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PubMed1990

Ciliary activity and pollution.

The mucociliary clearance is an important part of the nonspecific defense mechanism of the human airways. Coordinated beats of cilia in the nose, trachea, and bronchi propel the mucous layer toward the pharynx, and with it inhaled microorganisms and other particles captured in the mucus. Normal function of this system depends on the efficacy of the ciliary beating and the properties of mucus. Primary ciliary dyskinesia--a congenital respiratory disease characterized by ultrastructural defects and motility disturbances of cilia--has provided us with valuable information about the role the mucociliary function plays in the protection against harmful agents in the inhaled air. Secondary ciliary dyskinesia is described in inflammatory disorders of the respiratory tract, but also some components in air pollution results in malfunction of cilia, damage of ciliated epithelium, or alteration in the mucus. This review will focus on investigations found in the literature concerning the influence of pollutants on ciliary activity. The methods applied will be evaluated.

Air Pollution↗
PubMed1990

[Clinical and ultrastructural study on primary ciliary dyskinesia].

We evaluated laboratory and radiological findings and examined tracheobronchial cilia by transmission electron microscopy in 9 patients with primary ciliary dyskinesia (PCD), in order to elucidate the clinical pictures of PCD and the relationship between PCD and diffuse panbronchiolitis (DPB) which was proposed as a new disease entity in Japan in 1969. The clinical pictures of our PCD patients were almost the same as that already described in several articles in Europe and North America; early onset of respiratory symptoms, high incidence of chronic sinusitis and otitis media exudative as well as infertility, continuous infections in the lower respiratory tracts (Hemophilus influenzae, Pseudomonas aeruginosa etc.). Tracheobronchial cilia obtained by brushing technique were immotile (6 out of 8 patients) or dyskinetic (2 out of 8 patients). Ultrastructural study of cilia revealed the lack of dynein arms in all patients: the lack of both outer and inner arms (4 patients), the lack of outer arms (2 patients), the lack of inner arms (2 patients). Chest X-ray films revealed situs inversus in six out of nine patients. According to the radiological findings (chest X-ray film, CT-scan, bronchogram), the patients were divided into three groups; I: localized bronchiectasis (5 patients), II: diffuse micronodular lesions without definite bronchiectasis (3 patients), III: diffuse micronodular lesions with bronchiectasis (1 patient). Two patients of the second group satisfied the clinical diagnostic criteria for DPB (Chest 83:63, 1983). In conclusion, PCD can cause a variety of respiratory tract lesions such as bronchiectasis, DPB and other types of peripheral airway disorders.

Adult↗
PubMed1994

[Asthenozoospermia--a multifactorial symptom].

For the clarification of sperm motility disorders, changes in the flagella must be differentiated from influences exerted by the seminal fluid and extrinsic factors. The disorders of the flagella may be testicular or epididymal in origin. Only by taking account of these aspects of etiology and pathogenesis, will a rational therapeutic approach be possible.

Ciliary Motility Disorders↗
PubMed2005

Neutrophils potentiate platinum-mediated injury to human ciliated epithelium in vitro.

Exposure to platinum salts, such as may occur in the platinum refining industry, can be associated with the development of airway disorders such as asthma. However, there have been no studies investigating the direct effects of platinum salts on human ciliated epithelium. We have investigated the effects of platinic chloride on human ciliated epithelium, obtained by brushing the inferior nasal turbinate of healthy human volunteers. Ciliary beat frequency was measured using a phototransistor technique, and damage to the structural integrity of the epithelium was measured using a visual scoring index. Platinic chloride at concentrations between 0.25 and 25 microM caused a dose-dependent slowing of ciliary beating and damage to the structural integrity of the epithelium. These direct injurious effects were not affected by catalase, but were almost completely attenuated by preincubation of the epithelium with cysteine. The effects of platinic chloride on ciliary beating and structural integrity were enhanced by the presence of neutrophils and were partially attenuated by preincubation of the epithelial strips with catalase, suggesting that the direct effects of the metal were enhanced in this experimental system by reactive oxidants produced by activated neutrophils. This study documents that platinum salts have an injurious effect on human ciliated epithelium in vitro. If such effects also occur in vivo they may play a role, at least partly, in the pathogenesis of airway disorders that may manifest in exposed workers.

