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PubMedDate not supplied

[Neutrophil chemotaxis. II. Clinical implications and therapeutic indications in children].

Defects of neutrophil chemotaxis are usually accompanied by recurrent or chronic infections of the skin and the respiratory tract. The onset of clinical symptoms may occur early in infancy; infections tend to be severe and they are generally due by organisms which are of relatively low pathogenicity in the healthy subject. Abnormalities of neutrophil chemotaxis were classified and described as humoral, cellular and unclassified defects. The relevance of neutrophil chemotaxis in the single clinical entities was discussed, taking in particular account the most recent views on the argument. Some details on practical and theoretical therapeutic approaches were also reviewed.

Adolescent↗
PubMedDate not supplied

[Primary mucociliary transport insufficiency].

Case histories of 3 patients with primary mucociliary transport failure are described. Two of them had immotile cilia syndrome and one cystic fibrosis. In all three patients the same clinical picture was found and they had the same treatment, although the etiology of diseases was not the same. The treatment in two patients was not much successful since they had associated respiratory failure.

Adolescent↗
PubMed1990

[Pollinosis and bronchial asthma: pathogenesis, immunology, clinical aspects].

Rhinoconjunctivitis induced by pollen exposure and bronchial asthma are generally easily recognizable clinically. In asthma a number of differential diagnoses such as ciliary dyskinesia, cystic fibrosis and gastro-oesophageal reflux must be considered. The predominant symptoms are coughing and wheezing. Investigations into the complex nature of mediator release and IgE synthesis have established a predominantly inflammatory pattern of reactions largely responsible for induction and maintenance of bronchial hyperresponsiveness due to both acute and chronic processes. Future therapeutic consequences may be derived from anti-inflammatory strategies. This has already lead to reassessment and upgrading of use of corticosteroids in paediatric asthma.

Allergens↗
PubMed1990

[The immotile cilia syndrome as a cause of sterility].

We investigated the relationship between ciliary dyskinesia--commonly referred to as inmotile cilia syndrome--and sterility. In the past two years, we have accurately diagnosed 6 new cases of a total of 20 suspected as having this condition. To make the diagnosis, complete clinical, radiological, ultrastructural and spermatic work up was performed. All males in the fertile age were found to be sterile with spermatozoides with no motility. Sterility may initially go undetected in these patients due to early intense ORL and respiratory symptoms they present. However, mild forms of this disease entity may be asymptomatic and patients may consult for sterility with a clinical picture of scantily florid chronic bronchitis like that of smokers. Coincidental situs inversus may be useful in making the diagnosis.

Adolescent↗
PubMed1990

Atypical basal bodies in the oviductal mucosa (ampulla) of gilts with primary ciliary dyskinesia (PCD).

This report describes atypical basal bodies observed in the oviductal mucosa (ampulla) of two gilts suffering from immotile cilia syndrome. Roughly 3% of atypical basal bodies were found. The most surprising feature seen in this study was the presence of defective basal bodies of so-called 'half-centrioles' type in a fairly high percentage (1.8%). It is worthwhile to remember the centrioles and basal bodies shown an unusual degree of constancy in their size, shape, ultrastructure and location. In this respect they are unique among the cell organelles.

Animals↗
PubMed1990

Suspected ciliary dysfunction in Chinese Shar Pei pups with pneumonia.

Chronic pneumonia was investigated in a litter of young Chinese Shar Pei in which 4 of 6 dogs were affected. Serum immunoglobulin concentrations (IgA, IgG, IgM) determined by radial immunodiffusion varied over time, but were not consistently lower in affected dogs, compared with control dogs. Two dogs that died had hydrocephalus and lymphoid depletion, in addition to severe broncho-pneumonia. Evaluation of ciliary ultrastructure in 2 affected dogs revealed random orientation of adjacent respiratory tract or oviductal cilia and a greater number of microtubular disarrangements, compared with control dogs. In vivo tracheal mucociliary clearance of 99mtechnetium macroaggregated albumin was absent in 1 dog examined. The ciliary abnormalities were suspected to have resulted in an inefficient mucociliary transport system predisposing to the development of pneumonia. Further evaluation of 1 Chinese Shar Pei revealed lymphocyte mitogenesis results that were not consistently less than those of a control dog, normal total hemolytic complement values, and normal blood neutrophil chemotaxis.

Animals↗
PubMed1990

[The saccharin test in comparison with cytologic findings in assessing nasal mucosa function].

The authors emphasize the importance of two aspects of investigation of the nasal mucosa: morphologically by means of cytology, and functionally using the saccharin test. The results are based upon an investigation of 104 patients (208 saccharin tests and 208 cytological slides). The cytological analysis was performed on a mucous smear taken from each nostril separately. The slides were prepared by the May-Grünwald-Giemsa method and examined under the light microscope (magnification 63 and 630). The functional analysis of the nasal mucosa at the site from which the cytological smear was taken was performed by a saccharin test. There were four possible results of such an analysis: normal cytological findings (normal amount of ciliary cells presented) and normal function of mucociliary transport system (normal transport rate values), normal cytological findings and lack of mucociliary transport system and, finally, abnormal cytological findings followed by normal mucociliary transport rates. Possible reasons for the results are discussed. A three-dimensional picture of the nasal mucosa is obtained: morphological, functional and biologically by a combination of the two methods.

