Effect of inhalation of single dose of beclomethasone on airway epithelium.

Inhaled corticosteroids are being recommended for the treatment of bronchial asthma for their anti-inflammatory properties and reduction of airway hyperreactivity. The first tissue coming to the contact with all inhaled substances is the airway epithelium. In this experiment, the immediate effect of a single MDI dose of beclomethasone on the ultrastructure of the tracheal and bronchiolar epithelium was studied.

Acute and chronic hypoxia as well as 7-day recovery from chronic hypoxia affects the distribution of pulmonary mast cells and their MMP-13 expression in rats.

Chronic hypoxia results in pulmonary hypertension due to vasoconstriction and structural remodelling of peripheral lung blood vessels. We hypothesize that vascular remodelling is initiated in the walls of prealveolar pulmonary arteries by collagenolytic metalloproteinases (MMP) released from activated mast cells. Distribution of mast cells and their expression of interstitial collagenase, MMP-13, in lung conduit, small muscular, and prealveolar arteries was determined quantitatively in rats exposed for 4 and 20 days to hypoxia as well as after 7-day recovery from 20-day hypoxia (10% O2).

Local administration of 2% trimecaine affects the content of fucosylated glycoconjugates in goblet cells in rabbit tracheal epithelium.

The proportion of fucosylated glycoconjugate-containing rabbit tracheal goblet cells after intratracheal application of trimecaine was studied to evaluate its possible unfavourable effects. This lapine model is comparable with diagnostic findings in humans because airway epithelia in humans and rabbits are similar; tracheal epithelium is also practically identical to bronchial epithelium in both species. Local trimecaine anaesthesia caused a proportional decrease in percentage of the tracheal goblet cells containing both alpha(1-2)- and alpha(1-6)-, alpha(1-3)- and alpha(1-4)-fucosylated glycoconjugates as revealed 10 min postexposure using lectin histochemistry.

The effects of intravenously administered methylxanthines on the proportion of goblet cells containing fucosylated glycoconjugates in rabbit tracheal epithelium.

The proportion of goblet cells containing various fucosylated glycoconjugates was evaluated with the use of lectin histochemistry in rabbit tracheal epithelium at 15 and 30 min after intravenous administration of either aminophylline (Syntophyllin) or a mixture of etophylline and theophylline (Oxantil). Methylxanthine derivatives are nonspecific inhibitors of phosphodiesterases that are used to treat bronchial asthma; the proportion of fucosylated glycoconjugates strongly affects rheologic properties of respiratory tract mucus. It is concluded that administration of Syntophyllin dramatically lowered the proportion of goblet cells containing fucosylated glycoconjugates in rabbit tracheal epithelium, especially at 30 min after exposure.

The effects of intravenously administered methylxanthine preparations on the glycoconjugate composition of goblet cells in rabbit tracheal epithelium.

Effects of methylxanthine derivatives, which are inhibitors of phosphodiesterases I-IV used against bronchial asthma, on the composition of glycoconjugates in goblet cells were evaluated in tracheal epithelium of rabbits at 15 and 30 min after intravenous administration of aminophylline (Syntophyllin) and a mixture of etophylline and theophylline (Oxantil), respectively. Percentages of tracheal goblet cells containing neutral, total acidic, sulphated acidic, and sialylated acidic glycoconjugates were assessed using both conventional and lectin histochemistry. No significant changes were found in both experimental groups at 15 min after exposure.

[Leber's hereditary optic nerve neuropathy].

The authors present a case report of 26 years old man with bilateral optic nerve neuropathy. Detection of heteroplasmic mutation of mitochondrial DNA at G3460A site confirmed the suspicion on Lebers hereditary optic nerve neuropathy (LHON). Genetic and environmental factors of the disease and various accompanying neurologic and other symptoms, which can together with the optic nerve defect participate in the development of of the LOHN clinical pattern are discussed.

[Leber's hereditary optic neuropathy].

Background: Leber's hereditary neuropathy of the optic nerve (LHON) is manifested by bilateral affection of the eyes with acute or subacute loss of vision. The disease is caused by point mutations in the mitochondrial DNA (mtDNA) and is one of the most frequent mitochondrial diseases in the population. In patients with LHON 18 different point mutations in the mtDNA were described which correlate partly with the rate of progression of the disease and the severity and prognosis of the final affection of vision.

[Various manifestations of the A8344G mtDNA heteroplasmic mutation in 4 families with the MERRF syndrome].

Background: The most frequent manifestation of mitochondrial DNA (mtDNA) mutation 8344 A-->G is MERRF syndrome (Myoclonic Epilepsy and Myopathy with Ragged Red Fibres). Less frequent symptoms include ataxia, perceptive type of deafness, cardiomyopathy or external ophthalmoplegia and mental and motor retardation in children. We describe heterogeneity of clinical symptoms and results of biochemical and molecular investigations in four families with the heteroplasmic mutation 8344 A-->G in mtDNA.

[Lactate acidosis in childhood].

Background: Secondary lactate acidosis is found in children with hypoxaemia, with impaired tissue perfusion, in hepatic and renal failure or in intoxications. Primary lactate acidosis is usually caused by hereditary metabolic disorders. The objective of the trial was to analyze the causes which lead in childhood to the development of primary hyperlactacidaemia.

