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Biomedical subjects

M Pedersen

Publications and source records attributed to M Pedersen.

At least 127 records · Page 7Linked to original sources

Defective neutrophil motility in patients with primary ciliary dyskinesia.

Microtubules are important in the regulation of the motile functions of a variety of cells, including leukocytes, ciliated cells and spermatozoa. Polymorphonuclear leukocyte function was studied in ten patients with primary ciliary dyskinesia, an inherited disorder of microtubules in sperm tails and cilia. Neutrophil chemotaxis in Boyden chambers was slightly reduced, but only one patient showed a migration below normal values. In vivo mobilization of polymorphonuclear leukocytes into skin windows was also slightly decreased. In contrast, neutrophil polarization and orientation was normal. The bactericidal activity of neutrophils from patients with primary ciliary dyskinesia was normal, while the ingestion of bacteria was decreased. The abnormalities of neutrophil function in patients with primary ciliary dyskinesia are related to motility. It is suggested that the microtubule defect responsible for the abnormal pattern of ciliary beating is a general abnormality also responsible for the depression of polymorphonuclear leukocyte motility.

Adolescent↗

Blind quantitative electron microscopy of cilia from patients with primary ciliary dyskinesia and from normal subjects.

Cilia from the nasal cavity of 27 patients with congenital ciliary dyskinesia (immotile cilia syndrome) and of 15 normal persons were examined by transmission electron microscopy. The patients had on average a significantly higher number of cilia with absent or markedly reduced numbers of outer or inner dynein arms, of cilia with various types of abnormal configuration of the microtubules, except for compound cilia, and of cilia with an abnormally large variation of the beating axis. Compound cilia were equally frequent in patients and controls. The main structural defect in 9 patients was absent or abnormality low number of outer dynein arms in 4 in conjunction with an abnormally large axis variation and in 1 with a markedly reduced number of inner dynein arms also in 1 with supernumerous axonemal microtubules also. In 9 patients the main defects were abnormal configuration of the axonemal microtubules and a large axis variation. Seven of these had eccentrically located central pairs of microtubules and radial spoke defects, 5 in conjunction with abnormally low numbers of inner dynein arms. In one patient there were various types of abnormal configuration of the axonemal microtubules together with absent inner dynein arms, in another cilia with supernumerous microtubules only were present. The only abnormal finding in 1 patient was an extreme variation in the ciliary axes. Completely normal ciliary ultrastructure was found in 8 patients.

Adolescent↗

Specific types of abnormal ciliary motility in Kartagener's syndrome and analogous respiratory disorders. A quantified microphoto-oscillographic investigation of 27 patients.

Twenty seven patients with clinical evidence of lack of mucociliary activity of cilia, which include Kartagener's syndrome and termed in recent years immotile cilia syndrome or primary ciliary dyskinesia, were studied by a newly developed microphoto oscillografic technique, to determine different qualities in the ciliary motility pattern in vitro. Abnormal ciliary motility was compared to the findings at transmission electron microscopy. The patients could be allocated to groups, characterized by different abnormalities in ultrastructure and function. One patient who lacked both inner and outer dynein arms, and had total ciliary immotility. Eight who only lacked outer dynein arms had a low beating frequency, reduced number of cells with motile cilia and ciliary asynchrony. Seven with microtubular disarrangement (spoke defect) had cilia which beat asynchroneously and with short, stiff strokes. Two patients had random orientation of ciliary axes and one supernumerous microtubules, all were characterized by slight ciliary asynchrony. Eight patients had normal cilia ultrastructure, but an abnormal beating pattern, usual characterized by hyperfrequent and trembling ciliary movements (hypermotile). These groups seem to constitute separate types of congenital ciliary defects.

Adolescent↗

Nasal mucociliary transport, number of ciliated cells, and beating pattern in naturally acquired common colds.

Repeated samples of nasal epithelium were taken for in vitro study of the number and motility of ciliated cells (microphoto-oscillographic technique), and nasal mucociliary clearance transport rate was measured (saccharin test) in 26 subjects with naturally acquired common colds. The transport rate was markedly reduced during the disease, and a slight impairment remained even after 32 days. There was a considerable fall in the number of ciliated cells, and regeneration was slow. A moderate and shortlasting change in beating frequency and intracellular synchrony was also observed. It is concluded, that a common cold, as a rule, results in marked and long-lasting impairment of nasal mucociliary clearance function, and this may be the cause of some otherwise unexplainable symptoms from the nose and throat.

Adult↗

Bronchopulmonary symptoms in primary ciliary dyskinesia. A clinical study of 27 patients.

Lower airway symptoms in 27 patients with primary ciliary dyskinesia (Kartagener's syndrome, "immotile cilia syndrome") are presented. Nine of the patients had reduced ciliary beating (defective dynein arms), ten asynchroneous ciliary beating pattern (7 spoke defects and 3 other microtubular abnormalities) and eight a newly described type of abnormal bearing "hypermotile cilia" (normal cilia ultrastructure). Seventeen (63%) had partial or total situs inversus. All had absent or markedly reduced mucociliary transport in the nose and chronic rhinosinusitis since early childhood, whereas lower airway symptoms had a tendency to start later on in life. This was especially found in patients with hypermotile cilia, who also had less pronounced pulmonary disease. The most characteristic feature of the disease was a slow chronic course with a daily productive cough. Half of the patients were not even affected in their daily activities. Fifteen (56%) had bronchiectasis, and atelectasis occurred in 12 (44%). With a few exceptions the lung function was normal or only slightly reduced. Haemophilus influenzae was the most common pathogen found in sputum, but some had chronic pulmonary pseudomonas or coli infection. Improvement was seen in the patients who were regularly given antibiotics and prophylactic treatment against mucus accumulation.

