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Results for “Ciliary Motility Disorders”

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PubMed1989

[A case of immotile cilia syndrome and a review of Japanese cases].

A case of immotile cilia syndrome (ICS) is presented. A 34-year-old male, who had suffered from recurrent bronchitis, sinusitis and otitis media since early childhood, was admitted to Keio University Hospital complaining of productive cough and infertility. A saccharin test showed prolonged nasal clearance time, and semen analysis revealed immotile sperm. By electron microscopic observation of cilia of the nasal mucous epithelium and the sperm, inner and outer dynein arm defect, abnormal microtubular arrangement and compound cilia were detected and he was diagnosed as ICS. Thirty eight Japanese cases of ICS collected from the literature were analyzed concerning clinical manifestations, airway clearance, family history and ciliary ultrastructural abnormalities (Table 2, 3). Recurrent bronchitis, male infertility, chronic sinusitis, otitis and decrease in airway clearance were very common in these patients. Female infertility was more common than anticipated. The prevalence rate of situs inversus was more than 50%, probably due to more attention having been paid to Kartagener's syndrome in Japan. Recent studies show that the cilia of this syndrome is not always immotile but characterized by hypomotility or asynchrony, and have attempted to determine the relationship between each ciliary ultrastructural abnormality and motility pattern. It is necessary that more patients including incomplete and mild cases should be investigated.

Adult↗
PubMed1989

Physiology and pathology of the mucociliary system. Special regards to mucociliary transport in malignant lesions of the human larynx.

After a short historical introduction, the current state of knowledge of the anatomy and physiology of the mucociliary system is reviewed. Description and discussion of the clinical and experimental methods of investigation and measurement of the mucociliary transport and of the ciliary activity then follow. Light and electron microscopy are also considered. Next, the pathology, pathophysiology and pharmacology of the mucociliary system are reported. The introduction ends with the consideration of specific diseases related to the mucociliary system, such as the immotile cilia syndrome. The main interest of this investigation is directed to changes in the ciliary epithelium in laryngeal malignancies. Therefore the literature relevant to physiology is critically evaluated. Pilot studies of the method used for investigating the physiology of the ciliary epithelium were mainly undertaken on the more accessible nasal mucosa as a model for ciliary epithelium. The use of the saccharin test for demonstration of nasal mucociliary transport is thoroughly assessed and the suitability of a new, improved, marking substance was tested. The quantification of cell types for cytological assessment of nasal smears under the phase contrast microscope was appraised. The method of determination of ciliary frequency by microphotometry was evaluated critically by a series of experiments. The author's electron microscopic findings on ciliary epithelium taken from human larynges affected with cancer are also discussed. After these pilot studies the method of the saccharin test and the cytological smear appeared to be unsuitable for oncological investigations. Therefore, mucociliary transport in the subglottic space of 75 human larynges excised for malignancy was investigated by the development of a suitable method. This method demonstrated abnormal mucociliary transport pathways. Investigations of the normal pathway of mucociliary transport in the larynx were obtained in animal experiments and by endoscopic investigations in man.

Animals↗
PubMed1989

[Chronic dysplasia of the respiratory ciliary epithelium].

We report on a 14-year-old Turkish boy who suffered for more than seven years from recurrent pneumonia and bronchiectases. This led to the surgical removal of the left lower lobe. Brush biopsies taken from the bronchus and nose revealed severe dysmorphy of the ciliary epithelium with an altered cell-distribution of the normal surface pattern.

Bronchi↗
PubMedDate not supplied

[Clinical syndromes in congenital defects and developmental anomalies of the nasal cavity].

Clinical syndromes that accompany congenital diseases and developmental abnormalities of the nasal cavity are presented. The data are derived from clinical and functional investigations (rhinospirometry, olfactometry, rhinofibroscopy, rhinomicroscopy, examination of the motor function on the ciliated epithelium, etc) of 160 patients with the above pathologies. Based on the examinations, the following clinical syndromes can be identified: nasal asymmetry, nasal obstruction, respiratory, olfactory, mucociliary and reflex syndromes. It is concluded that further investigations in th s direction are needed and that the syndrome approach is valuable from the diagnostic point of view.

Adolescent↗
PubMed1989

[Clinical and functional aspects of endonasal operation of the maxillary sinuses].

A total of 118 detailed questionnaires sent out to patients 3-5 years after endoscopic endonasal maxillary sinus surgery were evaluated. Forty-four patients (37.3%) were re-examined endoscopically. The procedure involves no complications, and 87% of the patients judged the endoscopic treatment of chronic maxillary sinusitis to have been successful. Supraturbinal antral windows remained patent, and a higher percentage were functionally intact compared with infraturbinal ones. The mucociliary pathways of the maxillary sinus via the antral window were restored postoperatively.

Ciliary Motility Disorders↗
PubMed1989

[Effect of warm air inhalation on the nasal ciliated epithelium].

It is known from in-vitro-studies, that temperatures exceeding 45 degrees C can damage the function and structure of the nasal cilia irreversibly. We tested 30 healthy volunteers by inhalation therapy, exposed to moist vapor over a 10 min period. The ciliary function was assessed by vital cytological sampling, and included measurement of ciliary beat frequency. The beat frequency increased during inhalation therapy, but the number of viable ciliated cells did not change significantly. Thus warm air inhalation therapy does not damage the nasal ciliated epithelium, probably due to the air conditioning capacity of the nose.

Adolescent↗
PubMed1989

[Malignant lymphoma of the central nervous system in a boy with immotile cilia syndrome].

