Cystic fibrosis (CF) is a multisystem disorder, but progressive inflammatory lung disease causes the greatest burden of morbidity and death. Recent translational and mechanistic studies of samples from patients, and observations in animal models, indicate that platelets may drive lung injury and contribute to dysregulated host defense in CF lung disease. In this issue of the JCI, Ortiz-Muñoz and Yu et al. explored the role that the cystic fibrosis transmembrane conductance regulator (CFTR) plays in platelet-related inflammation. The authors used mouse and human model systems to show that CFTR dysfunction in platelets increased calcium entry though the transient receptor potential cation channel 6 (TRPC6), causing hyperactivation and consequent experimental lung inflammation. The study persuasively suggests that platelets are critical thromboinflammatory effector cells in CF lung disease. In the context of platelet-related organ injury seen in a variety of other diseases and syndromes, platelets may also contribute to nonpulmonary manifestations and comorbidities of CF.

囊性纤维化（CF）是一种多系统疾病，但是进行性炎症性肺病会导致最大的发病和死亡负担。最近对患者样品的转化和机理研究以及在动物模型中的观察结果表明，血小板可能会导致肺部损伤，并导致CF肺部疾病的宿主防御功能失调。在JCI的这一期中，Ortiz-Muñoz和Yu等人。探索了囊性纤维化跨膜电导调节剂（CFTR）在血小板相关炎症中的作用。作者使用小鼠和人类模型系统显示，血小板中的CFTR功能障碍会通过瞬时受体电位阳离子通道6（TRPC6）增加钙的进入，从而引起过度活化和随后的实验性肺部炎症。这项研究有说服力地表明，血小板是CF肺部疾病的关键血栓炎症效应细胞。在其他多种疾病和综合征中发现的血小板相关器官损伤的情况下，血小板也可能导致CF的非肺部表现和合并症。