Search

Oxygen tension in the blood and tissues, lipid peroxidation (LP) intensity (content of malonic blood dialdehyde and activity of superoxide dismutase in erythrocytes), spectrum of fatty acids and phospholipid composition of erythrocytic membranes, their passive K(+) permeability, total ATP content in erythrocytes, some other metabolic indices were investigated in 24 patients with blue type congenital heart disease. It was shown that these patients showed a reduced oxygen tension in the blood and tissues, LP intensification, changes of the lipid phase of erythrocytic membranes, disorders of their permeability, decrease of intraerythrocytic ATP reserves. Beta-adrenoblocking agents (obsidan, anaprilin were used in 10 patients. Reported are clinico-metabolic findings indicating a favourable effects of these drugs on LP processes, lipid spectrum of erythrocytic membranes, their permeability and bioenergy reactions.

Study of the environmental pollution (ambient air, drinking water, food and fodder) in southern Ukraine industrial region and study of congenital developmental defects were carried out. 78,678 newborns were examined. The most significant environmental factors responsible for congenital developmental defects, as authors proposed, were the ambient air pollution and environmental radioactivity.

As many as 618 children with different varieties of congenital developmental abnormalities were examined for the status of the immune system. Immunologic studies were carried out in 89 patients. They included the NBT test, determination of the content of T-, B- and O-lymphocytes, IgA, IgM and IgG. Morpho-histological studies of the spleen and thymus were performed according to the data of 529 autopsies. It has been established that 3/4 of cases with congenital developmental abnormalities are characterized by morphofunctional immune deficiency, which is more pronounced in multiple abnormalities (of chromosomal etiology in particular) as well in some systemic abnormalities, largely of the CNS.

A study was made of the prevalence, structure and risk factors of congenital developmental abnormalities (CDAs) of the CNS seen over the recent 17 years in the newborn (217,000 births) and 7845 children of different age, who died during the same period. The stage I work involved a retrospective analysis of the medical documentation up to a broad-scale introduction of ultrasonography of CDAs in the fetus; stage II was concerned, with an analysis of the genetic monitoring after this method was introduced into public health practice, with the coverage of the pregnant amounting to 98%. The recent years have witnessed a stable tendency toward a decrease of both the total incidence of CDAs of the CNS in the newborn and dead children and of the incidence of certain CDAs, namely anencephaly + and hydrocephalus, most accurately diagnosed on ultrasonography. The ratio of pregnancy (the 4th and subsequent ones), pregnancy pathologies (gestosis, infectious diseases), prematurity, intrauterine hypotrophy, previous exposure to ionizing radiation of the future child's mother and father (radiotherapy, industrial hazards, and so forth) are significant risk factors of CDAs of the CNS in the fetus.

Tissue oxygen tension, peroxidation of lipids (malonic dialdehyde levels, superoxide dismutase activity), fatty acid, spectrum and phospholipid composition in the red blood cells, Na+, K(+)-ATPase were studied in 11 healthy children and 21 with cyanotic heart disease. The beta-adrenoblocker obsidan was used in the multimodality treatment of 10 patients. Unlike healthy children, the patients had decreased tissue oxygen tension, intensified lipid peroxidation, an altered lipid profile in the red blood cell membranes, their abnormal permeability, diminished intracellular ATP depot. The conventional tools of intensive care (oxygenation, cardiotropic drugs, goal-oriented fluid therapy) are low beneficial. There is evidence for the supplementation of obsidan, a beta-adrenoblocker, to a therapeutical complex for this group of patients. Clinical and metabolic evidence for the positive action of the drug on lipid peroxidation, lipid spectrum in the red blood cell membranes, their permeability and biological energy reactions.

In the last 15 years 6,173 autopsy records were studied in the pediatric regional prosectorium and 3,938 children aged from one week to 15 years were examined in the nephrological clinic. It was established that the frequency of urinary system anomalies among children who had died ranged from 3.8 to 4.4%, while the fraction of multiple anomalies including lesions of the organs of the urinary system had somewhat increased (p less than 0.05). To determine exactly the possible role of genetic factors in the pathogenesis of anomalies of the urinary system organs, the authors studied the distribution of antigens of the HLA system in patients with this pathological condition. A total of 100 children with anomalies of organs of the urinary system, their parents, and 120 healthy individuals were examined. Typing was conducted by means of monospecific sera by the microlymphocytotoxic method. There was a tendency to an increase of the frequency of identification (as compared to the controls) of antigen B40 in the group of patients with uropathology, as well as B12 and B35. The authors calculated the relative risk of the development of the anomalies in children who have the listed antigens. The results of the study may be used as an additional criterion in differential diagnosis of uropathies.

