当前位置: X-MOL 学术Resp. Med. › 论文详情
Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)
Non-CF bronchiectasis: Orphan disease no longer.
Respiratory Medicine ( IF 3.5 ) Pub Date : 2020-03-27 , DOI: 10.1016/j.rmed.2020.105940
Jaafer Saadi Imam 1 , Alexander G Duarte 1
Affiliation  

Bronchiectasis is a complex, chronic respiratory condition, characterized by frequent cough and exertional dyspnea due to a range of conditions that include inherited mucociliary defects, inhalational airway injury, immunodeficiency states and prior respiratory infections. For years, bronchiectasis was classified as either being caused by cystic fibrosis or non-cystic fibrosis. Non-cystic fibrosis bronchiectasis, once considered an orphan disease, is more prevalent worldwide in part due to greater availability of chest computed tomographic imaging. Identification of the cause of non-cystic fibrosis bronchiectasis with the use of chest imaging, laboratory testing, and microbiologic assessment of airway secretions can lead to initiation of specific therapies aimed at slowing disease progression. Nonpharmacologic therapies such as airway clearance techniques and pulmonary rehabilitation improve patient symptoms. Inhaled corticosteroids should not be routinely prescribed unless concomitant asthma or COPD is present. Inhaled antibiotics prescribed to individuals with >3 exacerbations per year are well tolerated, reduce airway bacteria load and may reduce the frequency of exacerbations. Likewise, chronic macrolide therapy reduces the frequency of exacerbations. Medical therapies for cystic fibrosis bronchiectasis may not be effective in treatment of non-cystic fibrosis bronchiectasis.



中文翻译:

非CF支气管扩张:不再是孤儿疾病。

支气管扩张是一种复杂的慢性呼吸道疾病,由于一系列疾病,包括遗传性粘膜纤毛缺陷,吸入性气道损伤,免疫缺陷状态和先前的呼吸道感染,导致频繁咳嗽和劳累性呼吸困难。多年来,支气管扩张被归类为由囊性纤维化或非囊性纤维化引起。非囊性纤维化支气管扩张症,曾经被认为是一种孤儿病,在全球范围内更为普遍,部分原因是胸部计算机断层扫描成像的可用性更高。通过胸部成像,实验室检查和气道分泌物的微生物学评估来鉴定非囊性纤维化支气管扩张的原因,可以导致针对疾病进展的特异性疗法的启动。非药物疗法,例如气道清除技术和肺部康复,可改善患者症状。除非存在合并的哮喘或COPD,否则不应常规开具吸入糖皮质激素的处方。每年对≥3次急性发作的个体开具的吸入性抗生素耐受性良好,可减少气道细菌负荷,并可能减少急性发作的频率。同样,慢性大环内酯类药物治疗可减少病情加重的频率。囊性纤维化支气管扩张的医学疗法可能无法有效治疗非囊性纤维化支气管扩张。每年可耐受3次急性发作,减轻呼吸道细菌负荷,并可能减少急性发作的频率。同样,慢性大环内酯类药物治疗可降低病情加重的频率。囊性纤维化支气管扩张的医学疗法可能无法有效治疗非囊性纤维化支气管扩张。每年可耐受3次急性发作,减轻呼吸道细菌负荷,并可能减少急性发作的频率。同样,慢性大环内酯类药物治疗可减少病情加重的频率。囊性纤维化支气管扩张的医学疗法可能不适用于非囊性纤维化支气管扩张。

更新日期:2020-03-27
down
wechat
bug