[Calcification and ossification of lungs].

The different types of lung calcification and ossification including idiopathic and developing after various diseases are in the article. We have described rare lung pathology - pulmonary alveolar microlithiasis. We have drawn attention to the possible mechanisms of lung calcification and ossification development.

[Cardiopathies in children].

Cardiomyopathies in children are rather rare, but extremely severe disorders that are little known in practical healthcare. Many of them are a cause of sudden death and they are familial. By using their findings and the data available in the literature, the authors describe the etiology, pathogenesis, morphology, and clinical presentation of dilated, hypertrophic, arhythmogenic right ventricular, histiocytoid cardiomyopathies, as well as the non-compact myocardium, and a type of cardiac connective tissue dysplasia. By keeping in mind the frequency of inheritance of cardiomyopathies, it is necessary to improve the diagnosis of these diseases not only for timely treatment, but also medical genetic counseling.

[Partial trisomy 13 for the distal long arm and its prenatal diagnosis]. / Chastichnaia trisomiia po distal'nomu segmentu dlinnykh plech khromosomy 13 i ee prenatal'naia diagnostika.

The results of prenatal diagnosis of fetal karyotype in a woman carrier of reciprocal t(13; 21) (q22; q22) translocation during her second pregnancy are presented. The first pregnancy ended in a term delivery of male twins with multiple malformations typical of the Patau syndrome. No cytogenetic investigation was carried out in this case. In the second pregnancy, unbalanced fetal 46,XX,21q+ karyotype was determined in amniotic cell cultures. The pregnancy was terminated after 19 weeks using intraamniotic PgF2 alpha. Phenotypical and pathoanatomical description of the aborted embryo presented corresponds to the clinical picture of the Patau syndrome. The 46,XX,21q+ karyotype was confirmed in fetal skin fibroblast cultures (skin biopsy specimens obtained during induced abortion), namely, partial trisomy for the distal part of the long arm (13q22) of chromosome 13 translocated to chromosome 21, as a result of inherited unbalanced variant of maternal translocation, was demonstrated. Comparative data on phenotype/karyotype correlation of individuals with partial trisomy 13 for distal part of the long aem of varying length are discussed.

[Morphogenesis of bronchiectasis in children of preschool age]. / Osobennosti morfogeneza bronkhoéktaticheskoi bolezni u detei doshkol'nogo vozrasta.

Surgical material from 24 children aged 2-6.5 years was investigated for studying morphological features and morphogenesis of bronchiectatic disease (BED) associated with lung immaturity. Leading role in the development of BED belongs to the deficiency of the lung defense at the time of the disease onset and premorbid background. Purulent-destructive alterations of the lung are the basis of grave persistent recurring BED.

[Hyaline membrane disease (idiopathic respiratory distress syndrome of newborn infants)]. / Bolezn' gialinovykh membran novorozhdennykh detei (idiopaticheskii sindrom rasstroistva dykhaniia novorozhydennykh).

A review of literature on the morphology and pathogenesis of the disease of hyaline membranes (DHM) in children is presented. The role of predisposing factors in the development of the disease such as inheritance, perinatal asphyxia, prematurity of newborns, diabetes in the mother, Cesarian section, is analyzed. The results of electron microscopic, immunohistochemical and dynamic histological examinations of the lungs in DHM are presented. The current concepts on the association of this disease with a defficiency of a surfactant, alpha1-antitrypsin, hypoperfusion and reduction of fibrinolytic activity of the lung tissue, and with the condition of the vegetative nervous system are discussed.

[Morphofunctional state of the APUD-system cells of the lungs in hyaline membrane disease of newborn infants]. / Morfofunktsional'noe sostoianie kletok APUD-sistemy legkikh pri bolezni gialinovykh membran novorozhdennykh detei.

Examination of these cells in newborn infants with hyaline membrane disease. (HMD) has demonstrated involvement of neuroendocrine elements in its patho- and morphogenesis. A crucial factor in HMD development is immaturity of the lungs, one sign of which is the presence of numerous serotonin-containing cells. Degranulation of APUD cells and serotonin lease under exposure to pathogenic factors initiate pathologic reactions in the lungs and account for the earliest manifestations of the disease.

[Marfan's disease in a pregnant woman and her fetus]. / Bolezn' Marfana u beremennoi zhenshchiny i ploda.

An observation of Marfan's disease occurring simultaneously in mother and her fetus is described indicating an intrauterine formation of the alterations specific for this disease. Specific for Marfan's disease changes in the fetal aorta are shown. The problem of the influence of pregnancy on the course and outcome of Marfan's disease is discussed.

[The importance of the cytological study of the tracheobronchial aspirate in newborn infants with the respiratory distress syndrome who are on artificial pulmonary ventilation]. / Znachenie tsitologicheskogo issledovaniia trakheobronkhial'nogo aspirata u novorozhdennykh detei s respiratornym distress-sindromom, nakhodiashchikhsia na iskusstvennoi ventiliatsii legkikh.

