[Sanfilippo Syndrome].

Sanfilippo syndrome (mucopolysaccharidosis type III) is a lysosomal disorder caused by a defect in the catabolism of heparan sulfate. Mucopolysaccharidosis type III is the most common type of all mucopolysaccharidoses. The pathogenic basis of the disease consists of the storage of undegraded substrate in the central nervous system. Progressive cognitive decline resulting in dementia and behavioural abnormalities are the main clinical characteristics of Sanfilippo syndrome. Mucopolysaccharidosis type III may be misdiagnosed as otherforms of developmental delay, attention deficit/hyperactivity disorder and autistic spectrum disorders because of lack of somatic symptoms, presence of mild and atypical forms of the disease. Patients with Sanfilippo syndrome may have comparatively low urinary glycosaminoglycans levels resulting in false negative urinary assay. Definitive diagnosis is made by enzyme assay on leucocytes and cultured fibroblasts. There is currently no effective treatment of mucopolysaccharidosis type III, though ongoing researches of gene, substrate reduction and intrathecal enzyme replacement therapies expect getting curative method to alter devasting damage of central nervous system in near future.

[Sensitivity of hepatocytes to the effect of factors stimulating and suppressing cell proliferation in hepatocarcinogenesis]. / Chuvstvitel'nost' gepatotsitov k deistviiu faktorov, stimuliruiushchikh i podavliaiushchikh proliferatsiiu kletok, pri hepatokantserogeneze.

Sensitivity of hepatocytes to the proliferative stimulus (partial hepatectomy) and mitoinhibitory effect of carcinogen (toxic dose after operation) was studied during hepatic carcinogenesis induced by the chronic administration of diethylnitrosamine. At early stages of hepatocarcinogenesis (4-6 weeks of carcinogen treatment) in the rat liver tissue there appears and then increases a subpopulation of hepatocytes insensible to proliferation mediators. The cell number in this subpopulation essentially increased in the period preceding the development of microscopically detected neoplastic nodules.

[Interaction of growth factors and oncogenes in the tumorous transformation of cells]. / Vzaimodeistvie faktorov rosta i inkogenov pri opukholevoi transformatsii kletok.

Different aspects of the interaction of polypeptide growth factors with their receptors are reviewed. The problem of structural and functional interactions of the normal and transforming growth factors as well as the protein products of oncogenes during transmittance of a mitogenic signal and the proliferation regulation of normal and tumour cells is discussed.

[Cellular proliferation of the regenerating liver in rats after a single administration of N-nitrosodiethylamine]. / Proliferatsiia kletok regeneriruiushchei pecheni krys posle odnokratnogo vvedeniia N-nitrozodiétilamina.

Proliferative kinetics of hepatocytes and nonparenchymal cells of the regenerating rat liver has been studied after a single injection of the carcinogen in S- or G2-M-phase cell cycle of hepatocytes. Disturbances of proliferation in the mentioned cell populations and resistance of normal proliferating hepatocytes during the S-phase of the mitotic cycle to the cytotoxic effect of the carcinogen are established. Possible mechanisms of the resistance of the proliferating cells to the toxic action of the carcinogen are discussed.

[Heat shock proteins and proliferative activity of cultured hepatocytes after hyperthermia]. / Belki teplovogo shoka i proliferativnaia aktivnost' gepatotsitov v kul'ture posle gipertermii.

Hepatocytes in cell culture have been studied for their sensitivity to mitogenic effect of the epidermal growth factor, epidermal growth factor and insulin, 10% of cattle blood serum after hyperthermia. It is established that heat shock (43 degrees C, 90 min) induces hypersensitivity of hepatocytes to the mentioned mitogens, its maximum being observed 20-24 h after hyperthermia and coinciding in time with the development of thermotolerance and intensification of protein biosynthesis after the heat shock 68, 70, 94 kD.

[Characteristics of hepatocarcinogenesis after single exposure of synchronized cells of the regenerating liver to N-nitrosodiethylamine and subsequent administration of phenobarbital]. / Osobennosti gepatokantserogeneza pri odnokratnom vozdeistvii N-nitrozodiétilaminom na sinkhronizirovannye kletki regeneriruiushchei pecheni krys i posleduiushchem vvedenii fenobarbitala.

The frequency of tumour incidence in rats subjected to the single action of diethylnitrosamine to regenerating liver cells synchronized in different phases of the mitotic cycle was maximal in rats exposed to carcinogen when 62.5% of regenerating liver hepatocytes were in the S-phase. Dietary added phenobarbital increased the number and size of hepatocarcinomas especially in rats which received DENA in the G2-M phase of hepatocytes or during maximal proliferation of nonparenchymal cells. The role of proliferation of different cell populations in initiation and promotion of carcinogenesis is discussed.

