Mutation in NADPH oxidase 3 (NOX3) impairs SHH signaling and increases cerebellar neural stem/progenitor cell proliferation.

Abnormalities in cerebellar structure and function may cause ataxia, a neurological dysfunction of motor coordination. In the course of the present study, we characterized a mutant mouse lineage with an ataxia-like phenotype. We localized the mutation on chromosome 17 and mapped it to position 1534 of the Nox3 gene, resulting in p.Asn64Tyr change. The primary defect observed in Nox3eqlb mice was increased proliferation of cerebellar granule cell precursors (GCPs). cDNA microarray comparing Nox3eqlb and BALB/c neonatal cerebellum revealed changes in the expression of genes involved in the control of cell proliferation. Nox3eqlb GCPs and NSC produce higher amounts of reactive oxygen species (ROS) and upregulate the expression of SHH target genes, such as Gli1-3 and Ccnd1 (CyclinD1). We hypothesize that this new mutation is responsible for an increase in proliferation via stimulation of the SHH pathway. We suggest this mutant mouse lineage as a new model to investigate the role of ROS in neuronal precursor cell proliferation.

[Urgent surgical correction of ventricular septal defects in children of first months of life].

The article presents the results of urgent surgical correction of ventricular septal defects (VSDs) and a range of associated congenital heart diseases (CHDs) in 123 children of the first 6 months of life, hospitalized between 1988 and 2002 in a severe or critical condition. In 102 patients the progression of clinical symptoms was caused by a big single VSD and in 21 patients--by multiple VSDs. 70 patients had significant associated CHDs. Significant extracardiac anomalies were revealed in 75 patients. The condition of 55.3% of children had deteriorated due to pneumonia. Correction of defects with cardiopulmonary bypass was performed after urgent examination and intensive therapy. Hemodynamically significant VSD were closed in 117 (95.2%) patients. Two patients were reoperatied due to the presence of residual VSD. Partial or full simultaneous correction of associated CHDs was performed in 68 patients. 93.5% of patients with isolated VSDs survived the operation. The survival rate of patients with associated CHDs and extracardiac anomalies was 81.5%. Postoperative mortality risk factors were found to be critical condition at hospitalization and preoperative activity of infectious process (P < 0.01 and < 0.05, respectively). Accumulation of experience and introduction of a special approach allowed an 8-time decrease of the hospital mortality, which has not exceeded 3.4% within last 3 years. Good and excellent follow-up results were obtained in 98% of the survived patients within 10 to 67 months after operation. Precise diagnostics of associated CHDs and extracardiac anomalies, intensive preoperative treatment and weighed estimation of indications and risk factors allow significant improvement in the results of primary correction of VSD and accompanying cardiovascular anomalies in children of the first months of life in a heavy and critical condition upon admission.

[Humoral immunological aspects of the pathogenesis of rheumatism, nonrheumatic carditis and juvenile rheumatoid arthritis in children]. / Gumoral'ni imunologichni aspekty patogenezu revmatyzmu, nerevmatichnogo kardytu ta iuvenil'nogo revmatoïdnogo artrytu u ditei.

The state has been studied of the humoral component of immunological reactivity according to the content of the serumal complement, complement-bounding antibodies, immunoglobulins and immunoglobulin-antibodies of the main classes in the time-related course of rheumatism, nonrheumatic carditis and juvenile rheumatoid arthritis (JRA) in children. Tissue, enzymic, streptococcal antigens have been used together with those of nucleotides, nucleases. The obtained results suggest to us that the humoral component of bodily immunological reactivity has an important part in pathogenesis of the above medical conditions. It can be used in the diagnosis of affections of the heart.

[Comparative characteristics of lipids, lipoproteins and free radical processes in blood of rabbits after ionizing irradiation and dietary cholesterol administration]. / Sravnitel'naia kharakteristika spektra lipidov, lipoproteinov, a takzhesostoianie svobodnoradikal'nykh protsessov krovi u krolikov posle vozdeistviia ioniziruiushchego izlucheniia i vvedeniia kholesterina v dietu.

