[The role of Fas/FasL system in the regulation of tumor-immune system interactions in papillary thyroid carcinoma in children and adolescents]. / Sistema FAS/FASL i ee znachenie dlia reguliatsii vzaimootnoshenii opukholi i immunnoi sistemy pri papilliarnom rake shchitovidnoi zhelezy u detei i podrostkov.

Fas(APO-1/CD95) is a transmembrane protein that induces apoptosis in susceptible normal and neoplastic cells upon cross-linking by its ligand (FasL). Changes in the Fas and FasL expression were suggested to be a potential mechanism of tumor immune evasion. Thyroid cancer in children and young adults of the Republic of Belarus is the consequence of the Chernobyl accident. Papillary thyroid cancer (PTC) is the most common variant of thyroid carcinoma. This study was performed on the formalin-fixed and paraffin-embedded tissues obtained after surgery from 43 patients with PTC. The presence of Fas and FasL in thyroid tissue specimens was examined immunohistochemically. Positive staining for FasL was observed on neoplastic thyrocytes, whereas staining of normal thyroid cells was weak or absent. The pattern of staining was membranous and cytoplasmic. Staining of lymphocytes both in tumor tissue and in lymph nodes for FasL was weak or absent. Fas expression was found on normal thyroid cells, cancer cells and lymphocytes both in tumor and in lymph nodes with metastases. In lymph node metastases, in lymphocytes adjacent to FasL cancer cells morphological signs of apoptosis were observed.

[Various aspects of immunohistochemical and molecular-biological characteristics of papillary cancer of the thyroid in children]. / Nekotorye aspekty immunogistokhimicheskoi i molekuliarno-biologicheskoi kharakteristiki papilliarnogo raka shchitovidnoi zhelezy u detei.

The dependence of the tumor cells differentiation upon the tumor histoarchitectonics, tumor type and patients' sex is studied immunohistochemically and by the in situ hybridization in 57 cases of thyroid carcinoma in children from the Belarus republic. A significant decrease of thyroglobulin synthesis and accumulation in the solid structures of papillary carcinoma is found. A decrease of the degree of the tumor biological differentiation in males is observed regardless of tumor histoarchitectonics. Increased accumulation of thyroglobulin (without change in its synthesis) in normal follicular epithelium found in parallel with increased intrathyroidal carcinoma dissemination and the size of the dominant tumor needs further investigation.

[Thyroid pathomorphology in children of the Republic of Byelarus subjected to radiation exposure as a result of the accident at the Chernobyl Atomic Electric Power Station]. / Patomorfologiia shchitovidnoi zhelezy detei Respubliki Belarus', podvergshikhsia radiatsionnomu vozdeistviiu v rezul'tate avarii na Chernobyl'skoi AES.

Early and late effects of short-living iodine isotopes on the thyroid of children in Byelarus are described. Early effects manifested with an increase in the thyroid functional activity in children up to one year of age during 1986. Follicular cell hypertrophy is considered as a marker of the radiation effect at a dose level from 0.1 to 1 krad. Late effects are thyroid carcinomas the incidence of which in the country rose ten-fold within the last 5 years.

[Tissue reactions in children treated with hemodynamic dextran and detoxication polyvinylpyrrolidone plasma substitutes]. / Tkanevye reaktsii pri ispol'zovanii gemodinamicheskikh dekstranovykh i dezintoksikatsionnykh polivinilpirrolidonovykh zamenitelei plazmy krovi u detei.

By means of light and electron microscopy tissue reactions produced by repeated use of polyvinylpyrrolidone (PVP) and dextran plasma substitutes are studied on the material of 20 autopsies and 4 biopsies as well as in 65 experimental cases. Delayed type of PVP accumulation in tissues after the hemodes administration is determined as well as its dependence on the dose, time, kidney function and the presence of inflammatory foci. The differences in tissue reactions after administration of dextran and PVP substitutes are established. The results indicate that medicinal thesaurismosis may develop only after repeated infusions of PVP substitutes, while dextran accumulation is of a transitory type and results in characteristic morphological changes in the renal epithelium only.

[Iatrogenic diseases in children based on the data from pathologicoanatomic autopsies]. / Iatrogennye zabolevaniia u detei po dannym patologoanatomicheskikh vskrytii.

Iatrogenic diseases were studied on the material of 500 autopsies taken without selection of children who died in 1988-1989. Iatrogenic diseases were found in 185 cases (37%), most frequently at the age of 7-28 days (65.8% of all dead children), least frequently at the age up to 7 days (18.5%). Total number of iatrogenic diseases (270) exceeded the number of observations. Nosological structure is given: complications of reanimation and intensive therapy were most frequent (71.5% of all iatrogenesis), surgical iatrogenesis was 19.2%. Iatrogenic diseases were the only cause of death or an important factor in the death in 13.6% of all autopsies of children born alive.

