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We describe here a case of a man with Kartagener's syndrome, whose parents were cousins each other, associated with infundibular pulmonic stenosis, chronic renal failure and azoospermia. These combined abnormalities have not been previously reported in one patient with Kartagener's syndrome, as far as English literatures are concerned.
Nitric oxide (NO) plays role in a great range of important functions in the organism, such as vasodilatation, relaxation of muscles, neurotransmission, neuromediation, and host defense reactions. In the upper airways, nasal cavities and paranasal sinuses are the main sources of this biological mediator. Although the exact role of NO in nasal physiology remains poorly understood, the functions are thought to be host defense, ciliary motility and improved ventilation-perfusion ratio in the lungs by auto-inhalation. Low NO concentrations were reported in certain diseases such as primary ciliary dyskinesia, cystic fibrosis, and acute and chronic maxillary sinusitis whereas high concentrations were detected in upper airway infection, allergic rhinitis and nasal polyposis. Additionally this ubiquitous radical is being implicated in the regulation of cochlear blood flow, sensorineural hearing loss, middle ear effusions, and outer hair cell and vestibular functions. Solid tumors is another area where NO appears to have both tumor-promoting and tumor-inhibiting effects. The presence of NO with high levels within the nose and paranasal sinuses makes it reasonable to believe that this pluripotent gas is involved in a variety of physiological as well as pathophysiological events in the airways. Although NO has an ever-increasing role in various areas related to the practice of otolaryngology, further research is required to understand fully the role of NO in the upper airways.
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OBJECTIVE: The purpose of this study was to determine whether the pattern and distribution of bronchiectasis shown on CT scans can be used to discriminate between idiopathic cases and those with an identifiable cause. MATERIALS AND METHODS: The CT scans of 168 patients with chronic purulent sputum production and who were suspected of having bronchiectasis were analyzed (117 patients with idiopathic bronchiectasis, 15 with allergic bronchopulmonary aspergillosis, 15 with hypogammaglobulinemia, 15 with impaired mucociliary clearance, and seven with cystic fibrosis diagnosed in adult life). The scans were analyzed in random order by two observers. The extent, site, type, and lobar distribution of bronchiectasis and the severity of bronchial dilatation and bronchial wall thickening were scored. The frequency of these features in the known-cause groups was compared with that in the idiopathic group to identify any significant differences. RESULTS: Compared with idiopathic bronchiectasis, no significant lobar predominance was seen in any of the known-cause groups, apart from a higher frequency of lower lobe involvement in the patients with syndromes of impaired mucociliary clearance (p < .02). The bronchiectasis of allergic bronchopulmonary aspergillosis and adult cystic fibrosis was more often widespread (five or six lobes involved (p < .001 and p < .01, respectively) than idiopathic bronchiectasis. Central bronchiectasis was more common in allergic bronchopulmonary aspergillosis (p < .005), although the sensitivity when this was used as a diagnostic feature was only 37%. In all groups, cylindrical bronchiectasis was the most common type, with varicose and cystic bronchiectasis occurring more frequently in allergic bronchopulmonary aspergillosis (p < .01). On multiple regression analysis, allergic bronchopulmonary aspergillosis and adult cystic fibrosis showed more extensive disease than idiopathic bronchiectasis (p < .0005 and p < .001, respectively), independent of other CT features. In hypogammaglobulinemia, dilatation of the bronchial lumen was less than in idiopathic bronchiectasis (p < .02) independent of disease extent and bronchial wall thickness. CONCLUSION: Although differences in distribution and morphology of bronchiectasis may be seen on CT scans in groups of patients with bronchiectasis of different causes, CT findings applied to individual patients are of limited value in discriminating between idiopathic bronchiectasis and bronchiectasis of various known causes.
Primary ciliary dyskinesia is a congenital condition that may cause chronic rhinitis and bronchopneumonia. Primary ciliary dyskinesia may be diagnosed by induction of ciliogenesis by use of in vitro cell culture. Induction of ciliogenesis allows for differentiation between primary and secondary ciliary dyskinesia.