Comparative study of spectrum of lypoids and lipoproteins has been conducted and also of parameters of free radical processes in blood after exposure of organisms of experimental animals to ionizing irradiation in dose of 5 Gy and inclusion of cholesterol into diet. Intake of cholesterol to experimental animals irradiated caused specific impact on indices studied testifying about deep changes in metabolism. It is important during analysis of complex set of postradiation metabolic symptoms observed in clinics.

[The cytogenetic effects of 137Cs gamma quanta]. / Tsitogeneticheskie éffekty gamma-kvantov 137Cs.

The data on the human chromosomal level have been first obtained showing that the values of relative biological effectiveness of the 137Cs radionuclide exceed 1. These findings were allowed for during intracavitary radiotherapy in more than 50 patients with uterine body cancer.

[The composition and physicochemical properties of the blood lipoproteins in rats exposed to external gamma irradiation]. / Sostav i nekotorye fiziko-khimicheskie svoistva lipoproteinov krovi krys pri vozdeistvii vneshnego gamma-oblucheniia.

Content of lipids, character of chemiluminescence of blood plasma and certain classes of lipoproteins have been studied. Geometrical parameters, nature and quantity of charged groups of lipoprotein particles accessible for titration have been determined 1 and 30 days after a single external gamma irradiation of rats in a dose of 3 Gy. The used irradiation dose exerts an expressed hyperlipidemic effect retained for one month after irradiation. The disturbances in the spectrum of blood lipids and lipoproteins are of hyper-beta and hyper-prebeta lipoproteinemia character. Considerable disturbances of physicochemical properties of different classes of lipoproteins have been detected. They are exhibited in changes of the pattern of free-radical processes, state of the charge of surface ionogenic groups and geometrical parameters of lipoprotein particles. Changes registered by the methods of potentiometric titration and correlation spectroscopy are most expressed in lipoproteins of very low density and those of low density.

[Comparative characterization of the lipoprotein spectrum and status of free radical processes in the blood of rabbits exposed to external gamma-irradiation and cholesterol administration]. / Sravnitel'naia kharakteristika lipoproteinovogo spektra i sostoianiia svobodnoradikal'nykh protsessov v krovi u krolikov pri vneshnem gamma-obluchenii i vvedenii kholesterina.

Characteristic of lipoprotein spectrum and state of free-radical processes in the animals under action of ionizing radiation and cholesterin diet are comparatively studied. The content of lipids in the blood serum increases on the 5th day after irradiation and then decreases on the 15th and 30th days. Cholesterin diet increases the content of cholesterin under practically unchanged content of triglycerides in the blood serum in all the terms of investigations. Total decrease of the intensity of induced chemiluminescence of the blood serum and erythrocytes after irradiation is shown. Peroral introduction of cholesterin evokes differently directed changes in the light sum of chemiluminescence--a decrease in erythrocytes and increase in the blood serum.

[Effect of a single and fractionated gamma-irradiation on lipid content in rat blood and tissues]. / Vliianie odnokratnogo i fraktsionirovannogo gamma-oblucheniia na soderzhanie lipidov krovi i tkanei krys.

Contents of lipids, character of blood plasma chemiluminescence and separate classes of lipoproteins as well as the content of the lipids of tissues under single and fractionated gamma-irradiation of rats in a dose of 3 Gr. The development of hyperlipidemia and hypercholesterolemia for four weeks after irradiation which are more expressed under a single irradiation, a decrease of total content of cholesterol and triglycerides in the heart and liver tissues to the end of the experiment 24 hour after irradiation are registered in the both groups of animals.

[Blood lipids and characteristics of free radical processes in specific classes of lipoproteins in rats in the early periods after gamma-radiation]. / [Lipidy krovi i kharakteristika svobodnoradikal'nykh protsessov v otdel'nykh klassakh lipoproteinov u krys v rannie sroki posle gamma-oblucheniia.

