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囊性肺间质纤维化的治疗进展
作者:刘顺事  岳红梅  刘浩 
单位:兰州大学第一医院 呼吸内科, 甘肃 兰州 730000
关键词:囊性纤维化跨膜转导调节因子基因 囊性肺间质纤维化 治疗进展 文献综述 
分类号:R563.9
出版年·卷·期(页码):2017·36·第一期(123-125)
摘要:

传统上治疗囊性肺间质纤维化主要针对该病所致的黏液阻塞及继发感染进行对症治疗。近几年通过对囊性纤维化跨膜转导调节因子(CFTR)基因进行干预,使该病在治疗上取得了重大进展。作者对目前临床上囊性肺间质纤维化的常规治疗方法及相关新疗法的进展作一综述。

参考文献:

[1] KONSTAN M W,RATJEN F.Effect of dornase alfa on inflammation and lung function:potential role in the early treatment of cystic fibrosis[J].J Cyst Fibros,2012,11(2):78-83.
[2] AMIN R,SUBBARAO P,LOU W,et al.The effect of dornase alfa on ventilation inhomogeneity in patients with cystic fibrosis[J].Eur Respir,2011,37(4):806-812.
[3] CONRAD C,LYMP J,THOMPSON V,et al.Long-term treatment with oral N-acetylcysteine:affects lung function butnotsputum inflammation in cystic fibrosis subjects.A phase Ⅱ randomized placebo-controlled trial[J].J CystFibros,2015,14(2):219-227.
[4] ELKINS M R,ROBINSON M,ROSE B R,et al.A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis[J].N Engl J Med,2006,354(3):229-240.
[5] BUONPENSIERO P,De GREGORIO F,SEPE A,et al.Hyaluronic acid improves "pleasantness" and tolerability of nebulized hypertonic saline in a cohort of patients with cystic fibrosis[J].Adv Ther,2010,27(11):870-878.
[6] FURNARI M L,TERMINI L,TRAVERSO G,et al.Nebulized hypertonic saline containing hyaluronic acid improves tolerability in patients with cystic fibrosis and lung disease compared with nebulized hypertonic saline alone:a prospective,randomized,double-blind,controlled study[J].Ther Adv Respir Dis,2012,6(6):315-322.
[7] ROS M,CASCIARO R,LUCCA F,et al.Hyaluronic acid improves the tolerability of hypertonic saline in the chronic treatment of cystic fibrosis patients:a multicenter,randomized,controlled clinical trial[J].Aerosol Med Pulm Drug Deliv,2014,27(2):133-137.
[8] AITKEN M L,BELLON G,De BOECK K,et al.Long-term inhaled dry powder mannitol in cystic fibrosis:an international randomized study[J].Am J Respir Crit Care Med,2012,185(6):645-652.
[9] BILTON D,DAVISKAS E,ANDERSON S D, et al.Phase 3 randomized study of the efficacy and safety of inhaled dry powder mannitol for the symptomatic treatment of non-cystic fibrosis bronchiectasis[J].Chest,2013,144(1):215-225.
[10] ACCURSO F J,MOSS R B,WILMOTT R W,et al.Denufosol tetrasodium in patients with cystic fibrosis and normal to mildly impaired lung function[J].Am J Respir Crit Care Med,2011,183(5):627-634.
[11] RATJEN F,DURHAM T,NAVRATIL T,et al.Long term effects of denufosol tetrasodium in patients with cystic fibrosis[J].J Cyst Fibros,2012,11(6):539-549.
[12] MOGAYZEL P JJr,NAURECKAS E T,ROBINSON K A,et al.Cystic fibrosis pulmonary guidelines.Chronic medications for maintenance of lung health[J].Am J Respir Crit Care Med,2013,187(7):680-689.
[13] LANGTON HEWER S C,SMYTH A R.Antibiotic strategies for eradicating Pseudomonas aeruginosa in people with cystic fibrosis[J].Cochrane Database Syst Rev,2014(11):CD004197.
[14] TRAPNELL B C,MCCOLLEY S A,KISSNER D G,et al.Fosfomycin/tobramycin for inhalation in patients with cystic fibrosis with pseudomonas airway infection[J].Am J Respir Crit Care Med,2012,185(2):171-178.
[15] WATERS V,RATJEN F.Antibiotic treatment for nontuberculous mycobacteria lung infection in people with cystic fibrosis[J].Cochrane Database Syst Rev,2014(12):CD010004.
[16] LANDS L C,STANOJEVIC S.Oral non-steroidal anti-inflammatory drug therapy for lung disease in cystic fibrosis[J].Cochrane Database Syst Rev,2013(6):CD001505.
[17] BALFOUR-LYNN I M,LEES B,HALL P,et al.Multicenter randomized controlled trial of withdrawal of inhaled corticosteroids in cystic fibrosis[J].Am J Respir Crit Care Med,2006(173):1356-1362.
[18] RAMSEY B W,DAVIES J,McELVANEY N G.A CFTR potentiator in patients with cystic fibrosis and the G551d mutation[J].N Engl J Med,2011,365(18):1663-1672.
[19] DAVIES J C,WAINWRIGHT C E,CANNY G J,et al.Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551d mutation[J].Am J Respir Crit Care Med,2013,187(11):1219-1225.
[20] De BOECK K,MUNCK A,WALKER S,et al.Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation[J].J Cyst Fibros,2014,13(6):674-680.
[21] CLANCY J P,ROWE S M,ACCURSO F J,et al.Results of a phase Ⅱa study of VX-809,an investigational CFTR corrector compound,in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation[J].Thorax,2012,67(1):12-18.
[22] FLUME P A,LIOU T G,BOROWITZ D S,et al.Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation[J].Chest,2012,142(3):718-724.
[23] WAINWRIGHT C E,ELBORN J S,RAMSEY B W.Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR[J].N Engl J Med,2015,373(18):1783-1784.
[24] KEREM E,KONSTAN MW,De BOECK K,et al.Ataluren for the treatment of nonsense-mutation cystic fibrosis:a randomized,double-blind,placebo-controlled phase 3 trial[J].Lancet Respir Med,2014,2(7):539-547.
[25] ALTON E W,ARMSTRONG D K,ASHBY D,et al.Repeated nebulisation of non-viral CFTR gene therapy in patients with cystic fibrosis:a randomized,double-blind,placebo-controlled,phase 2b trial[J].Lancet Respir Med,2015,3(9):684-691.

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