[Clinical and morphological characteristics of renal hypoplasia in children].

The course of the disease was studied in children with renal hypoplasia on the basis of the results of clinical observations and analysis of autopsy protocols. The data of clinical observations of 236 children aged 0 to 15 years were analyzed. Complications and cause of death were assessed; concomitant malformations were analyzed with consideration for pathoanatomic findings. The results indicated that diverse hypoplastic malformations were in other organs and systems. The spectrum and location of congenital malformations were various. Bilateral involvement was most common in died children. There is a high rate of congenital malformations in the small pelvis and chest of these patients. The degree of renal dysfunction much varies in children with renal hypoplasia.

[Effect of anti-helicobacter therapy on the hepatic glucuronyl transferase system of adolescents with Gilbert's syndrome].

UNLABELLED: Bilirubin biotransformation occurs with the participation of the glucuronyl transferase (GTF) system of the liver and hepatocyte membranes. Disturbances in these systems may result in a rise of blood bilirubin levels and disbalance between direct and indirect bilirubin leading to jaundice. Gilbert's syndrome (GS) is a genetic disorder associated with the enhanced level of indirect bilirubin due to GTF insufficiency. MATERIALS AND METHODS: The study included adolescents aged 13.4 ± 0.42 yr divided into 2 groups depending on anti-Helicobacter therapy (AHBT). We measured levels of direct and indirect bilirubins, their ratio, and direct bilirubin content as percentage of total bilirubin at admission to and discharge from the hospital. The daily incremental growth of both bilirubin fractions was calculated RESULTS: Detailed analysis revealed negative effect of AHBT on the GTF system attributable to its impaired stability in patients with abnormal genotype. CONCLUSION: Prescription of AHBT to children with Gilbert's syndrome requires the thorough choice of medications and monitoring of their potential effect on the GFT system.

[Cholelithiasis and cholesterosis of a gallbladder: a comparison of pheno-genetypic characteristics of patients].

A study was and comparison was carried out of the phenotypical and genotypical features in 115 patients with cholelythiasis and 97 patients with cholesterosis of the gallbladder. The received data proves that cholelythiasis is characterized by the domination of (prevalence) of type O I blood type, decrease of blood types B III and AB IV in comparison to the control group and to the group with cholesterosis of the gallbladder (p < 0.05), increase of the frequency of gene O decrease of frequency of gene B, decrease of heterozygosis. Patients with cholesterosis of the gallbladder are characterized by the increase of frequency of gene A, decrease of ratio of patients with blood type B III and increase of patients with AB IV blood type (p < 0.05). The received data (results) show the presence of different and multidirectional phenotypical and genotypical characteristics in patients with cholelythiasis and cholesterosis of the gallbladder, and therefore differ genotypically.

[Duodenal atresia in the newborn].

The specific features of intrauterine development and pheno- and genotypic characteristics were studied in newborn infants with duodenal atresia. Main anthropometric parameters at birth (weight, height, and head and chest circumferences), concomitant developmental malformations, gene frequencies and phenotypic combinations of ABO and Rhesus factor blood groups were analyzed. The babies with duodenal atresia were found to have considerably impaired intrauterine development, multiple concomitant hypoplastic developmental abnormalities, significantly higher frequencies of the ORh+ phenotype, and lower frequencies of the ARh+ phenotype.

[Intestinal malrotation: genetics features and other congenital malformations in children].

The article is devoted to features of prenatal development of children with intestinal malrotation. Mass, height, head and chest circumferences of children after birth have been studied. In addition all associated malformations, a blood group (ABO system) and the rhesus-factor have been investigated. In comparison to the control group of newborn the disorders of prenatal development have included associated malformations, low anthropometric data after birth, decreasing of pA gene frequency. Notable, children with intestinal malrotation have had high frequency of malformations as in the abdominal and thoracic cavities as outside intestinal innervations: the small pelvis, the facial skeleton, the upper and lower extremity.

[Type 1 and type 2 interferon inductor (cycloferon) in therapy of children with pyelonephritis associated with herpes viruses].

