Recent Advances in Cystic Fibrosis

被引:13
|
作者
Lobo, Jason [1 ]
Rojas-Balcazar, Juan M. [1 ]
Noone, Peadar G. [1 ]
机构
[1] Univ N Carolina, Dept Med, Div Pulm & Crit Care Med, Chapel Hill, NC 27599 USA
关键词
Cystic fibrosis; CFTR; Gene modifiers; Pseudomonas aeruginosa; Bronchiectasis; Mucociliary clearance; QUALITY-OF-LIFE; RESISTANT STAPHYLOCOCCUS-AUREUS; MUCOID PSEUDOMONAS-AERUGINOSA; BURKHOLDERIA-CEPACIA COMPLEX; LUNG-DISEASE SEVERITY; ADULT PATIENTS; RESPIRATORY-TRACT; NONTUBERCULOUS MYCOBACTERIA; PULMONARY INFECTIONS; LIVER-DISEASE;
D O I
10.1016/j.ccm.2012.02.006
中图分类号
R56 [呼吸系及胸部疾病];
学科分类号
摘要
Cystic fibrosis (CF) is an inherited chronic disease that remains a common cause of morbidity and mortality in affected patients, mostly in the young. A wealth of knowledge has been gained into the genetics, pathophysiology, and clinical manifestation of the disease. In parallel with these new insights into the disease, novel treatments have been developed or are under development that have had a major impact on quality of life and survival. Improvement in the delivery of care to patients in CF centers, using a team-based approach, and constant review of process, and by quality improvement projects, have also had an impact on outcomes in CF.
引用
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页码:307 / +
页数:23
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