Vij Neeraj
Precision Theranostics Inc., Baltimore, MD 21202, USA.
VIJ Biotech, Baltimore, MD 21202, USA.
J Pers Med. 2021 Feb 3;11(2):96. doi: 10.3390/jpm11020096.
Cystic fibrosis (CF) is a genetic disease caused by a mutation(s) in the CF transmembrane regulator (CFTR), where progressive decline in lung function due to recurring exacerbations is a major cause of mortality. The initiation of chronic obstructive lung disease in CF involves inflammation and exacerbations, leading to mucus obstruction and lung function decline. Even though clinical management of CF lung disease has prolonged survival, exacerbation and age-related lung function decline remain a challenge for controlling the progressive lung disease. The key to the resolution of progressive lung disease is prognosis-based early therapeutic intervention; thus, the development of novel diagnostics and prognostic biomarkers for predicting exacerbation and lung function decline will allow optimal management of the lung disease. Hence, the development of real-time lung function diagnostics such as forced oscillation technique (FOT), impulse oscillometry system (IOS), and electrical impedance tomography (EIT), and novel prognosis-based intervention strategies for controlling the progression of chronic obstructive lung disease will fulfill a significant unmet need for CF patients. Early detection of CF lung inflammation and exacerbations with the timely resolution will not only prolong survival and reduce mortality but also improve quality of life while reducing significant health care costs due to recurring hospitalizations.
囊性纤维化（CF）是一种由囊性纤维化跨膜传导调节因子（CFTR）突变引起的遗传性疾病，其中反复加重导致的肺功能进行性下降是主要死因。CF患者慢性阻塞性肺疾病的发病涉及炎症和病情加重，进而导致黏液阻塞和肺功能下降。尽管CF肺部疾病的临床管理延长了患者的生存期，但病情加重和与年龄相关的肺功能下降仍是控制进行性肺部疾病的一大挑战。解决进行性肺部疾病的关键在于基于预后的早期治疗干预；因此，开发用于预测病情加重和肺功能下降的新型诊断和预后生物标志物将有助于对肺部疾病进行优化管理。因此，开发诸如强迫振荡技术（FOT）、脉冲振荡法系统（IOS）和电阻抗断层成像（EIT）等实时肺功能诊断方法，以及基于新型预后的干预策略来控制慢性阻塞性肺疾病的进展，将满足CF患者一项重大的未满足需求。早期检测CF肺部炎症并及时解决病情加重问题，不仅能延长生存期和降低死亡率，还能提高生活质量，同时减少因反复住院导致的巨额医疗费用。