Mucociliary transport is one of the most important local defense mechanisms of the airways, but it is prone to many and frequent acquired abnormalities and to inherited abnormalities. These abnormalities result in basic physiologic disturbances leading to a number of respiratory symptoms and signs. In order to critically evaluate the diagnostic value of parameters of this mucociliary cascade, the results of ciliary investigations in over 500 individuals (controls, acquired, and inherited abnormalities) were reviewed. Ciliary beat frequency, ciliary coordination and ultrastructural abnormalities were measured and evaluated in biopsies and after ciliogenesis in culture. There is a considerable overlap for all investigated parameters in biopsy material of controls, secondary, and primary ciliary dyskinesia. There is not one parameter that is diagnostic for primary ciliary dyskinesia or can be used as an exclusion criterion. After ciliogenesis in culture, cilia are always completely normal except for the inherited abnormalities. Absence of coordinated ciliary activity after ciliogenesis in culture is 100% sensitive and specific for the diagnosis of primary ciliary dyskinesia.
Diagnosing Primary Ciliary Dyskinesia can often be difficult. Physical findings suggest the disease, but definitive diagnosis should be made with a ciliary biopsy. Twenty biopsies were obtained from 16 patients and all underwent both light and electron microscopic examination. In 8/20 (40%) there was a discrepancy between the different imaging techniques. Therefore, light microscopy should be used to assess adequacy of biopsy and motion of the cilia along with electron microscopy to examine ultrastructure.
Mucociliary transport is one of the most important defense mechanisms of the airway. Mucociliary transport time or rate, as measured using the saccharin test or the radioisotope technique, respectively, is clinically the most relevant parameter, although subject to large intra- and interindividual variability. There is no correlation between mucociliary transport in vivo and ciliary beat frequency ex vivo. Preliminary evidence demonstrates that mucociliary transport correlates with ciliary structure and orientation as investigated with transmission and scanning electron microscopy. A correlation is presented between ciliary beat frequency and secondary ciliary abnormalities. This correlation can best be described according to the logistic sigmoid model (r = 0.69). Based on these functional data, an ultrastructural distinction is proposed among normal (less than 5%), light (5 to 15%), moderate (15 to 25%), and severe (more than 25%) secondary ciliary dyskinesia.
In chronic sinusitis, the disturbance of mucociliary transport explicitly represents the pathology of the mucous membrane. It was established by a photo-electric method that in this disease, mucociliary transport is disturbed as the result of not only change in secretory function but also a decrease in the frequency of ciliary beating. In the in vitro experiment using mucous membrane samples from 10 cases of chronic sinusitis, the frequency of ciliary beating was lowered slightly to about 409 +/- 84 beats per minute at 30 degrees C. In the most active phase of action, human cilia in sinusitis beat about 720 times per minute at 30 degrees C in vitro. The ciliary actions of the mucous membrane in human chronic sinusitis were classified into three types: 1) gathering type, 2) bifocal type, and 3) scattering type.
The previous paper (Part I) in our study described how a decrease in the frequency of ciliary beating was responsible for the deceleration of mucociliary transport in chronic sinusitis. In the present work, it was examined whether or not the reduction in ciliary beating frequency is associated with morphological changes in the mucosal epithelium. As a result, a very close correlation was found between them: the more severe the morphological changes, the more the beating frequency was reduced. The morphological changes of the mucosal epithelium in chronic sinusitis were swelling of the ciliary membrane, formation of compound cilia, dropping of epithelial cells, and metaplasia of a squamous epithelium. On the basis of these observations on ciliary beating and the fine structure of the mucosal epithelium, the severity of pathological change of the mucous membrane in chronic sinusitis was graded.
It is known that beta-adrenergic stimulants enhance mucociliary transport in the respiratory tract. The present study clearly indicated that such an enhancement resulted from not only the alteration in secretory function but also the direct stimulation of ciliated cells. However, the enhancement of ciliary beating depended on the morphological changes in ciliated cells. On the other hand, the effect of terbutaline aerosol, a beta-adrenergic stimulant, on patients with chronic sinusitis was evaluated by paranasal sinus radiography. Consequently, marked improvements in shadows were achieved after 4 weeks in 40% of the cases examined.
