-
1
-
-
84892619977
-
-
Cystic Fibrosis Foundation [homepage on the Internet]. [cited 2011 Sep 20]. Available from
-
Cystic Fibrosis Foundation [homepage on the Internet]. Patient Registry 2009: annual report [cited 2011 Sep 20]. Available from: http://www.cff.org/UploadedFiles/research/ClinicalResearch/Patient-Registry-Report-2009.pdf
-
Patient Registry 2009: Annual report
-
-
-
2
-
-
66849106907
-
Pulmonary complications of cystic fibrosis
-
Flume PA. Pulmonary complications of cystic fibrosis. Respir Care 2009;54:618-27.
-
(2009)
Respir Care
, vol.54
, pp. 618-627
-
-
Flume, P.A.1
-
3
-
-
0024424270
-
Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
-
Riordan JR, Rommens JM, Kerem B, Alon N, Rozmahel R, Grzelczak Z et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 1989;245:1066-73.
-
(1989)
Science
, vol.245
, pp. 1066-1073
-
-
Riordan, J.R.1
Rommens, J.M.2
Kerem, B.3
Alon, N.4
Rozmahel, R.5
Grzelczak, Z.6
-
4
-
-
84892568713
-
Cystic fibrosis overview and update on infant care
-
Padman R, Passi V. Cystic fibrosis overview and update on infant care. OJPed 2012;2:187-96.
-
(2012)
OJPed
, vol.2
, pp. 187-196
-
-
Padman, R.1
Passi, V.2
-
5
-
-
36248953255
-
Cystic fibrosis pulmonary guidelines: Chronic medications for maintenance of lung health
-
Flume PA, O'Sullivan BP, Robinson KA, Goss CH, Mogayzel PJ Jr, Willey-Courand DB et al. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med 2007;176:957-69.
-
(2007)
Am J Respir Crit Care Med
, vol.176
, pp. 957-969
-
-
Flume, P.A.1
O'Sullivan, B.P.2
Robinson, K.A.3
Goss, C.H.4
Mogayzel Jr., P.J.5
Willey-Courand, D.B.6
-
6
-
-
0029010510
-
Lower respiratory infection and inflammation in infants with newly diagnosed cystic fibrosis
-
Armstrong DS, Grimwood K, Carzino R, Carlin JB, Olinsky A, Phelan PD. Lower respiratory infection and inflammation in infants with newly diagnosed cystic fibrosis. BMJ 1995;310:1571-2.
-
(1995)
BMJ
, vol.310
, pp. 1571-1572
-
-
Armstrong, D.S.1
Grimwood, K.2
Carzino, R.3
Carlin, J.B.4
Olinsky, A.5
Phelan, P.D.6
-
7
-
-
47049115524
-
Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report
-
Farrell PM, Rosenstein BJ, White TB, Accurso FJ, Castellani C, Cutting GR et al. Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report. J Pediatr 2008;153:S4-14.
-
(2008)
J Pediatr
, vol.153
-
-
Farrell, P.M.1
Rosenstein, B.J.2
White, T.B.3
Accurso, F.J.4
Castellani, C.5
Cutting, G.R.6
-
9
-
-
28344436257
-
Cystic fibrosis at a Brazilian center of excellence: Clinical and laboratory characteristics of 104 patients and their association with genotype and disease severity
-
Alvarez AE, Ribeiro AF, Hessel G, Bertuzzo CS, Ribeiro JD. 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;80:371-9.
-
(2004)
J Pediatr (Rio J)
, vol.80
, pp. 371-379
-
-
Alvarez, A.E.1
Ribeiro, A.F.2
Hessel, G.3
Bertuzzo, C.S.4
Ribeiro, J.D.5
-
10
-
-
30944466084
-
A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis
-
Elkins MR, Robinson M, Rose BR, Harbour C, Moriarty CP, Marks GB et al. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 2006;354:229-40.
-
(2006)
N Engl J Med
, vol.354
, pp. 229-240
-
-
Elkins, M.R.1
Robinson, M.2
Rose, B.R.3
Harbour, C.4
Moriarty, C.P.5
Marks, G.B.6
-
11
-
-
0025647882
-
Recombinant human DNase I reduces the viscosity of cystic fibrosis sputum
-
Shak S, Capon DJ, Hellmiss R, Marsters SA, Baker CL. Recombinant human DNase I reduces the viscosity of cystic fibrosis sputum. Proc Natl Acad Sci U S A 1990;87:9188-92.
-
(1990)
Proc Natl Acad Sci U S A
, vol.87
, pp. 9188-9192
-
-
Shak, S.1
Capon, D.J.2
Hellmiss, R.3
Marsters, S.A.4
Baker, C.L.5
-
12
-
-
0028129568
-
Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis
-
The Pulmozyme Study Group
-
Fuchs HJ, Borowitz DS, Christiansen DH, Morris EM, Nash ML, Ramsey BW et al. Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group. N Engl J Med 1994;331:637-42.