[Aplasia of the radius and mechanisms of deformity of the arm]. / Aplaziia luchevoi kosti i mekhanizmy deformatsii verkhnei konechnosti.

A complex study (roentgenographic, arteriographic, anatomic, anthrometric and microscopic) of 16 upper extremities of stillborn and deceased infants with aplasia and hypoplasia of the radius has been carried out. The etiologic factors of the defect were chromosomal and monogenic mutations and multifactor causes. It has been demonstrated that: 1) in cases of reduction of the radius the structure of the arteries, nerves and muscles in destroyed mostly in the radial border of the forearm and hand; 2) the severity of the changes in the anatomic depends on the type of the defect; 3) the pathomorphologic manifestations of the forms of the defect characterized by the same severity are mostly similar. The mechanisms of deformations of the hand in aplasia (hypoplasia of the radius) are discussed.

[The prospective directions of research in teratology]. / Perspektivnye napravleniia issledovanii v teratologii.

Unsolved problems of modern teratology are discussed. The monitoring of the congenital malformation incidence is one of the variants of evaluation and control of the mutation pressing in the population. The investigation of human foetuses obtained in artificial abortions may be very helpful in this respect. The investigation of the phenotypical manifestations of malformations in the human prenatal ontogenesis and the use of the results for the creation of notion on the malformation morphogenesis seems to be perspective. The definition of the tissue dysplasias and their classification (dystopia, dyssynchronia, hamartomas) are given. The issue of the tissue malformations during the postnatal development is not similar. They may be asymptomatic, or to disturb the function of the organ concerned, or to predispose to chronic inflammation or neoplastic growth.

[The disseminated intravascular coagulation syndrome in endotoxin shock]. / Sindrom disseminirovannogo vnutrisosudistogo svertyvaniia krovi pri éndotoksinovom shoke.

Dynamics of blood coagulation and morphological changes after the intravenous administration of Salmonella typhimurium endotoxin is studied experimentally in Chinchilla rabbits. The stages of blood coagulation are established characteristic for the above syndrome. The signs of the developing blood stasiopathy with the domination of vascular wall damage and thrombocyte aggregation are observed. No considerable consumption of the blood coagulation factors is found. It is concluded that a leading role in the pathogenesis of the endotoxin shock microcirculatory disturbances belongs to the vascular damage and aggregation thrombocytopenia and not to the coagulopathy consumption.

[Astrocytic glia in the pathogenesis of Creutzfeldt-Jakob disease]. / Astrotsitarnaia gliia v patogeneze bolezni Kreittsfel'dta--Iakoba.

The role of astrocytes in pathogenesis of Creutzfeld-Jakob disease (CJD), one of the slow virus infections of human central nervous system caused by an unconventional virus (prion) has been studied. Histological and ultrastructural examinations of the brain of both dead people and experimentally infected guinea pigs, combined with the data on morphofunctional state of infected monolayer glial culture analysis have shown astrocytes, as well as neurons, to be target-cells for the causative agent of the disease. The astrocyte changes observed were evaluated as proliferative-degenerative ones with the activation prevailing over the cell destruction. It was determined that both fibrous and protoplasmic astrocytes are sensitive to the cell-destructive effect of the CJD agent. Only few astrocytes possess phagocytic activity against the background of CNS degenerative changes in CJD. It is supposed that activated astrocytes are involved in new intercellular reactions, this involvement aggravating the pathological process.

[Morphofunctional evaluation of the testes in cryptorchism]. / Morfofunktsional'naia otsenka iaichek pri kriptorkhizme.

In both abdominal and inguinal cryptorchism, complex morphological changes are observed with age in the undescended testis. Significant changes in the size of the seminiferous tubules are observed morphometrically; the most informative index was the surface of the tubuli and their relative volume in the total volume of the organ. The main importance in the development of cryptorchism belongs, in the authors' view, to the hormonal disturbance of the anterior lobe of hypophysis and Leydig cells which are practically absent in the undescended tests in fetuses, newborns and children of the first year of life with multiple congenital developmental abnormalities.

[Clinico-laboratory and pathomorphologic study of intravascular blood coagulation syndrome in experimental amniotic embolism]. / Kliniko-laboratornye i patomorfologicheskie issledovaniia DVS- sindroma pri émbolii okoloplodnymi vodami v éksperimente.

The time-course of hemocoagulative and morphological changes affected by intravenous administration of amniotic fluids was examined in experiments on Chinchilla rabbits weighing 2.0-2.5 kg. Laboratory studies revealed profound phasic changes in hemocoagulation (hyper- and hypocoagulation) which were typical of the DIC syndrome. At the same time, pronounced hemocirculatory abnormalities were pathomorphologically detected chiefly in the lung; there were also highly moderate phenomena of platelet aggregation and thrombus formation. These findings suggest that the contribution made by the demands of coagulative factors is not so significant in the pathogenesis of microcirculatory abnormalities in amniotic embolism as it is generally accepted today. The paper provides evidence for expediency of performing special investigations to elucidate the real role which the DIC syndrome plays in the pathogenesis of microcirculatory abnormalities in other diseases.