The effect of HR-6 solution containing adenosine triphosphate 10 mg/ml on the impaired nasal mucocililary function in 13 patients was studied. The nasal mucociliary function measured with the radioisotopic method using 99Tc-labelled human serum albumin as a tracer substance was markedly impaired in all patients. The patients had had symptoms of upper and/or lower respiratory tract infections for 8 1/2 years, on the average. The nasal mucociliary function ranged from 0 to 5.0 mm/minute, mean 2.0 mm/minute. In this single dose study the test drug improved nasal mucociliary function by at least 1.4 mm/minute in 77% of the patients and by at least 2.8 mm/minute in 54% of the patients. This preliminary study indicates the need for further examinations with the HR-6 in patients with impaired mucociliary function.
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.
Cultures of respiratory epithelial cells were obtained from nasal polyps collected in patients with and without primary ciliary defect. The ciliary beating frequency and the ciliary beating heterogeneity were determined on native and cultured tissues. We observed a significantly higher (p < 0.01) ciliary beating frequency of cultured ciliated cells, when compared with ciliated cells from the native tissue. The ciliary beating frequency of the cultured ciliated cells from the patient with primary defect (7.9 +/- 2.1 Hz) was significantly lower when compared with the beating frequency of the ciliated cells from the control subject (12.4 +/- 2.0 Hz). In addition, the percentage of ciliated cells characterized by a beating frequency lower than 8 Hz was 90.7% in the native tissue and 47.5% in the cultured tissue from the patient with ciliary primary defect. In the patient without ciliary primary defect, 90% of the cultured ciliated cells had a homogeneous ciliary beating, whereas in the patient with primary ciliary defect, only 47% of the ciliated cells had a homogeneous ciliary beating. These results suggest that the culture of respiratory cells associated with the functional activity measurement of the ciliated cells represent another way of precisely determining the extent of the primary ciliary dyskinesia defect.
The ciliary ultrastructure studied by scanning electron microscopy (SEM), nasal mucociliary transport rate (MTR) measured by a radioisotopic method and their correlation with each other were studied in 56 patients with recurrent or chronic respiratory infections. Patients were divided into three groups according to MTR. The number of pathological SEM findings increased remarkably in groups with moderate or poor MTR. Loss of ciliated cells and microvilli were seen in 86% of specimens with poor MTR, in 71% with moderate MTR, and in 50% with good MTR. There was ciliary disorientation in 45% of cases with poor MTR compared with 25% in cases with good MTR. Epithelial metaplasia and the number of short cilia increased in groups with poor or moderate MTR.
Ciliary beat frequency (CBF) was measured at room temperature in nasal epithelial cells from 10 control individuals at multiple time points during sequential monolayer-suspension culture. CBF was 8.4 +/- 1.6 Hz in the biopsy and decreased in monolayer culture to approximately 6 Hz. During monolayer culture the interindividual variability was increased (3.0 Hz), but not the intercellular and intracellular variability. Immediately after ciliogenesis in suspension culture, CBF was approximately 8 Hz, with a low interindividual variability. After 3 weeks in suspension culture CBF was 8.6 +/- 0.9 Hz. There was no difference in intercellular and intracellular variability of CBF in the different culture conditions. Ciliogenesis in the sequential monolayer-suspension culture results in quantitative normal ciliary activity, with low variability. The most important parameter of ciliary activity that changes during ciliary deterioration and ciliogenesis is the CBF itself.
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In subjects with an inherited lack of mucociliary transport, so called immotile-cilia syndrome (ICS), coughing effectively clears particles deposited in larger airways of the tracheobronchial region. The present study investigated clearance in smaller airways of 111In-labeled 6-microm (aerodynamic diameter) monodisperse Teflon particles in six subjects with ICS. The particles were inhaled at an extremely slow flow, 0.05 L/s. Theoretical calculations and experimental data in healthy subjects using this slow flow support particle deposition mainly in smaller ciliated airways, i.e., in bronchioli (generations 12-16). This contrasts with the more centrally deposited pattern obtained using a normal inhalation flow, 0.5 L/s. Lung retention was measured at 0, 24, 48,72 and 96 h. Clearance was significant every 24 h measured over the first 72 h, whereupon it slowed down. The fractions of retained particles were significantly (p < .01) larger than those found for healthy subjects using the slow inhalation flow and those found for ICS subjects using a normal inhalation flow. The results indicate that clearance of particles in smaller airways is incomplete and that cough cannot fully compensate for the lack of mucociliary transport in this region.