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PubMed1992

[Current understanding of mucociliary transport].

This review article deals with current understanding of mucociliary transport and its significance for clinical practice. The ultrastructure of cilia and principles of mucociliary clearance in the upper respiratory tract are outlined. Practicable diagnostic measures described are the saccharine test, vital cytological sampling of the nasal mucosa and electron microscopy for ultrastructural studies. Special pathologies such as the immotile cilia syndrome and mucoviscidosis are outlined in their relation to mucociliary transport. Recommendations are given as to how mucociliary transport might be influenced therapeutically.

Ciliary Motility Disorders↗
PubMed2003

[Comparison of the mucociliary transport rate of rhinitis sicca and atrophic rhinitis].

OBJECTIVE: To study the mucociliary transport function of rhinitis sicca and atrophic rhinitis, and to explore the standard of diagnosis. METHOD: The MTR of normal control group, the rhinitis sicca group and the atrophic rhinitis were determined by using saccharin, and then compared. Then MTR of rhinitis sicca treatment group were compared before and after treatment. RESULT: The MTR of normal group: (9.15 +/- 0.86) mm/min; the rhinitis sicca group: (5.84 +/- 0.48) mm/min and the atrophic rhinitis group: (3.36 +/- 0.07) mm/min. There were significant difference among them (P < 0.05). 25 patients of rhinitis sicca were treated by administering the pill of Gelomyrtol forte in 2 weeks. The MTR of rhinitis sicca were no significant difference before and after treatment (P > 0.05). CONCLUSION: Rhinitis sicca is a separate nasal disease, which is different from atrophic rhinitis. It is important to find an effective treatment for the disease.

Adolescent↗
PubMed2003

[Pathophysiology of chronic obstructive pulmonary disease].

Chronic obstructive pulmonary disease (COPD) is a disease state characterised by airflow obstruction that is not fully reversible and progressive. Symptoms, as cough, sputum production and dyspnoea, functional impairment and complications of COPD can all be explained on the basis of the underlying lung inflammation and the resulting pathology. The chronic airflow obstruction is caused by a mixture of small airway disease (obstructive bronchiolitis) and parenchymal destruction (emphysema). On one hand, chronic inflammation causes remodelling and narrowing of the small airways. On the other hand, destruction of the lung parenchyma, also by an inflammatory process, leads to the loss of alveolar attachments to the small airways and decreases lung elastic recoil. In turn, these changes reduce the ability of the airways to remain open during expiration.

Airway Obstruction↗
PubMed2004

[To the optimization of the diagnosis of primary ciliary dyskinesia].

Functional and morphological studies of the mucociliary system in primary ciliary dyskinesia were conducted using a model of the Zivert-Cartagener syndrome. They established the specific features of the mucociliary system and the expediency of a direct evaluation of mucociliary clearance by a non-invasive method in vivo, by taking into account the pronounced degree of mucociliary insufficiency. This makes it possible, no matter whether the situs inversus is absent or present, to reduce a cumbersome diagnostic complex usually used to verify this pathology and to undertake a study of the ultrastructure of bronchial epithelial cilia immediately. This approach enhances the accuracy of a diagnosis of this pathology, decreases its scope and time, and reduces its cost.

Bronchi↗
PubMed2004

[Differential diagnosis and treatment of chronic cough].

Postnasal drip, asthma and gastroesophageal reflux disease are the underlying causes in almost 90% of cases with chronic cough. Causal treatment is successful in the majority of patients, although in the event of a long-standing cough, it might need to be continued over several weeks. Smoking complicates the identification of cough as a clinical early symptom of an underlying tumor. Cardiac causes are rare, and in most cases are due to the use of ACE-inhibitors. Cough may be triggered by a variety of causes and the therapeutic palette must include several spectra. Since the individual causes often cannot be unequivocally identified, it may be necessary to take a polypragmatic therapeutic approach targeting the three most common causes simultaneously for 7-10 days.

Angiotensin-Converting Enzyme Inhibitors↗
PubMed2005

[A case of Kartagener's syndrome].

This case describes a 57-year-old woman in whom situs inversus had been noted at her birth. She had bronchial asthma and bilateral sinusitis during her childhood. She married and experienced childbirth. In December 2003, she was admitted to our Division complaining of wheezing, expectoration and dyspnea on effort. Bronciectasis was visualized on chest X-ray and CT. Electron microscopic examination of the nasal cavity epithelium and bronchial epithelial cilia revealed a deficit of bilateral dynein arms. These findings, helped establish a diagnosis of Kartagener's syndrome, which is characterized by primary ciliary dyskinesia. The restrictive and obstructive pulmonary dysfunction with increase of residual volume in the lung function tests and diffuse centrilobular small nodules with hyperinflation on chest CT were consistent with the findings of diffuse panbronchilitis (DPB) and suggested extended obliterative peripheral airway disease. Clarithromycin which is highly effective for DPB failed to prevent the aggravation of airway infection, arousing the concern about the progression into chronic respiratory failure.

