[Current problems of ante- and perinatal pathology]. / Aktual'nye problemy ante- i perinatal'noi patologii.

The paper provides strong evidence for further postmortem investigations in the ante- and perinatal periods, in the combination of those of the afterbirth. The very early antenatal abnormalities (gametopathy and blastopathy) have been less studied. There are predominant congenital malformations of the organs concurrent with placental incompetence among embryopathies. In the fetal period, the authors identify an abnormality in the early fetal phase when placental insufficiency, fetal hypoxia and intrauterine death prevail; that in the mid fetal phase concomitant with infectious fetal injuries and acute and chronic placental failures due to maternal diseases; and that in the late fetal phase that covers perinatal death. The time course of reasons for stillbirth and neonatal death was analyzed in Moscow in 1986-1989. Etiological causes of early and late abortions have been identified, which is a real reserve of reduction in ante- and perinatal mortality rates in this country.

[Congenital immunodeficiency syndromes]. / Vrozhdennye immunodefitsitnye sindromy.

The definition of the congenital immunodeficiency syndrome (CIDS) is given. Structural alterations of the thymus in combination with zonal disturbances and cell composition of the peripheral lymph organs are main morphological manifestations of CIDS. The most reasonable classification of CIDS is that based on etiology and thus making necessary to reconsider the existing terminology with an exclusion of such terms as "primary" and "secondary" immunodeficiency. The crucial point in the CIDS pathogenesis is a time of structural disturbances resulting from the action of an altering factor on the immune system in ontogenesis. A list of diseases of the histiomonocytic system is given which can be used as a basis for developing the classification of CIDS of the above system as the most ancient element in the immune response in the phylo- and ontogenesis.

[Morphology of Chediak-Higashi syndrome]. / Morfologiia sindroma Chediaka-Khigasi.

The authors observed two boys born to cousin sib parents. The boys presented with a variant of primary immunodeficiency--Chediak-Higashi syndrome. The diagnosis was based mainly on the presence of giant azurophil granulations in proband phagocytes. The disease studied morphologically ran a specific course with development of reactive generalized histiocytosis and erythrophagia. The latter are thought to arise in response to defective phagocytosis. The patients suffered recurrent infections from which they died.

[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.

[Nosology of the fetus and child as a problem of general pathology]. / Nozologiia ploda i rebenka kak problema obshchei patologii.

Alteration is a common pathological process inherent to kymatogenesis and responsible for congenital defects which are the basic nosological unit of early kymatogenesis. Intrauterine compensatory adaptation triggers proliferation of mesenchymal tissue or hyperplasia of parenchymal cellular elements with accelerated maturation. Blastomogenesis occurs in children primarily during kymatogenesis. This fact can explain rapid progression of malignant growth during the first five years of life, the tumors being of embryonic or visceral tissue. Circulatory disorders prevail in perinatal period. They are represented by such nosological units as intrauterine hemorrhages and pneumopathies and are related to tissue immaturity. Fetal inflammation can be attributed to productive and alterative components. It is prone to generalized infection typical for early months of life. Opportunistic and viral infection is a common pathology in early infancy being the main nosological unit in this period. Later on the infection becomes local. Chronicity of infection rests upon persistent tissue immaturity.

[Structure of the thymus, immune status and the pathological process]. / Struktura timusa, immunnyi status i patologicheskii protsess.

Morphological changes in the immune system organs are described which allow the pathologist to diagnose primary syndromes of immunodeficiency. The criterion for the evaluation of the infant immune status is a thymic structure at various stages of ontogenesis as compared to the structure of a normal thymus after an antigenic stimulation.

[The nature of the major pathology in the perinatal period at the present time]. / Kharakter osnovnoi patologii perinatal'nogo perioda na sovremennom étape.

The main pathology leading to mortinatality has been determined. It is shown that in 57.8% of cases mortinatality is associated with placental insufficiency, the reason for which can be found out by the pathoanatomical study of the afterbirth. It is proposed to differentiate afterbirth disorders of implantation, placentation, circulation and inflammatory changes. Fifty per cent of neonates are shown to die from the respiratory failure associated with the formation of atelectasis, hyaline membranes and edema with pulmonary hemorrhages. Next in frequency are cephalic ventricle extravasations. The main pathogenetic factor of both neonatal pathologies is prematurity with functional and morphological tissue immaturity.

[Various hereditary immune deficiencies linked to the X-chromosome]. / Nekotorye nasledstvennye immunodefitsity, stseplennye s X-khromosomoi.

The organs of immune system in hereditary immunodeficiencies linked to X-chromosome, particularly in Nezelof's syndrome and chronic granulomatosis (4 cases) were studied histologically. The alterations of thymus found in Nezelof's syndrome suggest the disturbance of its morphogenesis at the embryonal stage, those found in chronic granulomatosis suggest the postnatal disturbance of thymus morphogenesis. The children die of polytopic infections foci. Generalized BCG-itis is observed in children with chronic granulomatosis.

