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PubMedDate not supplied

Yield of bronchial forceps biopsies in addition to nasal brushing for ciliary function analyses in children.

BACKGROUND: The diagnosis of primary ciliary dyskinesia (PCD) is unlikely, if ciliary beat frequency (CBF) is normal. The aim of this study was to test the diagnostic value of an additional bronchial biopsy in cases where nasal CBF are abnormal. PATIENTS, METHODS: In a paediatric bronchitis population nasal brush biopsies and bronchial forceps biopsies were taken. In both samples we measured CBF and compared results to nasal CBF of infants and children without respiratory disease. RESULTS: Patients with bronchitis (n = 31; 0.3 to 14.6 years; 10 girls) had a normal CBF in their nasal biopsies in 68 %, and in bronchial biopsies in 48 %, compared to the reference group (n = 72; 0.5 to 17.5 years; 23 girls). One patient had an abnormal nasal, but a normal bronchial ciliary activity. When cilia were beating at both sites (n = 14), nasal CBF agreed well with bronchial CBF (mean difference -0.78 Hz, 95 % confidence interval -1.81 Hz to 0.25 Hz). CONCLUSIONS: By adding the investigation of bronchial mucosa to the measurement of nasal CBF the diagnostic yield to exclude PCD was only improved from 68 % to 71 %. Consequently, if nasal ciliary activity is abnormal in infants and children with bronchitis, we do not recommend additional bronchoscopy to obtain another biopsy.

Adolescent↗
PubMed2006

[A new breath actuated dry powder inhaler (Auto-Jethaler)].

BACKGROUND: The aim was to examine the handling of the recently developed breath actuated dry powder inhaler Auto-Jethaler (PulmoTec GmbH/Höchstädt, launch by Ratiopharm and CT Berlin). METHOD: 75 patients suffering from asthma, cystic fibrosis or primary ciliary dysfunction (age: 3 to 34 years; 29 female, 46 male) with mild or moderate bronchial obstruction took part in the study. Lung function testing including body plethysmography was performed to measure bronchial obstruction independent of effort. Peak Inspiratory Flow (PIF) was measured using a Fleisch pneumotachograph equipped with or without a Auto-Jethaler. Instead of the commercially available drug tablet a stainless steal ring device of equivalent resistance, easy to disinfect, was used. Actuation of the rotation mechanism which was triggered by inspiration at an inspiratory flow of about 40 L/min was accompanied by a rattling noise. Aims of the study were to examine, whether the subjects were able to handle the new device, and to measure PIF without and via Auto-Jethaler. RESULTS: Handling of the Auto-Jethaler was found to be easy. All patients managed to reach or surpass the critical value of 40 L/min, even those with mild to moderate bronchial obstruction. PIF without Jethaler was 85 to 599 L/min, via Auto-Jethaler 40 to 215 L/min. PIF was significantly age dependent (p<0.001). CONCLUSIONS: These findings suggest that the Auto-Jethaler will be an appropriate device for drug administration in children older than 3 years provided that they understand the inspiratory breathing manoeuvre.

Adolescent↗
PubMed1996

[Mucociliary clearance function and medicamentous modification].

Mucociliary clearance represents a complex self-cleaning mechanism of the lung and is based on the functional unity of ciliated columnar cells and the special viscoelastic properties of the secretion produced in the tracheobronchial system. It has been known for a long time that intubation and mechanical ventilation can impair mucous transport and lead to morphological damage to the tracheobronchial mucosa. Recent studies made it clear, however, that mechanical ventilation during anaesthesia using an appropriate anaesthesiological technique has no deleterious effect on mucus transport in patients with healthy lungs. Ventilated patients in the intensive-care unit frequently have impaired mucus transport, which is associated with the development of retention of secretion and nosocomial pneumonia. In these patients a number of factors combine to form a formidable potential insult to the mucociliary transport mechanism. Numerous drugs can affect mucociliary transport. Beta-mimetics and theophylline, in particular, have a favourable effect on mucociliary transport, whereas the effect of mucolytic agents is controversial.

Adrenergic beta-Agonists↗
PubMed1999

[Diagnostic procedure in primary ciliary dyskinesia].

