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Bevezetés A T-sejtes nagy granuláris lymphocytás leukémia egy ritka, rett T-sejtes neoplázia, mely változatos klinikai lefolyást mutat és gyakran társul autoimmun szövődményekkel. Esetismertetés Idős férfi betegünknél lymphocytosis és neutropenia hátterében igazolt T-LGLL mellett több autoimmun betegség is kialakult, mint gluténérzékenység, primer biliáris cirrhosis, tiszta vörösvértest-aplázia. A súlyos, rendszeres vörösvértest-transzfúziót igénylő tiszta vörösvértest-aplázia a standard immunszuppreszív gyógyszerekre refrakternek bizonyult, végül alemtuzumab hatására remisszióba került. Megbeszélés Bemutatjuk a T-LGLL-ben előforduló autoimmun szövődmények pathomechanizmusát, illetve röviden tárgyaljuk a refrakter esetek kezelési lehetőségeit.

The genetic profiling of renal tumors has revealed genomic regions commonly affected by structural changes and a general genetic heterogeneity. The VHL, PTEN, and BAP1 genes are often mutated in renal tumors. The frequency and clinical relevance of these mutations in renal tumors are still being researched. In our study, we investigated VHL, PTEN, and BAP1 genes and the sequencing of 24 samples of patients with renal tumors, revealing that VHL was mutated at a noticeable frequency (25%). Six of the investigated samples showed mutations, and one genetic polymorphism (rs779805) was detected in both heterozygote and homozygote forms. PTEN gene mutation was observed in only one sample, and one specimen showed genetic polymorphism. In the case of the BAP1 gene, all of the samples were wild types. Interestingly, VHL mutation was detected in two female patients diagnosed with AML and in one with oncocytoma. We assume that VHL or PTEN mutations may contribute to the development of human renal cancer. However, the overall mutation rate was low in all specimens investigated, and the development and prognosis of the disease were not exclusively associated with these types of genetic alterations.

Boric acid is a significant radioactive waste generated during the operation of nuclear power plants. Cementitious materials have been widely studied for the immobilization of boric acid. The generally used natural boric acid has been replaced by enriched boric acid for geochemical reasons and are expected to have varied behaviors in cementitious matrices. Results showed that simulated enriched/natural boric acid liquid wastes mostly contain boron in $${\mathrm{B}(\mathrm{OH})}_{4}^{-}$$ B ( OH ) 4 - and $${{\mathrm{B}}_{5}{\mathrm{O}}_{6}(\mathrm{OH})}_{4}^{-}$$ B 5 O 6 ( OH ) 4 - ionic forms, but the mass ratio of these species is higher in enriched boric acid solutions. In function with the concentration of enriched/natural boric acid, the solidified cementitious materials show different mineralogy.

Annual average indoor radon activity concentration was studied in 88 Hungarian kindergartens in 76 towns of 10 different counties. Annual average indoor radon activity concentration in the kindergartens was 61 Bq m− 3, maximum was 160 Bq m− 3. In the kindergartens the seasonal variation of radon is not so strong like in dwellings, because of the permanent ventilation and the closed period during the summer break.

This study investigates radon and thoron activity concentrations in adobe houses of Angola. Activity concentrations were recorded by passive detectors in rainy and dry seasons in 40 dwellings located at three areas with different climatic and geological backgrounds (Cabinda, Huambo, Menongue). Regarding seasonal and spatial variations, radon activity concentrations are higher in the rainy than in the dry season and both radon and thoron levels are the highest in Huambo, the central part of the country. The number of adobe houses above certain radon and thoron levels were estimated and the contribution of thoron to the inhalation dose was significant.