Acridines↗
PubMed1993

Usher syndrome type I associated with bronchiectasis and immotile nasal cilia in two brothers.

Usher syndrome type I is an autosomal recessive disease characterised by congenital sensorineural deafness, involvement of the vestibular system, and progressive visual loss owing to retinitis pigmentosa. Here we report the association of this disease with bronchiectasis, chronic sinusitis, and reduced nasal mucociliary clearance in two sibs and we suggest Usher syndrome type I could be a primary ciliary disorder.

Abnormalities, Multiple↗
PubMed1997

Otological manifestations of primary ciliary dyskinesia.

Primary ciliary dyskinesia is a hereditary defect in the ultrastructure of cilia, leading to poor ciliary motility. The sinonasal and the bronchial manifestations of the disease are well documented; whereas its otological aspects have received less attention. In this report, we describe the clinical profile of 16 patients with primary ciliary dyskinesia laying particular emphasis on the otological manifestations. All children (11 patients) had bilateral otitis media with effusion. Of the five adults, three had tympanosclerosis; one had bilateral cholesteatoma; and one patient had bilateral keratosis obturans in combination with tympanosclerosis. Hearing improvement and a dry ear was achieved in all the children treated by tympanostomy tube insertion. The study suggests that otitis media is a prominent feature of this disorder. Most subjects suffer from protracted bilateral otitis media with effusion throughout childhood.

Adolescent↗
PubMed2002

[Ultrastructural changes of the nasal mucosa in primary ciliary dyskinesia].

Primary ciliary dyskinesia syndrome (PCD) is a rare, autosomal receive disorder. Kartagener's syndrome is a subgroup of the PCD with situs inversus, bronchiectasis, and sinusitis. The symptoms results from an abnormal ultrastructural morphology of the cilia such as absence of dynein arms and other changes. As a consequence ciliary motility is disturbed. A 25-year-old man was examined because he suffered from recurrent severe pneumonia and Aspergillus infections of the lungs. On electron micrographs, ciliary abnormalities including deficiency of inner and outer dynein arms, dysmorphic outer dynein arms, and disorientation of the cilia were demonstrated. The diagnosis of PCD requires electron-microscopic investigations of the ciliated mucosa. Special attention should be given to ultrastructural changes of nasal or bronchial mucosa if a young patient suffers from recurrent severe respiratory infections.

Adult↗
PubMed2006

The humidification and filtration functions of the airways.

The mucociliary elevator is a highly evolved organ that humidifies inspired gases and protects the lungs from particulate, chemical, and microbiologic matter. Studies of disorders mucus and ciliary function have improved the understanding of this forgotten organ. The clinical implications of this understanding have yet to be explored.

Cilia↗
PubMed1999

[Adult bronchiectasis revealing familial ciliary anomaly].

We report a case of bronchiectasis in a 26-year-old man associated with the following congenital abnormalities: deafness, purulent bronchorrhea, nasal polyps, dysmorphic physical pattern and chronic sinusitis. Situs inversus was absent. A sampling was performed on the posterior nasal mucous membrane and displayed structural ciliary abnormality: a deficiency of the intern dynein-arm. The patient's bother was affected and had similar features: congenital bronchiectasis, deafness, mental deficiency and sinusitis. Young's syndrome was relevant in this case. Hereditary ciliary dyskinesia should be considered in adults with bronchiectasis together with rhinologic and alimentary canal disorders. Nasal biopsies are safe and allow cilia examination.

Adult↗
PubMed1984

The dyskinetic cilia syndrome in childhood. Modifications of ultrastructural patterns.

