Division of Pulmonary and Critical Care Medicine, University of North Carolina at Chapel Hill, 130 Mason Farm Road, CB#7020, Chapel Hill, NC 27599-7020, United States.
Curr Opin Pharmacol. 2010 Jun;10(3):294-9. doi: 10.1016/j.coph.2010.04.003. Epub 2010 May 1.
Mucociliary clearance (MCC) in CF lung disease is limited by airway dehydration, leading to persistent bacterial infection and inflammation in the airways. Agents designed to rehydrate the airway mucosa lead to improved MCC. Hyperosmolar agents, such as hypertonic saline and mannitol, create a luminal osmotic gradient, drawing water into the dehydrated ASL. Ion transport modulators function to activate alternative chloride channels and/or to block sodium hyperabsorption that occurs through a dysregulated ENaC channel. Combinations of these therapies may result in a synergistic improvement in airway hydration, and thus, restore MCC. Active ongoing phase II and III trials of new pharmacotherapeutics are covered in this review.
在 CF 肺部疾病中,黏液纤毛清除(MCC)受到气道脱水的限制,导致气道中持续存在细菌感染和炎症。旨在使气道黏膜再水合的药物可导致 MCC 改善。高渗剂,如高渗盐水和甘露醇,在气道上皮表面形成渗透梯度,使水分进入脱水的表面活性物质层。离子转运调节剂的作用是激活替代的氯离子通道,或阻断通过失调的 ENaC 通道发生的钠过度吸收。这些治疗方法的联合可能会协同改善气道水合作用,从而恢复 MCC。本综述涵盖了新的药物治疗的正在进行的 II 期和 III 期临床试验。