Absztrakt: Bevezetés: Nemzetközi tapasztalatok alapján a COVID–19-fertőzött, kórházban kezelt betegek több mint 30%-ában vénás, artériás és microvascularis thrombosis, disszeminált intravascularis véralvadási zavar, illetve thromboembolia alakul ki. Ennek laboratóriumi jellemzői a magas D-dimer- és fibrinogénszint, valamint a megnyúlt prothrombinidő és aktivált parciális thromboplastinidő. Módszer: Az esetbemutatások során intenzív osztályon kezelt, COVID–19-fertőzött betegek (n = 3) ClotPro® trombolasztométeres (EX-test, IN-test, FIB-test, RVV-test és TPA-test) eredményeit értékeltük a klinikai állapottal összefüggésben. Eredmények: A ClotPro®-paraméterek procoagulatiót, hypercoagulatiót, fibrinolysist vagy annak „shut down” megnyilvánulását jelezték, és összhangban voltak a hagyományos alvadási tesztekkel, illetve a kompenzált disszeminált intravascularis coagulopathia meghatározásával. Következtetés: Eredményeink magyarázatot adhatnak a vénás thromboemboliás szövődményekre, támogatják a terápiás alvadásgátló kezelés és a thrombocytaaggregáció-gátlás fontosságát. A gyógyszer típusának, adagolási algoritmusának, optimális időtartamának meghatározásához intervenciós klinikai vizsgálatok szükségesek, ezek engedélyeztetése folyamatban van. Orv Hetil. 2020; 161(22): 899–907.

Objective The aim of the present study was to analyse the incidence, prevalence, mortality and cause of death data of adult SLE patients and matched controls in a full-populational, nationwide, retrospective study. Methods This non-interventional study was based on database research of the National Health Insurance Fund of Hungary. A total of 7888 patients were included in the analyses, within which two subgroups of incident patients were created: the ‘All incident SLE patients’ group consisted of all incident SLE patients (4503 patients), while the ‘Treated SLE patients’ group contained those who received relevant therapy in the first 6 months after diagnosis (2582 patients). Results The median age of the SLE population was found to be 46.5 years (women 85%). The incidence rate was 4.86 and 2.78 per 100 000 inhabitants in the ‘All incident SLE patients’ and ‘Treated SLE patients’ groups, respectively. The standardized mortality ratio was 1.63 and 2.09 in the ‘All incident SLE patients’ and ‘Treated SLE patients’ groups, respectively. Overall survival was significantly lower (P Conclusion Infections, especially sepsis, had the largest positive effect on top of the extra mortality of SLE. This highlights that SLE patients are at increased risk of infection-related death.

Today, in the European multi-level and cooperative constitutional area the European Convention on Human Rights, the constitutional value provisions of the EU Treaties together with the Charter of Fundamental Rights of the EU, as well as the constitutions of the member states of the EU function as parallel constitutions. The legal remedies offered by international forums by nature are subsidiary, because it is desirable that legal issues of human rights be solved by the states at national level. The obligation of the exhaustion of domestic legal remedies as a procedural precon- dition is needed in order that the national level should have the chance to remedy the violation of human rights within its own legal system. The present paper focuses on Art. 8 para. (1) of Act LVIII of 2010 on the legal status of government officials, which states that the employer has the right to terminate the contract of goverment officials by two months’ notice period without any justification. The research is of considerable interest because the dismissed officials – who, in my opinion, de facto suffered injury by violation of human rights – were forced to turn to international forums because of the fact that the Hungarian legal system was not able to grant them adequate reparation. Therefore, the examination also evaluates the current level of fundamental rights arbitration and the jurisdiction using fundamental principles in Hungary.

Due to the Chernobyl nuclear power plant accident, contaminated air masses, containing