Materials on urinary diseases collected by maternity hospitals and children's outpatient clinics and pertaining to 145,979 children of different ages including neonates were compared to the ultrasound data on 10,396 children of the same age. According to the ultrasound data, the incidence of urinary system abnormalities in children is 1.83 +/- 0.196%. The highest rate of the diseases was recorded in neonates. Later it was found to decrease. The method of ultrasound diagnosis can be used as a screening stage in mass prophylactic examinations of the childhood population of all ages including the earliest one. It is advisable that the method be introduced in medical practice of the city (regional) and interregional outpatient clinics.

The prevalence and structure as well as risk factors for congenital developmental abnormalities (CDA) of the central nervous system (CNS) have been studied over recent 17 years among the newborn's population (217,000 births) and 7,845 dead children of varying age. The stage I work involved a retrospective analysis of the medical documentation up to broad-scale introduction of ultrasonography (US) of fetal abnormalities during pregnancy, the stage II work consisted in an analysis of the genetic monitoring after the introduction of US of the fetal states, comprising the pregnant, which approximated 98%. The significant risk factors for CDA of the CNS were the pregnancy rate (the IV and consequent ones), some types of pregnancy pathology (toxemia of the first half of pregnancy, infectious diseases) as well as prematurity, intrauterine hypotrophy, previous exposures of the mother and father of the future child to ionizing radiation. Over the recent years a stable tendency has been noted toward decrease of both the prevalence of CDA of the CNS among the newborn and dead children and of anencephalies and hydrocephalus, the developmental abnormalities most clearly diagnosed on US.

The necessity of conducting regional genetic monitoring (GM) is substantiated, an experience on its organization and the procedure of its carrying out with the help of computer technology is provided. The analysis of GM indicated that the incidence of developmental defects registered and listed is in the population limits. Proceeding from the example of anencephaly , the role of GM in the assessment of the effectiveness of comprehensive measures on prevention of birth of children with grave defects has been demonstrated.

In one of the southern regions of the Ukraine, analysis was made over the recent three years of the genetic monitoring data on the incidence and structure of congenital developmental abnormalities in the newborn as related to the status of environmental pollution (the content of dust, carbon black, carbon and nitrogen oxides, nitrogen and sulfur dioxides, hydrogen sulfide, formaldehyde, phenol, fluorine compounds in the atmospheric air; the content of toxic chemicals and pesticides in foods, soil, water and feeds) as well as to the radiation situation. In the region under study, areas with varying degree of environmental pollution were distinguished. That was mainly done in terms of the characteristics of the atmospheric air and (to a less degree) varying annual dose of external radiation on conversion to one inhabitant. Analysis of the genetic monitoring has demonstrated the prevalence of congenital abnormalities, in the genesis of which the mutation component was of paramount importance, to be significantly higher in an ecologically unsafe area. The data obtained can be used in the regional programs aimed at the improvement of the environment, a reduction of the influence of exogenous risk factors implicated in the formation of congenital developmental abnormalities in children.

The study was undertaken to examine 80 children with congenital heart and vessel malformations, their parents (50 married couples), and 100 families with healthy children. The antigen typing of the HLA system was accomplished by using the Terasaki microlymphocytotoxic test. A statistically significant correlation was found between B35 antigens and septal malformations, vascular diseases, and congenital cardiomyopathies. The unfavorable obstetric history was shown to play a role in the occurrence of developmental malformations. The data of an analysis of HLA antigens in the parents of the children with congenital malformations suggest that a marked HLA antigen compatibility of married couples is one of the cause of pregnancy complications and developmental malformations in their offspring.

Birth-histories and files of 42,275 newborn infants, ambulatory outpatient files of 38,280 children aged between 6 days and 15 years, and also findings of 4,153 autopsies were studied. The study was conducted for the years 1979-87. It was found that on the whole the incidence of congenital malformations (CM) in children 0-15 years old was 2.83 percent, last three years this indicator being 3.27 percent. Among stillborns the proportion of CM was 10.2 percent, among newborns--13.8 per 1,000 and for the last three years it rose up to 18.2. The leading congenital defects in newborn infants, according to prevalence, were malformations of organs of movement, abdominal wall and gastrointestinal tract, malformations of cardiovascular system. The analysis of CM structure among dead children indicated that the most prevalent were malformations of the central nervous system and also two last above-mentioned groups. The majority of children with CM among the dead were children of the first year of life. Proceeding from these findings the principal ways of CM prevention were suggested and main risk groups with regard to birth defects were identified.