[Congenital anophthalmos]. / Vrozhdennyi anoftal'm.

The authors analyze 8 cases of congenital anophthalmia (CA). In all the cases this condition has been a component of multiple congenital developmental abnormalities; in 4 cases the defect has been nosologically diagnosed. Developmental defects of the central nervous system have been revealed in all the cases. Histologic studies have made it possible to single out, with due consideration for the normal embryogenesis of the eye, the following variants of CA: (1) complete anophthalmia, (2) partial anophthalmia--(a) purely neuroectodermal, (b) mixed mesodermal/ectodermal, (3) anophthalmia with cysts, (4) anophthalmia with intracranial synophthalmus. The possible mechanisms of teratogenesis acting in every case of CA are discussed.

[Structure of surgical and biopsy material according to the findings of the centralized pediatric autopsy laboratory of Minsk]. / Struktura operatsionnogo i biopsiinogo materiala po dannym Tsentralizovannoi detskoi prozektury Minska.

Structure of the surgical and biopsy specimens from 9765 children aged from 1 day to 14 years was studied. According to the data obtained at the pediatric autopsy laboratory, organs of the alimentary, reproductive, urinary systems, skin, lungs and bones are most frequently subjected to an intravital histologic study. Most common among general pathologic processes are: inflammation (66,3%), tumours (13,5%) and congenital malformations (4,7%). The incidence of benign tumours is 81,5%, of malignant ones--5,2%, and that of tumour-like processes--13,3%. In most cases (49,9%) the tumours are of dysontogenetic origin. Such congenital malformations as atresia and stenosis on different esophageal levels, as well as aganglionosis, renal and ureteral dysplasias, ovarian dysplasia and hypoplasia occur most frequently. Inflammatory processes, mainly nonspecific ones, localize in the vermiform appendix, lymph nodes, bones, kidneys, lungs. Hyperplastic processes, immune reactions and reactive states (lymph nodes, tonsils) are not infrequent.

[The main etiologic groups of congenital developmental defects and the problems of diagnosis and thanatogenesis]. / Osnovnye étiologicheskie gruppy vrozhdennykh porokov razvitiia i nekotorye voprosy diagnostiki i tanatogeneza.

The proportion of congenital developmental defects (CDD) in children decreased in perinatal or early age is increasing. According to national and foreign data this figure is in the range of 21-41%. The etiologic structure of CDD for the last 5 years (1980-1984) basing on the data from the Minsk Teratologic Center has not practically changed. As in the previous 5 years, 50.8% of CDD belong to multifactorial ones, 14.3% are due to single gene mutations (8.4% of them are inherited according to dominant type and 5.9%--according to recessive type). Chromosome and gene mutations caused 7.9% of CDD. About 2% of CDD are induced by the effect of environmental factors on the embryo (less frequently on the fetus). Etiology of 25.1% of CDD is not clear.

[Pathological anatomy of monogenic syndromes of multiple congenital developmental defects]. / Patologicheskaia anatomiia monogennykh sindromov mnozhestvennykh vrozhdennykh porokov razvitiia.

Morphological study, with no selection, is performed of 1106 stillborns and children died before one year of age with congenital malformations (CM), 536 (46.9 +/- 1.5%) among them having multiple CM. Syndrome diagnosis is made in 44.1 +/- 2.1% cases: in 26.9 +/- 1.9% among them these were syndromes of chromosomal and in 17.9 +/- 1.7% syndromes of non-chromosomal etiology. Monogenic hereditary forms among non-chromosomal syndromes were in 42.1 +/- 4.8%. Comparative analysis of frequency and types of CM of various systems in monogenic and chromosomal syndromes and unclassified CM showed that the monogenic syndromes by their phenotypic manifestations are close to the chromosomal syndromes. The uniformity of alterations of certain morphological structures in these syndromes appear to be the consequence of common ways of realisation of both mutant genes in monogenic syndromes and their imbalance in chromosomal syndromes.

[Bowen-Conrad syndrome]. / Sindrom Bouéna-Konradi.

An observation of multiple malformation defects (Bowen-Conradi syndrome) is presented. The main morphological manifestations included: marked prenatal hypoplasia, facial dysplasias, microgeny, clinodactylia of little fingers, hypospadia, cryptorchism, changes in the central nervous system. All the patients with this syndrome die within the first year of life. The disease is inherited by the autosome-recessive type.

[Multiple developmental defects. The Neu-Povýsilová syndrome]. / Mnozhestvennye poroki razvitiia. Sindrom Noia-Povyshilovoi.

An observation of Neu-Povýsilová lethal syndrome of multiple congenital malformations and data on 6 cases of this syndrome described in the literature are presented. The main morphological manifestations of the syndrome include markedly manifest prenatal hypoplasia, defects of the CNS and the extremities. The presumed type of heredity is autosomal-recessive.