Polymorphonuclear neutrophil function in Kartagener's syndrome--report of two cases

The clinical triad of Kartagener's syndrome (KS) includes situs inversus, sinusitis and bronchiectasis or at least chronic bronchitis. Immotile cilia with various ultrastructural defects have been seen in KS. Because the enhanced susceptibility to infections seen in KS it had been suspected due to significant abnormality of neutrophil function in addition to a defect of ciliary function. In this report, neutrophil functions such as PMN phagocytosis, PMN chemotaxis, Migration inhibition test and PMN bacterial killing test were studied in two cases of KS at a time when they were free of infection. The defects observed by us were only slight. We conclude that although neutrophil motility of patients is normal, the bactericidal activity is depressed in the early stage. Whether this abnormality enhances patient susceptibility to bacterial infections is not yet clear. The significance and actural mechanism for his delayed killing are now under investigation.