Recent advances in cystic fibrosis

被引:26
作者
Ratjen, F. [1 ]
机构
[1] Hosp Sick Children, Div Resp Med, Toronto, ON M5G 1X8, Canada
关键词
cystic fibrosis; lung disease; airway inflammation; CFTR; airway infection;
D O I
10.1016/j.prrv.2008.01.004
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Considerable advances in cystic fibrosis (CF) research have translated into improved patient care, reflected by a continuing trend of improving life expectancy in CF patients. This review summarises some of the major findings of CF research that have occurred in the past year. The review specifically focuses on those developments that have direct implications for patient care or those in which clinical trials suggest benefits that may impact on the treatment of CF patients in the near future. (C) 2008 Elsevier Ltd. All rights reserved.
引用
收藏
页码:144 / 148
页数:5
相关论文
共 43 条
[1]   Evidence for airway surface dehydration as the initiating event in CF airway disease [J].
Boucher, R. C. .
JOURNAL OF INTERNAL MEDICINE, 2007, 261 (01) :5-16
[2]  
CHENG K, 2000, COCHRANE DB SYST REV
[3]   DEFECTIVE INTRACELLULAR-TRANSPORT AND PROCESSING OF CFTR IS THE MOLECULAR-BASIS OF MOST CYSTIC-FIBROSIS [J].
CHENG, SH ;
GREGORY, RJ ;
MARSHALL, J ;
PAUL, S ;
SOUZA, DW ;
WHITE, GA ;
ORIORDAN, CR ;
SMITH, AE .
CELL, 1990, 63 (04) :827-834
[4]   No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations [J].
Clancy, John P. ;
Rowe, Steven M. ;
Bebok, Zsuzsa ;
Aitken, Moira L. ;
Gibson, Ron ;
Zeitlin, Pam ;
Berclaz, Pierre ;
Moss, Rick ;
Knowles, Michael R. ;
Oster, Robert A. ;
Mayer-Hamblett, Nicole ;
Ramsey, Bonnie .
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY, 2007, 37 (01) :57-66
[5]   Cultural policy and the Tunisian Ma'luf + Arabic songs: Redefining a tradition [J].
Davis, R .
ETHNOMUSICOLOGY, 1997, 41 (01) :1-21
[6]   Phase 2 randomized safety and efficacy trial of nebulized denufosol tetrasodium in cystic fibrosis [J].
Deterding, Robin R. ;
LaVange, Lisa M. ;
Engels, Jean M. ;
Mathews, Dave W. ;
Coquillette, Sarah J. ;
Brody, Alan S. ;
Millard, Steve P. ;
Ramsey, Bonnie W. .
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 2007, 176 (04) :362-369
[7]   Cystic fibrosis mortality and survival in the UK: 1947-2003 [J].
Dodge, J. A. ;
Lewis, P. A. ;
Stanton, M. ;
Wilsher, J. .
EUROPEAN RESPIRATORY JOURNAL, 2007, 29 (03) :522-526
[8]   A double-blind randomized placebo-controlled phase III study of a Pseudomonas aeruginosa flagella vaccine in cystic fibrosis patients [J].
Doering, Gerd ;
Meisner, Christoph ;
Stern, Martin .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2007, 104 (26) :11020-11025
[9]   Mucus clearance and lung function in cystic fibrosis with hypertonic saline [J].
Donaldson, SH ;
Bennett, WD ;
Zeman, KL ;
Knowles, MR ;
Tarran, R ;
Boucher, RC .
NEW ENGLAND JOURNAL OF MEDICINE, 2006, 354 (03) :241-250
[10]   Oral corticosteroid therapy in cystic fibrosis patients hospitalized for pulmonary exacerbation - A pilot study [J].
Dovey, Mark ;
Aitken, Moira L. ;
Emerson, Julia ;
McNamara, Sharon ;
Waltz, David A. ;
Gibson, Ronald L. .
CHEST, 2007, 132 (04) :1212-1218