The content of lipids and lipoproteins, the state of free-radical processes and the level of eicosanoids (prostaglandin F-1L and thromboxane B-2) have been studied in the blood plasma and in certain classes of lipoproteins in rats 2 and 24 h after external gamma-radiation in doses of 1.2 and 3 Gy. Hypertriglyceridemia and hyperprebetalipoproteinemia, whose expressivity increases with a radiation dose are revealed. The activation of free-radical processes, an increase of the thromboxane V-2 content and a decrease of the prostaglandin F-1L content are observed with a dose of 1 Gy.

[The content of ceruloplasmin, fibronectin and selenium in the blood of patients with an acute myocardial infarct]. / Soderzhanie tseruloplazmina, fibronektina i selena v krovi bol'nykh ostrym infarktom miokarda.

The content of ceruloplasmin, fibronectin and selenium was evaluated in the blood plasma of acute myocardial infarction patients within the first three days after onset if the disease. Results showed a reduction of fibronectin during the first hours after onset of the disease. The content of ceruloplasmin was increased, selenium reduced during the entire period of examination indicating pronounced disturbances of lipid peroxidation in the acute period of myocardial infarction as well as potentiation of antioxidant processes.

[Dynamics of the radiation conditions and evaluation of the radiation dosage of the inhabitants of Kiev following the accident at the Chernobyl Atomic Electric Power Station]. / Dinamika radiatsionnoi obstanovki i otsenka doz oblucheniia zhitelei Kieva posle avarii na Chernobyl'skoi AES.

The estimation of the effective dose equivalents of exposure to accidental and non-accidental sources was made on the basis of the studying of radiation situation variations in Kiev after the Chernobyl accident in 1986-1989. The total annual effective dose equivalents to the Kiev population from all sources of exposure for 1986-1989 were 9.19, 7.29, 5.34 and 5.03 mSv, respectively. The necessity of radiation health measures aimed at limiting the intake of long-acting radionuclides of Chernobyl nature and reducing the exposure doses by optimizing the x-ray procedure and by regulating the natural radioactivity of building materials are under discussion.

[Content of blood lipids and characteristics of dyslipoproteinemias in people exposed to radiation during the accident at the Chernobyl nuclear power station]. / Soderzhanie lipidov krovi i kharakteristika dislipoproteinemii u lits, podvergshikhsia radiatsionnomu vozdeistviiu pri avarii na Chernobyl'skoi AES.

Lipid and lipoprotein spectrum of the blood plasma was investigated and phenotyping of hyperlipoproteinaemias was carried out in men exposed to ionizing radiation and suffered from acute radiation illness as a result of the Chernobyl accident. An increase of the total cholesterol triglycerides and content frequency of atherogenic types of hyperlipoproteinaemias in comparison with average levels of population both a year and 3 years after irradiation was observed. No essential differences were found between the obtained indices 1 and 3 years after irradiation which testified to the stability of the revealed disturbances.

[Use of some biochemical and physico-chemical indices for the evaluation of lung function in healthy school-age children]. / Ispol'zovanie nekotorykh biokhimicheskikh i fiziko-khimicheskikh pokazatelei dlia otsenki funktsii legkikh u zdorovykh detei shkol'nogo vozrasta.

The biochemical and physicochemical characteristics of nonrespiratory pulmonary functions were determined in 255 children of school age according to the condensate of exhaled air. As compared to the younger children, those belonging to the senior age groups manifested an increase of specific respiratory moisture loss. As compared to winter, specific respiratory moisture loss in summer was found to be decreased; the condensate of exhaled air was discovered to have a higher content of total protein, elevated concentration of surface active substances in the lung surfactant, and to show a certain tendency towards reduction of the intensity of the processes of free radical oxidation. No sex-associated differences were established between the characteristics under study.

[Assessment of oncogenic risk of the irradiation of the thyroid gland in humans]. / Otsenka onkogennogo riska oblucheniia shchitovidnoi zhelezy cheloveka.