The effects of alpha-tocopherol and its derivatives on the energy metabolism in mitochondria were studied. It was shown that alpha-tocopherol derivatives with short hydrocarbon chain produce uncoupling effects, while alpha-tocopherol itself as well as its long-chain derivatives (tocopheryl acetate and tocopheryl stearate) exert no effect. All the derivatives studied produced an inhibitory effect on DNP-stimulated mitochondrial ATPase. As far as these compounds did not influence the activity of mitochondrial F1 factor, it was concluded that the effects of the compounds were due to membrane modification.

The authors examined case records and autopsy protocols of 6173 dead children aged 0-15 years made in the regional pediatric dissecting room over the past 15 years. The incidence of congenital malformations (CM) in the dead children was found to range from 22.5 to 29.2%. The central nervous and cardiovascular systems, and gastrointestinal tract proved to be the most affected organs, some increase in the incidence of CM observed in the last years occurring by higher affliction rates of just these systems. As for the proportion of multiple CM (MCM) in the total number of malformations, it accounts for 30.4%. The trends in the incidence of MCM have not substantially changed among dead children over the past 15 years. The contribution of CM as a cause of death among children has also remained unchanged. In 77.5% of the cases, CM is regarded as the major abnormality in the autopsy diagnosis. Children with CM predominantly die in neonatal (up to 29.2%), perinatal (up to 16.2%) periods, and, in general, in infancy (greater than 90%).

The authors analyze clinical material and autopsy findings of fetuses and children dying at the age from 0 to 15 years during the last 15 years. For etiological evaluation of developmental congenital malformations the authors analyzed anamnesis, genealogy, dermatoglyphics, table materials, biochemical examination, karyotyping. The most frequent organs involved were the central nervous system, cardiovascular system, gastrointestinal tract and to a lesser degree--the genitourinary, osseous and respiratory systems. Congenital developmental malformations were registered in 10.2% of cases: in the prenatal period from 13.1 to 16.2% at different age. Among the children who died with developmental congenital malformations most were in the first year of their life.

A study was made of the immunogenetic parameters--the physicochemical properties of the circulating immune complexes (CIC) and their interrelation with HLA antigens--in 70 children aged 1 to 15 years with primary acquired glomerulonephritis (PGN). It has been demonstrated that the active period of PGN is characterized by high conceptration of the CIC of medium size (molecular mass) in children with HLA antigens. This characterizes the predisposition of the persons with the established phenotype to PGN as well as evidences that CIC play an undoubtful role in the pathogenesis of autosensitization in PGN.

The effect of azide on the activity and phosphorylation of ATPase in rat liver mitochondria was studied. It was shown that in the absence of exogenous Mg2+ azide inhibits both the activity and phosphorylation of ATPase. In the presence of exogenous Mg2+ azide inhibits ATPase hydrolysis, but does not inhibit the enzyme phosphorylation. It is concluded that the one-sided effect of azide on the activity of the ATPase (ATP-synthetase) mitochondrial complex is realized by participation of Mg2+. It was found also that the inhibitory effect of azide depends on the exogenous Mg2+ concentration.

In normal children of the first 6 months of life the erythrocyte membrane stability assessed from the characteristics of its passive K+-permeability depends on the feeding pattern. The greater stability is observed upon natural feeding while the least upon feeding by means of cow's milk dilution. Apparently, varying stability of cell membranes lies at the basis of appreciable disorders of membranes permeability during acute pneumonia in children kept on irrational feeding (with simple mixtures). Thus, in this group of the patients, there are higher indicators of passive K+-permeability of the erythrocyte membrane and the most significant changes in some of the characteristics of bioenergetic processes (intracellular content of potassium, ATP, transmembranous potassium distribution ratio).

The inhibition of succinate- and NADH-oxidase activities of submitochondrial particles by 4,7-diphenyl-1,10-phenantroline was studied. The inhibition was shown to increase when the particles were pretreated with SH-reagents. The treatment of submitochondrial particles with ethanol in the presence of 1,10-phenantroline resulted in a complete inactivation of succinate oxidase and succinate: tetramethyl-n-phenyldiamine reductase; the succinate PMS reductase activity was only partially inhibited after such treatment. It is concluded that tetramethyl-n-phenyldiamine and phenazine metasulfate react with different sites of the succinate dehydrogenase complex. The changes in the properties of submitochondrial particles after ethanol--phenantroline treatment are apparently due to the effect of non-polar solvent rather than to the extraction of non-haem iron.