-
(1994)
N Engl J Med
, vol.331
, pp. 637-642
-
-
Fuchs, H.J.1
Borowitz, D.S.2
Christiansen, D.H.3
Morris, E.M.4
Nash, M.L.5
Ramsey, B.W.6
-
14
-
-
77957273192
-
Dornase alfa improves the health-related quality of life among Brazilian patients with cystic fibrosis-a one-year prospective study
-
Rozov T, de Oliveira VZ, Santana MA, Adde FV, Mendes RH, Paschoal IA et al. Dornase alfa improves the health-related quality of life among Brazilian patients with cystic fibrosis-a one-year prospective study. Pediatr Pulmonol 2010;45:874-82.
-
(2010)
Pediatr Pulmonol
, vol.45
, pp. 874-882
-
-
Rozov, T.1
de Oliveira, V.Z.2
Santana, M.A.3
Adde, F.V.4
Mendes, R.H.5
Paschoal, I.A.6
-
15
-
-
33645990651
-
Linguistic validation of cystic fibrosis quality of life questionnaires
-
Rozov T, Cunha MT, Nascimento O, Quittner AL, Jardim JR. Linguistic validation of cystic fibrosis quality of life questionnaires. J Pediatr (Rio J) 2006;82:151-6.
-
(2006)
J Pediatr (Rio J)
, vol.82
, pp. 151-156
-
-
Rozov, T.1
Cunha, M.T.2
Nascimento, O.3
Quittner, A.L.4
Jardim, J.R.5
-
17
-
-
69549135253
-
Inhaled medication and inhalation devices for lung disease in patients with cystic fibrosis: A European consensus
-
Consensus Working Group
-
Heijerman H, Westerman E, Conway S, Touw D, Döring G; Consensus Working Group. Inhaled medication and inhalation devices for lung disease in patients with cystic fibrosis: a European consensus. J Cyst Fibros 2009;8:295-315.
-
(2009)
J Cyst Fibros
, vol.8
, pp. 295-315
-
-
Heijerman, H.1
Westerman, E.2
Conway, S.3
Touw, D.4
Döring, G.5
-
18
-
-
16344378766
-
Measuring and reporting quality of life outcomes in clinical trials in cystic fibrosis: A critical review
-
Abbott J, Hart A. Measuring and reporting quality of life outcomes in clinical trials in cystic fibrosis: a critical review. Health Qual Life Outcomes 2005;3:19.
-
(2005)
Health Qual Life Outcomes
, vol.3
, pp. 19
-
-
Abbott, J.1
Hart, A.2
-
20
-
-
84892613135
-
-
Grupo Brasileiro de Estudos de Fibrose Cística [homepage on the Internet]. [cited 2013 Feb 21]. Available from
-
Grupo Brasileiro de Estudos de Fibrose Cística [homepage on the Internet]. Registro Brasileiro de Fibrose Cística: primeiro relatório anual-ano 2009 [cited 2013 Feb 21]. Available from: http://www.gbefc.org.br/gbefc/estudo_gbefc_2009.pdf
-
Registro Brasileiro de Fibrose Cística: Primeiro relatório anual-ano 2009
-
-
-
21
-
-
38049007046
-
Incidence of cystic fibrosis in five different states of Brazil as determined by screening of p. F508del, mutation at the CFTR gene in newborns and patients
-
Raskin S, Pereira-Ferrari L, Reis FC, Abreu F, Marostica P, Rozov T et al. Incidence of cystic fibrosis in five different states of Brazil as determined by screening of p. F508del, mutation at the CFTR gene in newborns and patients. J Cyst Fibros 2008;7:15-22.
-
(2008)
J Cyst Fibros
, vol.7
, pp. 15-22
-
-
Raskin, S.1
Pereira-Ferrari, L.2
Reis, F.C.3
Abreu, F.4
Marostica, P.5
Rozov, T.6
-
22
-
-
80052014087
-
Inflammatory responses to individual microorganisms in the lungs of children with cystic fibrosis
-
Gangell C, Gard S, Douglas T, Park J, de Klerk N, Keil T et al. Inflammatory responses to individual microorganisms in the lungs of children with cystic fibrosis. Clin Infect Dis 2011;53:425-32.
-
(2011)
Clin Infect Dis
, vol.53
, pp. 425-432
-
-
Gangell, C.1
Gard, S.2
Douglas, T.3
Park, J.4
de Klerk, N.5
Keil, T.6
-
23
-
-
0036125965
-
Pulmonary infections in patients with cystic fibrosis
-
Rajan S, Saiman L. Pulmonary infections in patients with cystic fibrosis. Semin Respir Infect 2002;17:47-56.
-
(2002)
Semin Respir Infect
, vol.17
, pp. 47-56
-
-
Rajan, S.1
Saiman, L.2
-
24
-
-
14844303461
-
Prospective evaluation of emerging bacteria in cystic fibrosis
-
Steinkamp G, Wiedemann B, Rietschel E, Krahl A, Gielen J, Bärmeier H et al. Prospective evaluation of emerging bacteria in cystic fibrosis. J Cyst Fibros 2005;4:41-8.
