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PubMed1984

Absence of cilia and basal bodies with predominance of brush cells in the respiratory mucosa from a patient with immotile cilia syndrome.

his report describes the ultrastructural alterations observed in the tracheal epithelium of a 13-year-old male with a history of recurrent pneumonia, chronic bronchitis, chronic otitis media, and situs inversus. The epithelium consisted of globlet and basal cells with many columnar cells that lacked cilia and basal bodies. The surface of these cells had regular microvilli and cytoplasmic features typical of brush cells.

Adolescent↗
PubMed1985

Atypical cilia in hyperplastic, metaplastic, and dysplastic human bronchial mucosa.

Twenty-two cases displaying potentially precancerous epithelial changes of the bronchial mucosa--including basal and goblet cell hyperplasia, epidermoid metaplasia, and dysplasia--showed a wide variety of cilial abnormalities on the ultrastructural level. The changes comprised abnormal configurations of the ciliary plasma membrane, variations in the amount of ciliary matrix, disorganization of basal bodies, and diverse alterations of the axonemal microtubular pattern. More than one such alteration was observed in one and the same case, and there was no correlation with the actual type of epithelial lesion nor the degree of dysplasia. These changes, interpreted as abnormal ciliary regeneration in severely damaged ciliated cells, are potentially reversible and seem to represent a nonspecific response of the affected cells to an ample spectrum of noxes. Their diagnostic significance is poor, especially with regard to the modification of the axonemal microtubular pattern, unless the entire clinical picture suggests an immotile cilia syndrome.

Adult↗
PubMed1987

Ultrastructural diagnosis in the immotile cilia syndrome.

This article reports on the experience with 38 biopsies from nasal mucosa, submitted with the question of immotile cilia syndrome. Fixation in glutaraldehyde with MgSO4 is preferable. At least 50 cilia should be scrutinized in the electron microscope, and dynein arms, radial spokes, sheaths, nexin links and orientation should be tabulated. Six cases displayed virtual absence of inner and outer dynein arms. The orientation of these cilia was random. Two of the patients had situs inversus. In biopsies considered not to represent the immotile cilia syndrome, about four inner and seven outer dynein arms were found per cilium.

Adolescent↗
PubMedDate not supplied

Ciliary ultrastructure in primary ciliary dyskinesia and other chronic respiratory conditions: the relevance of microtubular abnormalities.

Twenty-eight subjects with chronic respiratory disease were investigated for clinical data, ciliary beat frequency of nasal mucosa (10 cases), and ciliary ultrastructure. The cases were divided into two groups: those considered compatible with primary ciliary dyskinesia (genetic), and those not fitting into this category (others). A case was defined as genetic if one or more of the following were present: dextrocardia, ciliary beat frequency less than 10 Hz, or an average dynein arm count (outer, inner, or both) of less than two per ciliary cross-section. In each of the genetic cases at least two of these parameters were present. The percentage of malformed microtubules was calculated from the total number of evaluated cross-sections for each case. Ciliary microtubular abnormalities of any kind were no more frequent in cases of primary ciliary dyskinesia than in other cases. The same was true for transposition and radial spoke defects.

Adolescent↗
PubMedDate not supplied

The role of electron microscopy in evaluating ciliary dysfunction: report of a workshop.

This report summarizes the proceedings of a workshop organized with the purpose of bringing together many of those with substantial experience in this troublesome area of pathology for an active interchange of ideas, opinions, problems, and solutions. Recognition was given the fact that current knowledge and technical capabilities are woefully inadequate for dealing with the diagnostic questions now being asked. Until such time as these inadequacies can be remedied, a very conservative approach to the interpretation of ultrastructural studies is advocated.

Adolescent↗
PubMedDate not supplied

Microtubular discontinuities as acquired ciliary defects in airway epithelium of patients with chronic respiratory diseases.

A critical relationship exists between ordered ciliary ultrastructure and optimal mucociliary clearance in the respiratory airways. Structurally defective cilia derived from heritable syndromes or from epithelial cell injury may promote or exacerbate chronic disease processes. A lesion of airway epithelial cilia characterized by microtubular discontinuities and previously associated with primary ciliary dyskinesia (PCD) has been documented in other forms of chronic airways diseases, including cystic fibrosis (CF). Nasal cilia obtained by curettage of the inferior nasal turbinate from 89 patients without CF but exhibiting symptoms favoring PCD were evaluated by transmission electron microscopy. Of the 89 patients in the study group, 19 (21.4%) were diagnosed with PCD. Among the PCD patients, 16 (84.2%) exhibited microtubular discontinuities. Nine patients from this group without ultrastructural evidence of PCD also exhibited these defects, however. Furthermore, seven of eight nasal biopsy specimens from patients with CF in a separate disease control group exhibited microtubular discontinuities. Microtubular discontinuities were quantitatively negligible among control groups of healthy human subjects and individuals experimentally and naturally subjected to acute airway injury. These data provide evidence that ciliary microtubular discontinuities represent acquired ciliary defects reflective of chronic airway disease injury and are not components of a primary structural abnormality in PCD.

