1/17. Different frequency of cilia with transposition in human nasal and bronchial mucosa. A case of acquired ciliary dyskinesia.Nasal and bronchial cilia and spermatozoa of a patient with a high clinical suspicion of a ciliary dyskinesia syndrome were ultrastructurally studied and quantified. Defective cilia showed two types of axonemal patterns: 9d 0s and 8d 1d. Of these, 9d 0s cilia prevailed in the proximal region, whereas 8d 1d prevailed in the distal region. Translocation of a peripheral doublet to the central position occurred at the middle region of cilia lacking the central pair, probably to compensate for its absence. Quantitative analysis showed that the percentages of anomalous cilia were 5.32 /-0.93 in nasal samples and 43.17 /-2.34 in bronchial samples. spermatozoa without the central pair or with a translocated microtubular doublet were rarely observed, but a variety of nonspecific defects were seen. Even though transposition is generally considered to be an inherited ciliary defect and one of the causes of primary ciliary dyskinesia, in this case quantitative ultrastructural analysis and clinical data indicate that this is an acquired ciliary defect.- - - - - - - - - - ranking = 1keywords = mucosa (Clic here for more details about this article) |
2/17. Chronic cough and infertility: a report of two cases.OBJECTIVE: To report two cases of infertility caused by primary ciliary dyskinesia in patients who presented with an associated complaint of a chronic cough. DESIGN: Case report. SETTING: University teaching hospital.Patient(s): Two patients presenting with unexplained infertility and an associated history of long-term chronic cough. INTERVENTION(s): patients underwent a nasal mucosal biopsy by an otolaryngologist. Electron microscopy (EM) examination of biopsy specimens was performed. MAIN OUTCOME MEASURE(s): diagnosis and appropriate treatment for functional tubal factor infertility. RESULT(s): Both patients were diagnosed with primary ciliary dyskinesia based on EM of the nasal biopsy specimens. Given this diagnosis, they immediately underwent IVF-ET. Both patients became pregnant with their first IVF-ET cycle. CONCLUSION(s): Other investigators have shown that almost 20% of patients with a chronic cough will have EM evidence of ciliary dyskinesia. patients presenting with idiopathic infertility and an associated unexplained chronic cough should be referred for nasal biopsy with EM evaluation to rule out primary ciliary dyskinesia. infertility in these cases, which is due to a functional tubal factor, is best treated with IVF-ET rather than superovulation and intrauterine insemination treatments.- - - - - - - - - - ranking = 0.25keywords = mucosa (Clic here for more details about this article) |
3/17. Obstructive azoospermia associated with chronic sinopulmonary infection and situs inversus totalis.We describe 2 cases of obstructive azoospermia associated with situs inversus and sinopulmonary infection due to ciliary defects. Electron microscopy of testicular sperm flagella demonstrated normal morphology with nine peripheral doublets surrounding a central pair and complete sets of inner and outer dynein arms. Electron microscopy of the nasal mucosa revealed partial defects of the dynein arms of cilia, although the "9 2" morphology was preserved. Our cases were considered unique variants of Young's syndrome but also had characteristic features of kartagener syndrome, and thus support the hypothesis that Young's syndrome has a genetic etiology similar to that of kartagener syndrome.- - - - - - - - - - ranking = 0.25keywords = mucosa (Clic here for more details about this article) |
4/17. Abnormal length of cilia--a cause of primary ciliary dyskinesia--a case report.A 7-year-old Turkish boy had suffered from chronic coughing from early childhood. Severe bronchiectasis in the right lung was confirmed by bronchography. Ciliary beat frequency determined in a bronchial mucosal biopsy was markedly decreased (5.7 Hz). Electron microscopy revealed cilia with a length of 15 microns. No structural abnormality was found. A possible link between the abnormally long, slow beating cilia and the clinical symptoms is discussed.- - - - - - - - - - ranking = 0.25keywords = mucosa (Clic here for more details about this article) |
