bluuebunny/crossref_metadata_2025 huggingface dataset:
Virtual Museum in the Library: Models and Technologies of Creation
Authors: Kuleva Olesya V.
Published: October 2018

The article describes the history of creation of virtual museums (VM) in global Internet space. The author considers the research areas of this resource, including the phenomena of VM development; defines the aspects of VM existence (information, social, technical) and its main types, in particular, representation of a real Museum and a virtual Museum existing only in the Web-space, and VM as an effective PR-tool. The paper presents the results of monitoring of Russian and some foreign virtual museums in libraries and describes their current state. The main specific features of the museums in the Russian libraries are the original object of exposure (book) and additional information materials (bibliography, alphabetical index, search system, etc.). Information content of the VM is mainly created basing on free programs and with minimal use of multimedia technologies; communication links to Web 2.0 services are weak. The main advantages of VM in the foreign libraries are simplicity of collections presentation and navigation through them, as well as aesthetics and dynamism, achieved by the use of original Web design (beautiful typography, sliders, tile (block) interfaces, etc.). Further development of memorial space of Russian libraries and creation of VM require theoretical study of the issue, namely the modeling of this resource. Analysis of scientific publications shows that there are different approaches to VM modeling, such as functional (educational, information, marketing models), complex (complex hardware — software system) and structural (structural model, including the following blocks: collections, library, exhibition, lecture and reference). Priority approach to the modeling of library VM at the stage of changing technological paradigm can be the technological one, which allows to reveal the set of the given resource properties, the process of the model design, consisting of the following blocks: resource, software and technology, preparation of objects for exhibiting, resource promotion, educational, cultural-educational and analytical.
Physical and Mathematical Sciences in the New Edition of Library Bibliographic Classification Schedules
Authors: Yakovleva Galina V.
Published: October 2018

The article presents changes in the structure and content of Schedules of Library Bibliographic Classification (LBC) to reflect the modern literature on Physical and Mathematical Sciences. The author describes specific features of separating the literature inside subdivision and between other divisions of the LBC Medium Schedules. The article considers the principles of separation of literature between Mathematics and Logic, representing the greatest difficulty in the systematization of literature. The range of issues belonging to the complex of computer sciences removed beyond the Division of Mathematics. Special sections on Hydro — and Aerodynamics collect literature, reflecting the applied directions in connection with a variety of technical applications, as well as separate research areas, where the studies are very intensive. The main series of subdivision on Physics is updated due to the introduction of new division, which reflects the new, rapidly developing areas of research in the Physics of soft condensed matter and Nanophysics. In this regard, there was conducted separation with the relevant subdivisions of Chemistry. Modern Radio physics turned from the applied science that supports Radio engineering in the extensive independent field of Physics. In this regard, it was decided to collect the literature on Radio physics in the Physics division, and to add the Radio band to the Schedule of special type sections on frequency ranges of electromagnetic waves. The new division of Laser Physics is introduced for the literature on physical processes related to the generation and amplification of optical radiation. The author describes the principles of separating with the subdivision on engineering technology, where lasers are considered as optical quantum generators. The Astronomy division for the first time presented the literature, reflecting studies on the detection of gravitational waves and on the origin and early evolution of the Solar system.
A Critical Review on Mathematical Functions Employed for Heptane Plus Characterization in Gas Condensate Reservoirs: Lessons Learned and Future Development
Authors: Chandra Steven
Published: October 2018

Characterizing heptanes plus fraction in PVT analysis has been a complex problem since its first inception. In this publication, the author is reviewing available mathematical functions employed for this task, whilst pointing out advantages and weaknesses for each of them and proposing a new method that is capable of complex characterization.

This publication addresses a new method that is capable of accurately characterize heptane plus fraction especially in discontinued areas where errors could leap up to 40%. The author modifies natural logarithmic function to be used as an accommodation to discontinuities. The modified distribution provides better accuracy in modeling the discontinuities as a straight-line function, making them ideal for real gas condensate composition characterization. The new method is tested against several test data used by previous researchers, and applied to 3 sets of field data. The results have shown that this new method is capable of lowering CPU requirement whilst making better accuracy for all test data.
The Optimization and Analysis of Hydrocarbon Recovery under Injection of Biopolymer, Synthetic Polymer and Gels in a Heterogeneous Reservoir
Authors: Putra Dike Fitriansyah , Temizel Cenk
Published: October 2018

Water injection is a conventional method which increases the recovery percentage by providing pressure support and displacing oil in the heterogeneous porous medium. In such a displacement process, (low) mobility ratio is important for a more efficient oil displacement by the injected fluid. As such, the mobility ratio can be reduced using the fluids involving gelling agents for increasing in the volumetric sweep. While polymers degrade and break up on experiencing sudden shear stresses and high temperatures, polymer macromolecules are forced to flow into narrow channels and pores where molecular scission processes can take place. Thus, it is of utmost importance to have a strong understanding of the use of the right type and amount of viscosity as a reduction agent.

For polymer injection, a comparison of xanthan polymer and synthetic polymer mechanisms was conducted. A commercial full-physics reservoir simulator was coupled with a robust optimization and uncertainty tool to run the model, where a simplified gel kinetics was assumed to form a microgel with no redox catalyst. Water injection continues over all six layers for 450 days, followed by gel system injection for 150 days in the bottom two layers. Water injection was continued to four years. The top four layers have higher horizontal permeabilities, and a high permeability streak is at the bottom of the reservoir to reduce any helpful effects of gravity. Control and uncertainty variables were set to investigate the sensitivity of this process using the coupled optimization and uncertainty tool.

