Repeated respiratory tract infections, sinusitis,
persistent rhinorrhea, otitis media with hearing loss, male infertility.
Bronchiectasis may develop in infancy and is common in early adult life
secondary to recurrent pneumonia. Lung function shows an obstructive
pattern, which may become restrictive later. Pulmonary hypertension and
right ventricular dysfunction develop in later stages of the disease.
Abnormal neutrophil migration has been demonstrated in vitro; however, its
clinical significance is unknown.