5/17. Recurrent respiratory infections in a child with fucosidosis: is the mucus too thin for effective transport?fucosidosis is caused by a deficiency of the lysosomal enzyme alpha-l-fucosidase (ALF) leading to an accumulation of glycoproteins in a variety of cells. Infants and young children with this disorder are prone to recurrent sinus and pulmonary infections and often die of pneumonia. We studied the mucociliary and systemic immune function in a 6 year old girl with fucosidosis and recurrent respiratory infections. All measurements of systemic immune function were normal. sweat chloride was normal when measured on angiokeratotic skin but was greater than 65 mg/L on uninvolved areas. During the placement of tympanic ventilation tubes, tracheal mucus was gently aspirated and a mucosal biopsy was taken. Tracheal mucus transport was not measured. The biopsy material was examined under phase contrast microscopy and revealed ciliated cells with apparently normal beating. TEM of these cells showed a characteristic pattern of vacuoles in the cytoplasm as described in other tissues from patients with fucosidosis. Ciliary ultrastructure was normal. mucus viscoelasticity was measured in a magnetic microrheometer. The loss tangent was 2 SD above the mean for normal mucus and mechanical impedance was about 2 SD below the mean. These changes are similar in direction but double in magnitude to what has been described with methacholine administration in dogs. The high compliance of the mucus may be due to incomplete assembly of mucus glycoprotein or to decreased secretion of glycoproteins in respiratory secretions. This leads to mucus that is abnormally watery and thus difficult to clear from the airway.- - - - - - - - - - ranking = 0.25keywords = mucosa (Clic here for more details about this article) |
6/17. Freeze-fracture analysis of the respiratory cilia from the bronchial mucosa of a patient with primary ciliary dyskinesia.Respiratory cilia of the bronchial mucosa from a 5-year-old boy with clinical evidence of classical Kartagener's syndrome (situs inversus, bronchiectasis and sinusitis) were first examined by means of transmission electron microscopy for identification of the axonemal defects described as typical for primary ciliary dyskinesia (PCD). Additional oscillography was performed on the cilia in vitro, which showed absence of a coordinated ciliary beat frequency. After clear classification of the case as PCD, a freeze-fracture examination of the respiratory cilia was performed, which revealed a higher density of intramembrane particles on the outer fracture face (E-face) than on the inner fracture face (P-face). The results were discussed with regard to probable pathogenetic aspects on PCD.- - - - - - - - - - ranking = 1.25keywords = mucosa (Clic here for more details about this article) |
7/17. Primary ciliary dyskinesia with transverse colon carcinoma.Primary ciliary dyskinesia, characterized by chronic airway infection, impaired fertility, and situs inversus in half the number of cases, was detected in a 58-year-old Japanese woman, along with carcinoma of the transverse colon. She was of complete situs inversus and subfertile and had chronic bronchitis and nasal polyposis. Ultrastructurally, the cilia of the nasal mucosa lacked dynein arms. This is the first documentation of this disease coupled with cancer.- - - - - - - - - - ranking = 0.25keywords = mucosa (Clic here for more details about this article) |
8/17. 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 = 0.5keywords = mucosa (Clic here for more details about this article) |
9/17. Aplasia of nasal cilia with situs inversus, azoospermia and normal sperm flagella: a unique variant of the immotile cilia syndrome.A 23-year-old man with situs inversus and bronchiectasis was investigated because of azoospermia. serum levels of testosterone and pituitary gonadotropins were normal. Scrotal exploration revealed no abnormalities of the testes, epididymides or vasa deferentia. Electron microscopy of a testicular biopsy specimen revealed a normal sperm flagellum structure, including the presence of dynein arms but 2 separate biopsies of the nasal mucosa revealed complete ciliary aplasia. This case, which bears a striking resemblance to Young's syndrome (bronchiectasis and azoospermia), represents a unique variant of the immotile cilia syndrome.- - - - - - - - - - ranking = 0.25keywords = mucosa (Clic here for more details about this article) |
10/17. 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 = 0.5keywords = mucosa (Clic here for more details about this article) |
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