Results demonstrate deep penetration of gel and blocking of the high permeability bottom layers. Sensitivity studies indicate the relative merits of biopolymer, xanthan polymer in terms of viscosity effects vs synthetic PAM in terms of resistance factor vs in-situ gelation treatments and their crossflow dependence. Adsorption and retention of polymer and gel are permeability dependent.

Considering the potential for application of gel solutions in the U.S. and throughout the world, this study illustrates the relative advantages of different treatments in terms of viscosity reduction in the same model in a comparative way, while outlining the significance of each control and uncertainty variable for better management of reservoirs where displacement efficiency is highly critical.
Features of cellular structure of the induced sputum and profile of cytokines at sintropiya of bronchial asthma and obesity at young patients
Authors: Soloveva I A , Sobko E A , Demko I V , Kraposhina A Yu , Loktionova M M , Gordeeva N V
Published: October 2018

Aim. To estimate changes of cellular structure of the induced sputum at young patients with bronchial asthma at interrelations with BMI and level of cytokines in blood plasma. Materials and methods. 164 patients with bronchial asthma were divided into 2 groups taking into BMI: the 1st group included patients with bronchial asthma and BMI from 18 to 25 kg/m2, patients with bronchial asthma and BMI from 30 to 40 kg/m2 entered into the 2nd group. The group of control was made by 40 almost healthy volunteers. Estimated existence of excess weight and defined obesity degree according to recommendations of World Health Organization. Studied the level of control of bronchial asthma, cellular structure of the induced sputum, the IL-2, IL-4, IL-6, IL-8, IL-10, IL-12, IL-15, IL-17, TNF-α, INF-γ levels in plasma of peripheral blood. Results. There are presented the results of the research of cellular profile of the induced sputum and profile of cytokines at patients with bronchial asthma depending on BMI and severity of the disease. The received results testify to prevalence of eosinophilic type of an inflammation in the group of patients with BMI less than 25 kg/m2 whereas at patients mainly paucigranulation inflammation decided on obesity. The highest content of the Il-17 was registered at patients with bronchial asthma and obesity as in comparison with indicators of patients with normal BMI, and with almost healthy that, perhaps, is the reason of low effect of steroid therapy at these patients. Conclusion. Endotype assessment before basic antiinflammatory therapy at patients with the first time diagnosed bronchial asthma, will be able to help with selection of the most optimum treatment to each specific patient.
Correlations between indicators of interleukin-10 and interleukin-6 in patients with periodic disease
Authors: Jndoyan Z T , Bablumyan A Yu , Ginosyan K V , Shekoyan S V
Published: October 2018

Aim. Determination of the concentration of interleukin-10 (IL-10) and interleukin-6 (IL-6) in serum of patients with periodic disease (PD) before and after treatment with colchicin, as well as the identification of correlation between the indicators of these parameters. Materials and methods. We examined 188 patients with PD (89 men, 99 women) aged from 12 to 69 years, as well as 44 patients with rheumatoid arthritis (RA) as a comparison group and 41 healthy people of the control group. Patients were divided into groups: 1 - PD colchicinotherapy patients with seizures without amyloidosis that do not respond to treatment maximum dose of colchicine 2.0 mg/day; 2nd-PD patients without amyloidosis, not responding to treatment 1.5 mg/day colchicine; 3rd - PD patients that responds to certain doses of colchicine (0.5 to 2.0 mg/day); 4th - PD patients without amyloidosis who did not receive treatment; 5-I of the healthy persons of the control group; 6-I - RA patients. The concentration of IL-10 in blood serum was determined by enzyme immunoassay ELISA, and IL-6 - immunochemiluminescent method. For statistical processing the computer program SPSS is used. The results were considered statistically significant at the level of reliability p
Clinical significance of the determination of surfactant proteins A and D in assessing the activity of lung sarcoidosis
Authors: Beketov V D , Lebedeva M V , Mukhin N A , Serova A G , Ponomarev A B , Popova E N , Yanakaeva A Sh , Solomka V A , Kondrashov A V , Konovalov D V
Published: October 2018

The results of a clinical study showing the importance of surfactant proteins A and D in assessing the activity and progression of idiopathic pulmonary fibrosis and chronic lung sarcoidosis are presented. Aim. To study the clinical significance of SP-A, SP-D in assessing the activity of idiopathic pulmonary fibrosis and sarcoidosis. We examined 81 patients with morphologically confirmed diagnoses of idiopathic pulmonary fibrosis (ILF) and sarcoidosis, a control group of 20 healthy individuals. The MSCT of the thoracic organs of the chest was performed, the diffusivity of the lungs was examined, oxygen saturation was determined. In the serum, the surfactant proteins SP-A and SP-D were determined by the enzyme-linked immunosorbent assay. Results. A significant increase in SP-A and SP-D (p
The influence of weather-climatic and social factors on population mortality from circulatory diseases in Russia
Authors: Zolotokrylin A N , Titkova T B , Bokuchava D D
Published: October 2018

