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[Ciliary function in bronchopulmonary infections in childhood].

It was the aim of this study to examine the influence of bacterial or viral infections of the airways on the ciliary beat rate in childhood. In 21 children with bacterial bronchopulmonary infections a mean ciliary beat rate of 9.1 +/- 2.4. Hz was found that did not differ significantly from that of the group of the healthy subjects (9.9 +/- 1 Hz). In 7 of the 21 patients we could identify an infection of the respiratory tract with Haemophilus influenzae; in those children there was a marked reduction of the mean ciliary beat rate at 8 Hz. 13 children with viral bronchopulmonary infections had a mean ciliary beat rate of 11.8 +/- 1.8 Hz, which is significantly enhanced when compared with that of the healthy group. Compared with the mean ciliary beat rate of bacterial infections of the respiratory tract there is a significant difference. In viral infections of the airways no value below 9 Hz was found. In case of markedly reduced ciliary beat rate a bacterial infection must be assumed.

Adolescent↗

[Mucociliary transport in patients with pulmonary tuberculosis complicated by bronchial obstruction].

The examination of 98 patients with newly diagnosed destructive pulmonary tuberculosis was undertaken to find the relation between the condition of the bronchial tree, level of obstruction and degree of mucociliary insufficiency. The latter was shown to depend on the condition of the bronchial free and was most manifest in diffuse endobronchitis. At the same time, the degree and level of obstruction of the bronchial tree also had influence on the rate of mucociliary transport.

Adult↗

[Serous otitis and ciliary dyskinesia syndrome].

Ciliary dyskinesia syndrome or immotile cilia syndrome, is a congenital defect in the ultrastructure of cilia, which result in a clinical expression diverse: recurrent respiratory infections, recurrent otitis media, infertility in the adult male, and half of those situs inversus. A case of ciliary dyskinesia syndrome is presented, who suffered of recurrent pneumonia and relapsing secretory otitis media.

Adenoidectomy↗

[A case of immotile cilia syndrome accompanied by retinitis pigmentosa].

A case of immotile cilia syndrome accompanied by retinitis pigmentosa is reported. This syndrome involves congenital ciliary ultrastructural abnormality. A 27-year-old male complained of repeated pneumonia, sinusitis, and middle otitis. In addition, he had sperm motor insufficiency and electron microscopic finding of cilia led to the diagnosis of the present syndrome. Both fundi presented remarkable degeneration of retinal pigment epithelium and choroid and marked arterial narrowing. Constriction of the visual field and extinguished ERG were also noted. Abnormality of cilia of the retinal pigment epithelium was suggested. It was proposed that retinitis pigmentosa may be caused by abnormal cilia of the retinal pigment epithelium.

Adult↗

The reversibility of impaired mucociliary function after lung transplantation.

Impairment of mucociliary function occurs after lung transplantation and may predispose patients to repeated pulmonary infections. The purpose of this study is to determine whether and how soon such mucociliary function may recover. Ten dogs underwent left lung autotransplantation. Within 3 weeks five of these dogs underwent study for proximal airway clearance by observation through a bronchoscope of the movement of carbon particles placed at different locations on the tracheobronchial mucosa. The mechanical properties of collected mucus from specific sites were determined by magnetic rheometry. The right lung, which was not operated on, served as a paired control. Similar studies were conducted in the remaining five dogs at 12 weeks after autotransplantation. Lung autotransplantation caused significant depression of proximal airway clearance and a 35% increase in mucous rigidity (p = 0.05) soon after operation. At 12 weeks after operation, there was a partial recovery of proximal airway clearance. Mucous changes were no longer consistent. Histologic and electron microscopic examinations initially revealed focal denudation of ciliated cells and loss of the bronchial glands. At 12 weeks there was a regeneration of cilia and a reappearance of the bronchial glands. We conclude that the mucociliary function, observed to be depressed early after lung autotransplantation, recovers partially during the late postoperative period. Thus the mucociliary functional recovery should be attributed to revascularization rather than to reinnervation, since the latter is unlikely to occur during this period.

Anastomosis, Surgical↗

Ultrastructure of respiratory cilia of WIC-Hyd male rats. An animal model for human immotile cilia syndrome.

The WIC-Hyd rat is a mutant from the Csk: Wistar-Imamichi rat, with spontaneous hydrocephalus. In male rats, the hydrocephalus is severe and about one half of hydrocephalic male littermates possess situs inversus totalis. Ependymal cilia in these animals are immotile, and this defect is regarded as a mechanical cause of hydrocephalus. This paper presents the ultrastructural features of respiratory cilia in these rats in comparison with those in human immotile cilia syndrome. The respiratory cilia in these rats also are immotile and the dynein arms are missing, as in human cases. Previously only eight dogs with immotile cilia syndrome and a mutant hydrocephalic-polydactyl mouse were reported with respect to these phenomena. However the WIC-Hyd rat is the first useful animal model for human immotile cilia syndrome, and further studies may serve to clarify the genetic background of this condition.

Animals↗

Kartagener's syndrome.

Kartagener's syndrome is characterized by situs inversus, chronic paranasal sinusitis and bronchiectasis. The pathogenesis of this rare condition is an ultrastructural defect that results in immotile cilia. Radiologic features may include findings consistent with bronchiectasis.

Abnormalities, Multiple↗

Primary ciliary dyskinesia in pigs.

The first description of a familial immotile cilia syndrome diagnosed through ovario-hysterectomy in six siblings of pigs has been performed. This report may indicate another possible cause of reproductive failure in domestic animals. In fact, the immotile cilia syndrome has not been considered from this point of view in veterinary medicine. This case emphasizes that a study about ciliary motility in the female reproductive tract in domestic animals is absolutely necessary to improve current knowledge about different causes of infertility. Up to date, it remains unknown what degree of ciliary motility is necessary for mammalian fertility in females.

Animals↗

[The immotile cilia syndrome. A case report].

A case of congenital bronchiectasis associated with hypoplasia [correction of hyperplasia] of maxillary sinuses and sinusitis in a 3-year-old boy is presented. The ultramicroscopic examination of nasal mucosa showed abnormalities in the ultrastructure of the mucociliary system (spare cilia, absence of basal corpuscles, cytoplasmatic projections) clearly suggesting the ciliary immotility syndrome.

Bronchiectasis↗

[Diagnostic procedures in primary ciliary dyskinesia. The usefulness of nasal biopsy].

Eight patients are studied who are suspected to have primary ciliary dyskinesia (PCD). All cases presented from the first year of live repetitive respiratory infections, chronic cough, mucopurulent rhinorrhea, radiologic signs of sinusitis and one patient also presented situs inversus. Bronchiectasis were found in four cases, they were discarded in two cases, and in two other cases they could not be found nor discarded. The definite diagnosis was achieved by the study of the ultrastructure of the cilia by nasal biopsy. In three cases, nasal biopsy discarded the diagnosis of PCD and confirmed such diagnosis in other three cases. One case of PCD was diagnosed by a bronchial biopsy after two unsuccessful attempts to obtain a nasal sample containing ciliary epithelium. One case remains undiagnosed since after a nonvalid biopsy, we did not consider necessary to obtain another one given that the patient was asymptomatic during the last three years.

Adolescent↗