BACKGROUND: Primary ciliary dyskinesia (PCD) is usually diagnosed by ultrastructural investigations of nasal or bronchial mucosa. Less invasive techniques for quick and cost-effective diagnosis of PCD should be evaluated. METHODS: In 32 patients with suspected PCD, saccharin transport time, ciliary beating of nasal respiratory epithelium, and ultrastructure of nasal mucosa biopsies were investigated. RESULTS: In 13 patients, PCD was excluded by a normal saccharin transport time (< 20 min). In 19 patients, respiratory cells were obtained by nasal brushing. Frequency, coordination, and amplitude of ciliary beating were examined using interference contrast microscopy and scored. PCD was excluded in 10 of 19 patients, who revealed normal ciliary activity. In the remaining 9 patients, nasal mucosa specimens for ultrastructural investigation were obtained. Ultrastructural alterations indicating PCD were found 4 of 9 specimens, in 2 specimens no cilia were found and in 3 specimens alterations indicating secondary inflammatory alterations were found. CONCLUSIONS: PCD may be efficiently excluded in several cases using cost-effective diagnostic techniques. The definite diagnosis in the remaining cases requires ultrastructural investigations which should be performed in specialized centers. In the present study, PCD in a carefully preselected group had a surprisingly high prevalence.

Adolescent↗
PubMed1998

[Tolerance, subjective complaints and mucociliary clearance in rhinitis sicca before and after nasal irrigation with Rhinomer Force 1].

BACKGROUND: Rhinitis sicca is a widespread disease, caused by a variety of factors. There are many different treatments, but none is more reliable than the other. METHODS: In a prospective study we examined 12 patients before and after a ten-day course of nasal irrigation with Rhinomer Force 1 regarding their symptoms, the mucociliary clearance measured with the saccharin test according to Andersen and tolerance of therapy. RESULTS: Improvement of nose breathing (57%), sensation of mucous running in the pharynx (42%), feeling of a dry nose (42%, significant p = 0.0313). The overall improvement was significant (67%, p = 0.054). Mucociliary transport analysed for each side of the nose (24 sides) resulted in 38% improvement (up 10 min in average), 12% same result, 50% worsening (down 12 min in average). The acceptance was reported as very good in 91% of the study group. CONCLUSIONS: Depending on the symptoms, a high to significant amount of improvement is documented after therapy with isotonic salt water solution. In our study the measurement of the mucociliary transport does not correlate with subjective symptoms. It does not appear to be an adequate tool for diagnosing rhinitis sicca or evaluating the success of treatment.

Adult↗
PubMed1998

[Glands of the human nasal mucosa--electron microscopy and immunohistochemical studies].

BACKGROUND: Ultrastructural characteristics of the nasal glands are presented and possible changes concerning different diseases of the nasal mucosa are investigated. METHODS AND PATIENTS: Specimens from 23 patients suffering from primary ciliary dyskinesia, allergic rhinopathy, and chronic inflammatory hyperplasia were the subject of electron microscopic and immunohistochemical studies. RESULTS: All preparations exclusively showed glands of the serous type. The end segments of the glands were surrounded by contractile myoepithelial cells in a basket-like fashion. Distinct differences in the fine structure of the glands were not observed between the individual groups. The epithelial cells of the efferent ducts consisted of numerous rows forming one layer with pronounced interlockings. These cells in particular showed a high content of mitochondria. This segment of the duct probably has the function of changing the content of water and ions in the nasal secretion. CONCLUSIONS: Our immunofluorescence and electronmicroscopic investigations present the ultrastructure of the nasal glands in detail. We assume an influence of the efferent ducts on the composition of the nasal secretion.

Biopsy↗
PubMed1995

[Evaluation of nasal mucociliary clearance with a modified saccharin test].

To measure the complex nasal clearance we propose to perform our variation of the Andersen saccharin method as a saccharin liquid test (SLT). The application of 3 molar aqueous sodium saccharin solutions with a Wiretrol capillary on the concha nasalis inferior or media allows simple handling, exact dosage, minimal nasal irritation and an increase in reproducibility. Application of 1 microlitre is sufficient to carry out the test. In the present study 48 healthy volunteers without any nose disease or history of sinusitis showed a saccharin transport rate of 10.4 (SD 4.2) minutes.

Adolescent↗
PubMed1990

[Possible pathogenesis of swimming sinusitis].

