Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Ciliary Motility Disorders”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 613 records · Page 34Linked to original sources
PubMed1983

Supernumerary microtubules in the cilia of two siblings causing "immotile cilia syndrome".

The function and ultrastructure of nasal cilia in 2 siblings with unexplained chronic sinusitis and bronchitis were investigated. Both patients lacked nasal mucociliary clearance (greater than 30 min). On electron microscopy, the ultrastructure of nasal cilia showed an extra doublet or supernumerary microtubules with disorganized microtubular network. Other axonemal components were normally present. It is conceivable that an "extra" component of the axoneme may be added to those "defects" already recognized as a cause of the "immotile-cilia syndrome".

Adolescent↗
PubMed1984

Clinical, pathologic, and ultrastructural features of situs inversus and immotile-cilia syndrome in a dog.

Situs inversus, rhinitis, and bronchitis were diagnosed in a 7-month-old male Golden Retriever. Electron microscopic examination of tracheal and bronchial biopsy specimens revealed 9% of the cilia with abnormal axonemal pattern and 1.2% with a fibrous ring between the circle of microtubular doublets and the cell membrane. A permanent response to treatment with antibiotics was not obtained and the dog was euthanatized. At necropsy the thoracic and abdominal organs were found to be in reversed position. Microscopic examination revealed evidence of chronic bronchitis as well as cystic and distended distal tubules of the kidneys. Foci of fibrosis were observed in the renal cortex. The diagnosis was immotile-cilia syndrome.

Animals↗
PubMed1984

[Stroboscopic study of the respiratory mucosal ciliary beat frequency].

Abnormalities of the ciliary function have been found as an important factor in chronic respiratory diseases. For the study of the ciliary beat frequency, we developed a stroboscopic method using a stroboscopic illuminator ( Strobo Hertz) substituted to the normal light of an inverted optic microscope (Leitz). The ciliated cells obtained either by nasal scraping or tracheobronchial brushing were maintained in a culture medium (IP 199) at 37 degrees C. The ciliary beat frequency was measured at various times, at least one hour after the sampling. Nasal scraping, was performed with a curette in 17 children from 10 months to 16 years who complained of chronic respiratory diseases. Ciliated cells were obtained in 14 samples. In one of them 5 years old with a situs inversus, although the ciliated cells were numerous, the cilia appeared immotile. In the 13 others, the frequency was from 7 to 11.5 Hz with a mean of 10.8 Hz. These results were compared to those obtained by tracheobronchial brushings performed in 7 patients from 4 months to 46 years who underwent an endoscopic investigation. The ciliary frequency was 7 to 10 Hz with a mean of 8.5 Hz. The study of the ciliary beat frequency obtained by nasal scraping seems to be a reliable method for the detection of abnormal ciliary function. Nasal scraping is a non invasive method. Moreover, the stroboscopic method is a non expensive tool.

Adolescent↗
PubMed1984

[Young syndrome. Chronic sino-pulmonary infection and infertility].

Young's syndrome is a combination of chronic sinopulmonary infections and obstructive azoospermia in men. Cystic fibrosis and the "immotile cilia syndrome" must be ruled out. The most important methods in differential diagnosis are determination of sweat chloride and sodium levels, investigation of cilia ultrastructure and analysis of semen samples. A case of Young's syndrome in a 42-year-old patient is reported.

Adult↗
PubMedDate not supplied

Testis factors that may regulate gene expression: evidence from a patient with Kartagener's syndrome.

Patients with Kartagener's syndrome characteristically have immotile cilia and flagella. Ultrastructural analysis of the efferent ducts of the patient described in this report revealed that the cilia totally lacked dynein arms. Based on clinical findings his respiratory cilia also were not functional. In contrast, this patient's spermatozoa were motile and displayed normal ultrastructure, including dynein arms. This suggests that two genes exist that code for slightly different proteins with analogous functions; one of these genes functions in somatic tissue and the other in the male germ tissue. Differential gene expression in somatic and male germ tissue may be related to the syncytial nature of male germ cells, low scrotal temperature, or haploid gene expression.

