1 Department of Medicine.
2 Gregory Fleming James Cystic Fibrosis Research Center.
Ann Am Thorac Soc. 2018 Nov;15(Suppl 3):S177-S183. doi: 10.1513/AnnalsATS.201806-407AW.
The airway surface functional microanatomy, including the ciliated airway epithelium and overlying mucus layer, is a critical component of the mucociliary escalator apparatus, an innate immune defense that helps to maintain a clean environment in the respiratory tract. Many genetic and acquired respiratory diseases have underlying pathophysiological mechanisms in which constituents of the airway surface functional microanatomy are defective. For example, in cystic fibrosis, mutations in the cystic fibrosis transmembrane conductance regulator gene, which normally produces a secretory anion channel protein, result in defective anion secretion and consequent dehydrated and acidic mucosal layer overlying the airway epithelium. This thick, viscous mucus results in depressed ciliary beating and delayed mucociliary transport, trapping bacteria and other pathogens, compromising host defenses and ultimately propagating disease progression. Thus, developing tools capable of studying the airway surface microanatomy has been critical to better understanding key pathophysiological mechanisms, and may become useful tools to monitor treatment outcomes. Here, we discuss functional imaging tools to study the airway surface functional microanatomy, and how their application has contributed to an improved understanding of airway disease pathophysiology.
气道表面功能微观结构，包括纤毛气道上皮和覆盖的黏液层，是黏液纤毛清除装置的一个关键组成部分，该装置是一种先天免疫防御机制，有助于维持呼吸道的清洁环境。许多遗传性和获得性呼吸道疾病的潜在病理生理机制是气道表面功能微观结构的组成部分有缺陷。例如，在囊性纤维化中，囊性纤维化跨膜电导调节基因的突变，通常会产生一种分泌阴离子通道蛋白，导致阴离子分泌缺陷，继而导致气道上皮上方的黏膜层脱水和呈酸性。这种厚而粘稠的黏液导致纤毛摆动减弱和黏液纤毛转运延迟，使细菌和其他病原体被捕获，宿主防御功能受损，最终导致疾病进展。因此，开发能够研究气道表面微观结构的工具对于更好地理解关键病理生理机制至关重要，并且可能成为监测治疗效果的有用工具。在这里，我们讨论了用于研究气道表面功能微观结构的功能成像工具，以及它们的应用如何有助于改善对气道疾病病理生理学的理解。