To evaluate whether the use of a commercially available dimethylxanthine theophylline compound (SpermMobil®) for artificial sperm activation would negatively affect clinical, obstetric and perinatal outcomes. Artificial sperm activation (ASA) was used when sperm motility after preparation was low or absent in our clinical standard procedure practice. ICSI cycles using either testicular or ejaculated sperm with concentration smaller than 5 million/ml from August 2012 to January 2018 were retrospectively analyzed (n = 815) and divided into two groups, a control group where no ASA was needed and the SpermMobil® group with ASA. The fertilization rate was significantly higher in the control group, but pregnancy and implantation rates did not differ significantly. Number of embryos transferred, good quality embryos for ET and number of frozen blastocysts were similar in both groups. Clinical pregnancy loss was significantly reduced in the SpermMobil® group, which was reflected in slightly better live birth rates than in the control group. Furthermore, there were no significant differences regarding gestational age, weight, height and z score for singletons or multiples in the SpermMobil® (n = 27 and n = 10) or control (n = 144 and n = 67) groups. There were no reports of malformation, perinatal mortality or intensive therapy in the SpermMobil® group, whereas in the control group, 12 babies needed intensive care, besides one intrauterine death. The use of SpermMobil® in samples with mostly immotile sperm not only facilitates the embryologists work but also optimizes the treatment outcomes for those patients with a bad prognosis. This is the first report of obstetric and perinatal outcomes after applying a theophylline derivative in human clinical use.

Abstract: Introduction: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant multisystemic vascular disease with a worldwide prevalence of 1 : 5000 – 1 : 10 000. Diagnosis is based on clinical Curacao criteria. Approximately 85% of HHT cases have heterozygous family-specific mutations in the ENG or ACVRL1 genes. Aim: We investigated 23 Hungarian HHT families, established the genetic diagnosis, executed family-screening and confirmed founder effects. Method: Probands were identified by the stratified population screening of the primary attendance area of our institution and from individuals contacting our study group voluntarily. Diagnosis is based on the otorhinolaryngological physical examination completed with characteristic telangiectasis sites, a visceral arteriovenous malformation screening and the sequence analysis of ENG and ACVRL1 genes. The family screening consists of physical examination and genetic screening for the family-specific mutation, followed by the arteriovenous malformation screening in patients with definite/suspected HHT and/or in individuals with the mutation. Results: Sixty-three individuals with family-specific mutations were identified in 22 families, 48 of them with definite and 12 with suspected HHT. Seven ENG and ACVRL1 mutations were detected, respectively; most of these are pathogenic. Three founder mutations were observed. One proband with definite HHT had wild-type alleles in all tested HHT-specific loci. Conclusions: The significance of genetic testing is confirming or excluding HHT in young asymptomatic individuals in families with pathogenic mutations. As ENG and ACVRL1 mutations result in overlapping fenotypes, the genetic testing lacks any prognostic value. The identification of founder effects might simplify the genetic diagnosis of new HHT patients from a given region. Orv Hetil. 2019; 160(18): 710–719.

One of the challenges of the present century is to limit the greenhouse gas emissions for the mitigation of climate change which is possible for example by a transitional technology, CO2 geological storage. Clay minerals are considered to be responsible for the low permeability and sealing capacity of caprocks sealing off stored CO2. However, their reactions are not well understood for complex simulations. This work aims to create a kinetic geochemical model of Na-montmorillonite standard SWy-2 supported by solution and mineral composition results from batch experiments. Such experimentally validated numerical models are scarce. Four 70-hours experiments have been carried out at atmospheric conditions, and with CO2 supercritical phase at 100 bar and 80 °C. Solution samples have been taken during and after experiments and their compositions were measured by ICP-OES. The treated solid phase has been analyzed by XRD and ATR-FTIR and compared to in-parallel measured references (dried SWy-2). Kinetic geochemical modelling of the experimental conditions has been performed by software PHREEQC. Experiments and models show fast reactions under the studied conditions and increased reactivity in presence of scCO2. Solution composition results cannot be described by the change of the uncertain reactive surface area of mineral phases. By considering the clay standard’s cation exchange capacity divided proportionally among interlayer cations of Na-montmorillonite, the measured variation can be described on an order of magnitude level. It is furthermore indicated that not only the interlayer cations take part in this process but a minor proportion of other, structural ions as well, differently in the reference and scCO2 environments.