The syndrome of cilia dyskinesia is known as a heterogenous ciliary dysfunction caused by morphological defects of the dynein arms, the nexin links, the radial spokes and by the transposition of microtubules. Supernumerary tubules have been regarded as acquired morphological defects on the background of other bronchopathies. The report of a 9-year-old girl with the clinical signs of ciliary dyskinesis is considered to be an attribution to the clinical and pathological features of this syndrome. The girl's history of chronic bronchopulmonary infections and nasal polyposis resistant to therapy made her suspected to be ill of Kartagener's syndrome. The results of ultrastructural investigations of the mucosa from ciliated epithelium revealed a ciliary structural defect in the bronchi as well as in the nose and the sinuses with supernumerary microtubular doublets and singles, a decentration of the central tubules and shortened dynein arms. The regularity of the electron optical abnormalities implicates a systemic disorder of ciliated epithelium, which is to be summarized to the syndrome of cilia dyskinesis.

Bronchi↗
PubMedDate not supplied

[The absence of the internal arms of dynein as a cause of the immotile cilia syndrome].

Two children aged 9 and 8 years respectively presenting upper and lower respiratory tract infections from early childhood are presented. In both, nasal mucociliary transport was impaired (greater than 30 min) and electron microscopic examination showed cilia devoided of the inner dynein arms. This ciliary defect is one of those recognized as a cause of the Immotile Cilia Syndrome but is very rare, having been described only once in a boy affected with Kartagener's syndrome, which in turn has been reclassified in the Immotile Cilia Syndrome. Therefore the absence of inner dynein arms observed by us in the respiratory cilia of these two children clearly indicated that similarly to many other genetic disorders the immotile cilia syndrome may be determined by many defects in the axonemal structure.

Child↗
PubMed1993

A treatment for primary ciliary dyskinesia: efficacy of functional endoscopic sinus surgery.

Primary ciliary dyskinesia (PCD) is an inherited disorder manifested in children as chronic otomastoiditis, recurrent pneumonia, and chronic sinusitis. The failure of the ciliary beat pattern to effectively function in the respiratory tract produces stasis of secretions with secondary inflammation, edema, and infection. The authors report three young children with PCD who presented with variable severities of symptoms. Each had the aforementioned respiratory tract problems. The child with the most severe symptomatology was treated with a variety of medical options, including long-term gamma globulin injections, but hospitalizations persisted twice per month because of severe sinopulmonary illness. All three of the children underwent functional endoscopic sinus surgery (FESS). In addition, two children received pressure equalization (PE) tubes. One child required a revision procedure. Their surgical outcomes are discussed. Follow-up of FESS in three children with this disorder shows a marked improvement in symptomatology with a decreased incidence of hospitalization and a somewhat decreased need for medical therapy.

Child, Preschool↗
PubMed1989

Congenital ciliary aplasia in two siblings. A primitive disregulation of ciliogenesis?

Congenital ciliary aplasia was demonstrated in two siblings with clinical history of primary ciliary dyskinesia. Ultrastructural histochemistry of successive bronchial biopsies revealed the predominance of immature mucous cells and the total absence of ciliated or preciliated cells in the respiratory epithelium. This original disorder may represent a unique variant of primary ciliary dyskinesia with primitive disregulation of ciliogenesis.

Biopsy↗
PubMed2004

Cilia and flagella revealed: from flagellar assembly in Chlamydomonas to human obesity disorders.

The recent identification in Chlamydomonas of the intraflagellar transport machinery that assembles cilia and flagella has triggered a renaissance of interest in these organelles that transcends studies on their well-characterized ability to move. New studies on several fronts have revealed that the machinery for flagellar assembly/disassembly is regulated by homologs of mitotic proteins, that cilia play essential roles in sensory transduction, and that mutations in cilia/basal body proteins are responsible for cilia-related human disorders from polycystic kidney disease to a syndrome associated with obesity, hypertension, and diabetes.

Animals↗
PubMed1983

[Disorders in mucociliary transport. Primary ciliary dyskinesia].

A survey is given of the mucociliary transport in the airways. Disturbances in rheologic qualities (viscosity and elasticity) of the mucuslayer may adversely influence mucociliary transport. This plays a role in cystic fibrosis and chronic bronchitis of other etiology. Disturbances in ciliary action can be secondary to for instance viral infections, inhalated substances and drugs. Inborn errors in the architecture of the cilia may cause immotility or ineffective motility of cilia, resulting in frequent airway infections. The clinical characteristics, diagnostic possibilities and a proposal for therapeutic measures are discussed.

Adult↗