Adult↗
PubMed1995

[The prevalence of primary dyskinetic ciliary syndromes in patients with sinusitis and bronchiectasis].

In this paper are studied the prevalence of this syndrome (the PDCS) in 18 patients affected either of chronic sinusitis or bronchiectasias. Fourteen cases (77%) fulfil the diagnostic requirements in order to be considered as PCDS. Clinical differential features with regard to the idiopathic group are: situs inversus, male's infertility, perennial rhinorrhea and secretory otitis media. Mucociliary transport is studied through an isotopic technique and resulted absent in the PDCS group, being normal in the idiopathic one. The ultrastructure of nasal cilia is normal in the idiopathic representative, while in the cases with PDCS was verified the total or partly lack of dyneine arms in 8 cases, without cilia 3 cases, surnumerary central microtubules 3 cases and surnumerary peripheral microtubules in 2 cases. One sufferer of Kartagener's syndrome showed cilia structure in accordance with standard pattern.

Adolescent↗
PubMedDate not supplied

[Pansinusitis: approach to its etiopathogenesis].

Local factors and external conditions are determinants for pansinusitis. We present 10 cases in which 20% are of unknown origin, 30% have allergic antecedents, 20% show hypersensitivity to aspirin, 20% have an immotile-cilia syndrome and 10% are caused by exogenous factors. Surgical treatment is performed in 70% of the cases, always followed by a long-term medical therapy. Forty per cent of the cases show a chronic progress despite the type of treatment applied.

Adult↗
PubMed1994

Atypical basal bodies in a cat with immotile-cilia syndrome.

Atypical basal bodies from the oviductal mucosa of a female, 2.5-years-old cat suffering from immotile-cilia syndrome are described. The microtubular pattern was observed in 250 cross-sectional basal bodies. Four basal bodies (1.6%) had a defective number of microtubular triplets resulting in the appearance of so-called 'half-centriole' type. Two out of 100 longitudinal basal bodies showed duplicating striated rootlets and only one had an additional set of nine subdistal appendages, so that two basal feet arose from a single basal body. Electron-dense material and isolated microtubules into the lumen of some basal apparatuses were occasionally seen. It is known that centrioles and basal bodies show an unusual degree of constancy in size, shape and geometrical configuration, so that their abnormalities are very rare.

Animals↗
PubMed1994

Immotile cilia syndrome: nasal mucociliary function and nasal ciliary abnormalities.

We present 17 patients with a typical symptomatology of immotile cilia syndrome, seven of them with complete situs inversus. Firstly, a study of the nasal mucociliary transport was made by means of the radioisotopic technique with serum albumin-Tc99m. In all cases there was absence of transport. Secondly, we studied the ultrastructure of the nasal cilia. Defects in the dynein arms were frequently found (65%). In two cases (11%) there were no cilia; in two other cases the cilia were normal, and in another two cases alterations of the central pair of microtubules were seen. Ciliary complexes were detected in all cases. We conclude that in patients with chronic or recurrent infections of the airways without known cause we must initiate a study of the nasal mucociliary transport. If this is absent or decreased, study of ciliary ultrastructure should be carried out. If mucociliary transport is normal, immotile cilia syndrome is ruled out and ultrastructural study of the cilia is not required.

Adolescent↗
PubMed1994

[The mechanisms of the development of respiratory tract ciliary dysfunction in nonspecific lung diseases].

An original TV system was employed to evaluate ciliary function of the respiratory epithelium in patients with acute pneumonia, acute bronchitis, prebronchitis, chronic bronchitis. Criteria for identification of early ciliary dysfunction have been developed. The effects on ciliary movements of eicosanoids, platelet-activating factor, protease, biogenic amines and mechanisms underlying ciliary dysfunction in nonspecific pulmonary diseases are considered.

Adolescent↗