[The immune system and its relation with infection process in children]. / Immunnaia sistema i ee sviaz' s kharakterom infektsionnogo protsessa u detei.

The immune system was studied in 30 cases of local infection (pneumonia) and 56 cases of generalized infection (sepsis). Predominantly children with immunologic deficiency of the humoral type (77% of the cases) characterized by unscheduled fatty transformation of the thymus, underdevelopment of B-zones of lymphoid organs, low level of IgM production and the lack of IgG and IgA production were found to die with pneumonia, whereas children with physiological immaturity of the immune system and in smaller numbers (41% of the cases) with deficiency of immunity of the cellular and phagocytic type as confirmed by immaturity of the thymic tissue or its dysplasia with hypoplasia of lymphoid organs died with sepsis. Immunological deficiency of the humoral type is accompanied by suppurative destructive lesions of the respiratory organs, immunodeficiency of the cellular and phagocytic type by necrotic changes in the septic focus and mucous membranes of the organs contacting the environment.

[Pathologic anatomy of childhood diseases today]. / Patologicheskaia anatomiia boleznei detskogo vozrasta na sovremennom étape.

The main problems developed by M. A. Skvortsov, the founder of pathology of childhood diseases in the USSR, are dealt with. M. A. Skvortsov worked on pathology mainly of infectious diseases analysing them from the point of view of the patterns of responsiveness of the child. New information which has at present enriched the theory of child's responsiveness is given: enzymatic systems and their defects, endocrine regulation and the importance of the enterine system, immune responsiveness and the importance of its defects. The current status of pathology of modern infectious diseases of childhood, in particular, umbilical sepsis, oncological diseases, etc. are dealt with. The role of organization of the childhood pathological service for further development of the M. A. Skvortsov's theory concerning pathology of childhood diseases is indicated.

[Principles of pathological diagnosis in children with congenital abnormalities]. / Printsipy postroeniia patologoanatomicheskogo diagnoza u detei s vrozhdennymi porokami razvitiia.

The features of establishment of pathological diagnosis in children with congenital malformations (CM) are discussed. An analysis of 1058 autopsy records of the centralized department of pathology of the Children's Clinical Hospital No. 1, Moscow, was carried out. CM were found in 236 cases (22.3% of the total number of autopsies). In 117 cases (49.6% of all CM observations) CM was the main disease, in 82 (34.7%) observations CM were combined with other diseases. In the establishment of pathological diagnosis in cases of combined diseases, the epidemiological situation, the importance of one or another process for statistical generalizations should be taken into consideration. In our opinion, preference should be given to cases of intrauterine, highly contagious, and nosocomial infections. In 37 (15.7%) observations CM exerted no effect on the course of the disease and was classified as the background condition.

[Significance of the immunodeficient state in human pathology]. / Znachenie immunodefitsitnykh sostoianii v patologii cheloveka.

Recurrent infectious processes, malignant tumors, allergic, and autoimmune diseases regularly occur in immunodeficient conditions. A classification and data are presented on the basis of which different types (cell-mediated, humoral, combined, complementary, phagocytic) and nosological forms of primary, predominantly hereditary immunodeficiency syndromes may be distinguished. Pathological processes and diseases in which secondary acquired immunodeficient conditions may develop are listed. According to the author, morphology of the thymus is most informative for a pathologist with regard to diagnosis of the primary or secondary nature of an immunodeficiency syndrome. The problem of pathological diagnosis in immunodeficient conditions is discussed.

[Features of sepsis in children today]. / Osobennosti sovremennogo sepsisa u detei.

High risk groups of infants with regard to sepsis are described. The first high risk group includes newborn babies, infants of the first 3 months of age, premature babies, the second babies with congenital defects of the immune system (classified and unclassified defects), the third babies with acquired immune deficiency conditions due predominantly to the pathology of therapy. Septicemia remains the main clinico-anatomical form of sepsis in infants. The morphological criteria of this form of sepsis are described. In the lack of decrease of sepsis incidence in infants pathology of a therapy plays a great role, but it should be remembered that sepsis nowadays is observed in babies who previously had died within a short period of time after birth or after the onset of the disease.

[Current aspects of the problem of thesaurismoses]. / Sovremennye aspekty problemy tezaurismozov.

The main changes occuring in the theory of thesaurismoses are presented briefly. Main groups of thesaurismoses characterized by disorders of one or another kind of metabolism are dealt with. Most frequently occuring forms of thesaurismoses are described and by their examples it is shown how new nosological entities are distinguished by the nature of metabolic disorders, by the defect of the enzyme, and by the degree of severity of the enzymatic block. The main criteria for morphological diagnosis of thesaurismoses are presented including the presence of accumulation cell, their localization in different organs, histochemical analysis of accumulation products and electron microscopic findings. Biochemical examinations are considered to be essential for diagnosis.