Factor XIII in bronchoalveolar lavage fluid from children with chronic bronchoalveolar inflammation.

BACKGROUND: Extravascular activation of the coagulation system and consequent fibrin deposition is involved in the pathomechanism of chronic bronchoalveolar inflammatory diseases. The turnover of extravascular fibrin is attenuated by its cross-linking with activated factor XIII (FXIII). OBJECTIVES: Determination of cellular and plasmatic forms of FXIII and their correlation with D-dimer level in the bronchoalveolar lavage fluid (BALF) from healthy children and from children with bronchoalveolar inflammation. PATIENTS AND METHODS: Highly sensitive immunoassays were used for the quantitation of cellular and plasma FXIII and D-dimer in the BALF of children with recurrent wheezy bronchitis and fibrosing alveolitis. BALF was investigated for FXIII-containing cells by flow cytometry. RESULTS AND CONCLUSIONS: In the BALF of controls a low amount of the cellular form of FXIII (FXIII A2) and D-dimer were measured, while plasma FXIII (FXIII A2B2) was absent. Alveolar macrophages represented the single cell population in BALF that contained FXIII. In the BALF of both patients' groups the concentration and the total amount of FXIII A2 was significantly elevated, and plasma FXIII also appeared in the BALF of most patients. The D-dimer concentration was also elevated in the patients' groups and it correlated both with plasma FXIII and neutrophil count. These findings suggest that FXIII A2 is released from activated or injured alveolar macrophages into the bronchoalveolar lining fluid and in bronchoalveolar inflammatory diseases, FXIII A2B2 also leaks out from the capillaries. By cross-linking fibrin and inhibitors of fibrinolysis to fibrin, FXIII might be a key regulator of fibrin turnover in the extravascular compartment.

Use of midazolam ('Dormicum') and flumazenil ('Anexate') in paediatric bronchology.

One hundred and seven bronchological examinations using midazolam narcosis in association with flumazenil were carried out in 100 children (mean age 3.5 years, range 4 months to 14 years) suffering from chronic non-specific lung disease. Rigid bronchoscopy was followed in 49 cases by bronchography. All patients were premedicated with atropine followed by midazolam (0.2 mg/kg intravenously). Ventilation was carried out with nitrous oxide and oxygen in 47 children and with oxygen only in 60 patients. After 3 mins, suxamethonium (2 mg/kg intravenously) was given for muscle relaxation and intubation carried out. Fifty-one of the children ventilated with oxygen only also received fentanyl (0.002 mg/kg intramuscularly), at the same time as atropine, to provide analgesia. After extubation, all patients were given flumazenil (0.1 to 0.2 mg intravenously) to reverse the effects of midazolam. The results showed that midazolam provided effective sedation and comfortable sleep (mean examination time 12 min 50 sec) and it was considered that the method using fentanyl rather than nitrous oxide for analgesia was the most satisfactory one. Patients awakened promptly (1 min) after flumazenil and quick and effective expectoration was noted, particularly important in those who had undergone bronchography. No complications were observed. Since this investigation, a further 500 bronchoscopics have been carried out using this method with the same results. Even though no narcosis equipment is required, it is recommended that, as with other procedures involving narcosis with muscle relaxation, bronchoscopy with these drugs should not be used in out-patients.

[Cases of acquired immunodeficiency syndrome (AIDS) in infancy]. / Szerzett immunhiány syndroma (AIDS) csecsemökori esetei.

The authors report about two cases of AIDS in infancy. Both infants were Rumanian citizen brought to Hungary for examinations. Hepatosplenomegaly, lymphadenopathy and recurrent upper respiratory tract infections respectively were the main clinical symptoms. The infections seemed to have nosocomical origin. Patients coming from Rumania are belonging to the high risk group as to the carrier state. Regular screening before or during hospital admission is advised therefore.

[Ultrastructural study of bronchial mucosa in suspicion of ciliary dyskinesia]. / A hörgónyálkahártya ultrastrukturális vizsgálata ciliaris dyskinesia gyanúja esetén.

In the ciliary dyskinesia (immotile cilia syndrome) shows the partial or total lacking of cilia's elements. The ciliary dyskinesia may be developed congenital or acquired. The authors report on the experience with 72 biopsies from bronchial mucosa of 68 children, submitted with the question of immotile cilia syndrome. On micrographs (M: 64,000x) of the specimens processed by routine electron microscopical method the number of outer and inner dynein arm, A and B peripheral tubules, central tubules and central sheet were determined to normal 9 + 2 structure. 50-100 ciliaris per case were examined. Total or partial lacking of dynein and non-dynein elements were expressed for the total number of ciliaris compartments. Considering any earlier quantitative examination with this expression there was ease to characterised the quantitative behaviour of the components of ciliaris. Seven Kartagener's syndrome cases was the positive control for determined the quantitative differences between the primer and secondary ciliary changes. In the primary ciliary defects where the situs inversus were presented the total lacking of outer or/and inner dynein arms, where the situs inversus were not presented only the total lacking of inner dynein arms could be found. In secondary ciliary defects the partial lacking of the dynein arms and tubular components were presented. The used method is suitable to characterise the primary or secondary ciliary defects of bronchial mucosa.