[Effect of sialadenectomy on hepatic carcinogenesis in rats induced by N-nitrosodiethylamine]. / Vliianie sialadenéktomii na gepatokantserogenez, indutsirovannyi u krys N-nitrozodiétilaminom.

It is established that sialadenectomy leads to inhibition of hepatocarcinogenesis induced in adult rats by diethylnitrosamine, which is confirmed by a decrease in the incidence of tumours, their delayed growth and an increase of longevity. But sialadenectomy 2 weeks after the beginning of carcinogen treatment has no effect on the hepatocarcinogenesis. A significant molecular heterogeneity of the epidermal growth factor-competing activity is observed in acid-ethanol extracts of the liver tumour of the sialadenectomized rats.

[Effect of the epidermal growth factor on 1,2-dimethylhydrazine-induced carcinogenesis in the rat intestinal mucosa]. / Vliianie épidermal'nogo faktora rosta na kantserogenez, indutsirovannyi 1,2-dimetilgidrazinom v slizistoi obolochke kishechnika krys.

The radioimmunological and radioreceptor methods have been used to show that sialadenectomy leads to the stable decrease of the epidermal growth factor (EGF) concentration in saliva and blood serum. The mean number of colon tumours per rat was significantly lower among the rats which had been sialadenectomized before injections of the carcinogen, than in the control. But a sharp stimulation of carcinogenesis in the duodenal mucosa was observed after sialadenectomy. The production of the alpha-transforming growth factor with the EGF-competing activity for the EGF-receptors was found in the chemically-induced rat colon tumours.

[Rapid isolation of highly purified epidermal growth factor by reverse-phase liquid chromatography]. / Uskorennoe poluchenie vysokoochishchennogo épidermal'nogo faktora rosta s pomoshch'iu zhidkostnoi khromatografii v obrashchennoi faze.

The first step in the isolation of murine epidermal growth factor (EGF) from submaxillary glands was the trace enrichment of the acid-ethanol extract on Sep-pak C18 cartridges using the step elution with ethanol. The fraction eluted by 60% ethanol contained above 75% of EGF. The final purification of EGF was achieved by reverse-phase liquid chromatography on Novapak C18 column in the gradient of acetonitrile. A comparison of the effectiveness of gel filtration (on Bio-gel P-60 column) and trace enrichment (on Sep-pak C18 cartridges) of the appropriate fractions were analyzed by the reverse-phase high-pressure liquid chromatography, which had demonstrated that hydrophobic separation of EGF had a number of advantages. The homogeneous character of EGF was confirmed by the silver staining of gels after disc-electrophoresis and isoelectric focusing (pI 4.8). The pronounced mitogenic effect of EGF on the primary culture of the rat hepatocytes is described.

[Use of the method of nucleus sedimentation fractionation for studying the dynamics of cell populations in the regenerating liver]. / Ispol'zovanie metoda sedimentatsionnogo fraktsionirovaniia iader dlia issledovaniia dinamiki kletochnykh populiatsii regeneriruiushchei pecheni.

In different periods after partial hepatectomy (0, 11, 18, 24, 48 and 72 hours) isolated liver cell nuclei were separated according to their sizes using free sedimentation at 1 g in the exponential 0.1-1.0 M concentration gradient of sucrose at 4 degrees C. A sedimentogram of the intact liver cell nuclei displays three peaks corresponding to the nuclei of stromal cells, and diploid and tetraploid nuclei of hepatocytes. At 18 and 24 hours after the operation, but not at 0 or 11 hours, a curve of 3H-thymidine incorporation is represented by three maxima suggesting the influx of hepatocytes of different ploidy in the S-period. Then, 48 and 72 hours after the partial hepatectomy two additional peaks appear corresponding to 8- and 16-ploid nuclei, and a decrease in the portion of diploid nuclei is seen up to the full absence of the corresponding peak. Thus, the method of sedimentation at unit gravity of isolated nuclei using radioisotope labeling allows to obtain the information on the structure of the heterogeneous proliferating cell population.

[Characteristics of hepatocyte proliferation in the regenerating rat liver with hydroxyurea synchronization of the proliferative processes]. / Osobennosti proliferatsii gepatotsitov regeneriruiushchei pecheni krys pri sinkhronizatsii proliferativnykh protsessov oksimochevinoi.

