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Results for “Ciliary Motility Disorders”

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At least 631 records · Page 35Linked to original sources
PubMed1994

A seven-year-old boy with sinusitis, otitis media, and asthma.

A 7-year-old boy with recurrent otitis media, bronchitis, pneumonia, asthma, and sinusitis was found to have primary ciliary dyskinesia. It was important to rule out other systemic diseases such as immune deficiency and cystic fibrosis. Electron microscopy of a properly obtained and prepared biopsy of the mucosal surface of the nose, trachea, or bronchus is essential.

Anti-Bacterial Agents↗
PubMed1993

Ultrastructural defects of the ciliary epithelium in a child with Kartagener's syndrome.

Kartagener's syndrome is a well known classical triad of presentations consisting of bronchiectasis, sinusitis and situs inversus. It is now recognized that the syndrome is an extreme presentation of primary ciliary dyskinesia, a large group of conditions with ultrastructural ciliary defects, leading to poor ciliary motility in various organ systems. A case of Kartagener's syndrome is presented in an eight year old Thai boy in whom the ultrastructural ciliary defects have been examined and described in detail for the first time in Thailand. Incomplete lack of dynein arms was recognized. In addition, disorientation of ciliary axis was noticed. Due to severe bronchiectatic changes of the right lower lobe and right lingular lobe which did not improve despite adequate antibiotics, these lobes were surgically removed. The child has done well since, but still suffers occasional and recurrent bouts of sinusitis.

Child↗
PubMed1993

[Physicochemical properties of sputum from patients with immotile cilia syndrome].

Sputum samples were collected from 15 immotile cilia syndrome (ICS) cases, 12 diffuse panbronchiolitis (DPB) cases, and 11 bronchiectasis without ICS (BE) cases, during stable clinical state, to clarify the physicochemical properties of sputum from patients with ICS and to compare them with the properties of sputum from patients with DPB and BE. We measured sputum rheological properties and concentrations of several biochemical components. In ICS, spinnability was higher than that in DPB. No significant difference was seen between ICS and the other cases regarding other rheological properties. Albumin was lower, but fucose, sialic acid, and IgA were higher in ICS than in DPB. Although no significant difference was seen between ICS and BE, the sialic acid/albumin ratio was higher and the sialic acid/fucose ratio was lower in ICS than in BE. These results revealed that sputum from ICS cases was characterized by an absolutely or relatively increased mucus component with high spinnability and by a decreased extravascular transudate component. In view of these rheological properties, sputum from ICS cases was not always indicated to be associated with efficiency in cough clearance. The results suggested that chronic airway inflammation in ICS is not such a serious problem compared with DPB and BE.

Adolescent↗
PubMed1993

[Muco-ciliary transport in chronic inflammatory pathology of the middle ear].

The Authors have studied the behaviour of nasal mucociliary transport (MCT) in the chronic phlogistic pathology of the middle ear, in subjects suffering from simple and cholesteatomatous otitis. In both groups the duration of MCT was longer than a control group of normal subjects; instead no significant difference was observed either between the two kind of pathologies nor between the two different phases of the otitis (quiescence and breakthrough). The Authors conclude therefore that, in the recurrent phlogistic pathology of the middle ear, the function of MCT must be carefully studied, possibly corrected if damaged, to prevent the evolution in the breakthrough.

Adolescent↗
PubMed1994

Abnormal length of respiratory cilia in a pig. An ultrastructural study.

The authors report the first case of abnormal length of respiratory cilia in a domestic animal (18 microns versus normal length about 5 microns). These cilia lie on the carpet of cilia of normal length. The width of some of these cilia is also abnormal, measuring 0.5-0.6 microns. Other cilia have hook-shaped tips that could be responsible for an effective beat. We suggest that the ineffective ciliary beat could also be due to the undulating movements of abnormally long cilia, and stress the need for further morphological and biochemical studies of respiratory cilia in pigs. Respiratory pathology is a very common finding in pigs and is responsible for remarkable economical losses.

Animals↗
PubMedDate not supplied

[Primary ciliary dyskinesia: functional and morphological study].

We present 7 patients with a typical symptomatology of immotile cilia syndrome, three of them with complete situs inversus. Nasal mucociliary transport was studied by sero-albumin marked with technetium 99m. In all cases there was an absence of transport. The ultrastructure of the nasal cilia was studied. The findings were: In four cases alterations in the dynein arms, in three cases alterations in the central and peripheral microtubules associated por not to the defects in the dynein arms, and in one cases absence of cilia were observed. The ciliary complexes were common to all cases.

Adolescent↗
PubMed1993

[The circadian rhythm of ciliary beat frequency of human nasal cilia in probands with healthy lungs and in patients with chronic obstructive lung disease. Includes adrenergic stimulation by terbutaline].

Brush biopsies taken from the human nose eight times during 24 hours were examined under a phase contrast microscope and the ciliary beat frequency was measured directly by a photosensitive cell. Ten healthy volunteers showed in two series of measurements, at 600, 1200, 1800, 2400 initially and 300, 900, 1500, 2100 six weeks later, a marked decrease in ciliary beat frequency towards the middle of the day and a statistically higher beat frequency in the early morning. The influence of the adrenergic drug terbutaline led to a cilioexcitation ranging from 0.1-1.4 Hz (mean: 0.9 Hz). In contrast to, neither a circadian variation of the ciliary beat frequency nor a stimulation by terbutaline could be observed in a group of fifteen patients with chronic bronchitis or bronchiectasis at 600, 1200, 1800 and 2400.