Aim. To study the links between the standard mortality rate of the population from circulatory system diseases (CSD) with factors: weather-climatic (inter-day jumps in air temperature and atmospheric pressure by seasons and for the year) and social (average annual income per person and the number of doctors of all specialties) in Russia for the period 1995-2015. Materials and methods. According to station data and data of reanalysis, seasonal and annual amounts of day-to-day jumps in air temperature were calculated more than the absolute value of 4° and 6°C and the atmospheric pressure more than the absolute value of 8 GPa. The links between climate variables and the mortality rate of the population, taking into account social factors, were investigated using factor analysis, including regression and variance analyses. Results. Annual amounts of temperature (pressure) jumps of different signs vary greatly on the territory: the maximum amounts are 3-4 times higher than the minimum ones. The geographical distribution of air temperature fluctuations differs from the distribution of atmospheric pressure fluctuations. The sum of temperature jumps in the absolute value of more than 6°C is about twice less than the sum of jumps more than 4°C, but they are characterized by similarity of geographical distribution. The sum of the jumps of temperature (pressure) is reduced during the summer is approximately two times compared to the winter. The maximum jumps are observed mainly in the Northern regions with low population density, but with high per capita income, while the minimum is observed in the South-Western parts of the European part of the country with high population density, as well as middle and low income. Global warming does not significantly affect the reduction of annual amounts of temperature (pressure) jumps. Factor analysis of social and climatic variables in the territory for each year indicates the dominance of the influence of the social factor (per capita income) on the mortality rate from CSD. Conclusion. Factor analysis is integrated in the annual scale climatic and social variables showed a dominant effect on the coefficient of mortality from CSD, the factor of standard of living (per capita income of the population). Then the significance of the impact factors is consistently reduced: negative atmospheric pressure jumps, average seasonal pressure, health care level, positive pressure jumps. The significance of temperature variables is the smallest.
Stem Cell Therapy for Perianal Crohn’s Disease
Authors: Knyazev O V , Fadeeva N A , Kagramanova A V , Belyakov N I , Orlova N V , Lishchinskaya A A , Konoplyannikov A G , Parfenov A I
Published: October 2018

Aim. To compare the effectiveness of the effect of combination therapy (local and systemic administration) with bone marrow mesenchymal stromal cells (MSC), anticlitokine therapy with infliximab (IFX), and antibiotic (AB)/immunosuppressive (IS) therapy on the frequency of healing of simple perianal fistulas in Crohn's disease. Materials and methods. In our study, the 1-st group of patients aged 19 to 58 years (Me-29) (n = 12) received MSCs culture systemically according to the scheme and locally. The 2-nd group of patients with CD (n = 10) from 20 to 68 years old (Me-36) received anticytokine therapy with infliximab (IFX) according to the scheme. The 3-d group of patients with CD (n=14) from 20 to 62 years old (Me-28) received antibiotics (AB) and immunosuppressors (IS). Efficacy was assessed by the index of perianal activity of Crohn's disease (PCDAI) and the frequency of relapses. Results. After 12 weeks among patients of the 1-st group, healing of simple fistulas was noted in 8/12 patients (66.6%), in the 2-nd group in 6/10 (60.0%) In the 3-d group, in 1/14 patients (7.14%). After 6 months in the 1-st group of patients, healing of simple fistulas was preserved in 8/12 (66.6%), in the 2-nd group - in 6/10 (60.0%). In the 3-d group - in 1/14 patients (7.14%). After 12 months in the 1-st group, healing of simple fistulas was preserved in 7/12 (58.3%), in the second group - in 6/10 (60.0%). In the 3-d group - in 2/14 patients (14.3%). After 24 months, among the patients of the 1-st group, fistula closure was maintained in 5/12 patients (41.6%), in the 2-nd group - in 4/10 (40.0%). In the 3-d group - in 0/14 patients (0.0%). Conclusion. Combined cellular and anticytokine therapy of CD with perianal lesions significantly contributes to the more frequent and prolonged closure of simple fistulas, as compared to antibiotics/immunosuppressors, and to a decrease in the frequency of recurrence of the disease.
Management of acute coronary syndrome in older adults (data from russian federal acute coronary syndrome registry)
Authors: Ocshepkova E V , Sagaydak O V , Chazova I E
Published: October 2018

The frequency of cardiovascular diseases is increasing progressively with age, and the global aging of the population poses the problem of treatment of patients of older age groups in a row with other relevant medical and socio-economic problems. Aim. In the present study was to investigate the quality of medical care for patients with acute coronary syndrome (ACS) old age and compliance of the treatment current guidelines. The data is exported from the system of the Federal register OKS. Materials and methods. Analyzed medical history 33 893 patients with ACS entered in the system registry of ACS for the period from 01.01.2016 to 31.12.2016. a comparison was made of the quality of care in patients with ACS elderly (75 years and older, n=8773) and in younger patients. Results. The results of the study showed that in patients of senile age, when compared with younger patients, comorbid conditions are significantly more often observed, as well as a significantly higher risk of hospital and 6-month death, calculated on the GRACE scale. Conclusion. Patients of senile age with ACS are almost twice less likely to undergo percutaneous coronary interventions than younger ones, which worsens the prognosis in these patients and increases mortality.
Combination therapy is a new standard for treatment of pulmonary arterial hypertension
Authors: Avdeev S N , Tsareva N A , Gaisin I R
Published: October 2018

At present, compelling evidence has been obtained that combined therapy of pulmonary arterial hypertension (PAH) with specific drugs can significantly slow progression of PAH. Therefore, in current guidelines combination therapy is already considered as standard treatment for a significant proportion of patients with moderately severe and severe forms of PAH. However, the quality of life and long-term prognosis of patients receiving combination therapy, should be the object of further research. The future research is absolutely necessary to identify the most optimal strategy of treatment of patients with PAH, such as initial combination therapy or rapid sequential combination therapy, double or triple combinations as well as exploring new signaling pathways PAH, which can become targets for new specific drugs PAH.
Effect of N-acetylcysteine on mucosal immunity of respiratory tract
Authors: Kalyuzhin O V
Published: October 2018