Adult↗
PubMedDate not supplied

Mucous gel transport in a simulated cough machine: effects of longitudinal grooves representing spacings between arrays of cilia.

Under some conditions, such as inhalation injury, an array of longitudinal channels without cilia may be formed on the epithelial surface, affecting mucus transport in the lung during coughing. Moreover, in certain cases of the diseased state of the lung, immotile cilia remain embedded in the serous fluid and may form channels in the direction of air motion during coughing, providing a resistance-free pathway for serous layer fluid and assist in mucus clearance. To understand this phenomenon, we have conducted experiments with mucous gel simulants (MGS) in a simulated cough machine (SCM) using soap solution as a serous layer simulant (SLS). The channel structure of the airway surface was modeled by introducing bottom plates with longitudinal grooves about 10 cm long having different depths, widths and number of grooves. It was shown that mucous gel transport increases as the cross-sectional area occupied by channel grooves increases. The effects of sinusoidal constriction and mucous gel filance were also investigated in this model system. It was found that mucous gel transport increases in the constricted case (minimum gap, 3 mm, and maximum gap, 9 mm) in comparison with that in the parallel case (constriction gap of 9 mm). The effect of increasing the mucous gel filance was to decrease the transport,, as reported in previous studies.

Cilia↗
PubMed1991

[Ultrastructural abnormalities of the cilia in human nasal epithelia].

Nasal mucosal cilia were observed with electron microscope in 14 patients with immotile cilia syndrome (ICS), 9 with nasal papilloma (NP), 23 with sinobronchial syndrome (SB), 2 with sinusitis combined with dextrocardia (SC), 1 with Kartagener's syndrome (KS), and 5 normal controls (C). Abnormalities such as complex cilia, cilia with abnormal axonemes and cilia with randomly oriented central microtubules were frequently found in the groups of ICS (8.1%) and NP (10.4%) while less in other groups: SB (4.9%), SC (5.3%), KS (4.7%) and C (3.9%). The percentage of cilia with defective dynein arms (DA) was the highest in the ICS group (94.0%), followed by the groups of SC (53.7%), SB (47.5%), NP (41.2%), C (35.8%) and KS (33.3%). The ICS group was found to be the largest in the number of defective DA per a cilium (4.1), followed by the groups of NP (1.0), SB (0.6), SC (0.7), KS (0.4) and C (0.4). Increased rates of defective DA were also recognized in cilia of tracheal mucosa and flagella of sperm in 7 patients with ICS examined. In conclusion, neither abnormal cilia nor defective DA of cilia are specific findings for ICS. However, when we observe these findings in high percentage in nasal mucosa as well as in other organs, we may define this condition as ICS.

Adult↗
PubMed2001

Ciliary dyskinesia associated with hydrocephalus and mental retardation in a Jordanian family.

OBJECTIVE: To describe the presentation and genetic transmission of ciliary dyskinesia syndrome associated with hydrocephalus and mental retardation in 3 generations of a family. PATIENTS AND METHODS: A large Jordanian family included 9 individuals in 3 generations with recurrent pulmonary infections; 4 male siblings have been diagnosed as having mental retardation, and a maternal uncle was believed to have been similarly affected. Chromosome analysis of the family showed a normal karyotype. RESULTS: Electron microscopy of the nasal cilia from 3 affected siblings showed features of primary ciliary dyskinesia. Computed tomographic scans of the brains of all 4 affected siblings showed hydrocephalus. CONCLUSIONS: The recurrent pulmonary infections and hydrocephalus in this large Jordanian family are likely related to ciliary dyskinesia, which appears to follow an autosomal recessive mode of inheritance. The unusual presentation of ciliary dyskinesia, hydrocephalus, and mental retardation may be due to a new genetic mutation.

Abnormalities, Multiple↗
PubMed2004

Primary ciliary dyskinesia in a Staffordshire bull terrier.

Primary ciliary dyskinesia (PCD) is a diverse group of inherited structural and functional abnormalities of the respiratory and other cilia, which results in recurrent respiratory tract infections. Primary ciliary dyskinesia was diagnosed in a 14-week old Staffordshire bull terrier that had a history of respiratory disease from 7 weeks of age. Pneumonia was diagnosed on thoracic radiographs and transtracheal aspirate. Transmission electron microscopy of the bronchi and trachea indicated the presence of both primary and secondary ciliary dyskinesia. The most prominent primary defects consisted of absent inner dyneim arms, absent radial spokes and absence of the central microtubules. These defects accounted for 62% of the total number of cross-sections screened. Non-specific ciliary abnormalities encountered most often were compound cilia, swollen cilia, addition/deletion of peripheral doublets and disorganised axonemes (26%). To the authors' knowledge, this is the first case of PCD described in the Staffordshire bull terrier and the first report of PCD in South Africa.

Animals↗
PubMed2004

Exhaled markers of inflammatory lung diseases: ready for routine monitoring?

