1/13. A possible mechanism of primary ciliary dyskinesia: a case of a segmental defect in ciliary microtubules.We report here a 13-year-old woman with cough, sputum and fever. The patient had both chronic sinusitis and bronchitis. Chest X-ray and computed tomographic scan of the chest revealed mucous bronchial filling and bronchiectasia in bronchi of bilateral lower lobes, right middle lobe and left upper lobe. Aerosol inhalation scintigraphy with 99mTechnetium demonstrated delays of the discharged tracer. On the basis of these findings, primary ciliary dyskinesia was suggested. This was confirmed by the findings from nasal biopsy with transmission electron microscopy where all of the microtubules were segmentally defected near the basal body in the cilia. On the basis of these findings, we diagnosed the patient with primary ciliary dyskinesia which may be due, at least in part, to segmental defect of ciliary microtubules.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
2/13. Young's syndrome associated with situs inversus totalis.Primary ciliary dysfunction causes recurrent sino-pulmonary infections and shares the pathophysiology of the reproductive tract. A 34-year old man with bronchectasis and situs inversus totalis was investigated because of azoospermia. Endocrinological evaluations were all normal and scrotal exploration revealed the distended caput, but atrophic body to tail, of the epididymis. light microscopy of testicular biopsy showed normal spermatogensis and motile sperms were easily obtained by testicular sperm extraction. Electron microscopy demonstrated normal sperm structure, whereas 65% of nasal cilias showed defect of central microtubules. This case, which bears a resemblance of Kartagener's syndrome (situs inversus totalis), is considered a unique variant of Young's syndrome.- - - - - - - - - - ranking = 43.195287332668keywords = tract (Clic here for more details about this article) |
3/13. Aplasia of respiratory tract cilia.We report on ciliary aplasia of the respiratory tract, a rare disorder of the mucociliary apparatus, that is insufficiently recognized as a distinct entity. A culture method for ciliogenesis was developed by our laboratory and offers the advantage of studying cilia free of secondary changes associated with infection. Three cases of primary ciliary aplasia were documented histologically in direct biopsy specimens and also in biopsy specimens cultured specifically for ciliogenesis. Primary ciliary aplasia should be differentiated from secondary ciliary aplasia in which basal bodies are present and ciliogenesis takes place in specific culture. Only hereditary ciliary abnormalities are expressed in cell cultures. We critically review the cases of ciliary aplasia reported to date.- - - - - - - - - - ranking = 66196.360754865keywords = respiratory tract, tract (Clic here for more details about this article) |
4/13. hydrocephalus, bronchiectasis, and ciliary aplasia.A girl presented in the neonatal period with hydrocephalus, bronchiectasis, and ciliary aplasia. A common defect both in respiratory tract cilia and in ventricular ependyma cilia may explain the association of the two diseases.- - - - - - - - - - ranking = 13239.272150973keywords = respiratory tract, tract (Clic here for more details about this article) |
5/13. Immotile-cilia syndrome with azoospermia: a case report and review of the literature.A case of immotile-cilia syndrome associated with azoospermia is presented. This diagnosis is based on a typical history of bronchitis, sinusitis, situs inversus, impaired nasal mucociliary clearance and characteristic ultrastructural defect in the respiratory tract cilia and in the sperm tail. semen analysis showed azoospermia with no evidence of obstruction in the epididymis or vas deferens; there was normal spermatogenesis.- - - - - - - - - - ranking = 13239.272150973keywords = respiratory tract, tract (Clic here for more details about this article) |
6/13. Ciliary abnormalities in respiratory disease.One hundred and sixty seven children, ranging in age from 5 weeks to 16 years, with chronic upper or lower respiratory tract problems, or both, were investigated for ciliary dyskinesia. Abnormal ciliary function was found in 18 cases all of whom had chronic lower respiratory disease and most of whom also had upper respiratory problems. Fifteen of the 18 cases had reduced ciliary beat frequencies (less than 10 Hz) associated with dyskinesia and the other three showed apparent absence of ciliated cells. Of the 15 cases with reduced ciliary beat frequencies, ciliary ultrastructure was normal in seven cases but abnormal with missing dynein arms and occasional abnormalities of microtubular arrangement in eight. Respiratory symptoms in the perinatal period were more common in children with abnormal ciliary function and present in all those with ultrastructural abnormalities or absence of ciliated cells compared with 34 (26%) of 132 children, in whom symptoms were recorded, with normal ciliary function. This study would suggest that all children with unexplained chronic respiratory disease, in particular those with symptoms starting in the perinatal period, should be investigated for ciliary dyskinesia.- - - - - - - - - - ranking = 13241.272150973keywords = respiratory tract, tract, upper (Clic here for more details about this article) |