Malignant lymphoma of the central nervous system in a thirteen-year-old boy with immotile cilia syndrome (ICS) is reported. He had frequent upper respiratory tract infections, chronic sinusitis and pneumonia during in childhood. Bronchiectasis was demonstrated by bronchography. The diagnosis of ICS was confirmed by the lack of dynein arms of cila in the nasal mucosa with electronmicroscopy. In 1987, he complained of headache and vomiting and a space occupied mass lesion in the left frontoparietal lobe was found by head CT scan, which was subtotally resected. Histological studies showed large cell type non-Hodgikin lymphoma of B-cell phenotype. He received radiotherapy (41Gy) to the whole brain and systemic chemotherapy consisting of adriamycin, cyclophosphamide, vincristine, prednisolone, L-asparaginase and intrathecal methotrexate, and the patient maintained complete remission for eight months. However, relapse occurred and the patient died twelve months after the initiation of treatment.

Adolescent↗
PubMed1989

[Effect of antitubercular preparations on the function of the ciliary epithelium of the respiratory tract mucosa].

The effect of various concentrations of streptomycin, isoniazid, rifampicin and ethambutol on the function of the siliated epithelium of the frog oral mucosa was studied (110 experiments with 50 frogs). The level of inhibition of the siliated epithelium function depended on the properties of the drugs and their concentration in solution. More pronounced inhibition was induced by 15 per cent ethambutol solution and 10 per cent isoniazid solution. The least inhibition of the siliated epithelium function was observed with the use of 6.25 per cent streptomycin solution, 5 per cent isoniazid solution, 7.5 per cent ethambutol solution and 3.75 per cent rifampicin solution.

Animals↗
PubMed1985

Ultrastructural abnormalities of respiratory cilia. Descriptive and quantitative study of respiratory mucosa in a series of 33 patients.

Ultrastructural abnormalities of human respiratory tract cilia have been studied in 33 patients: 21 were adults, 18 with chronic bronchitis, and 12 were children, two with situs inversus. Abnormalities, such as the lack of a few dynein arms or the loss of a peripheral doublet were observed quite frequently in both children and adults. However, congenital abnormalities associated with the "immotile cilia syndrome" were rare and were observed in only three of the children and none of the adults.

Adolescent↗
PubMed1986

[Electron microscopy studies of cilia of the bronchial tree in children with chronic bronchopulmonary diseases].

The results of an ultrastructural analysis of cilia in 30 children at the age of 11 month to 16 years are presented. Children with marked symptoms of chronic bronchitis were consciously chosen for examination. One group of 15 children was suffering from chronic bronchitis with bronchiectases. No bronchiectasis was found in the second group of 15 children with chronic bronchitis. Specific changes were more evident in the first group, whereas nonspecific changes were of equal frequency in both groups. Only in 3 patients of group I generalized specific defects could be demonstrated. In these cases mucociliary clearance was nearly not present. Ultrastructural changes of cilia in children with chronic nonspecific lung diseases enclose a wide spectrum. Compared to it generalized structural defects are rare and only in 3 cases of our study demonstrable (negative selection of a bronchological centre).

Adolescent↗
PubMed1988

Primary ciliary dyskinesia: cytological and clinical features.

Thirty patients with functional and/or morphological abnormalities of respiratory tract cilia were identified. The diagnosis of primary ciliary dyskinesia was based on observed abnormalities of ciliary ultrastructure or beating in vitro (beat pattern, beat frequency or percentage of motile cilia). Beat frequency and motility indices approached the normal range in some cases and suggests that the term 'immotile cilia syndrome' is not appropriate. Morphological abnormalities were most commonly due to deficiency of dynein arms, affecting the outer arms (n = 7), inner arms (n = 3) or both (n = 10). Examples of radial spoke and microtubular defects were also identified but in seven subjects ciliary ultrastructure was normal. In six patients paired samples of nasal and bronchial cilia were obtained and showed consistent abnormalities of motility and ultrastructure. Adenosine triphosphate and adenosine triphosphatase did not restore in vitro motility when added to dynein deficient cilia. The clinical picture was of life-long sinusitis and recurrent bronchial infection but the spectrum was broader than that encompassed by Kartagener's triad (dextrocardia, sinusitis and bronchiectasis). Fourteen patients had normal cardiac situs and definite or highly suggestive evidence of bronchiectasis was present in only 17 patients. Radiological evidence of sinusitis was common but absence of frontal sinuses was not universal. Chronic serous otitis media was a frequent finding but deafness was rarely profound. Fertility problems were common but were not universal in female subjects. Lung function testing revealed evidence of airflow obstruction but this was mild in most cases.

Adenosine Triphosphatases↗
PubMed1988

Ciliary defects: cell biology and clinical perspectives.

Considerable progress has been made in achieving a perspective of the pathophysiology of ciliary defects in human disease in the interval between Siewert's and Kartagener's early descriptions of KS. Not only have we achieved a better understanding of some of the mechanisms involved in the pathogenesis of congenital ciliary syndromes, but also we have come to appreciate a new spectrum of ciliary defects, those of acquired etiology. The advent of modern electron microscopy has been a significant element to this progress and its importance is reflected by the numerous studies of ciliary defects that have come in the wake of the pioneering ultrastructural investigations of Afzelius and colleagues. However, each discovery appears to generate additional questions. This is indicative of the importance of the subject to both basic and clinical science and of the vitality of the investigators in this field as a comprehensive understanding of ciliary diseases is sought.

Adult↗