+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Cystic fibrosis compared with the immotile-cilia syndrome. A study of mucociliary clearance, ciliary ultrastructure, clinical picture and ventilatory function

Cystic fibrosis compared with the immotile-cilia syndrome. A study of mucociliary clearance, ciliary ultrastructure, clinical picture and ventilatory function

Scandinavian Journal of Respiratory Diseases 59(6): 297-306

Patients with cystic fibrosis (CF) were investigated for mucociliary clearance (with and without stimulation by terbutaline), clinical picture, ventilatory function and ultrastructure of cilia. The results were compared with those of patients with congenitally immotile cilia (immotile-cilia syndrome). Mucociliary clearance could be demonstrated in all the seven CF patients who succeeded in inhaling the test aerosol. Ciliary ultrastructure from a deceased CF patient was normal. Patients with the immotile cilia syndrome had no substantial clearance and defective cilia. The CF patients coughed more during the clearance measurements than any other group studied earlier, and their coughing was effective. One patient succeeded in avoiding coughing in both measurements and had faster clearance when he got terbutaline than when he got the vehicle. Although younger, the CF patients tended to be more obstructed in their lungs and more handicapped than the patients suffering from the immotile-cilia syndrome. The latter patients had more discomfort from rhinitis, sinusitis and otitis than had the CF patients. An impairment of the mucociliary transport rate is hence unlikely to be a primary pathogenic factor for the respiratory tract disease in CF patients.

Please choose payment method:

(PDF emailed within 1 workday: $29.90)

Accession: 039727383

Download citation: RISBibTeXText

PMID: 373089

Related references

Immotile cilia syndrome: nasal mucociliary function and nasal ciliary abnormalities. Rhinology 32(3): 109-111, 1994

Nasal mucociliary clearance and ciliary beat frequency in cystic fibrosis compared with sinusitis and bronchiectasis. Thorax 36(9): 654-658, 1981

Mucociliary clearance in obstructive lung diseases. Correlations to the immotile cilia syndrome. European Journal of Respiratory Diseases. Supplement 146: 295-301, 1986

Acquired ciliary defects compared to those seen in the immotile-cilia syndrome. European Journal of Respiratory Diseases. Supplement 127: 5, 1983

Nasal mucociliary transport and ciliary ultrastructure in cystic fibrosis. A comparative study with healthy volunteers. International Journal of Pediatric Otorhinolaryngology 40(1): 27-34, 1997

Cystic fibrosis mutations and immotile cilia syndrome. Clinical Genetics. 47(6): 328-329, 1995

How useful is the study of cilia ultrastructure of the respiratory tract for the diagnosis of a so called immotile cilia syndrome. Acta Anatomica 120(4): 241, 1984

Mucociliary and long-term particle clearance in airways of patients with immotile cilia. Respiratory Research 7: 10, 2006

Recurrent respiratory disease, azoospermia, and nasal polyposis. A syndrome that mimics cystic fibrosis and immotile cilia syndrome. Archives of Internal Medicine 145(12): 2201-2203, 1985

In vitro nasal ciliary motility and peripheral blood neutrophil function in immotile cilia syndrome. Clinical Research 32(1): 132A, 1984

A morphological study of ciliary abnormalities in patients with immotile cilia syndrome. La Pediatria Medica e Chirurgica 17(5): 411-415, 1995

Mucociliary function, ciliary ultrastructure, and ciliary orientation in Young's syndrome. Thorax 47(3): 184-187, 1992

Kartagener's syndrome with motile cilia and immotile spermatozoa: axonemal ultrastructure and function. American Review of Respiratory Disease 134(6): 1233-1236, 1986

The dyskinetic cilia syndrome. Ciliary motility in immotile cilia syndrome. Chest 78(4): 580-582, 1980

Congenital defects in the ultrastructure of respiratory cilia in the immotile cilia syndrome. Clinical Research 26(6): 818A, 1978