7/13. Primary ciliary dyskinesia: ciliary activity.In primary ciliary dyskinesia (PCD) the cilia of the respiratory tract are immotile or they show an incoordinate, abnormal beating pattern. Consequently, mucociliary clearance is lacking. Most patients with PCD have ultrastructural abnormalities in their cilia. In some patients, however, the ciliary ultrastructure is completely normal. In this study a semi-quantitative scoring method is described for studying ciliary motility in biopsies of nasal mucosa. For every cell the frequency, coordination and amplitude of the ciliary beat are scored. Thereafter, a total score of ciliary activity per cell can be calculated. This method is simple and has proved to be reproducible. By using this scoring method the presence of a PCD can be established with certainty, whether the ciliary ultrastructure is abnormal or not. In patients with a normal ciliary ultrastructure the cilia have a rather high beating frequency (vibration). By scoring the ciliary motility in mucosal biopsies, the time-consuming electronmicroscopic investigation of the cilia is no longer necessary in most patients.- - - - - - - - - - ranking = 13239.272150973keywords = respiratory tract, tract (Clic here for more details about this article) |
8/13. Respiratory infections may reflect deficiencies in host defense mechanisms.Serious respiratory tract infections are rare in the healthy individual and most of the nuisance morbidity that occurs results from nasopharyngeal viral infections that many people get once or twice a year. The economic impact from these upper respiratory tract infections is appreciable, however, in terms of absenteeism from school or work, but unfortunately there is little that can be done to ward them off in a practical way. pneumonia is an infrequent lifetime experience for most non-smoking adults and when it occurs, unusual circumstances may pertain--a particularly virulent microorganism is in circulation, or perhaps one has been exposed to a newly recognized germ, such as has occurred with legionella species in the past 8 years or so. What protects us the great majority of the time is a very effective network of respiratory tract host defenses. These include many mechanical and anatomical barrier mechanisms concentrated in nose and throat; mucociliary clearance, coughing and mucosal immunoglobulins in the conducting airways and in the air-exchange region of the alveolar structures, phagocytes, opsonins, complement, surfactant and many other factors combine to clear infectious agents. The ability to mount an inflammatory response in the alveoli may represent the maximal and ultimate expression of local host defense. In some way these host defenses are combating constantly the influx of micro-organisms, usually inhaled or aspirated into the airways, that try to gain a foothold on the mucosal surface and colonize it. But many general changes in overall health such as debility, poor nutrition, metabolic derangements, bone marrow suppression and perhaps aging promote abnormal microbial colonization and undermine the body's defenses that try to cope with the situation. It is a dynamic struggle. The departure from normal respiratory health may not be obvious immediately to the patient or to the physician and repeated episodes of infection or persisting symptoms of cough, expectoration and sinus or ear infections may develop before serious assessment of the situation is taken and appropriate diagnosis gotten underway. Obvious explanations for respiratory infections may be apparent and, nowadays, side effects from antineoplastic chemotherapy or immunosuppressive therapy for a variety of diseases that create an immunocompromised host are common. In a few subjects, especially young adults who present with a cumulative history of frequent but mild infections in childhood and youth, a subtle deficiency in host defenses may exist and have been partially masked because of attentive pediatric medical care and prompt use of broad spectrum antibiotics.(ABSTRACT TRUNCATED AT 400 WORDS)- - - - - - - - - - ranking = 103304.42838006keywords = respiratory tract infection, respiratory tract, upper respiratory tract, tract infection, tract, upper (Clic here for more details about this article) |
9/13. Enhancement of ciliary action by a beta-adrenergic stimulant.It is known that beta-adrenergic stimulants enhance mucociliary transport in the respiratory tract. The present study clearly indicated that such an enhancement resulted from not only the alteration in secretory function but also the direct stimulation of ciliated cells. However, the enhancement of ciliary beating depended on the morphological changes in ciliated cells. On the other hand, the effect of terbutaline aerosol, a beta-adrenergic stimulant, on patients with chronic sinusitis was evaluated by paranasal sinus radiography. Consequently, marked improvements in shadows were achieved after 4 weeks in 40% of the cases examined.- - - - - - - - - - ranking = 13239.272150973keywords = respiratory tract, tract (Clic here for more details about this article) |
10/13. Ciliary abnormalities in children (hockey-stick cilia). Study by scanning electron microscope. Report on three paediatric cases.The authors report three cases of patients suffering from repeated infection of the upper and lower respiratory tracts. mucociliary clearance, measured in the nasal fossae by coloured indicators, is reduced. Both transmission and scanning electronic ultramicroscopic study of the vibratile cilia show a morphological malformation of "hockey-stick" appearance, while the structure of the exonemal microtubular apparatus is normal and complete. This morphological ciliary abnormality is held to be responsible for the respiratory pathology in the three cases observed.- - - - - - - - - - ranking = 13240.272150973keywords = respiratory tract, tract, upper (Clic here for more details about this article) |
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