The outcome of diseases accompanied or caused by mucostasis depends both on the restoration of drainage function of the airways and on the effectiveness of immune mechanisms against pathogens. N-acetylcysteine (NAC) is widely used as mucolytic and antioxidant remedy in clinical practice. In this regard, the data of the scientific literature on the direct and indirect effects of NAC on the mucosal immunity of the respiratory tract have been reviewed. NAC possesses pleiotropic immunomodulating properties, most of which contribute to the regression of clinical manifestations of acute and chronic inflammatory diseases of the respiratory tract. Biological and pharmacological effects of NAC include improvement in rheological properties of mucus, reduction of excess mucin production, restoration of mucociliary clearance and production of sIgA, suppression of excess production of IgE and IgG4, destruction of biofilms and inhibition of their formation, suppression of adhesion of pathogenic bacteria to epithelial cells, antioxidant activity, regulation of the production of pro-inflammatory and profibrotic cytokines. There was no convincing evidence that NAC is able to suppress any component of mucosal immunity. For final conclusions on this subject, further research are required.
Primary lymphangiectasia of the gastrointestinal tract
Authors: Esedov E M , Abasova A S , Akhmedova F D , Musaeva L N
Published: October 2018

Intestinal lymphangiectasia is a very rare pathology, characterized by the presence of enlarged lymphatic vessels in all layers of the intestinal wall and in the mesentery. As a result, "lymphatic lakes" are formed, through which lymph exudates into the lumen of the intestine. The main manifestation is hypoproteinemic edema. Diagnosis of the disease is based on laboratory-instrumental methods of investigation, including by determining in the stool α-1 antitrypsin as a screening test. Treatment includes: a diet rich in calcium, trace elements, be sure to use medicinal foods enriched with medium chain triglycerides, substitution and symptomatic therapy.
Acute ischemic stroke in the setting of essential thrombocytemia (clinical cases)
Authors: TANASHYAN M M , KUZNETSOVA P I , RASKURAZHEV A A , SUBORTSEVA I N , MELIKYAN A L
Published: October 2018

This article describes several clinical cases of acute ischemic stroke among patients suffering from essential thrombocytemia. Ambiguity of etiological factors of stroke is demonstrated among patients with this pathology. Thrombocytosis and high allele load in the Jak2 gene play an important role (even with normal platelet count) in progression of cerebrovascular disease. Also the question of effectiveness of preventive and etiological therapy is considered.
Coinheritance of HbD-Punjab/β+-thalassemia (IVSI+5 G-C) in patient with Gilbert's syndrome
Authors: PETRENKO A A , PIVNIK A V , KIM P P , DEMIDOVA E Yu , SURIN V L , ABDULLAEV A O , SUDARIKOV A B , PETROVA N A , MARYINA S A
Published: October 2018

Thalassemia and qualitative hemoglobinopathy are hereditary disorders of Hb synthesis that lead to change in the Hb conformation or a decrease in the synthesis of structurally normal Hb, and consequently, to erythron pathology. Many variants of Hb are unstable or have altered affinity for oxygen, and, in heterozygous form can be associated with clinical and hematological manifestations (hemolytic anemia, hypochromic microcytic anemia, erythrocytosis). HbD-Punjab [β121 (GH4) Glu → Gln; HBB: C.364G> C] is variant of Hb carrying the amino acid substitution in the 121 position of β-globin chain. In all cases reported so far, patients with HbD-Punjab/β+-thalassemia (IVSI+5 G-C) combination experienced typical thalassemia with hypochromic microcytosis. HbD-Punjab was detected by electrophoresis from 37 to 94% of total Hb. The article describes rare clinical case of the cohabitation of HbD-Punjab/β+-thalassemia (IVSI+5 G-C) in a patient with homozygous variant of Gilbert's syndrome observed in AS Loginov Moscow Clinical Scientific Center.
Diagnostics and treatment challenges of Ph-like acute lymphoblastic leukemia: a description of 3 clinical cases
Authors: ZARUBINA K I , PAROVICHNIKOVA E N , BASKHAEVA G A , KRASILNIKOVA A E , GAVRILINA O A , BIDERMAN B V , SUDARIKOV A B , BONDARENKO S N , DAVYDOVA Y O , GALTSEVA I V , SOKOLOV A N , TROITSKAYA V V , SAVCHENKO V G
Published: October 2018

B-cell acute lymphoblastic leukemia (B-ALL) is a diverse group of malignant blood disorders both with regard to the biological properties of the tumor and to therapeutic approaches. Immunophenotyping, molecular genetic techniques, whole-genome sequencing characterize B-ALL as a very diverse group for sensitivity to chemotherapy and prognosis. We present three clinical cases of patients with B-ALL and expected good response to standard therapy, in whom standard protocol treatment failured: refractoriness, persistence of minimal residual disease (MRD), and progression (MRD increase). The remission in these patients was achieved after chemotherapy change to immunological targeted therapy. Nowadays a unified therapeutic approach to all primary patients of the B-ALL is considered generally outdated. Great efforts are carrying out to develop molecular genetic classifications. The molecular dissection of subtypes of B-ALL goes on, and new protocols for selective treatment with targeting are clearly outlined for each subtype of B-ALL.
Literature review and clinical observation of acquired idiopathic hemophilia with a new missense mutation in the factor VIII gene (His2026Arg)
Authors: ERSHOV V I , GADAEV I Yu , BUDANOVA D A , PERINA F G , SURIN V L , SALOMASHKINA V V , PSHENICHNIKOVA O S , ZOZULYA N I
Published: October 2018

