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Results for “Ciliary Motility Disorders”

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At least 523 records · Page 29Linked to original sources
PubMed1988

Immotile cilia syndrome (primary ciliary dyskinesia) and inflammatory lung disease.

Abnormal mucociliary clearance can either be due to defects of mucus production and rheology or to abnormalities of the cilia. Ciliary dysfunction can be inherited as a genetically determined defect in axonemal structure (called the immotile cilia syndrome or primary ciliary dyskinesia) or acquired defects can result from airway injury. This article examines the defense of the lung as related to ciliary activity and addresses ways in which this activity can be modified by inflammatory mechanisms.

Cilia↗
PubMed1988

The clinical effect and the effect on the ciliary motility of oral N-acetylcysteine in patients with cystic fibrosis and primary ciliary dyskinesia.

The effect of peroral N-acetylcysteine (NAC) in patients with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) was investigated. 41 CF patients and 13 PCD patients completed the study which was a double-blind, placebo-controlled, cross-over trial. The patients received either NAC or placebo for two periods of three months followed by a three month follow-up period. Active treatment consisted of NAC, either 200 mg x 3 daily (patients weighing less than 30 kg) or 400 mg x 2 daily (greater than 30 kg). The effect was evaluated in terms of a subjective clinical score, weight, sputum bacteriology, blood leucocyte count, sedimentation rate, titres of specific antimicrobial antibodies, lung function parameters and measurement of the ciliary function. No effect was seen in PCD patients, but in CF patients an improved lung function was seen in the period when the patients suffer most from lower airway infections.

Acetylcysteine↗
PubMed1988

Orientation of human respiratory cilia.

Ciliary orientation was studied on the respiratory epithelium of the nasal cavity or the sphenoidal sinus of ten adult nonsmokers without respiratory disease. The ciliary orientation was evaluated from micrographs by measuring the angle between the plane defined by the central tubules and reference line (with a semiautomatic image analyser (IBAS I]. The standard deviation of the angles of cilia population was counted in every field. The standard deviation of the measurements described the ciliary alignment. It varied from 12.1-41.2 degrees. The mean standard deviation was 27.3 +/- 7.4 degrees. 58% of all measured cilia were within +/- 0-20 degrees of the mean and 85% of cilia were within +/- degrees. However, a few cilia or small groups of cilia were found in most fields which differed dramatically from the main orientation. The size of these groups was always less than ten cilia. On the normal respiratory epithelium the standard deviation of ciliary orientation varies between +/- 10-40 degrees (at about 97% probability). For diagnostic conclusions more than 60 cilia should be measured.

Adult↗
PubMed1987

[Immotile cilia syndrome].

The case of a patient with immotile cilia is reported. This syndrome is characterized by infertility caused by immotile spermatozoa and by bronchiectases owing to inadequate function of ciliated cells. Electron microscope studies showed gross abnormalities of the axial threads in the flagella of the spermatozoa examined.

Adult↗
PubMed1987

[Radionuclide evaluation of the mucociliary function of the mucosa of the nasal cavity in chronic lung diseases].

The authors presented their first experience of radionuclide assessment of the nasal mucosal transport (NMT) in 39 patients with chronic bronchopulmonary pathology (14 adults and 25 children) to detect patients among them with suspected syndrome of primary ciliary dyskinesia (SPCD). In 18 patients the test was positive excluding SPCD as a cause of a chronic inflammatory pulmonary process. In 21 patients NMT was absent, 2 of these patients had Kartagener's triad serving as a kind of model confirming the data on NMT. The authors proposed the method as a screening test due to its non-invasiveness and simplicity in patients with chronic inflammatory pulmonary diseases in order to detect among them patients with suspected SPCD for further examination using invasive methods.

Adolescent↗