The effect of coughing on the elimination of inhaled 6 micrometer radioactively tagged teflon particles in humans was studied by external measurements of the radioactivity retained in the lungs before and after 1--2 min of voluntary coughing. In six healthy subjects coughing produced no substantial elimination of the particles. Six out of eight patients with lung disease produced expectorate and also eliminated particles from the lungs by coughing. The other two patients had no phlegm, did not produce any expectorate and did not eliminate particles by coughing. An increased amount of tracheobronchial secretion thus seems to be necessary for coughing to be effective. In the patients, the elimination of particles by coughing was fairly reproducible, suggesting that the test model may be useful for investigation of the influence of physiological and pharmacological factors on the elimination process.

Adult↗

PubMed1978

Kartagener's syndrome does exist.

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Cell Movement↗

PubMed1978

[Documentation on bromhexine: What is the truth?].

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Bromhexine↗

PubMed1978

On the function of cilia in the female reproductive tract.

A recently discovered syndrome is characterized by congenital immotility of the cilia. It consists of chronic infections in the respiratory system, male infertility, and, in about one-half of the cases, situs inversus. The syndrome thus includes Kartagener's syndrome. The syndrome provides a unique opportunity to gain insight into the role of the cilia in the human body. Five women evidently suffering from this syndrome were examined. They had no gynecologic disorders. Three of the five women had tried to become pregnant and two have suceeded. A review of the literature indicates that men with Kartagener's syndrome are usually infertile, whereas the women are not. This fact and the data presented herein constitute strong evidence that ciliary motility is not essential for female fertility. More conclusive proof could be obtained if any of the affected women would consent to ultrastructural investigation of the oviductal cilia.

Adolescent↗

PubMed1978

Tracheobronchial clearance in patients with emphysema associated with alpha1-antitrypsin deficiency.

Tracheobronchial clearance was studied in five patients who had emphysema associated with deficiency of serum alpha1-antitrypsin but no history of chronic bronchitis. After the patients had inhaled an aerosol of 6 micrometer teflon particles tagged with 99mTc, the radioactivity in the lungs was followed externally during 2 h. Clearance in the emphysematous patients was normal or even rapid as compared with clearance in healthy subjects, and was significantly more rapid than clearance in patients with a history of chronic bronchitis. The results indicate that emphysema can develop in patients with alpha1-antitrypsin deficiency without their having an impairment of mucociliary transport.

Aged↗

PubMed1978

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

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.

Adult↗

PubMed1978

On the pathogenesis of obstructive lung disease. A study on the immotile-cilia syndrome.

The role of mucociliary transport in man can be evaluated by studying persons with the recently recognised "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. Fourteen persons with the immotile-cilia syndrome have been 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 years; there were ten men and four women. Mucociliary transport in the lungs was extremely slow in all subjects. Spirometry demonstrated airway obstruction in nine subjects. Two of these subjects had radiological evidence of pulmonary emphysema. The results indicate that an absent mucociliary transport predisposes to the development of obstructive lung disease. Patients with classic chronic bronchitis have earlier been 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.

Adult↗

PubMed1977

The immotile-cilia syndrome. A congenital ciliary abnormality as an etiologic factor in chronic airway infections and male sterility.

We investigated six men and a woman suspected of suffering from congenital immotility of cilia. All had chronic airway infections, and the men had immotile spermatozoa. The woman and three men had Kartagener's syndrome. The investigations included measurements of the mucociliary transport in the lower airways and ultrastructural studies of the sperm tails or respiratory cilia (or both). Mucociliary transport was significantly delayed. Sperm tails lacked dynein arms in five patients. Respiratory cilia from the women and two men lacked dynein arms and were irregularly oriented. The results support the hypothesis that a congenital defect in the cilia and sperm tails will cause chronic respiratory-tract infections and male sterility--the immotile-cilia syndrome. In about half these patients there will also be a situs inversus--i.e., Kartagener's syndrome.

Adult↗