The article provides review of possible mechanisms of inhibitor coagulopathies, in particular of acquired hemophilia A. This pathology is an extremely rare disease occurring in 1-2 cases in 1 million per year. In the present study we provide data for two clinical cases of hemophilia A in women. These cases had different development mechanisms, although both women have a newly discovered missense mutation His2026Arg in the VIII factor gene. The matter of main interest is the description of the disease development in the patient with an acquired idiopathic hemophilia A with a possible disease occurrence due to an asymmetric X-chromosome inactivation (lyonization). In this particular case lyonization led to the late manifestation of the hemophilia A carrier’s state and development of severe form of the inhibitor-associated acquired hemophilia A. We also discuss therapeutic approaches to these forms of the disease, considering there are no concise protocols for case management due to an extreme rarity of the pathology. Acquainting the clinical personnel working it the different areas of medicine with suchlike inhibitor coagulopathies has a major practical importance.
Structure and significance of cytogenetic abnormalities in adult patients with Ph-negative acute lymphoblastic leukemia
Authors: PISKUNOVA I S , OBUKHOVA T N , PAROVICHNIKOVA E N , KULIKOV S M , TROITSKAYA V V , GAVRILINA O A , SAVCHENKO V G
Published: October 2018

Objective. To evaluate occurrence, variety, structural peculiarities and prognostic meaning of cytogenetic abnormalities in adult patients with Ph-negative acute lymphoblastic leukemia (ALL) receiving therapy according to ALL-2009 protocol. Materials and methods. The study included 115 adult patients with firstly diagnosed Ph-negative ALL: 58 male and 57 female aged from 15 to 61 years (mean age 26.5 years), who underwent treatment from September 2009 to September 2015 in National Medical Research Center for Hematology MH RF (n=101) and in hematology departments of regional hospitals (n=14). All patients received therapy of ALL-2009 protocol (ClinicalTrials.gov, NCT01193933). The median follow-up was 24.5 months (0.2-94.4 months). As a part of the study results of a standard cytogenetic assay (SCA) were analyzed and fluorescence hybridization in situ (FISH) with the use of DNA-probes was performed on archived biological material for structural changes in gene locuses MLL/t(11q23), с-MYC/t(8q24), TP53/ deletion 17p13, CDKN2A/ deletion 9p21, translocation t(1;19)/E2A-PBX1 и t(12;21)/ETV6-RUNX1; iAMP21 identification. Results. Karyotype was defined using SCA in 86% of patients. Normal karyotype was found in 48.5% of them, chromosome aberrations in 51.5% (structural changes were found in 19.2%, hyperploidy in 27.2%, and hypoploidy in 5.1%). In 17.2% of patients complex karyotype abnormalities were found. With the use of FISH technique aberrations were found in 67% of patients: 9p21/CDKN2A deletion in 24.3%, MLL/t(11q23) gene abnormalities in 7.8%, 17p13/TP53 deletion in 5.2%, abnormalities of c-MYC/t(8q24) in 1.7%, t(1;19)/E2A-PBX1 in 0.8%, and iAMP21 in 0.8%, other abnormalities (additional signals/absence of signals from gene locuses) in 26.4%, t(12;21)/ETV6-RUNX1 was not found. FISH technique use in addition to SCA allows to increase aberrant karyotype location from 51.5 to 67%. A statistically significant correlation of 9p21/CDKN2A deletion with high serum lactate dehydrogenase activity (p=0.02); MLL/t(11q23) gene abnormalities - with leucocytosis and high blast cells level in blood (p=0.0016), hyperploidy - with normal leukocyte count (p=0.02) was shown. In groups with different cytogenetic abnormalities no statistically significant differences of treatment with ALL-2009 protocol were found (in terms of complete remission, early mortality and treatment resistance). When connection of cytogenetic abnormalities and their combinations with long-term results were analyzed according to ALL-2009 protocol, only two characteristics - MLL/t(11q23) and c MYC/t(8q24) gene abnormalities had a statistically significant influence on disease-free survival (HR - 176.9; p
Immunoglobulinopathies in patients with angioimmunoblastic T-cell lymphoma
Authors: CHERNOVA N G , SOBOLEVA N P , MARIINA S A , SIDOROVA Y V , SINITSYNA M N , DVIRNYK V N , BADMAZHAPOVA D S , VINOGRADOVA Y E , ZVONKOV E E , SAVCHENKO V G
Published: October 2018

Contex. Angioimmunoblastic T-cell lymphoma (AITL) is a rare form of non-Hodgkins lymphoma, characterized by generalized lymphadenopathy, hepatosplenomegaly and dysproteinemia. Hypergammaglobulinaemia is revealed in 50-83% pts with AITL. However, the characteristics of immunoglobulinopathies observed in AITL are scarce. Objective: The aim of the study was to characterize quantitative and qualitative immunoglobulinopathies in patients with AITL at the onset of the disease. Patients and methods. 55 patients with newly diagnosed AITL were enrolled in the study, the male/female ratio was 30/25; median age was 61 (29-81) years. Diagnosis was based on standard WHO criteria. Immunochemical studies of blood serum included serum protein electrophoresis/immunofixation, nephelometric quantification of total immunoglobulins, serum free light chain assay. Results. Quantitative and qualitative immunoglobulinopathies were determined in 49 (89,1%) of 55 pts. Quantitative immunoglobulinopathies were revealed in 47 (85.5%) of 55 cases, qualitative - in 14 (25,5%). Combination quantitative and qualitative immunoglobulinopathies was observed in 12 (21,8%) of 55 pts. The detected immunoglobulinopathies were divided into 4 groups: polyclonal hypergammaglobulinaemia, hypogammaglobulinaemia, oligoclonal gammapathy, and monoclonal gammapathy. Polyclonal hypergammaglobulinaemia was marked in 41 (74.5%) of 55 pts, elevated level of IgG was determined in 27 (49,15%) of 55 cases, IgM - in 18 (32,7%) and IgA - in 21 (38.2%). Interestingly, polyclonal IgE hypergammaglobulinaemia was detected in 12 (48,0%) of 25 cases of performed studies. Hypogammaglobulinaemia was detected in 8 (14,5%) of 55 cases. Oligoclonal gammapathy was determined in 4 (7.3%) of 55 pts. Monoclonal gammapathy was revealed in 11 (20,0%) of 55 cases. The amount of monoclonal immunoglobulin varied from 2.6 to 14.1 g/l. Monoclonal immunoglobulin Gk was detected in 5 of 11 pts, Gλ - in 2, Mλ - in 2, Mk - in 2. Monoclonal gammapathy was accompanied by polyclonal hypergammaglobulinaemia in 9 of 11 cases, hypogammaglobulinaemia - in 2. Conclusions. Quantitative and qualitative immunoglobulinopathies are observed in most patients at the onset of AITL. Quantitative abnormalities were determined more often than qualitative. Monoclonal gammapathy can be a manifestation of lymphoproliferation and other concomitant disorders. The prognostic value of immunochemical parameters is still unclear and requires dynamic observation and study.
Hematopoietic stem cell transplantation with alpha/beta T-lymphocyte depletion and short course of eculizumab in adolescents and young adults with paroxysmal nocturnal hemoglobinuria
Authors: SHASHELEVA D A , MASCHAN A A , SHELIKHOVA L N , PETROVA U N , KURNIKOVA E E , ILLARIONOVA O I , BOYAKOVA E V , NOVICHKOVA G A , MASCHAN M A
Published: October 2018