To investigate the pathogenesis of swimmer's sinusitis, a group of 20 swimmers and 20 controls were interviewed and examined. Clinical examination was followed by rhinomanometry, sonography of the maxillary sinuses, a saccharin test to assess mucociliary transport in the nose, and a viable cytological sampling of the nasal mucosa. Anamnesis showed a preponderance of symptoms of sinusitis and chronic rhinitis in the swimmer group, as well as a reddening of the nasal mucosa and a swelling of the adenoids. Sonography showed mucosal thickening in the antrum in more cases among the swimmers. Rhinomanometry, the saccharin test, and viable cytological sampling revealed no significant differences between the groups. It is argued that pathogenesis is more probable through cooling of the skin of the face and the whole body than through entrance of water into the upper respiratory tract.

Adolescent↗
PubMed1989

[Reactions of the ciliated epithelium of the respiratory tract to intubation anesthesia].

During insufflation anaesthesia the ciliary beat frequency of the ciliated cells of the trachea and the nasal cavity was assessed via vital cytological sampling from these sites before and after anaesthesia. The percentage of vital and dead ciliated cells as well as the percentage of squamous cells was counted in a chamber.-It was found that the ciliary beat frequency did not change significantly, whereas the number of vital ciliated cells in the tracheal samples was significantly reduced and the vitality of the epithelia in the nose showed a minor change. It must be presumed that the observed noxious effects on the tracheal epithelium are due to inhaled gases, since the temperature and humidity in the ventilation circuit are within a range known not to violate the ciliary epithelium. Apart from that, the intubation itself might produce reflexes in the mucosal layer.

Anesthesia, Endotracheal↗
PubMed1984

Young's syndrome. Obstructive azoospermia and chronic sinopulmonary infections.

We studied 29 men with Young's syndrome, a combination of obstructive azoospermia and chronic sinopulmonary infections. Men with this syndrome have only mildly impaired respiratory function and normal spermatogenesis; the azoospermia is due to obstruction of the epididymis by inspissated secretions. The diagnosis is based on the occurrence of chronic sinopulmonary infections, persistent azoospermia, normal spermatogenesis, and characteristic epididymal findings, as well as exclusion of cystic fibrosis and the immotile-cilia syndrome. The sperm themselves appear to be normal in Young's syndrome. Pregnancies had occurred in five couples; in three paternity was documented by genotyping. Thus, improved microsurgical and medical therapy might restore fertility. We suggest that Young's syndrome has a prevalence comparable to that of Klinefelter's syndrome and is a common cause of both chronic sinopulmonary infection and azoospermia.

Adult↗
PubMed2002

Identification of dynein heavy chain 7 as an inner arm component of human cilia that is synthesized but not assembled in a case of primary ciliary dyskinesia.

Although the basic structure of the axoneme has been highly conserved throughout evolution, the varied functions of specialized axonemes require differences in structure and regulation. Cilia lining the respiratory tract propel mucus along airway surfaces, providing a critical function to the defense mechanisms of the pulmonary system, yet little is known of their molecular structure. We have identified and cloned a dynein heavy chain that is a component of the inner dynein arm. Bronchial epithelial cells were obtained from normal donors and from a patient with primary ciliary dyskinesia (PCD) whose cilia demonstrated an absence of inner dynein arms by electron microscopy. Cilia from normal and PCD cells were compared by gel electrophoresis, and mass spectrometry was used to identify DNAH7 as a protein absent in PCD cilia. The full-length DNAH7 cDNA was cloned and shares 68% similarity with an inner arm dynein heavy chain from Drosophila. DNAH7 was induced during ciliated cell differentiation, and immunohistochemistry demonstrated the presence of DNAH7 in normal cilia. In cilia from PCD cells, DNAH7 was undetectable, whereas intracellular DNAH7 was clearly present. These studies identify DNAH7 as an inner arm component of human cilia that is synthesized but not assembled in a case of PCD.

Amino Acid Sequence↗
PubMed2004

The secondary nature of ciliary (dis)orientation in secondary and primary ciliary dyskinesia.

