Dorgan Daniel J, Hadjiliadis Denis
Division of Pulmonary, Allergy and Critical Care, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA.
Expert Rev Respir Med. 2014 Jun;8(3):315-26. doi: 10.1586/17476348.2014.899906. Epub 2014 Mar 21.
Despite advances in medical care, patients with cystic fibrosis still face limited life expectancy. The most common cause of death remains respiratory failure. End-stage cystic fibrosis can be treated with lung transplantation and is the third most common reason for which the procedure is performed. Outcomes for cystic fibrosis are better than most other lung diseases, but remain limited (5-year survival 60%). For patients with advanced disease lung transplantation appears to improve survival. Outcomes for patients with Burkholderia cepacia remain poor, although they are better for patients with certain genomovars. Controversy exists about Mycobacterium abscessus infection and appropriateness for transplant. More information is also becoming available for comorbidities, including diabetes and pulmonary hypertension among others. Extra-corporeal membrane oxygenation is used more frequently for end-stage disease as a bridge to lung transplantation and will likely be used more in the future.
尽管医疗护理取得了进步，但囊性纤维化患者的预期寿命仍然有限。最常见的死亡原因仍是呼吸衰竭。终末期囊性纤维化可通过肺移植进行治疗，且肺移植是该手术实施的第三大常见原因。囊性纤维化患者的治疗效果优于大多数其他肺部疾病，但仍有限（5年生存率为60%）。对于晚期疾病患者，肺移植似乎可提高生存率。洋葱伯克霍尔德菌感染患者的治疗效果仍然较差，不过某些基因变种的患者治疗效果较好。对于脓肿分枝杆菌感染以及移植的适宜性存在争议。关于合并症（包括糖尿病和肺动脉高压等）的信息也越来越多。体外膜肺氧合在终末期疾病中作为肺移植的桥梁使用得更为频繁，并且未来可能会使用得更多。