The main goal is to optimize hematopoietic stem cell transplantation (HSCT) approach among adolescents and young adults with paroxysmal nocturnal hemoglobinuria (PNH) by means of Graft-versus-host disease (GVHD) and post-transplant complications risk lowering. Materials and methods. We report our experience of HSCT from HLA-matched unrelated donors using TCR alfa/beta and CD19 depletion in 5 pts (1M/4F) with PNH, developed after successful immunosuppressive therapy (IST) of acquired aplastic anemia (AA). Median age of pts at the moment of transplantation was 17,8 years (range 14,5-22,7), median interval from IST to PNH was 4 years (5mo - 6,5 y). In all patients non-severe pancytopenia was present: granulocytes 0,8х109/l (0,8-1,8 х109/l) platelets 106 х109/l (27-143 х109/l) and Hb -78 g/l, median PNH clone size in granulocytes was 94 (range 75-99)%. One pts previously developed sinus thrombosis. Conditioning consisted of thoraco-abdominal irradiation 4-6 Gy, cyclophosphamide 100 mg/kg, fludarabine 150 mg/m2 and anti-thymocyte globulin (ATG) or alemtuzumab. Eculizumab was given from day (-7) till day (+14) (every 7 days, only 4 times). GVHD prophylaxis was tacrolimus ± methotrexate. Results. Infusedgraft characteristics were: CD34+ - 8,1х106/kg, CD3TCRab·150х103/kg, CD3gd+ - 7,3х106/kg, СD19+ - 221х103/kg, NK -6,4х108/kg. Engraftment was achieved in all 5 pts with a median of 15(12-18) и 13(10-18) days for granulocytes and platelets, respectively. Skin acute GVHD grade I developed in only 1 pt, and subsided with short course of glucocorticoids. CMV reactivation occurred in 1 pt; there were no episodes of Epstein-Barr Virus (EBV) o rAdenovirus (AdV) reactivation. Full donor myeloid chimerism was established in all pts by day +30. Immune reconstitution was delayed until 6 months after transplant but no severe infections occurred. All pts are alive 1,7-5,5 years (med 4 years) after HSCT with normal hematopoiesis and immune function, full donor chimerism and no late sequelae. Conclusions. Transplantation of TCRalfa/beta and CD19 depleted hematopoietic cells from matched unrelated donor after immunoablative conditioning and supported with short course of eculizumab is perfectly safe and efficient technology leading to cure in young patients with PNH.
Maternal incompatibilities with fetal human platelet alloantigens -1a, -1b and -15 are the main causes of neonatal alloimmune thrombocytopenia in Russia
Authors: KHASPEKOVA S G , GOLOVKINA L L , DONUSH E K , GOLUBEVA N V , SHUSTOVA O N , MAZUROV A V
Published: October 2018

The aim. Mechanisms underlying the development of neonatal alloimmune thrombocytopenia (NAIT) in in Russia have been studied. Materials and methods. Genetic polymorphisms of human platelet alloantigens (HPA) -1, -2, -3, -4, -5, and -15 were evaluated in 27 families having the newborns with NAIT. NAIT was diagnosed according to the following criteria: (1) newborn with thrombocytopenia; (2) mother with no thrombocytopenia and no increase of platelet associated IgG, (3) presence of antibodies reacting with paternal platelets in maternal plasma / serum. HPA genotyping revealed incompatibilities in 23 out of 27 tested families. In these 23 families HPA-1 conflicts were detected in 16 ones (70%). In 8 cases mothers were homozygous carriers of rare HPA-1b allele and in another 8 cases - of HPA-1a allele which cased incompatibilities with fetal HPA-1a and HPA-1b respectively. In 5 out of 23 families (22%) there were incompatibilities with fetal HPA-15 (HPA-15a, n=2 and HPA-15b, n=3), in 1 family - with HPA-5b (4%), and in 1 family - with HPA-3b (4%) alloantigens. In conclusion the main causes of NAIT in Russia were HPA-1a and -1b conflicts and HPA-15 conflicts were the second frequent ones.
First experience of using Brentuximab vedotin and modified program NHL-BFM-90 in the front-line treatment of patient with anaplastic large-cell lymphoma: a case report and a review of literature
Authors: CHERNOVA N G , ZVONKOV E E , BADMAZHAPOVA D S , SINITSYNA M N , GREBENYUK L A , SIDOROVA Y V , KOSTINA I E , KOVRIGINA A M , OBUKHOVA T N , SUDARIKOV A B , SAVCHENKO V G
Published: October 2018