[Mitochondrial myopathy, deafness and type 2 diabetes mellitus with tRNALeu(UUR) point mutation in mitochondrial DNA].

Background: A heteroplasmic A3243G point mutation in tRNALeu(UUR) gene of mitochondrial DNA (mtDNA) is found in patients with MELAS syndrome (Mitochondrial Encephalomyopathy, Lactic Acidosis and Stroke-like episodes), less frequently in patients with other dominating clinical features, such as deafness, diabetes mellitus type 2, hypertrophic cardiomyopathy, renal problems or inborn development defects. Present report describes histochemical, enzymatic and molecular biology studies of the family with clinical variant of meals syndrome.

Methods And Results: A 45-year-old woman with progressive muscle weakness, external ophtalmoplegia, perceptive deafness, ischemic heart disease, diabetes mellitus type 2 and hyperlactacidemia was metabolically investigated because the multiorgan problems indicated mitochondrial origin of the disease.

[DNA diagnosis of the fragile X chromosome syndrome--FRAXA using PCR].

Background: Fragile X syndrome is gonosomal recessive mental retardation with the frequency 1:1000 in male population. Fragile X syndrome is caused by amplification of CGG repeat in 1. exon of FMT-1 gene.

Effect of an adrenergic agonist and a cholinergic antagonist on the airway epithelium.

The ultrastructure of the rabbit tracheal epithelium was studied 30 minutes after intratracheal administration of two puffs of salbutamol and ipratropium bromide, respectively. The injury to the tracheal epithelium due to the treatment with both bronchospasmolytic drugs was considered moderate to severe. In both experimental groups, the degree of goblet cells' stimulation did not differ significantly, the ciliated cells were less damaged compared with the goblet ones and the morphological signs of the impaired self-cleaning ability were revealed.

Ultrastructure of the tracheal epithelium in rabbits after acetylcholine administration.

The ultrastructure of the tracheal epithelium was studied 5 and 20 min after intravenous (i.v.) administration of 0.

Comparison of the effect of a high and low dose of atropin on the ultrastructure of the tracheal epithelium.

The ultrastructure of rabbit tracheal epithelium was studied 20 min after injection of 0.04 and 0.5 mg of atropin.

An abnormally high occurrence of malformed kinocilia in the tracheal epithelium of a clinically healthy rabbit.

An abnormally high occurrence of malformed kinocilia containing axonemes with different number or arrangement of microtubules compared with the typical 9+2 pattern of motile cilia was encountered in the tracheal epithelium of one clinically healthy rabbit. The malformed cilia amounted to 6.87% of all kinocilia.

[Airway epithelium and its reaction to harmful substances].

The trachea and large bronchi are lined with a pseudostratified columnar ciliated epithelium. The secretory elements are represented by goblet cells. In healthy subjects the majority of goblet cells are filled with mucus, only 3% of them discharge secretion.

Ultrastructure of rabbit tracheal epithelium after the administration of Hexabrix--a hexaiodated contrast agent for tracheobronchography.

The ultrastructure of the tracheal epithelium after the application of 1 ml of Hexabrix (a hexaiodated contrast agent for tracheobronchography) into the airways was studied. Severely altered pseudostratified columnar epithelium was found in the trachea. Eighty-one +/- 5% of the goblet cells had been stimulated to discharged mucus.

Quantitative evaluation of the degree of damage to tracheal epithelium.

Quantitative evaluation of the injury to the tracheal epithelium was carried out under various experimental conditions. The damage of the epithelium was classified in three groups, according its severity. In several impaired epithelium all the goblet cells were stimulated to release their secretion.

[The effect of a single therapeutic dose of Intussin on the ultrastructure of the epithelium of the respiratory tract].

The effect of administration of a single therapeutic dose of Intussin on the ultrastructure of the rabbit tracheal epithelium was investigated. Twenty minutes after the application of five drops of Intussin only slight reaction of the pseudostratified ciliated epithelium was recorded. The ciliated cells manifested only slight signs of pathological alteration.

The effect of Hexabrix tracheobronchography on the ultrastructure of the airway epithelium.

The ultrastructure of the airway epithelium was studied after experimental Hexabrix tracheobronchography. Serious damage to the tracheal epithelium due to Hexabrix was recorded. 81 +/- 5% of goblet cells were stimulated to discharge mucus.

Effect of bronchoalveolar lavage on the ultrastructure of the tracheal epithelium in rabbits.

Serious damage to the tracheal epithelium due to bronchoalveolar lavage (BAL) was recorded. Immediately after BAL 99 +/- 2% of goblet cells were exhausted and degenerated. Their regeneration began 24 h after BAL resulting in hyperplasia of goblet cells with the formation of endoepithelial mucous glands.

Regeneration of changes produced in rabbit tracheal epithelium by saline lavage of the airways.

The authors studied the course of the repair of changes induced in the rabbit tracheal epithelium by saline lavage of the airways. The tracheal epithelium was examined 2, 24, 48 and 72 hours after treatment. Saline lavage stimulated the goblet cells to instantaneous discharge of their secretion.