-
(2005)
J Cyst Fibros
, vol.4
, pp. 41-48
-
-
Steinkamp, G.1
Wiedemann, B.2
Rietschel, E.3
Krahl, A.4
Gielen, J.5
Bärmeier, H.6
-
25
-
-
77952728027
-
No positive effect of rhdnase on the pulmonary colonization in children with cystic fibrosis
-
Bonestroo HJ, Slieker MG, Arets HG. No positive effect of rhdnase on the pulmonary colonization in children with cystic fibrosis. Monaldi Arch Chest Dis 2010;73:12-7.
-
(2010)
Monaldi Arch Chest Dis
, vol.73
, pp. 12-17
-
-
Bonestroo, H.J.1
Slieker, M.G.2
Arets, H.G.3
-
26
-
-
33748672304
-
Effect of aerosolized rhDNase (Pulmozyme) on pulmonary colonization in patients with cystic fibrosis
-
Frederiksen B, Pressler T, Hansen A, Koch C, Høiby N. Effect of aerosolized rhDNase (Pulmozyme) on pulmonary colonization in patients with cystic fibrosis. Acta Paediatr 2006;95:1070-4.
-
(2006)
Acta Paediatr
, vol.95
, pp. 1070-1074
-
-
Frederiksen, B.1
Pressler, T.2
Hansen, A.3
Koch, C.4
Høiby, N.5
-
27
-
-
0035666058
-
A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities
-
Quan JM, Tiddens HA, Sy JP, McKenzie SG, Montgomery MD, Robinson PJ et al. A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities. J Pediatr 2001;139:813-20.
-
(2001)
J Pediatr
, vol.139
, pp. 813-820
-
-
Quan, J.M.1
Tiddens, H.A.2
Sy, J.P.3
McKenzie, S.G.4
Montgomery, M.D.5
Robinson, P.J.6
-
28
-
-
78650492873
-
Longitudinal assessment of health-related quality of life in an observational cohort of patients with cystic fibrosis
-
Sawicki GS, Rasouliyan L, McMullen AH, Wagener JS, McColley SA, Pasta DJ et al. Longitudinal assessment of health-related quality of life in an observational cohort of patients with cystic fibrosis. Pediatr Pulmonol 2011;46:36-44.
-
(2011)
Pediatr Pulmonol
, vol.46
, pp. 36-44
-
-
Sawicki, G.S.1
Rasouliyan, L.2
McMullen, A.H.3
Wagener, J.S.4
McColley, S.A.5
Pasta, D.J.6
-
29
-
-
77951228925
-
Dornase alfa for cystic fibrosis
-
[serial on the Internet]. [cited 2013 Feb 20]. Available from
-
Jones AP, Wallis C. Dornase alfa for cystic fibrosis. Cochrane Database Syst Rev [serial on the Internet]. 2010(3):CD001127 [cited 2013 Feb 20]. Available from: http://www.ncbi.nlm.nih.gov/pubmed/20238314
-
(2010)
Cochrane Database Syst Rev
, Issue.3
-
-
Jones, A.P.1
Wallis, C.2
-
31
-
-
56049116219
-
Improvements in lung function outcomes in children with cystic fibrosis are associated with better nutrition, fewer chronic pseudomonas aeruginosa infections, and dornase alfa use
-
McPhail GL, Acton JD, Fenchel MC, Amin RS, Seid M. Improvements in lung function outcomes in children with cystic fibrosis are associated with better nutrition, fewer chronic pseudomonas aeruginosa infections, and dornase alfa use. J Pediatr 2008;153:752-7.
-
(2008)
J Pediatr
, vol.153
, pp. 752-757
-
-
McPhail, G.L.1
Acton, J.D.2
Fenchel, M.C.3
Amin, R.S.4
Seid, M.5
-
32
-
-
16344385427
-
The measurement of health-related quality of life (QOL) in paediatric clinical trials: A systematic review
-
Clarke SA, Eiser C. The measurement of health-related quality of life (QOL) in paediatric clinical trials: a systematic review. Health Qual Life Outcomes 2004;2:66.
-
(2004)
Health Qual Life Outcomes
, vol.2
, pp. 66
-
-
Clarke, S.A.1
Eiser, C.2
-
33
-
-
0035094310
-
A case-controlled study with dornase alfa to evaluate impact on disease progression over a 4-year period
-
Shah PL, Conway S, Scott SF, Rainisio M, Wildman M, Stableforth D et al. A case-controlled study with dornase alfa to evaluate impact on disease progression over a 4-year period. Respiration 2001;68:160-4.
-
(2001)
Respiration
, vol.68
, pp. 160-164
-
-
Shah, P.L.1
Conway, S.2
Scott, S.F.3
Rainisio, M.4
Wildman, M.5
Stableforth, D.6
|