The paper deals with one of the most urgent aspects of irradiation hygiene, namely assessment of risk for irradiation-induced cancers of the thyroid. A model is described to predict high mortality rates of thyroidal cancer in the population due to the catastrophe at the Chernobyl Atomic Power Station. With the model, life-time risk rates involving sex and age at the moment of irradiation, as well as an irradiation mode.

[Health-related evaluation of thyroid irradiation doses in inhabitants of the Ukrainian S.S.R. after the Chernobyl AES accident]. / Gigienicheskaia otsenka doz oblucheniia shchitovidnoi zhelezy zhitelei USSR posle avarii na Chernobyl'skoi AES.

The paper considers the radiation doses of the thyroid gland in the inhabitants from the Ukrainian SSR areas (Kiev, Zhitomir, Chernigov, and Vinnitsa Regions), which have been obtained by instrumental studies of thyroidal radioactivity in May-June, 1986, and calculated by the most conservative single-dose administration model. A hygienic evaluation has been made of the findings, taking into account the age and residence. The cumulative irradiation doses of the thyroid have been estimated for children and adults. Possible late sequelae for the areas in question may account for 1060 and 300 thyroid carcinoma cases during the whole life for children and adults, respectively.

[Thyroid irradiation doses and organization of endocrinological monitoring of the inhabitants of the Ukrainian S.S.R. after the accident at the Chernobyl AES]. / Pogloshchennye dozy v shchitovidnoi zheleze i organizatsiia éndokrinologicheskogo monitoringa zhitelei USSR posle avarii na Chernobyl'skoi AES.

The paper is devoted to one of the most important aspects of the accident at the Chernobyl Nuclear Power Station: thyroid irradiation in the residents of 8 areas in the Ukrainian SSR and the town of Pripyat, affected with radioactive iodine. Irradiation doses, prognosis of medical effects of the accident, methods and practical measures for the organization of endocrinological monitoring of the affected population were thoroughly considered. The authors proposed a scheme of health care of the population in these areas. They also discussed the structure of data bases for analysis of medico-dosimetric information on "iodine" effects of the accident. The main trends in medico-dosimetric problems of the effects of thyroid irradiation were outlined.

[Free-radical processes and blood antioxidant systems in the late period following acute radiation sickness]. / Svobodnoradikal'nye protsessy i antioksidantnye sistemy krovi v otdalennye sroki posle ostroi luchevoi bolezni.

Some indices of free radical processes and antioxidant blood protection systems were investigated in a group of persons exposed to ionizing radiation at the Chernobyl accident. Lipid peroxidation was shown to undergo changes against a background of decreased antiperoxide protection of erythrocytes 22-24 mos. after exposure in patients after acute radiation sickness, I degree. As compared to donors, the examinees demonstrated a tendency to the accumulation of primary products of lipid peroxidation--hydroperoxides (by 32.2%) and a significant decrease in erythrocyte peroxide stability.

[The cytogenetic effects of 22-MeV neutrons]. / Tsitogeneticheskie éffekty 22 MéV neitronov.

22-MeV neutrons affecting the culture of human peripheral blood lymphocytes have been studied for their cytogenetic peculiarities. Linear character of the dependence of chromosome aberrations yield within the dose range of 0.2-4.0 Gy is noted. Relative biological efficiency of 22-MeV neutrons reaches the highest values at low doses (18.8-2.4). Pair fragments and dicentrics prevail in spectrum of aberrations.

[Composition and physical structure of atherogenic lipoproteins in ischemic heart disease]. / Osobennosti sostava i fizicheskoi struktury aterogennykh lipoproteinov pri ishemicheskoi bolezni serdtsa.

The results of comparative studies in atherogenic lipoproteins of blood plasma under heart ischemic disease (HID) which is accompanied by hypercholesterinemia or proceeds without disturbances in the lipid metabolism, evidence for considerable differences in the composition and physical structure of very low-density lipoproteins (VIDL) and low-density ones (LDP) under the investigated states. The decrease in the surface charge density and in sizes of VLDL, as compared to normal, which are least expressed at HID and an increase in the surface charge density with certain increase of LDL radii under this pathologic state are revealed.