Ciliary Motility Disorders↗
PubMed2005

Culture of cells harvested with nasal brushing: a method for evaluating ciliary function.

OBJECTIVES: Usefulness and reliability of nasal brush samples in a monolayer cell culture was studied for evaluation of ciliary movement. METHODS: Cells for cultures were harvested under the middle turbinate from patients with chronic sinusitis and from controls. Ciliary function was analysed using a microscope equipped with a high-speed video camera. Ciliary beat frequency (CBF), ciliary amplitude, waveform and coordination were analysed from the cultures 4-6 h after the harvest of brush samples and 3 days after the culture. RESULTS: The average success rate of cell cultures was 82.5%. There were not statistically significant differences in CBF between patients and controls but there was significant difference between brush biopsy and cultured brush biopsy in controls. The ciliary beat amplitude and the waveform were normal in all samples in both groups. CONCLUSIONS: Miniculture method for culturing nasal cells from brush biopsies proved reliable and non-invasive for detailed analysing of ciliary function and for excluding possible secondary ciliary dyskinesia. Compared to conventional mucosal cell cultures where thick and invasive specimens are needed for successful cultures, it is easier and quicker to perform and well tolerated by patients. Thin monolayer cultures enable the evaluation of frequency, amplitude and the waveform of the cilia beat under the microscope whereas floating cells and cell clusters do not give this opportunity.

Adult↗
PubMed1992

[Mucociliary clearance in childhood].

AIM OF THE STUDY: A new, nearly monodisperse human serum albumin particle produced by air-pressure-nebulization was inhaled by children and lung-transplant patients. METHOD: After inhalation of the particles obtained with an air-pressure nebulizer, the initial deposition pattern showed a marked tracheobronchial deposition which could be reproducibly obtained without a special breathing technique, the alveolar deposition being not higher than 10%. With the use of 99mTc, the radiation exposure is limited to a level which is low enough for children, but images can be taken up to 24 hrs later. Further parameters for in vivo characterization of the mucociliary function are the 24 h retention pattern and the velocity of particle motion in the trachea. RESULTS: Within the first 45 minutes, the global clearance rate was 51% in healthy children, which is rather high in comparison with the literature, most likely due to size of particles and the selection of patients with a mean age of 10.7 years. In ciliary dysfunction, the initial clearance rate was 16% and 46% within 24 h. CONCLUSIONS: With simplification of the preparation, application, and examination technique, this method is to be used in children, so that a wider use can be anticipated. The normally fast initial elimination of particles allows quick differentiation of normal and impaired ciliary function.

Adolescent↗
PubMed2005

[The syndrome of dyskinetic cilia as the cause of chronic sinusitis].

UNLABELLED: The syndrome of dyskinetic cilia is the cause of chronic inflammatory processes of upper and lower airways. The research studies concerning cilia's structure were held during 50s. About 20 types of cilia's defects were characterized by now, classified as inborn defects. OBJECTIVES: The presentation of the case is because of both the rarity of incidence and diagnostical's problem and treatment. In this case, we observed who re-occurrence of chronic inflammation accompanied by having infections of lower respiratory tract it is necessary to expand diagnosis to find out the reasons of such disease. MATERIALS AND METHODS: A 7 years old girl was admitted to our Clinic. Since couple years she has been under assistance of Specialist Clinic. She was also hospitalized because of aggravation inflammatory state of lower airways. After computers tomography of sinuses she was qualified to surgical treatment. During operation a material to study of cilia immobility was taken. The electron microscope study confirm the diagnosis of the group of dyskinetic cilia. RESULTS: The follow-up examination was held 14 months after operation. During this time the girl did not take any antibiotic (before antibiotherapy was held couple times during the year). Clinical status proved. Better results were also on computer tomography. CONCLUSIONS: In this case, re-occurrence, inflammatory process both upper and lower respiratory tract are necessary to expand the diagnostics to find out the cause of illnesses. The methods of treatments the group of in-movement's cilia based on the symptomatic treatment, but the role of physiotherapy of respiratory system is stressed as the operation of removal of secretion's retention. There is an important role of regular laryngology care and surgical treatment allow partly to control the disease.

Child↗
PubMed2005

Ultrastructural ciliary findings in nasal obstructive diseases.