[Incidental finding: vascular ring anomaly]. / Nebenbefund: Vaskularring-Anomalie.

In two patients who inhaled a foreign body a vascular ring anomaly was additionally diagnosed. The importance of the anamnesis and careful endoscopy is discussed, equally the problems of the indication of surgical treatment. The authors suggest surgery to prevent later complications.

[20 years' experience using anesthesia in pediatric bronchology]. / Zwanzigjährige Narkoseerfahrungen in der Kinderbronchologie.

The authors inform about their experience with anesthesia gained in the last 20 years with 50,000 bronchological examinations in children. Different anesthesia methods are discussed. They conclude that local anesthesia in adults and in adolescents is a possible procedure using fiberscope. In infants and children the most preferred anesthesia is halothane-nitrous oxide-oxygen-anesthesia with relaxation by succinylcholine.

Broncho-vaxom therapy for children with chronic bronchitis.

The authors report on a clinical study conducted in 1985/86 for evaluating the efficacy of Broncho-Vaxom in children with chronic bronchitis. They observed, during the Broncho-Vaxom therapy, a significant reduction in the number and severity of the acute episodes as well as the significant reduction of the use of antibiotics. They recommend the use of Broncho-Vaxom in chronic, recurrent respiratory infections.

Fibronectin in bronchoalveolar lavage fluid and plasma from children with chronic inflammation of lungs.

Fibronectin/albumin ratios in plasma and in bronchoalveolar lavage fluid were evaluated in patients (1-6 years of age) with recurrent obstructive bronchitis and different interstitial lung diseases. These inflammatory reactions were characterized by increased influx of macrophages on the bronchoalveolar surface, but an increase in the proportion of lymphocytes or neutrophils was also detected in the group of patients with lymphocyte-macrophage or neutrophil-macrophage alveolitis. There was no considerable difference in plasma fibronectin concentrations obtained from healthy children and patients with moderate obstructive bronchitis and slight inflammation of the bronchial mucosa observed bronchoscopically. Levels of plasma fibronectin were elevated in patients with serious bronchial inflammation and different alveolitis, but they were within the normal range. A comparison of lavage fibronectin/albumin ratios with plasma fibronectin/albumin ratios with plasma fibronectin/albumin ratios indicated significant local production of fibronectin in subjects with serious bronchial inflammation and interstitial lung disorders. Fibronectin detected on the bronchoalveolar surface seems to be an important factor in mediating cell-to-cell interactions in the repair of the bronchoalveolar structures, and in tracing the activity of the inflammatory reactions not only in patients with interstitial lung diseases, but also in patients with serious chronic bronchial inflammation.

Chemotactic activity of bronchoalveolar lavage fluid from children with recurrent obstructive bronchitis.

Boyden's method was used to assess the chemotactic activity of bronchoalveolar lavage (BAL) fluid samples from patients aged 1-6 ys with recurrent obstructive bronchitis when signs of chronic mucosal inflammation were observed bronchoscopically. BAL was performed to reveal features of this mucosal inflammation by analysing the lavagable cells and soluble factors. In comparison with the chemotactic effect of casein, the activity of the fluid proved to be high for polymorphonuclear leukocytes (PMNs), but significantly low for mononuclear cells. After treatment of antibodies to C3 and C5, the activity was decreased considerably, but not to a minimum. Presumably, other chemo-attractants (leukotriens, enzymes) can also be found in the lavage fluid beside macrophage- or serum-derived and locally activated complement components. A comparison of complement components/albumin ratios in BAL fluid with serum complement components/albumin ratios suggested a significant local production and/or concentration of these factors. On the basis of our results, an accumulation of PMNs could be expected in the differential cell count, but an increased influx of macrophages was detected. Other amplifying or inhibitor systems may play an important role in the accumulation of PMNs in vivo.

Fibronectin on the bronchoalveolar surface in children with recurrent obstructive bronchitis.

Fibronectin is normally present in the lower respiratory tract. Significantly increased levels of it were detected in the lavage fluid in patients with interstitial lung diseases. Because this molecule appears to mediate a number of components of the inflammatory process, we evaluated the status of fibronectin in plasma and bronchoalveolar lavage in patients with recurrent obstructive bronchitis when signs of severe chronic mucosal inflammation were observed bronchoscopically. There was no considerable difference in plasma concentrations of fibronectin obtained from healthy children and patients. A comparison of lavage fibronectin/albumin ratios with plasma fibronectin/albumin ratios suggested significant local production, especially when the lavage and plasma ratios were measured in the same patients. Phagocytic activity of alveolar macrophages and blood granulocytes from the same patients was enhanced at both concentrations of fibronectin used. This concentrations referred to values quantified in the lavage fluid. The metabolism of fibronectin seems to be an important factor in tracing the inflammation process not only in adults with chronic interstitial lung diseases, but also in children with recurrent obstructive bronchitis.