Cytological and pH-metrical investigations of the tracheobronchial aspirate were performed in 43 children with a respiratory distress syndrome (RDS) on artificial pulmonary ventilation. The content over 40% in the cytogram of polymorphonuclear leukocytes (PNL) indicated infectious genesis and below 10%--noninfectious genesis of RDS. The content of PNL in the range of 11-39% requires additional clinical data which would help to establish RDS cause.

[Morphological changes in the primordia of the deciduous teeth during an exacerbated course of the antenatal period]. / Morfologicheskie izmeneniia zachatkov vremennykh zubov pri otiagoshchennom techenii antenatal'nogo perioda.

Histologic examination of 380 rudimentary deciduous teeth of 91 stillborn babies or corpses of newborns dead within 16 days after birth and comparison of the results with clinical data brought the authors to a conclusion that the diagnosis of enamel hypoplasia is erroneous because all dental tissues are involved during this condition. Pathohistologic changes in the enamel, dentin, and tissues adjacent to dental rudiment are described in detail, all these tissues developing under unfavorable conditions of antenatal odontogenesis.

[The antioxidative defense system in the liver of rat fetuses upon antenatal exposure to lead]. / Sostoianie sistemy antioksidantnoi zashchity v pecheni potomstva krys pri antenatal'nom vozdeistvii svintsa.

The activity of Zn, Cu-containing superoxide dismutase was studied in the liver of 20-day old fetuses of female rats exposed to different concentrations of lead in different periods and during pregnancy. There were significant changes in the enzyme activity depended on a used dose of lead and on the duration of exposure to this compound. Activation of free radical processes and impairment of the antioxidative defense system are likely to be one of the basic mechanisms responsible for hepatic damage during prolonged antenatal lead intoxication.

Effect of prenatal lead exposure on superoxide dismutase activity in the brain and liver of rat fetuses.

Prenatal lead exposure had a damaging effect on Cu/Zn superoxide dismutase activity in the brain and liver of rat fetuses (20 days of gestation). The decrease in Cu/Zn superoxide dismutase activity in the brain and liver of treated fetuses reflects activation of free radical processes and impairment of the antioxidant defense system during prenatal lead exposure.

[The pathogenesis and morphogenesis of noninflammatory lung diseases in premature infants]. / O pato- i morfogeneze nevospalitel'nykh zabolevanii legkikh nedonoshennykh detei.

Studies of the morphofunctional status of the lungs in premature infants showed that the factors responsible for the immaturity of the bronchoalveolar tissue played a major role in the patho- and morphogenesis of noninflammatory conditions: atelectasis and hyaline membrane disease. Of special significance is inadequate differentiation of the alveolar parenchyma and blood-air barrier as well as the presence of large amounts of serotonin-containing APUD cells in the immature lungs. When released into the surrounding parenchyma under the influence of pathogenic factors, serotonin brings about the development of broncho- and vasoconstriction, microthrombosis, and increased permeability of the vascular walls, which induces the development of hyaline membrane disease in premature infants. The findings open up new possibilities for the elaboration of new prevention and therapy patterns in combating this condition.

[Artificial late abortion for medical genetic indications]. / Iskusstvennoe preryvanie beremennosti v pozdnie sroki po mediko-geneticheskim pokazaniiam.

Late abortions have been induced for genetic indications in 96 women using intra-amniotic administration of 20% sodium chloride or Enzaprost. The results were compared within this group and with a control group of 90 women whose pregnancies were terminated for other indications at similar dates and with the same agents. The use of 20% sodium chloride was associated with significantly higher blood loss and greater lengths of abortions. Patterns and rates of complications were comparable in both groups. These data suggest a utility of 20% sodium chloride and, especially Enzaprost, in late pregnancy termination for genetic indications.

[Value of echoencephalography in the diagnosis of periventricular leukomalacia in children and the prognosis of their development]. / Znachenie ékhoéntsefalografii v diagnostike i prognoze razvitiia detei s periventrikuliarnoi leikomaliatsiei.

The investigation was conducted to reveal reliable echographic features of early (necrotic) stages of periventricular leukomalacia (PVL) on the basis of retrospective analysis of echograms of decreased children (with postmortem diagnosis of PVL) and survived newborns with pronounced cystic changes in the periventricular areas (advanced PVL stage), which were revealed during dynamic echoencephalography. Altogether 268 echograms of 78 children with a birth-weight from 700 to 2400 g were analysed. All children but one were born preterm. Ultrasound device "Aloka-SSD 118" (Japan), with a transducer frequency of 5mHz, was used during frontal fontanel echoencephalography. Three patterns of echographic image of periventricular areas compromised with the aforementioned disease were identified. In the detection of early PVL stages, the specificity of the technique was 94% and sensitivity, 86%. The followup that lasted from 9 months to 2.5 years revealed varying pronounced neurologic disorders in all the survived children with PVL.