Adult↗
PubMed1990

[The saccharin test in the pediatric broncho-pneumonologic outpatient clinic. A noninvasive screening method for the assessment of ciliary function of the respiratory tract in children].

The Saccharin test is a non-dangerous, inexpensive, suitable and repeatable method for assessing the mucociliary function of the respiratory epithelium. A small quantity of Saccharin (R) is deposited on the inferior nasal concha; the chemical agent will be transported by the respiratory epithelium (kinocilia) from the nasopharynx to the oropharynx and can be tasted here as "sweet". The time interval between the deposition of Saccharin and the "sweet" taste is the "nasal mucociliary transport time (nmctt)"; data in minutes. In 381 children (age: 3-17 years) we found an average nmctt of 6.6 (+/- 4.8) min (healthy controls) and 8.8 (+/- 5.2) min resp. (CNSRD children). A nmctt longer than 30 min leads one to suspect disturbances of the mucociliary function, the aetiology of which can be analysed by mucosa biopsy and subsequent examination under the electron microscope. We hold the view that Saccharin test is an essential part of the diagnostic program for children suffering from CNSRD; after 3 tests the mucociliary function can be evaluated correctly. The test can be used both for diagnosing the aetiology of respiratory diseases and for the assessment of the efficiency of therapeutic and prophylactic measures.

Adolescent↗
PubMed1989

[Are ciliary abnormalities always present in Kartagener's syndrome? A study of 16 patients].

Kartagener's syndrome is defined as the combination of bronchiectasis, sinusitis and situs inversus. Assessment of characteristic ultrastructural changes and of their clinical expression was possible by examining the ciliated cells in the airways of 16 patients (12 children). Respiratory cilia of 11/13 patients were found to have abnormal motility. Quantitative ultrastructural data were obtained for 15 patients. Findings were absolutely normal in 2 cases. In 13 cases, ciliary abnormalities affected all the cilia, the majority of them (70-90%) or some of them (20-40%) (n = 7, n = 4, n = 2, respectively). The most frequently encountered aberration was a lacking external dynein arm. Summing it up: 1) ultrastructural ciliary abnormalities do not appear consistently in Kartagener's syndrome; 2) the lack of external dynein arms of cilia is the most frequent anomalous finding: 3) the clinical expression of ciliary dysfunction varies and the exact correlation between ultrastructural data and the intensity of the clinical manifestations remains to be established.

Adult↗
PubMed1985

[Are there mucoviscidosis specific humoral factors? 1: Properties, prevalence, preparation, formation].

In 1967 Spock et al. reported on the serum of cystic fibrosis (CF) homozygotes containing a factor altering the coordination of ciliary motion in rabbit tracheal explants. Just in 1967 Mangos et al. found sweat and saliva from CF homozygotes having an inhibitory effect on sodium reabsorption in the rat parotid gland. Since that time the existence of CF specific humoral factors was supposed. Hitherto mainly biological tests (especially tests of ciliary dyskinesia) were used to prove these factors. These tests caused different results which even were doubtful with regard to the existence of CF specific proteins. Recently it is possible to differentiate between proteins with effects of ciliary dyskinesia and a CF specific protein by means of high sensitive biochemical and immunological methods of protein distinction. In future one can expect elucidation of question related to the importance of CF protein in pathogenesis and diagnosis of cystic fibrosis.

Blood Proteins↗
PubMed1989

[Immotile cilia syndrome--ultrastructural deviations of the nasal cilia].

This study deals with a transmission electron microscopic examination of the nose cilia. Dyskinetic or dysfunctional cilia result clinically in the immotile-cilia syndrome. Differentiation between congenital and acquired anomalies is important. An early diagnosis as part of the therapeutic approach should be considered.

Cilia↗
PubMed1989

Pulmonary permeability in primary ciliary dyskinesia.

Pulmonary clearance (Pcl) of aerosolized 99mTc-DTPA was studied in fourteen patients with primary ciliary dyskinesia (PCD), (median age 23.5 yrs, range 12-44 yrs) and nine normal individuals (median age 23 yrs, range 18-27 yrs). All had never smoked. Regional Pcl was studied for arbitrarily defined central and peripheral regions of the lung using a gamma camera method, whilst total Pcl was studied by a plasma sample method. The patients with PCD had significantly reduced total Pcl compared to the normal individuals (p less than 0.05) and also significantly lower total lung capacity (TLC), vital capacity (VC), forced expiratory volume in one second (FEV1), and FEV1/VC values (p less than 0.05). There was no correlation between Pcl and FEV1/VC. It is concluded that the reduced Pcl in the PCD patients may be associated with their small lung volumes. In addition, reduced bronchial clearance of surfactant in PCD may be associated with an increased alveolar lining fluid volume and/or an impaired movement of 99mTc-DTPA along the alveolar septa to the bronchoalveolar junction, where the epithelium may be more specialized for absorption.

Adolescent↗