Abstract: Introduction: Prognostic impact of the detection of minimal residual disease (MRD) in multiple myeloma (MM) has been confirmed in numerous studies. Aim: Retrospective examination of our patient database (107 newly diagnosed multiple myelomas between 2007 and 2017). Flow cytometry (FCM) was performed as MRD assessment. Method: MRD assessment was performed in 56 patients (median age: 68 years), after induction treatment of multiple myeloma. The treatment contained bortezomib in 91%, autologous haematopoetic stem cell transplantation (ASCT) was perfomed in 50%. MRD detection was performed on bone marrow samples, predominantly in our hospital (BD FACScan, 3 colour, panel: CD38, CD138, CD19, CD45, CD56, CD28, CD117, cyKappa, cyLambda, 100 000 events). Statistical analysis: SPSS 13.0. Results: The progression-free survival (PFS) and the overall survival (OS) were significantly longer in MRD negative (n = 22) patients (PFS: 54 months, OS: 79% after 5 years) than MRD positive patients (n = 34, PFS: 22 months, OS 21% after 5 years, p = 0.001). Patients achieving complete response (CR) (n = 29) have different PFS (MRD negative CR: 60 months, MRD positive CR: 21 months, p

Objectives: The aim of this study was to analyze the postoperative effects of extended nerve blocks and local infiltration analgesia (LIA) on postoperative pain control, muscle weakness, and blood loss after total knee arthroplasty (TKA). Patients and methods: Between February 24th 2020 and July 10th 2020, a total of 161 patients (55 males, 106 females; median age: 69.0 years [IQR 63.0-75.0], range, 41 to 81 years) who underwent primary TKA were randomly allocated into three parallel groups according to their concomitant procedure in a double-blind fashion: (i) those to whom nerve blockade was performed after competition of surgery under the duration of spinal anesthesia (n=50); (ii) those to whom LIA was performed during surgery (n=52), and (iii) control group (n=59). The content of LIA was 10-10 mL of 20 mg lidocaine with 0.01 mg adrenalin and 100 mg ropivacaine, 1 mL (30 mg) ketorolac, and 5 mL (500 mg) tranexamic acid was diluted by 50 mL 0.9% NaCl under aseptic conditions. Outcome parameters were the evaluation of pain until the evening of first postoperative day (24 to 36 h), mobilization, and blood loss within the first three postoperative days. Results: The pain was maximal between 4 and 8 h postoperatively, when the effect of the spinal anesthetic drugs disappeared. During this critical period, tolerable pain (Numerical Rating Scale, NRS ≤3) was observed in 52%, 42%, and 19% of nerve blockade in LIA and control groups, respectively. None of the patients complained of high-intensity pain (NRS >8) in the LIA group, which was a significant difference from the block and control groups (10% and 14%, p Conclusion: This study demonstrates that LIA technique offers a fast and safe treatment option for pain relief after TKA. No clinically relevant muscle weakness was observed among groups according to field block applications. Significant advantages were also achieved in blood loss.

We investigated CO2-free and naturally CO2-flooded sandstone samples from a deep saline aquifers formation, which represents potential carbon storage reservoirs. A descriptive geochemical model is also coupled to the laboratory study for the better understanding of geochemical interaction between sandstone and CO2. The studied area is located in the, Western Hungary in the Little Hungarian Plain where one of the largest CO2-producing fields in Europe can be found. In this region, we have the opportunity to compare rocks of the same sandstone formation without CO2 (not affected by natural CO2 flooding) and naturally CO2 flooded sandstone, where the CO2 was trapped around 7-4 million years ago. As boreholes sampled not only the parts of the formation, which were flooded by CO2 (Mihályi-Répcelak), but also the parts which were not affected at all by this flooding (Ölbő).Besides petrographic observations, scanning electron microscopy and mineral chemistry analyses, X-ray diffraction and infrared spectroscopy were used to determine 7 CO2-free and 6 CO2-flooded samples textural features, mineral compositions and the presence of OH-bearing minerals. We carried out thermodynamic-batch modelling with PHREEQC geochemical modelling software and compared to the laboratory results.The sandstone samples from the CO2 bearing reservoirs contain quartz, mica, kaolinite, K-feldspar and carbonates such as dolomite, calcite, ankerite and siderite. The CO2-free samples also contain chlorite, plagioclase and pyrite and all mentioned above. In the CO2-flooded samples a carbonate phase, dawsonite (NaAlCO3(OH)2) could be also observed in significant amounts (3-16 w/w%). This is an indicator mineral of large amount of CO2 inflow in the CO2-water-rock system. In addition, chlorite is apparently missing in the CO2-flooded samples. According to the petrographic observations and X-ray diffraction (XRD) results, it is clear that the plagioclase content is higher (∼ 11 w/w%) in the CO2-free samples compared to the CO2-flooded ones (2 flooded reservoir rocks. The lower amount of K-feldspar and plagioclase in the CO2-flooded samples can be explained by precipitation of dawsonite. These minerals can dissolve as a result of CO2-flooding and serve Na+ and/or Al3+ ion for dawsonite formation. The amount of the carbonate minerals also reveal systematic differences between the CO2-free and CO2-flooded sandstone, the amount of ankerite is higher (from 6 to 12 w/w%) in the later ones implying that some parts of the ankerite formed after the CO2 flooding event. The investigation of this unique area provides opportunity to study sandstone before interaction with CO2 and after millions of years being in contact with CO2.Acknowledgements:This research was financed by Hungarian Scientific Research Fund (K131353).Dóra Cseresznyés’ work is supported by the Cooperative Doctoral Programme granted by The Ministry for Innovation and Technology (ITM), National Research, Development and Innovation Office.

