[Comparative diagnostic value of the analysis of the skeletal muscle and lymphocytes in mitochondrial diseases]. / Sravnitel'naia diagnosticheskaia tsennost' analiza skeletnoi myshtsy ilimfotsitov pri mitokhondrial'nykh bolezniakh.

Cytochemical analysis of lymphocytes' enzymes may could be an alternative method to skeletal muscle biopsy in diagnosis of mitochondrial pathology. We investigated biopsies of skeletal muscles and cytochemical status of lymphocytes in 14 children with mitochondrial pathology. Lymphocytes were investigated also in 12 health children. The data on mitochondrial state in the skeletal muscles and peripheral blood lymphocytes were comparable. The less were the size and functional activity of single mitochondria in lymphocytes, the higher was mitochondrial insufficiency in the skeletal muscle. Enzymatic status of peripheral blood lymphocytes is not less indicative of mitochondrial insufficiency in children than mitochondrial characteristics in skeletal muscles. It reflects multisystem mitochondrial insufficiency. The results obtained are determined by both mitochondrial pathological modifications and their compensatory response to pathological process.

[Morphological and pathogenetic differences in the respiratory disorders of newborn infants in the first days of life based on data from the composition of tracheobronchial smears]. / Morfologicheskie i patogeneticheskie razlichiia dykhatel'nykh rasstroistv u novorozhdennykh detei v pervye sutki zhizhni po dannym sostava trakheobronkhial'nykh smyvov.

Six variants of pulmonary pathology are described basing on the differences in composition of tracheobronchial wash-offs. This composition was studied by electron microscopy and biochemical assay of phospholipids in 76 neonates during the first day of life as well as by structural investigation of the lungs in 16 children who had died within two days after birth. The composition of the tracheobronchial wash-offs was compared with lung alterations associated with intrauterine hypoxia, infection, delay of respiratory parenchyma development, brain trauma.

[Characteristics of infection in children during prolonged oxidative stress after radiation exposure]. / Osobennosti infektsionnogo protsessa u detei v usloviiakh dlitel'nogo okislitel'nogo stressa vsledstvie radiatsionnogo vozdeistviia.

In the article on discusses the problems of origin and duration of chlamydia and herpes-virus infection in children with chronic somatic pathology and different degrees of radiation stress in contaminated territories of Brianskaya region (the level of contamination according to 137Cs = 9.37-19.73 Ci/km2).

[Mitochondrial nature of cardiomyopathies in children (analysis of skeletal muscle biopsies)]. / Mitokhondrial'naia priroda kardiomiopatii u detei (analiz bioptatov skeletnykh myshts.

Morphological characteristics of the skeletal muscle biopsies from 10 children with idiopathic dilatational and hypertrophic cardiomyopathies are described. Methods of light and electron microscopy, histochemistry (mitochondrial enzymes, glycogen, lipids, calcium), morphometry and statistics were used. The presence of specific and nonspecific signs of systemic mitochondrial insufficiency, the character of histochemical distribution of the energetic substrates and the calcium suggest that this insufficiency is the basis of cardiomyopathies pathogenesis.

[Morphological alterations of the skeletal muscles in mitochondrial myopathies in children and their mothers]. / Kharakteristika morfologicheskikh izmenenii skeletnoi myshechnoi tkani pri mitokhondrial'nykh miopatiiakh u detei i ikh materei.

Skeletal muscle biopsies from 18 children and 8 from their mothers were studied in mitochondrial myopathies. It is shown that by means of electron microscopy, histochemistry, and morphometry the correct diagnosis of mitochondrial insufficiency is quite possible.

[Mitochondrial insufficiency in children]. / Mitokhondrial'naia nedostatochnost' u detei.

Structural and functional disturbances closely associated with mitochondrial insufficiency are found in the nervous system elements, skeletal and smooth muscles, myocardium, kidneys, liver, gastrointestinal tract, endocrine glands, systems of hematopoiesis and immunity. Nosological and syndromic forms of mitochondrial pathology associated with mitochondrial or nuclear DNA mutations are characterized. The disease variants with symptomatic (may be secondary) or with presumable mitochondrial pathology are analyzed. For the pathologist the most reliable diagnostic criteria are skeletal muscle changes, and the "ragged red fibres" phenomenon first of all. The comparisons of the clinicobiochemical and morphological data are of the decisive importance.

[The effect of reaferon and alpha-tocopherol on lipid peroxidation in experimental influenza]. / Vliianie reaferona i alpha-tokoferola na protsessy perekisnogo okisleniia lipidov pri éksperimental'noi grippoznoi infektsii.

Reaferon administered to outbred young mice with experimental influenza had a positive effect on morphologic changes in pulmonary tissues and on lipid peroxidation in the blood plasma and pulmonary tissues. Combined use of reaferon and alpha-tocopherol in mice infected with influenza A virus reduced the mortality rate, boosted the antiviral effect of reaferon proper, and led to virtually complete normalization of lipid peroxidation processes in the blood plasma and pulmonary tissues.

[Viral antigens in kidney biopsies of children with glomerulonephritis]. / Virusnye antigeny v bioptatakh pochechnoi tkani u detei s glomerulonefritom.

A relationship between clinical forms of glomerulonephritis and incidence of viral antigens in renal tissue was revealed: Hepatitis B virus antigens (HBsAg) are more frequently detected in the glomeruli in the patients with nephrotic glomerulonephritis, Herpes simplex antigens are detected in the glomeruli in mixed glomerulonephritis, and cytomegaloviral and Herpes simplex antigens are detected in the epithelium of the proximal canaliculi in patients with hematuric glomerulonephritis. No correlations between the persistence of Herpes simplex type 1, cytomegalovirus, and HBsAg in the renal tissue were detected. HBsAg is detected in the renal tissue mainly in the children without free HBsAg in the blood serum. This may be indicative of an important role of specific immune complexes in the pathogenesis of glomerulonephritis associated with hepatitis B viral infection. The results point to an appreciable contribution of a persistent viral infection to the progress of glomerulonephritis.

