis diagnosed from clinical symptoms: a 10-year retrospective study in a French region (Brittany). Pediatr Pulmonol 2003, 35(5):342-349.
    • (2003) Pediatr Pulmonol , vol.35 , Issue.5 , pp. 342-349
    • Siret, D.1    Bretaudeau, G.2    Branger, B.3    Dabadie, A.4    Dagorne, M.5    David, V.6
  • 139
    • 25844466580 scopus 로고    scopus 로고
    • The survival advantage of patients with cystic fibrosis diagnosed through neonatal screening: evidence from the United States Cystic Fibrosis Foundation registry data
    • Lai H.J., Cheng Y., Farrell P.M. The survival advantage of patients with cystic fibrosis diagnosed through neonatal screening: evidence from the United States Cystic Fibrosis Foundation registry data. J Pediatr 2005, 147(3 Suppl):S57-S63.
    • (2005) J Pediatr , vol.147 , Issue.3 , pp. S57-S63
    • Lai, H.J.1    Cheng, Y.2    Farrell, P.M.3
  • 140
    • 84874660385 scopus 로고    scopus 로고
    • Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis
    • Yen E.H., Quinton H., Borowitz D. Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. J Pediatr 2013, 162(3):530-535. e531.
    • (2013) J Pediatr , vol.162 , Issue.3 , pp. 530-535
    • Yen, E.H.1    Quinton, H.2    Borowitz, D.3
  • 141
    • 84861841848 scopus 로고    scopus 로고
    • Outcome measures for clinical trials assessing treatment of cystic fibrosis lung disease
    • VanDevanter D., Konstan M. Outcome measures for clinical trials assessing treatment of cystic fibrosis lung disease. Clin Invest 2012, 2(2):163-175.
    • (2012) Clin Invest , vol.2 , Issue.2 , pp. 163-175
    • VanDevanter, D.1    Konstan, M.2
  • 142
    • 0000471085 scopus 로고
    • Cystic fibrosis of the pancreas and its relation to celiac disease: a clinical and pathologic study
    • Andersen D.H. Cystic fibrosis of the pancreas and its relation to celiac disease: a clinical and pathologic study. Am J Dis Child 1938, 56(2):344-399.
    • (1938) Am J Dis Child , vol.56 , Issue.2 , pp. 344-399
    • Andersen, D.H.1
  • 143
    • 84912523010 scopus 로고    scopus 로고
    • Improvements in lung function and height among cohorts of 6-year-olds with cystic fibrosis from 1994 to 2012
    • VanDevanter D.R., Pasta D.J., Konstan M.W. Improvements in lung function and height among cohorts of 6-year-olds with cystic fibrosis from 1994 to 2012. J Pediatr 2014, 165(6):1091-1097. (e1092).
    • (2014) J Pediatr , vol.165 , Issue.6 , pp. 1091-1097
    • VanDevanter, D.R.1    Pasta, D.J.2    Konstan, M.W.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.