The clinical and laboratory efficacy of the treatment of children with pyelonephritis with addition of cycloferon, an inductor of early interferon of types 1 and 2, to the main therapy was studied. The mechanism of the cycloferon action was described. The clinical and laboratory remission within a year was observed in 64.3% of the patients treated with addition of cycloferon vs. 47.1% of the patients under the main therapy without the cycloferon addition. The number of the relapses lowered to 7.1% vs. 20.6% of the episodes in the control group. The minimal risk of the disease exacerbation (0.37) in the patients treated with cycloferon and the relative risk of the unfavourable outcomes among the patients under the therapy with addition of cycloferon (0.5967< or =1) were determined.

[The relationship between certain phenotypic traits and the function of individual links of immunity in children with acute pneumonia]. / Sviaz' mezhdu opredelennymi fenotipicheskimi priznakami i sostoianiem otdel'nykh zven'ev immuniteta u detei, bol'nykh ostroi pnevmoniei.

While studying immunogenetic indexes in 250 children with acute pneumonia, it is revealed that there is a connection between their phenotypic peculiarities (blood group B/III/, MN, P--dry type of ear-wax, low indexes of mass of the body and high ones of the body length during birth) and indexes of T-lymphocytes and immunoglobulins of blood serum A, M, G. It has permitted recommending to perform purposeful therapeutic and prophylactic measures including immunocorrection for these children.

[Adaptive norm and stabilizing selection for anthropometric characters at birth]. / Adaptivnaia norma i stabiliziruiushchii otbor po antropometricheskim priznakam pri rozhdenii.

Analysis carried out on medical documentation containing data on several thousand healthy and affected babies has revealed patterns of differential mortality and differential morbidity for newborns and infants which allow to estimate relative risk as a function of birth weight and body length. Adaptive norm for anthropometric traits at birth was defined as the weight or length interval in which mortality/morbidity rates were lower than the overall population level. The intensity of stabilizing selection associated with birth weight calculated from these data is several times higher in the neonatal period than for the age interval 1-12 months. Neonatal mortality in more than 50% cases can be attributed to the effects of stabilizing selection associated with birth weight.

[A population-genetics approach to the problem of nonspecific biological resistance of the human body. III. The ABO and rhesus blood group systems of healthy and sick children and their mothers]. / Populiatsionno-geneticheskii podkhod k probleme nespetsificheskoi biologicheskoi ustoichivosti chelovecheskogo organizma. Soobshchenie III. Gruppy krovi sistem ABO i rhesus u zdorovykh i bol'nykh detei i ikh materei.

ABO and Rhesus blood types have been specified in 2047 diseased newborns, diseased infants and children who died before the age of one, as well as in their mothers. 527 healthy children and their mothers were investigated as a control group. A significant difference in the ABO phenotype frequencies has been revealed between: i) healthy and dead children, ii) mothers of diseased newborns and mothers of healthy children, iii) dead children and their mothers. The significant increase in the incidence of maternal Rhesus-negative phenotype, as compared with the control group, was shown in the groups of diseased newborns, diseased infants and dead children. In the same groups, mothers differ significantly from their children with respect to the frequency of Rhesus phenotypes. The incidence of Rhesus-incompatible mother-child pairs in the groups of diseased newborns, diseased infants and dead children was shown to be two times higher than the respective frequency in the control group and the expected frequency. A certain increase in the frequency of ABO-incompatible pairs was revealed in the groups of diseased newborns and dead children, but the difference, as compared to the control group, did not prove to be statistically significant. A hypothesis was advanced to the effect that the mother-child incompatibility for Rhesus and ABO antigens may result not only in fetal wastage and haemolytic disease of newborns, but also in the decrease of child's resistance to diseases of different origin.

[Nonspecific resistance of the body and predisposition to acute leukemia in children]. / Nespetsificheskaia ustoichivost' organizma i predraspolozhennost' k zabolevaniiu ostrym leikozom u detei.

An analysis of 422 Moscow hospital records of children suffering from acute leukemia during the period from 1959 and continuing through to 1979 showed that the proportion of those with morphologically "average" weight and body length values at birth, was significantly lower in the group with leukemia, than in the control group of healthy children. Genetic structure of leukemic and healthy children was found to be significantly different by phenotypic combinations of 2 loci (ABO and Rhesus). The distribution of children for their age at the beginning of the disease was studied. The results revealed that children with the low weight and body length values at birth, as well as those having ARh+ and BRh+ blood groups, develop leukemia earlier than those with "average" characteristics and blood groups other than ARh+ and BRh+. The anamnesis of children which developed leukemia demonstrated a higher infectious-inflammatory disease frequency, as compared with the control group. By comparison of some parental characteristics of two groups of children, it was found that parents of children which became ill, were on the average older at the moment of baby's birth than parents of healthy children. Babies of 2nd, 3rd and subsequent deliveries were registered more frequently in the group with leukemia than in that of healthy children. Evidence presented confirms the efficiency of the population-genetic approach to the problem of nonspecific resistance of human organism to diseases.

