《Table 1 Main clinical features in patients with CF6and PCD7》

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《Chemoattractants and cytokines in primary ciliary dyskinesia and cystic fibrosis: key players in chronic respiratory diseases》

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Abbreviations:ABPA，allergic bronchopulmonary aspergillosis；CF，cystic fibrosis；CFRD，CF-related diabetes；PCD，primary ciliary dyskinesia；SA，situs ambiguus；SI，situs inversus.

The coordinated movement of motile cilia on the lung epithelium is responsible for the upward transportation of lung mucus，wherein pathogens and harmful particles are captured.This process is called mucociliary clearance and is essential for keeping the lungs infection free.1Primary ciliary dyskinesia（PCD）and cystic fibrosis（CF）are both rare，autosomal-inherited disorders associated with impaired mucociliary clearance.2，3Although both diseases have a different genetic and functional origin，they share common features such as chronic respiratory infections characterized by excessive neutrophil influx.Indeed，high neutrophil counts and elevated neutrophil chemoattractant levels，for example，C-X-C motif chemokine ligand 8/interleukin-8（CXCL8/IL-8），are present in the lungs of PCD and CF patients，even during stable conditions.4，5Despite the high neutrophil numbers and CXCL8/IL-8 levels，these patients suffer from persistent bacterial colonization and uncontrolled inflammation.3An overview of the most common clinical features in CF and PCD is listed in Table 1.