Specific ultrastructural findings have widely been described in case of obstructive nasal diseases due to congenital defects. Ciliary impairment has in particular been observed as the main pathological feature in these conditions. In this study, nasal mucosal samples from different pathologies have been collected via the "brushing" technique and analysed by transmission electron microscopy. TEM analysis was focused on specific features, such as the numerical array of peripheral and central doublets of the cilium axoneme, including eventual microtubular disarrangement; partial or total loss of inner and/or outer dynein arms; defects of radial spokes and nexin links; disorientation of the ciliary axis in closely adjacent cilia, calculating the angle between the line crossing the central microtubular core and the horizontal ciliary axis and compound cilia (CC). Statistical comparison was carried out between study and control groups. A significant incidence of organic ciliary defects was found not only in patients with inflammatory processes, but mostly in those supposed to have a long-lasting nasal respiratory disease due to mechanical stenosis in relation to septum deviation and turbinate hypertrophy. Prevalence and percentage of compound cilia were instead more related to inflammatory conditions. The "brushing" technique can be considered an easy and reliable method for the assessment of the condition of the nasal mucosa. According to the findings derived from this study, mechanical nasal obstruction seems to cause major alterations on the nasal ciliary arrangement, thus determining a functional impairment on the whole nasal function.

Adult↗
PubMed2006

[Neonatal respiratory distress caused by primary ciliary dyskinesia].

Two newborns, both boys, presented with unexplained respiratory distress. One developed recurrent pneumonias in the first neonatal week and was diagnosed with primary ciliary dyskinesia at the age of 2.5 years. The other had respiratory problems besides a situs inversus totalis and was diagnosed with primary ciliary dyskinesia in the neonatal period. Although 65-90% of children with primary ciliary dyskinesia present with neonatal respiratory distress, the disease is often diagnosed after a considerable delay. Primary ciliary dyskinesia should be considered in newborns with unexplained respiratory problems and in children with recurrent respiratory problems. The disease is diagnosed by taking a nasal brush biopsy of the cilia and examining it using electron microscopy or using phase contrast microscopy. Early diagnosis and adequate treatment may prevent further lung damage.

Ciliary Motility Disorders↗
PubMedDate not supplied

[Changes in the motor apparatus of the bronchial cilia. Comparative study of two types of myopathies: nemaline myopathy and the Werdnig-Hoffmann syndrome].

The Authors during an extensive applications of nasal brushing in infancy according to Rutland and Cole, remarked--first time in literature--the contemporary presence in a child of the nemaline myopathy syndrome and immotile cilia syndrome. Supposing the possibility that in other myopathy may be present the same ciliary immotile syndrome, they studied with nasal brushing three cases of Werdnig-Hoffman syndromes: but they presented normal ciliary conformation. The Authors suppose that in case in future it should be found in other cases of nemaline myopathy the contemporary presence of dinein arms lack, it should be demonstrated that this syndrome takes derivation from a genetic alteration both of the muscular apparatus and the ciliary mobility system of bronchial epithelius.

Bronchi↗
PubMedDate not supplied

[Results of nasal brushing in the study of ciliary conformation and function in chronic bronchopneumopathies in childhood].

UNLABELLED: For the frequent remark in childhood of relapsing bronchitis and/or broncho-pneumonic sickness we resolved to use for the differential diagnosis the nasal brushing method according to Rutland (for samples of ciliated epithelium from the deep surface of the inferior nasal turbinate using a 2 mm diameter nasal brush. The samples were used for ultrastructural study of cilia. RESULTS: Children studied 22:14 (6-14) y.old with relapsing bronchitis, 4 with relapsing broncho-pneumonitis, 1 Nemaline myopathy (n.m.) with heavy respiratory insufficiency; 3 Werdnig Hoffmann syndromes (W.H.). Control group of 4 normal children. In the control group and 14 relapsing bronchitis normal tubular pattern (9 + 2) and ciliary conformation were present; in the relapsing broncho-pneumonic syndrome group, 2 children presented deficiency of some brace dynein arms = chronic inflammation; in the Nemaline myopathy we noted total lack of interior dynein arms with random cilia orientation; in the 3 W.H. = normal cilia conformation. The authors noted for the heavy respiratory insufficiency of the Nemaline myopathy the presence of immotile cilia and discuss possibility that in all Nemaline myopathy syndromes this association may be present (a new syndrome?). The W.H. myopathy is characterized by normal cilia aspects and the respiratory insufficiency is caused by muscular respiratory insufficiency secondary to anterior spinal corn horn. In the Nemaline myopathy the same genetic defect can act in muscular fibres and on dynain arms (cytoskeleton). The technique described is suitable for processing epithelial brushings from other parts of the respiratory tract.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