[Genetic markers and diseases: the genetic, anthropometric and clinical characteristics of children with acute pneumonia]. / Gennye markery i bolezni: geneticheskie, antropometricheskie i klinicheskie osobennosti detei, bol'nykh ostroi pnevmoniei.

The paper presents new experimental evidence for the efficiency of an earlier developed approach (Yu. P. Altukhov, O. K. Botviniev, O. L. Kurbatova, 1979, Genetika, 15. No. 2) to the problem of nonspecific resistance of human organism to disease. Children suffering from acute pneumonia are shown to differ from a control group of healthy children by a complex of clinical, anthropometric and genetic characteristics. The group of children with acute pneumonia is characterised by a marked disposition to viro-bacterial diseases, high frequency of small developmental anomalies and by twofold decrease of proportion of children with average body length and weight at birth. Significant differences in 4 genetic systems (PGM1, ACP, MNSs and Rhesus) have been revealed. On the whole, children with pneumonia display lower level of average heterozygosity per locus and higher frequency of rare antigene combinations as well as rare electrophoretic protein variants.

[Population genetics approach to the problem of nonspecific biological resistance of the human body. II. Clinical characteristics, congenital anomalies and genetic structure of sick children with attention to birth weight and length]. / Populiatsionno-geneticheskii podkhod k probleme nespetsificheskoi biologicheskoi ustoichivosti chelovecheskogo organizma. Soobshchenie II. Klinicheskaia kharakteristika, vrozhdennye anomalii i geneticheskaia struktura bol'nykh detei s uchetom ikh vesa i dliny tela pri rozhdenii.

Clinical analysis of several groups of newborns and infants arranged by 2 metrical characters has shown that morphologically average individuals ("adaptive norm") are most resistant to the wide range of diseases including congenital malformations. The frequency of grave diseases and congenital malformations is much higher in newborns and infants with extreme (especially low) values of characters, each group of extreme variants being characterized by a specific set of diseases. Statistically significant differences in genetic structure of these groups are detected: departures from Hardy-Weinberg equilibrium and lower level of heterozygosity for ABO and Ww loci are observed in a group of children with low weight and body length values. These features are most pronounced in the group comprising dead newborns and infants.

[Population genetics approach to the problem of the nonspecific biological resistance of the human body. I. Statement of the problem and basis for the approach. Distribution parameters of anthropometric markers of normal and ill newborn infants and nurslings]. / Populiatsionno-geneticheskii podkhod k probleme nespetsificheskoi biologicheskoi ustoichivosti cheloveskogo organizma. Soobshchenie I. Postanovka problemy i obosnovanie podkhoda. Parametry raspredelenii antropometricheskikh priznakov novorozhdennykh i grudnykh detei v norme i patologii.

Distributions of two metrical characters-the weight and the body length at birth are considered in several groups of healthy, diseased and dead newborns and infants. Significant differences in statistical parameters of these distributions (mean, standard deviation, skewness and kurtosis) are demonstrated for normal and malformated children. The increased number of children with minimal deviations from the mean values of characters (adaptive norm) is observed among healthy newborns, while in groups of diseased and dead children the frequency of individuals with extreme (especially low) values is increased. The principle of classification of individuals based simultaneously on two characteris is suggested and the problem of "average phenotype" classification by the number of characters is discussed.

[Diurnal rhythm of the adrenal cortex function in patients with circulatory disorders]. / Funktsional'noe sostoianie kory nadpochechnikov v techenie sutok u bol'nykh s narusheniem krovoobrashcheniia.

The blood hydrocortisone and corticosterone content and the aldosterone level in the urine were determined for 24 hours in patients with congenital heart disease before and after operative treatment. In patients with symptoms of cardiac insufficiency both glucocorticoid and mineralocorticoid activity of the adrenal cortex was marked by deviations from the diurnal physiological rhythm. This is reflected in the continuous activation of the adrenal cortex during 24 hours; activation of mineralocorticoid function in the evening and during the night is a particularly characteristic phenomenon.