Nodal anaplastic ALK-negative large cell lymphoma (nALCL, ALK-) is a Т-cell lymphoma that is characterized by aggressive clinical course and low sensitivity to СНОР (cyclophosphamide, doxorubicin, vincristine, prednisolone) and other chemotherapy regimen. In the article we present a literature review and describe our clinical case of nALCL, ALK-. For the first time a combination of Brentuximab vedotin with modified program NHL-BFM-90 was used as a first-line therapy. As a result of immunochemotherapy a complete antineoplastic effect was obtained. For consolidation of this effect high-dose chemotherapy with following autologous blood stem cell transplantation was performed. The chosen treatment tactics allowed to achieve a complete remission in a medium risk group patient.
Wound analgesia in a patient with hemophilia in a highly traumatic operation
Authors: LEVCHENKO O K , POLYANSKAYA T U , ZORENKO V Y , GALSTIAN G M
Published: October 2018

Given a number of limitations on the use of perioperative analgesia in patients with hemophilia, wound analgesia may be one of the components of multimodal analgesia in this category of patients. The aim of the study was to describe the use of the wound analgesia method in a patient with hemophilia in the case of a clinical case. Materials and methods. A patient with severe hemophilia A underwent postoperative analgesia after total knee replacement (within the first 48 hours) with an extended infusion of local anesthetic (ropivacaine) into the wound. Results. During the first 8 hours, the patient received 20 mg of morphine (with the aid of a device for patient-controlled analgesia), the pain level ranged from 7 to 4 points. Further, there was a sufficient effect (NRS - 2 points), from anesthesia only with ropivacaine, using a system for anesthetizing surgical wounds. Complications and side effects were not noted. Conclusion. The clinical case demonstrates an effective and safe method of prolonged analgesia in patients with hemophilia. Considering encouraging data, further study of wound analgesia in this category of patients is necessary.
Constrictive pericarditis in a patient with inherited factor VII deficiency
Authors: PONOMAREV R V , MODEL S V , ZOZULYA N I , IVANOV V A , PODCHASOV D A , MERSHINA E A , SINITSYN V E , LUKINA E A
Published: October 2018

Constrictive pericarditis (CP) is the final stage of a chronic inflammatory process characterized by fibrous thickening and calcification of the pericardium that impairs diastolic filling, reduces cardiac output, and ultimately leads to heart failure. We present a clinical case of CP in a patient with rare inherited bleeding disorder - factor VII deficiency. Heart failure due to CP was suspected based on clinical symptoms, results of ultrasonic and radiological investigations. The diagnosis was verified by the results of cardiac magnetic resonance imaging. Pericardectomy was performed resulting in significant improvement in the patient’s condition.
ToDIGRA Diversity Workshop Special Issue: Introduction
Authors: Beavers Sian , Jayemanne Darshana
Published: October 2018

The inaugural DiGRA DiversityWorkshop, 'Gaming the System', was held at The University of Melbourne on 2nd July 2017; we thank and acknowledge the Wurundjeri people as Traditional Custodians of this land. The Workshop was an initiative of the Diversity Working Group that first met in 2015. The event drew together five formal papers (four of which have proceeded to peer review and appear in this special issue) followed by a general discussion. The aim of the Workshop was to critically interrogate what it would mean for Game Studies to be diverse, and to invite presentations that could expand our ideas about diversity. This included questioning whether �diversity� is an unalloyed good, the nature of the non-diverse �norm� from which it putatively offers a departure, and the function of diversity as a discourse operative within the contemporary academy.
Facilitating Diversity-Centred Adult Computing Education
Authors: Johnstone Danielle , Pelletier Caroline
Published: October 2018

This article draws from a dissertation composed of ethnographical study on the work of codebar London, a chapter of an organisation working to diversify the tech work force by offering free programming workshops to under-represented people. It delves into the role played by codebar�s organisers, considering how the problem of gender, ethnic and sexual under-representation in technology work leads to codebar�s particular effort to solve it. In exploring links between the organisers� work and existing theories about empowering pedagogy, it addresses the question: How is diversity practiced educationally?
Homo Includens: Surveying DiGRA�s Diversity
Authors: Butt Mahli-Ann Rakkomkaew , De Wildt Lars , Kowert Rachel , Sandovar Alyea
Published: October 2018

This article examines which bodies have access to participate in Digital Games Research Association (DiGRA) events, and to DiGRA as an organization. It is based on a survey (N=174), among subscribers to the DiGRA 'Gamesnetwork' mailing list. The survey included questions on age, gender, location and career level to gain insight into who is included in the DiGRA community, with further questions on problems and challenges faced by those who have had trouble accessing DiGRA. This paper does not proceed solely by statistical methodology, but draws on feminist theories of embodiment and qualitative methods. Through this diverse methodological approach, the paper analyzes which bodies have difficulties accessing DiGRA�s academic communities and conferences, which practices cause these difficulties, and which policies might be introduced to address these. The survey indicates that young, early-career and women�s bodies are in particularly precarious positions. This situation is perpetuated through various practices of economic and social inaccessibility. Upon reflection, the paper proposes a set of policies to address these practices. We conclude that this survey and its analysis are only a first step to making DiGRA a more diversely inclusive organization.
1789 Yılında Askerî Personel İçin Yazılmış Almanca-Türkçe Transkripsiyon Metni
Authors: BEKAR Beytullah
Published: October 2018