[Pathologic diagnostic criteria of cardiomyopathy in children]. / Patologoanatomicheskie diagnosticheskie kriterii kardiomiopatii u detei.

Pathologic diagnostic criteria of the hypertrophic and dilated cardiomyopathy and comparative histologic characteristics of chronic non-specific myocarditis and restrictive cardiomyopathy are presented. The basis of the idiopathic cardiomyopathies is according to the authors, destructive and degenerative processes in cardiomyocytes, their combination with a zonal-segmentary muscle hypertrophy, dilatation and myocardium sclerosis. Dependence of the cardiomyopathy upon the mitochondrial dysfunction and the aggravation of morphological changes by viruses is emphasized.

[Status and prospects for the development of a pediatric pathology service in the Russian Federation]. / Sostoianie i perspektivy razvitiia pediatricheskoi patologoanatomicheskoi sluzhby v Rossiiskoi Federatsii.

The paper presents the main points of the reports made at the constituent conference of the Russian Society of Pathologists. Some urgent measures for improving this service are suggested including the creation of independent pediatric bureaus of pathology and some others.

[Intrauterine and postnatal pneumonia in acquired immunodeficiency of infants]. / Vnutriutrobnye i postnatal'nye pnevmonii v usloviiakh priobretennogo immunodefitsita u detei rannego vozrasta.

The autopsy material of 15 children aged from 2 months to 3 years from the zonal group of increased risk of the ecologic pathology, acquired immunodeficiency and viral infections was assessed morphologically and clinically. Decreased number of T-cells (T4, T8), an increase of the level of serum IgA, IgE and immune complexes, HIV-antibodies (4 cases) were found in the patients. The method of the molecular hybridization by means of virus-specific 32P-DNA probes was used. Bronchopneumonia was the cause of death. Severe deficiency of the organs and cells of the immune system, alternative-proliferative lung inflammation, mainly in the form of pneumonitis and alveolitis, were found. The latter differed either individually or as a result of the predominant infectious agent (RNA- or DNA-viruses, pneumocysts, bacterial flora, fungi). Considerable immunity dysfunctions enhanced the intensity of the specific features in pneumonia morphology.

[The systemic disorder of connective tissue metabolism and hypoplasia of the nephron elements in hereditary nephritis in children]. / Sistemnoe narushenie metabolizma soedinitel'noi tkani i gipoplaziia élementov nefrona pri nasledstvennom nefrite u detei.

Clinical biochemistry and morphological methods were employed to examine 25 children aged 3 to 15 years with hereditary nephritis. Measurements were made of morphological alterations in renal biopsy specimens, excretion with urine of connective tissue metabolites (hydroxyproline, hydroxylysine glycosides, glycosaminoglycans), the level of the same metabolites and characteristic features of the cellular growth of skin fibroblasts in culture. The early stages of nephritis development were marked by hypoplasia of nephron elements, followed by dystrophy and destruction of its ultrastructural elements including collagen of the glomerular basal membranes. The status of the skin fibroblast cell culture corresponded with the changes seen in renal cells of mesenchymal origin. The conclusion is made that in children with hereditary nephritis, nephron cells and skin fibroblasts reflect systemic metabolic defect of the connective tissue.

[Dysplastic bases of the development of kidney diseases in childhood]. / Displasticheskie osnovy razvitiia nefropatii detskogo vozrasta.

Altogether 412 children aged 1 to 15 years with chronic nephropathies of various etiology were examined. Methods of clinical morphology were applied. It is marked that in 42 percent of cases the development of glomerular and interstitial nephropathies was caused by foregoing disorders in the formation of the renal structure, chiefly at the tissue and cell levels. It is assumed that the majority of those disorders are multifactorial in nature. Alterations in cell membranes and in supramembraneous biopolymeric layers were also seen, being associated with the definite clinical symptomatology. It is stressed that of paramount importance is the design of the methods of stimulating therapy for correction of renal developmental abnormalities with a decrease of the risk of immunopathologic responses, chronic inflammation and nephrosclerosis.

[Lipid peroxidation in experimental viral infection and the effect on it of interferons and alpha-tocopherol]. / Protsessy perekisnogo okisleniia lipidov pri éksperimental'noi virusnoi infektsii i vliianie na nikh interferonov i alpha-tokoferola.

Lipid peroxidation as well as effects of various interferons (native, from fibroblasts of new born and adult animals) on the peroxidation were studied in blood plasma, erythrocytes, liver and kidney tissues of mice with viral infection. These interferons, simultaneously with antiviral action, exhibited the positive effect on lipid peroxidation, while the interferon from fibroblasts of newborns produced the side reactions. Simultaneous use of the interferon and alpha-tocopherol removed these side reactions.

[The problem of tissue developmental defects in childhood]. / Problema tkanevykh porokov razvitiia v detskom vozraste.

Chronic diseases of the thymus, lungs and kidneys observed in children are viewed basing on analysis of the malformations detected in the children at tissue and cellular structural level, using autopsy and biopsy evidence. Multiple modality investigation was focused on dysplasia, hypoplasia and dyschronism. Dysplasia was characterized by congenital and acquired forms similar in the development and presentation while dyschronism by enhancement or inhibition of structural development seen in immaturity or premature postnatal involution. Immaturity shows the possibility of further development and structural and functional patency. The occurrence of tissue immaturity is not rare, being found in one or many organs of the child. Tissue malformations are chronic inflammation risk factors.