Adult↗
PubMed1993

Immotile cilia syndrome in pigs. A model for human disease.

This paper describes the ultrastructural alterations observed in the tracheal epithelium of six sibling swine suffering from porcine immotile cilia syndrome (PICS) compared with those in human immotile cilia syndrome (HICS). As in some human cases, the tracheal epithelium of these pigs was lined by cilia-lacking cells. A variety of dynein defects in other pigs suffering from PICS have been previously observed. The spectrum of defects affords evidence that the PICS is genetically heterogeneous. Available data suggests that there are many similarities between HICS and PICS. Therefore, it is proposed that PICS may prove to be a useful animal model for the human disease.

Animals↗
PubMed1993

[Inhibitory effect on sulfur dioxide on ciliary motility in rabbit tracheal epithelium and its prevention by intracellular cyclic AMP].

To elucidate the role of endogeneous cyclic AMP in the protection against airway mucosal dysfunction induced by air pollutants, we studied the effect of sulfur dioxide (SO2) on ciliary motility in rabbit cultured tracheal epithelium in vitro. Exposure of cells to perfusate bubbled with SO2 rapidly decreased ciliary beat frequency (CBF), as assessed by a photoelectric method, from 971 +/- 12 to 718 +/- 28 beats/min by 3 ppm SO2 and from 963 +/- 22 to 635 +/- 34 beats/min by 10 ppm SO2 (p < 0.001, in each case). This effect was reversed by washing out the SO2-containing medium and was accompanied by a corresponding decrease in intracellular levels of cyclic AMP. Preincubation of the epithelial cells with salbutamol, vasoactive intestinal peptide, prostaglandin E2 or 3-isobutyl-1-methylxanthine increased cyclic AMP levels and inhibited the decreases in both CBF and cyclic AMP in response to the subsequent application of SO2 at 3 ppm, whereas dexamethasone had no effect. These results suggest that SO2 decreases airway ciliary motility through the reduction of intracellular cyclic AMP concentration, and that drugs that increase endogeneous cyclic AMP may prevent the SO2-induced impairment of mucociliary transport in the respiratory tract.

Animals↗
PubMed1996

[Primary ciliary dyskinesia, immotile cilia syndrome, and Kartagener syndrome: diagnostic criteria].

Primary ciliary dyskinesia is the generic term for a heterogeneous group of inherited diseases in which ciliary ultrastructure is defective and as a consequence ciliary motility is disturbed. An international consensus on the diagnostic criteria has not yet been reached. This paper reviews some recent findings which are useful in the diagnosis of the disease and attempts to establish the best diagnostic criteria. The marker symptoms are chronic bronchitis, otitis, and sinusitis since childhood. Additionally, one or more of the following criteria must be present: Kartagener syndrome, a dextrocardia situation, markedly reduced frequency in ciliary motility, or an essential ultrastructure deviation in more than 20% of the square cuts (e.g. reduced number of dynein arms). Biopsy of the ciliated mucosa is usually required for the above criteria and is studied by vital microscopy and transmission electron microscopy. Primary and secondary ciliary dyskinesia can be distinguished by these methods and the rare case of PCD without ultrastructure deficiency ruled out. In special cases a cell culture is recommended for the diagnosis. Practical aspects of the sampling methods and diagnostic pitfalls are reviewed.

Cilia↗
PubMed1996

[Mucociliary clearance of radically operated maxillary sinuses and effect of endoscopic ethmoid bone revision on the mucociliary system].

Twenty-six maxillary sinuses (of 20 patients) were studied following Caldwell-Luc procedures. Surgery had been performed between 1 to 27 years previously. Follow-up studies included nasal endoscopy, coronal computed tomography and camera-sequence scintigraphy. Findings demonstrated that normalization of disturbed mucosal function was possible after surgery. Indications for revision endoscopic sinus surgery are discussed, as are the limitations of surgery.

Ciliary Motility Disorders↗
PubMedDate not supplied

[A morphological study of ciliary abnormalities in patients with immotile cilia syndrome].

The present study has the aim of studying the morphological aspects of ciliary abnormalities in patients affected by immotile-dyskinetik cilia syndrome. Five patients affected by primary ciliary dyskinesia have been studied, examining by TEM the cells obtained by bronchial brushing during fiberoptic bronchoscopy. Many types of anomalies have been observed, and a morphologic classification was proposed. The frequency of abnormal cilia appears to be correlated to the severity of respiratory pathology. The morphological anomalies are not specific for this pathology.

Adult↗
PubMed1996

Young's syndrome: a further cause of chronic rhinosinusitis.

Three males--aged 32, 35, and 27 years--presented Young's syndrome: a combination of obstructive azoospermia and chronic sinopulmonary infection. The evaluation of nasal mucociliary transport using an isotopic technique revealed mucociliary stasis in one case and decreased clearance in the others (< 2 mm/min). Ciliary ultrastructure was normal in two patients, while the other showed mucous hyperplasia and low ciliary density which made correct ciliary evaluation not possible. The clinical development of this syndrome is chronic, although less severe than in the other two syndromes that exhibit primary failure of mucociliary transport: cystic fibrosis and primary ciliary dyskinesia. Young's syndrome should be considered in the differential diagnosis of patients suffering from chronic rhinosinusitis, particularly with cystic fibrosis and primary ciliary dyskinesia syndrome.

Adult↗