Neutrophil function in the immotile cilia syndrome

Patients with ICS have a microtubular abnormality of cilia, which is thought to contribute to frequency respiratory tract infections. Neutrophils have a cytoplasmic microtubular system that, if abnormal and malfunctioning, could also contribute to recurrent infections. Neutrophil function has been assessed in 14 patients with ICS and compared with that of controls. The ICS patients included those with the dynein defect, the radial spoke defect, and microtubular transposition. Cell migration in each patient has been examined under agarose and by Boyden chamber techniques. No significant abnormality was detected in random or directed migration of neutrophils. Six patients representing the three different types of abnormal cilia had more extensive studies of neutrophil function performed. These included assessments of bacterial phagocytosis and killing, lysosomal degranulation, and oxidative pathway activity. Phagocytosis and killing of Staphylococcus aureus 502A in vitro was normal. Hexose monophosphate pathway activity, tetrazolium dye reduction, and lysosomal degranulation by neutrophils at rest and during phagocytosis were similar to those of control neutrophils. Our findings suggest that an abnormality of neutrophil function does not play an important role in the respiratory infections of patients with ICS.