Assessing airway inflammation is important for investigating the underlying mechanisms of many lung diseases, including asthma and chronic obstructive pulmonary disease (COPD). Yet these are not measured directly in routine clinical practice because of the difficulties in monitoring inflammation. The presence and type of airway inflammation can be difficult to detect clinically, and may result in delays in initiating appropriate therapy. Non-invasive monitoring may assist in differential diagnosis of lung diseases, assessment of their severity and response to treatment. There is increasing evidence that breath analysis may have an important place in the diagnosis and clinical management of asthma, COPD, primary ciliary dyskinesia (PCD) and other major lung disease. The article reviews whether current noninvasive measurements of exhaled gases, such as nitric oxide (NO), hydrocarbons, inflammatory markers exhaled breath condensate (EBC) are ready for routine use in clinical practice.

Alkanes↗
PubMed1999

Positive expiratory pressure in the physiotherapeutic management of primary ciliary dyskinesia in paediatric age.

The clinical features of primary ciliary dyskinesia are related to congenital impairment of mucociliary clearance, which represents the common aetiopathogenetic denominator. If untreated, the disease evolves with progressive, slowly developing, structural lung damage. Chest physiotherapy may play a relevant role in the therapeutic management of this pathological condition, owing to the improvement in mucus transport and the recruitment of the obstructed lung periphery. This may lead to modification of the natural history of the disease, if early intervention is implemented. The rationale behind this intervention and the advantages/disadvantages of the two main positive expiratory pressure techniques used are discussed here.

Child↗
PubMed1999

Asymmetry of cilia and of mice and men.

Evidence is given for the opinion that cilia in the early embryo, by their work, determine the laterality of the body; without ciliary work body laterality would be randomized. More exactly, monocilia in the primitive node are responsible for this determination. They have been described as being of the 9+0 type, but with dynein arms and with a gyrating movement. The orientation of the monocilia on the epithelium is of no importance but the direction of their gyration is, as may also be the shape of the node. The chirality of the cilia is thus reflected directly in the asymmetry of the body. The dynein arms go clockwise as seen from the base to tip and the ciliary rotation is in the same direction. The resulting waterflow is towards the left and so is the movement of the forming heart. In most subgroups of the immotile-cilia syndrome this mechanism does not work and equally many individuals will be born with situs inversus as with situs solitus. An exception is the immotile-cilia subgroup, named 'microtubule transposition', which is characterized by all cilia having a 9+0 structure throughout most of their length.

Animals↗
PubMed1999

[Sinobronchial syndrome].

A condition characterized by chronic paranasal sinusitis and simultaneous chronic pulmonary infection was recognized and reported as long ago as the early 1900's. This condition was termed "sinobronchial syndrome" (SBS) in 1966. Many types of SBS are reported including Kartagener's syndrome/primary ciliary dyskinesia, Young's syndrome, bare-lymphocyte syndrome, cystic fibrosis and various types of immunoglobulin deficiency. SBS is also found in Japan. Among them, the most important is diffuse panbronchiolitis. The pathogenesis of SBS might involve inherited predisposition, probably accompanied by some deficiencies in the host defense of the respiratory system.

Chronic Disease↗
PubMed1999

[Primary ciliary dyskinesia treated with living-donor lobar lung transplantation].

We report a case of primary ciliary dyskinesia in which a living-donor lobar lung transplant was performed. A 24-year-old woman with a diagnosis of primary ciliary dyskinesia and bronchiectasis was admitted to Shinshu University Hospital because of persistent dyspnea and pyrexia over a period of 4 months. Although she was given various antibiotics, neutrophilia, elevated plasma C-reactive protein (CRP) levels, and respiratory failure persisted. Chest roentgenograms and computed tomography disclosed severe bronchiectasis and diffuse infiltrative shadows in both lung fields. Pseudomonas aeruginosa was detected in a sputum culture. Although a variety of conventional therapies were administered, the patient's oxygenation progressively deteriorated. She was intubated and assisted by mechanical ventilation. The patient and her family proposed lung transplantation, and we concluded that a living-donor lobar lung transplant would be a suitable treatment for her disease. We transported the patient to Okayama University Hospital by helicopter 10 days after intubation. A living-donor lobar lung transplant was successfully performed with lung tissues donated by the patient's mother and sister for each transplant site.

Adult↗
PubMed1999

[First successful bilateral living-donor lobar lung transplantation in Japan].

No successful lung transplantation has been reported in Japan until recently when we performed the first successful bilateral living-donor lobar lung transplantation. A 24-year-old woman with primary ciliary dyskinesia began experiencing severe respiratory insufficiency and required mechanical ventilation. On October 28, 1998, she underwent bilateral living-donor lobar transplantation, receiving her sister's lower right lobe and her mother's left lower lobe under cardiopulmonary bypass. The patient was discharged from the hospital 61 days after transplantation. Six months postoperatively, she has returned to normal life and is able to carry out daily activities. She is in good physical condition with a vital capacity of 1.77 L.

Adult↗