PATHOGENESIS OF OBSTRUCTIVE LUNG-DISEASE - STUDY ON IMMOTILE-CILIA SYNDROME

  • 1 January 1978
    • journal article
    • research article
    • Vol. 59  (2) , 55-65
Abstract
The role of mucociliary transport in man can be evaluated by studying persons with the recently recognized immotile-cilia syndrome. Such persons have chronic or recurrent infections of the upper and lower airways and have ultrastructural defects of cilia and sperm tails. Men suffering from the syndrome usually are sterile and have immotile spermatozoa. About half of the subjects have complete situs inversus. Persons (14) with the immotile-cilia syndrome were studied here in an attempt to evaluate the role of an impaired mucociliary transport for the development of obstructive lung disease. Age range was 25-40 yr. There were 10 men and 4 women. Mucociliary transport in the lungs was extremely slow in all subjects. Spirometry demonstrated airway obstruction in 9 subjects. Two of these subjects had radiological evidence of pulmonary emphysema. An absent mucociliary transport possibly predisposes to the development of obstructive lung disease. Patients with classic chronic bronchitis earlier were shown to have a severely impaired mucociliary transport. The present findings in subjects with primary ciliary immotility make it probable that this impaired mucociliary transport is of pathogenetic importance for development of the obstructive lung disease often found in patients with chronic bronchitis.