Activities of Daily Living↗

Nose-, sinus- and ear-symptoms in 27 patients with primary ciliary dyskinesia.

Nose-, sinus- and ear-symptoms, signs and tests were studied in 27 patients with primary ciliary dyskinesia. All subjects had had daily nasal discharge since birth or early childhood. X-ray examination showed sinusitis, and most patients had frequent episodes of sinusitis symptoms. The frequency of common colds was apparently not increased. As a general rule, the patients had chronic secretory otitis media, but few episodes of acute otitis media. Repeated suctions, punctures, and insertion of tubes had been disappointing, but more radical surgery of the nose and sinuses had helped some of the patients. It is concluded that knowledge of the very characteristic ENT symptoms is important in respect of an early diagnosis and starting preventive treatment in order to inhibit the development of lung damage. The ENT symptoms, on the other hand, are more often overtreated than the reverse.

Adolescent↗

Intrinsic asthma and bacterial histamine release.

In this study of intrinsic asthma (IA) in children the pathogenic role of bacteria in respiratory disease was elucidated by a basophil histamine liberation technique. Several strains of bacteria caused release of histamine from peripheral leukocytes in vitro. Normal, non-infectious and non-atopic children frequently responded in a similar fashion, although positive responses were less frequent. It seems that two different mechanisms of bacterial histamine release exist: interaction with the basophil-bound IgE and a direct interaction with the cell surface. It is suggested that the histamine release takes place only in the lung of IA patients, where a defective pulmonary barrier could permit the bacteria to enter, but not in healthy individuals.

Adolescent↗

Postoperative radiotherapy in rectosigmoid cancer Dukes' B and C: interim report from a randomized multicentre study.

The design, and complications seen during the first 2 years, of a randomized trial of postoperative radiotherapy for rectosigmoid cancer Dukes' B and C are presented and discussed. It is concluded that the present complication rate-below 10% in 221 patients-permits continuation of the intake, which is planned to include 550 patients, to demonstrate a possible increase in crude 5-year survival by 15% (60-75% in Dukes' B and 25-40% in Dukes' C), on the basis of a 0·01 significance level and a probability that the experiment will be successful of 0·90.

Clinical Trials as Topic↗

Rhinitis, sinusitis and otitis media in Kartagener's syndrome (primary ciliary dyskinesia).

Ear, nose and throat symptoms and signs were studied in 15 patients with Kartagener's syndrome: a triad consisting of chronic rhinosinusitis, chronic bronchitis with bronchiectasis, and situs inversus. The triad is caused by primary ciliary dyskinesia and characterized by absent or considerably reduced mucociliary transport. Daily accumulation of secretions in the nose, chronic recurrent secretory otitis media and sinusitis occurred in all subjects. Characteristically, nasal discharge started at birth or was first detected in early childhood. Apparently, the frequency of common colds and of acute purulent otitis media was not increased. As primary ciliary dyskinesia can occur without situs inversus, knowledge of the typical ENT symptoms is essential for making an early diagnosis, which is important for the correct management of the disease. While a conservative surgical approach to treatment of the ENT symptoms is recommended, early and active treatment of the bronchial symptoms is probably important for prevention of further lung damage and development of bronchiectasis.

Adolescent↗

Studies on hypersensitivity to bacterial antigens in intrinsic asthma.

Twelve children, aged 4 to 14 years, with moderate to severe intrinsic asthma (IA) were studied. Symptom-Score charts were used to confirm the relationship of acute respiratory tract infections to exacerbations of asthma. Hypersensitivity to eight commonly occurring bacteria from the normal flora of the upper respiratory tract was studied by skin test, by crossed immunoelectrophoresis, and by basophil histamine release in vitro, using ultrasonicates of the bacteria as antigens. Skin tests were all negative. All children contained low titers of precipitating antibodies against most of the bacteria, but in this respect they did not differ from normal children. In contrast, release of histamine was induced in leukocytes from the IA children by all, or most sonicates, while such reactions, were less frequent in control children. The pattern of responses indicated an element of specificity. These was no correlation to precipitating antibodies, or to the microbial flora of the children. Positive responses were characterized by low values of maximal histamine release, and by a tendency to fluctuations with time. Because of these fluctuations, and because the IA children and control children were tested on separate occasions, we cannot be certain as to the real difference between these two groups. Our studies do, however, demonstrate that water-soluble constituents of all the bacterial strains tested were capable of causing the release of histamine in vitro, but that this phenomenon is not restricted to IA. The clinical significance of these findings awaits further investigations on the mechanism(s) of release in vitro by such agents.

Acute Disease↗