Türkçenin transkripsiyon metinleri, yabancılar

tarafından Türkçeyi öğretmek amacıyla hazırlanmış olan metinlerdir. Bazı

transkripsiyon metinleri yalnız sözlük şeklinde hazırlanmış, bazıları sözlük ve

gramer ilaveli hazırlanmıştır. Bazı transkripsiyon metinlerinde tematik olarak

hazırlanmış konuşma cümleleri bulunurken bazılarında deyimler, atasözleri ve

Nasreddin Hoca fıkralarına yer verilmiştir. Bununla birlikte bazı

transkripsiyon metinleri Türkçe öğrenmek isteyen herkese yöneliktir, bazıları

ise özel amaçla hazırlanmıştır. Thomas Chabert’in “Kurze Anleitung zur

Erlernung der türkischen Sprache, für Militär Personen. Sammt einer Sammlung

von nützlichen Gesprächen, Ausdrücken und Redensarten und einem Handlexikon der

gebräuchlichsten Wörter” adlı transkripsiyon metni de başlığında belirtildiği

üzere askeri personel için hazırlanmış bir eserdir.
Müellifi Bilinmeyen Bir Tıp Sözlüğü
Authors: MURAD Sibel
Published: October 2018

Sözlükler, bir dilin söz varlığını ortaya

koymaları, başka dildeki sözcüklerin öğrenilmesi, bir alanla ilgili terimleri

barındırması gibi birçok bakımdan önemli eserlerdir. Bilhassa belirli bir alana

ait olan sözcüklerin yer aldığı terim/alan sözlükleri yazıldıkları alana ve

ilgili kişilere hizmet ederler. 14. yüzyıldan sonra birçok bilim dalıyla ilgili

eserler verilmiştir. Bunlar arasında tıp ve sağlıkla ilgili metinler de

bulunmaktadır. Tıp ve eczacılıkta kullanılan birçok yabancı bitki, terkip,

ilaç, aş, cevher vs.nin başka dillerdeki karşılıklarını veren müstakil olarak

hazırlanmış tıp-ecza sözlüklerinin yazımına ise 17. yüzyıldan itibaren

başlanmıştır. Çalışmamızda Prof. Dr. Mehmet Fatih Köksal’ın şahsî

kütüphanesinde bulunan müellifi, müstensihi ve yazılış tarihi bilinmeyen bir

tıp sözlüğü tanıtılarak metni sunulacaktır. Eser 16 yaprak olup baş tarafı

eksiktir. Üç “fasıl”dan oluşan eserin ilk faslında tıbbî özellikteki bitki,

ilaç adlarının Türkçe, Arapça, Farsça ve Boşnakça –bir kelimenin Ermenice-

karşılıkları yer almakta, ikinci fasılda insan vücudunda bulunan kan, safra,

balgam, sevda unsurlarının oranlarına göre insan mizacına nasıl etki ettikleri

ve bunlara göre insan tabiatları tanıtılmaktadır. Son fasılda ise birçok

hastalık adının Türkçe, Arapça ve Farsça karşılıkları bulunmaktadır. Sözlüğün

madde başları çoğunlukla Arapça, Farsça olup yer yer Türkçe madde başlarına da

rastlanmaktadır. Çalışmamızda, çeşitli bitkilerin, devaların, hastalıkların

Arapça, Farsça, Türkçe ve Boşnakça karşılıklarının bulunduğu söz konusu sözlük

tanıtılarak çeviriyazı alfabesiyle metni sunulacaktır.
Ortaokul Türkçe Derslerinde Biyografi Türünün Kullanımı
Authors: KEMİKSİZ Ömer
Published: October 2018

Türkçe ders kitapları, öğrencilerin çeşitli edebî

türleri tanıyabileceği metinleri içerir. Bu edebî türlerden biri de

biyografidir. Bu araştırmanın amacı biyografi türünün ortaokul Türkçe

derslerindeki kullanım durumunu tespit etmektir. Doküman incelemesi ile

gerçekleştirilen çalışmanın örneklemini 7. ve 8. sınıftan beşer olmak üzere

toplam 10 adet öğretmen kılavuz kitabı oluşturmuştur. Araştırmada biyografi

türünün hem ders kitaplarındaki metinlerde hem de ders içindeki etkinliklerde

hangi sıklıklarda kullanıldığı tespit edilmeye çalışılmıştır. Biyografi

türündeki metinler ana ve yardımcı metinler şeklinde ele alınmış ve sınıf

seviyelerine göre dağılımlarına bakılmıştır. Biyografi türünün kullanıldığı

etkinlikler ise kişiye/kişilere yönelik ve türe yönelik etkinlikler olmak üzere

iki başlıkta incelenmiş; kişiye/kişilere yönelik etkinlikler kendi içinde

araştırmaya dayalı, bilgilendirmeye dayalı ve otobiyografi; türe yönelik

etkinlikler ise araştırmaya dayalı, bilgilendirmeye dayalı ve uygulamaya dayalı

şeklinde alt başlıklara ayrılmıştır. Elde edilen bulgular tablo ve şekiller

yardımıyla sunulmuş; bulguları desteklemek amacıyla etkinlik yönergelerine de

yer verilmiştir.  Araştırmada biyografi

türündeki metinlere daha çok Millî Kültür ve Kişisel Gelişim temalarında yer

verildiği belirlenmiştir. Biyografi metinlerinin yardımcı metin olarak

kullanıldığı etkinlikler ise genelde noktalama işaretlerinin öğretimiyle ilgili

etkinlikler olmuştur.  Etkinliklerin ise

büyük oranda kişilere yönelik etkinlikler olarak düzenlendiği, bu türden

etkinliklerin de daha çok araştırmaya dayalı çalışmalardan oluştuğu tespit

edilmiştir. Kişilere yönelik biyografi etkinliklerinin isimleri veya

özellikleri verilen kişiler üzerine hazırlandığı, etkinliklerde ise daha çok

isimleri verilen belirli şahısların araştırıldığı ortaya çıkmıştır. 