5-week old rats underwent a partial hepatectomy (2/3 of the liver was removed). The operated rats were repeatedly injected hydroxyurea, within 9-24 or 12-24 hours following operation. Proliferation of hepatocytes and non-parenchymal cells in the regenerating liver was studied. Five or six injections of hydroxyurea increased the labelling index of hepatocytes up to 62.5% and induced proliferation of bile duct cells significantly.

[The development of an automated system for computing the "standard" values of the economic loss brought by one case of infectious disease]. / Razrabotka avtomatizirovannoi sistemy rascheta "standartnykh" znachenii ékonomicheskogo ushcherba, nanosimogo odnim sluchaem infektsionnoi bolezni.

An original and technically realizable system for the determination of the values of economic damage produced by a single case of an infectious disease has been developed, based on a concrete socio-economic analysis. The system has been realized with the use of an IBM PC/AT by means of the Econ program module developed in the SuperCalc-4 tabulated processor program environment. The use of the average socio-epidemiological, national economic and demographic statistical data has made it possible to calculate the "standard" values of the economic damage produced by a single case of 15 infectious diseases (total average, as well as for different groups of patients and measures). Analysis of the values thus obtained shows their, on the average, 1.25-fold growth, as well as their considerable variability reflecting the clinico-epidemiological nature of infectious diseases.

[Effect of diethylnitrosamine on the protein-synthesizing cell system of the rat liver]. / Vliianie diétilnitrozamina na beloksinteziruiushchuiu sistemu kletok pecheni krys.

Diethylnitrosoamine is found to alter the course of the regenerative processes induced by partial hepatectomy, which is manifested in essentially other, than in the control, changes of polyribosomes and mRNA. In early terms following DENA injection one may observe a reduced content of total polyribosomes, a sharp decrease in the amount of membrane-bound polyribosomes, the inhibited incorporation of newly produced mRNA in both classes of polyribosomes. In later terms, a proportion of the membrane-bound polyribosomes is again diminished, while the ratio of the specific radioactivity of free and membrane-bound polyribosomes is raised.

[Biochemical characteristics of major fractions of rat liver cells in dimethylnitrosamine-induced carcinogenesis]. / Biokhimicheskaia kharakteristika osnovnykh fraktsii kletok pecheni krys pri kantserogeneze, indutsirovannom nitrozodimetilaminom.

The report deals with a comparative study of the morphological and biochemical indexes in the fractions of parenchymal and non-parenchymal isolated cells of rat's liver, both intact and treated with a polytropic carcinogen-nitrosodimethylamine (NDMA)-for seven months. A higher susceptibility of parenchymal cells to NDMA was established.

[The effect of ceruloplasmin on hepatocyte proliferation in the regenerating rat liver]. / Vliianie tseruloplazmina na proliferatsiiu gepatotsitov regeneriruiushchei pecheni krys.

The influence of ceruloplasmin on cell proliferation in regeneration liver of the rat has been studied. Ceruloplasmin stimulates cell proliferation in regeneration liver increasing functional activity of the mononuclear phagocytes.

[Iron metabolism, hemopoiesis, prevention and treatment of iron deficiency anemia in multiparous women]. / Osobennosti obmenazheleza, gemopoéza, profilaktiki i lecheniia zhelezodefitsitnykh anemii u mnogorozhavshikh zhenshchin.

A total of 160 multiparous women with iron deficiency anemia (IDA) who have 4 and more children were under study. Hemoglobin parameters in patients with non-severe anemia (n = 64) fluctuated from 119 to 90 g/l, in patients with moderate severity (n = 60) from 89 to 70 g/l, in those with severe stage of anemia--69 g/l and lower. The latent anemia group consisted of women whose hemoglobin values were 120 g/l and higher, however, the figures characterizing serum iron, transferrin and ferritin content evidenced iron deficiency. Parameters of serum iron, transferrin and ferritin metabolism were studied in all the patients. Decreased levels of serum iron and ferritin and a compensatory increase of transferrin content were recorded. Manifest signs of acquired dyserythropoiesis and a significant increase of ineffective erythropoiesis (34.96 +/- 0.89%) were demonstrated in myelograms. It has been recommended that routine measures used for the treatment of IDA in multiparous women should be supplemented by anabolic agents and blood substitutes for protein parenteral nutrition.

[Modification of collagen during action light].

In work chemical modification of collagen during action visible spectrum sunlight. These changes of collagen were found to indicate a photodegradation and photooxidation processes in collagen.