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2
CFTR: cystic fibrosis and beyond.CFTR：囊性纤维化及其他。
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Mucus-targeting therapies of defective mucus clearance for cystic fibrosis: A short review.针对囊性纤维化黏液清除缺陷的黏液靶向治疗：简短综述。
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CFTR, mucins, and mucus obstruction in cystic fibrosis.囊性纤维化中的 CFTR、黏蛋白和黏液阻塞。
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[Chinese experts consensus statement: diagnosis and treatment of cystic fibrosis (2023)].[中国专家共识声明：囊性纤维化的诊断与治疗（2023年）]
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本文引用的文献

1
Early Life Growth Trajectories in Cystic Fibrosis are Associated with Pulmonary Function at Age 6 Years.囊性纤维化患者的早期生长轨迹与6岁时的肺功能相关。
J Pediatr. 2015 Nov;167(5):1081-8.e1. doi: 10.1016/j.jpeds.2015.07.044. Epub 2015 Sep 2.
2
Outcomes and Treatment of Chronic Methicillin-Resistant Staphylococcus aureus Differs by Staphylococcal Cassette Chromosome mec (SCCmec) Type in Children With Cystic Fibrosis.囊性纤维化患儿中，耐甲氧西林金黄色葡萄球菌慢性感染的结局及治疗因葡萄球菌盒式染色体mec（SCCmec）类型而异。
J Pediatric Infect Dis Soc. 2015 Sep;4(3):225-31. doi: 10.1093/jpids/piu048. Epub 2014 Jun 9.
3
Repeated nebulisation of non-viral CFTR gene therapy in patients with cystic fibrosis: a randomised, double-blind, placebo-controlled, phase 2b trial.反复雾化非病毒 CFTR 基因治疗囊性纤维化患者：一项随机、双盲、安慰剂对照、2b 期试验。
Lancet Respir Med. 2015 Sep;3(9):684-691. doi: 10.1016/S2213-2600(15)00245-3. Epub 2015 Jul 3.
4
Efficacy and safety of ivacaftor in patients with cystic fibrosis who have an Arg117His-CFTR mutation: a double-blind, randomised controlled trial.依伐卡托特治疗携带 Arg117His-CFTR 突变的囊性纤维化患者的疗效和安全性：一项双盲、随机对照试验。
Lancet Respir Med. 2015 Jul;3(7):524-33. doi: 10.1016/S2213-2600(15)00201-5. Epub 2015 Jun 9.
5
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.鲁马卡托-依伐卡托用于携带苯丙氨酸508位缺失CFTR基因纯合突变的囊性纤维化患者。
N Engl J Med. 2015 Jul 16;373(3):220-31. doi: 10.1056/NEJMoa1409547. Epub 2015 May 17.
6
Lung transplantation for cystic fibrosis: results, indications, complications, and controversies.囊性纤维化的肺移植：结果、适应证、并发症及争议
Semin Respir Crit Care Med. 2015 Apr;36(2):299-320. doi: 10.1055/s-0035-1547347. Epub 2015 Mar 31.
7
Nontuberculous mycobacteria in cystic fibrosis and non-cystic fibrosis bronchiectasis.囊性纤维化和非囊性纤维化支气管扩张中的非结核分枝杆菌
Semin Respir Crit Care Med. 2015 Apr;36(2):217-24. doi: 10.1055/s-0035-1546751. Epub 2015 Mar 31.
8
Reduced risk of nontuberculous mycobacteria in cystic fibrosis adults receiving long-term azithromycin.接受长期阿奇霉素治疗的成年囊性纤维化患者非结核分枝杆菌感染风险降低。
J Cyst Fibros. 2015 Sep;14(5):594-9. doi: 10.1016/j.jcf.2015.02.006. Epub 2015 Feb 28.
9
A phase 3, open-label, randomized trial to evaluate the safety and efficacy of levofloxacin inhalation solution (APT-1026) versus tobramycin inhalation solution in stable cystic fibrosis patients.一项评估左氧氟沙星吸入溶液（APT-1026）与妥布霉素吸入溶液在稳定期囊性纤维化患者中的安全性和疗效的 3 期、开放标签、随机试验。
J Cyst Fibros. 2015 Jul;14(4):507-14. doi: 10.1016/j.jcf.2014.12.013. Epub 2015 Jan 13.
10
Novel Approaches for Potential Therapy of Cystic Fibrosis.囊性纤维化潜在治疗的新方法。
Curr Drug Targets. 2015;16(9):923-36. doi: 10.2174/1389450116666150102113314.

囊性纤维化肺病的当前及未来治疗选择：最新证据与临床意义

Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications.

作者信息

Edmondson Claire, Davies Jane C

机构信息

Royal Brompton & Harefield NHS Foundation Trust, Paediatric Respiratory Medicine, London, UK.

Imperial College London, Paediatric Respirology and Experimental Medicine, London SW7 2AZ, UK.

出版信息

Ther Adv Chronic Dis. 2016 May;7(3):170-83. doi: 10.1177/2040622316641352. Epub 2016 May 1.

DOI:10.1177/2040622316641352
PMID:27347364
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4907071/
Abstract

Treatment for cystic fibrosis (CF) has conventionally targeted downstream consequences of the defect such as mucus plugging and infection. More recently, significant advances have been made in treating the root cause of the disease, namely a defective CF transmembrane conductance regulator (CFTR) gene. This review summarizes current pulmonary treatment options and highlights advances in research and development of new therapies.

摘要

传统上，囊性纤维化（CF）的治疗针对该缺陷的下游后果，如黏液阻塞和感染。最近，在治疗该疾病的根本原因方面取得了重大进展，即囊性纤维化跨膜传导调节因子（CFTR）基因缺陷。本综述总结了当前的肺部治疗选择，并重点介绍了新疗法研发方面的进展。