OBJECTIVE: Ciliary orientation (COR) is an important parameter of mucociliary clearance and ciliary disorientation has been reported in cases of acquired abnormalities [secondary ciliary dyskinesia (SCD)] and in a very few cases as the single abnormality in primary ciliary dyskinesia (PCD). The etiology, pathogenesis, consequences and relevance of ciliary (dis)orientation are still unclear. MATERIAL AND METHODS: To elucidate the primary or secondary nature of ciliary (dis)orientation, COR was measured in 179 non-PCD and 59 PCD patients. COR was measured in biopsies and after ciliogenesis in culture and was correlated with a number of functional and ultrastructural parameters. COR was defined as the SD of the angles of lines through the central pair of microtubules using transmission electron microscopy. Internationally accepted normal values for COR are < or = 20 degrees; COR values of 20-35 degrees indicate increased disorientation; and COR values > 35 degrees represent a random orientation. RESULTS: For non-PCD biopsies, COR increased with increasing SCD, from 15 +/- 7 degrees (n = 54) for normal (< 5%) SCD to 28 +/- 8 degrees (n = 16) for severe (> 25%) SCD. No correlation was found between COR and ciliary beat frequency. However, increased COR values (28 +/- 8 degrees) were found for immotility (n = 8), compared to (coordinated) ciliary activity (19 +/- 9 degrees) (n = 121). After ciliogenesis no ultrastructural abnormalities were found and COR was normal (13 +/- 5 degrees; n = 308). COR can therefore be considered to be secondary in non-PCD and correlates with SCD percentage and ciliary motility. In biopsies from PCD patients with dynein deficiency and with normal ultrastructure, COR was increased, to 28 +/- 11 degrees (n = 32) and 21 +/- 7 degrees (n = 15), respectively, and in cases with central pair abnormalities COR was random (38 +/- 11 degrees; n = 12). After ciliogenesis COR remained random in the PCD group with central pair abnormalities (38 +/- 9 degrees; n = 15), and was increased in the PCD groups with dynein deficiency (24 +/- 10 degrees; n = 35) and normal ultrastructure (25 +/- 8 degrees; n = 17). Ciliary disorientation was never found as the single abnormality. CONCLUSION: COR can be considered to be secondary in PCD. Both ciliary (im)motility and SCD percentage contribute to COR.

Cilia↗
PubMedDate not supplied

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.

Adult↗
PubMedDate not supplied

Prolonged airway retention of insoluble particles in cystic fibrosis versus primary ciliary dyskinesia.

Patients with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) have been shown to have impaired large airway clearance of radiolabelled particles as measured by external gamma camera scanning up to 6 hours post deposition. Recent studies suggest that 24-hour retention of particles may reflect some airway retention in addition to alveolar retention. In a retrospective study, we analyzed the relationship between the deposition pattern and 24-hour retention (Ret24 hr) of technetium 99-radiolabelled iron oxide (99Tc-Fe2O3) particles in 20 patients with CF, 12 patients with PCD, and 17 normal subjects. By gamma camera analysis, initial aerosol deposition was analyzed in terms of central-peripheral (C/P) activity within the lungs. Gamma camera scanning was performed immediately following deposition and again at 24 hours to assess residual retention (Ret24 hr) as a percent of initial deposition. C/P analysis was also performed on the 24-hour scan (C/P24). For all subjects, initial deposition pattern (C/P) was inversely related to lung function (forced expiratory volume in 1 second [FEV1]%pred vs. C/P, r = -.54). Ret24 hr was also inversely related to initial deposition pattern for all subjects (Ret24 hr vs. C/P ratio, r = -.42). Analysis of covariance showed that for a given C/P ratio, CF patients had significantly greater Ret24 hr compared to normal subjects (9.8 +/- 2.8 [SE]%). In addition, the CF patients had similar C/P24 as the normal subjects (1.35 +/- 0.40 [SD] vs. 1.10 +/- 0.39, respectively). These results suggest that small airway clearance is compromised in CF patients compared to normal subjects. On the other hand, PCD patients had C/P24 similar to their initial deposition C/P ratios (2.78 +/- 1.72 vs. 2.45 +/- 0.87, respectively), significantly greater than 1.0, and significantly greater than CF or normal subjects, suggesting that PCD patients have prolonged particle retention associated with their large bronchial airways.

Adolescent↗
PubMedDate not supplied

Clinico-pathological evaluation of ciliary dyskinesia: diagnostic role of electron microscopy.

From November 1995 to May 2002, the authors studied 59 children with suspected primary ciliary dyskinesia (PCD). Samples of ciliated respiratory epithelium were obtained by nasal brushing from 44 patients and by biopsy of bronchial mucosa from 15 patients. Thirty-four/Fifty-nine samples were suitable to obtain a qualitative-quantitative evaluation of ultrastructural ciliary abnormalities. Many ciliary and axoneme alterations were described. This study revealed that quantitative and qualitative ultrastructural assessment of respiratory epithelial cilia plays an important role in the differentiation between primary, secondary, and borderline ciliary abnormalities. Early diagnosis of PCD with appropriate clinical follow-up and treatment is important to prevent irreversible lung tissue damage, namely bronchiectasis.

Child↗