Cystic fibrosis in adults - Current and future management strategies

被引：11
作者：
Morrissey, BM
Schock, BC
Marelich, GP
Cross, CE
机构：
[1] Adult Cyst Fibrosis Clin, Sacramento, CA USA
[2] Univ Calif Davis, Sch Med, Sacramento, CA 95817 USA
[3] Queens Univ Belfast, Sch Med, Resp Res Grp, Belfast, Antrim, North Ireland
[4] Kaiser Permanente, Sacramento, CA USA
来源：
CLINICAL REVIEWS IN ALLERGY & IMMUNOLOGY | 2003年 / 25卷 / 03期
关键词：
cystic fibrosis; CF; adult; CFTR; BRONCHIAL EPITHELIAL-CELLS; ALLERGIC BRONCHOPULMONARY ASPERGILLOSIS; PSEUDOMONAS-AERUGINOSA; LUNG-DISEASE; NONTUBERCULOUS-MYCOBACTERIA; PULMONARY DYSFUNCTION; RESPIRATORY-TRACT; OXIDATIVE STRESS; STOP MUTATIONS; CFTR GENE;
D O I：
10.1385/CRIAI:25:3:275
中图分类号：
R392 [医学免疫学];
学科分类号：
100102 ;
摘要：
Over 30,000 individuals in the United States of America are living with cystic fibrosis (CF). Despite incremental advances in care and understanding of its pathophysiology, CF remains a significantly life-limiting disease. Readily accessible newborn screening, genetic testing, and an improved awareness have increased the early recognition of CF, atypical presentations of CF, and the CF-related diseases. Improvements in medical management have led to continually improving life expectancy for patients with CF. Despite improved management strategies, severe lung disease remains the commonly life-limiting pathology. We review the pathophysiology, diagnosis, and management of the respiratory-tract manifestations of CF that represent the life-limiting aspects of the condition and summarize upcoming and possible future therapies for patients with CF.
引用
收藏
页码：275 / 287
页数：13
相关论文
共 80 条
[1]   Cystic fibrosis of the pancreas and its relation to celiac disease - A clinical and pathologic study [J].
Andersen, DH .
AMERICAN JOURNAL OF DISEASES OF CHILDREN, 1938, 56 (02) :344-399
[2]   Nacystelyn inhibits oxidant-mediated interleukin-8 expression and NF-κB nuclear binding in alveolar epithelial cells [J].
Antonicelli, F ;
Parmentier, M ;
Drost, EM ;
Hirani, N ;
Rahman, I ;
Donaldson, K ;
MacNee, W .
FREE RADICAL BIOLOGY AND MEDICINE, 2002, 32 (06) :492-502
[3]   Dose-finding and 24-h monitoring for efficacy and safety of aerosolized Nacystelyn in cystic fibrosis [J].
App, EM ;
Baran, D ;
Dab, I ;
Malfroot, A ;
Coffiner, M ;
Vanderbist, F ;
King, M .
EUROPEAN RESPIRATORY JOURNAL, 2002, 19 (02) :294-302
[4]  
BIRRER P, 1995, RESPIRATION, V62, P25
[5]   Bronchial artery embolization for the treatment of hemoptysis in patients with cystic fibrosis [J].
Brinson, GM ;
Noone, PG ;
Mauro, MA ;
Knowles, MR ;
Yankaskas, JR ;
Sandhu, JS ;
Jaques, PF .
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 1998, 157 (06) :1951-1958
[6]   Pulmonary dysfunction in cystic fibrosis is associated with oxidative stress [J].
Brown, RK ;
Wyatt, H ;
Price, JF ;
Kelly, FJ .
EUROPEAN RESPIRATORY JOURNAL, 1996, 9 (02) :334-339
[7]  
BRUCE MC, 1985, AM REV RESPIR DIS, V132, P529
[8]   α-tocopherol inhibits the respiratory burst in human monocytes -: Attenuation of p47phox membrane translocation and phosphorylation [J].
Cachia, O ;
El Benna, J ;
Pedruzzi, E ;
Descomps, B ;
Gougerot-Pocidalo, MA ;
Leger, CL .
JOURNAL OF BIOLOGICAL CHEMISTRY, 1998, 273 (49) :32801-32805
[9]   CFTR activation in human bronchial epithelial cells by novel benzoflavone and benzimidazolone compounds [J].
Caci, E ;
Folli, C ;
Zegarra-Moran, O ;
Ma, TH ;
Springsteel, MF ;
Sammelson, RE ;
Nantz, MH ;
Kurth, MJ ;
Verkman, AS ;
Galietta, LJV .
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY, 2003, 285 (01) :L180-L188
[10]   Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis [J].
Clancy, JP ;
Bobök, Z ;
Ruiz, F ;
King, C ;
Jones, J ;
Walker, L ;
Greer, H ;
Hong, J ;
Wing, L ;
Macaluso, M ;
Lyrene, R ;
Sorscher, EJ ;
Bedwell, DM .
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 2001, 163 (07) :1683-1692
12345678