Recently, boric acid enriched in B-10 has received attention over natural boric acid in nuclear industry, because the elevated content of B-10 is a prospective neutron absorber. Advantages connected to the use of B-10 enriched boric acid are the increased controllability of reactor core which results in use of reduced amount of boric acid and, subsequently, the reduction in the amount of the radioactive boric acid waste produced during reactor operation. In the other hand, consequent radioactive boric acid waste requires an adequate stabilization technology as it contains fission products of health concerns, importantly Cs-137. Cementation is one of the proven, commercially viable, durable, widely used, simple and flexible technology for immobilization of low-level radioactive wastes (Hyatt and Ojovan, 2019). General integrity and durability of the cementitious waste form containing boric acid is B-leachability dependent (Rostamiparsa et al, 2020). The B-10 enriched boric acid leaching is expected to control also the Cs-leaching. However, no study is found in which this is proven and the different geochemical behavior and phase distribution of the B and Cs might cause deviations. This calls for the investigation of the connection between B- and Cs-leaching behaviors in cementitious materials, in this case, especially focusing on B-10 enriched boric acid waste form. In this ongoing experimental work the B- and Cs-leaching behavior of cementitious materials are studied, which are made of Portland cement, boric acid enriched in B-10 isotope and CsCl. Boron- and Cs-leachability from the cementitious matrix are investigated in parallel by a standardized reference leaching test (ASTM, 2017). The tests are carried out by immersing the 28 days cured cement paste samples in deionized water in a glass bottle. Leachant renewal and solution sampling are done on a daily basis for the whole leaching test period of 11 days. Analysis of leached fractions are quantitatively measured by ICP-OES. Characterization of solid samples are conducted by XRD, SEM-EDX and Raman micro-spectroscopy methods. This is the first study to shed light on the connection between B-leaching and Cs-leaching in cementitious materials containing B-10 enriched boric acid. Acknowledgements Our special thanks goes to Környezettudományi Centrum, Eötvös Loránd University and the Prémium_2017-13 research grant. References ASTM (2017). Standard Test Method for Accelerated Leach Test for Diffusive Releases From Solidified Waste and a Computer Program to Model Diffusive, Fractional Leaching from Cylindrical Waste Forms. ASTM Standard C1308-08(2017), West Conshohocken, PA. Hyatt, N.C & Ojovan, M.I. (2019). Special Issue: Materials for Nuclear Waste Immobilization. Materials, 12(21), 3611. Rostamiparsa, M., Szabó-Krausz, Z., Fábián, M., Falus, G., Szabó, C., & Völgyesi, P. (2020). Experimental assessment of interaction between boric acid enriched in boron-10 and cementitious matrix. In EGU General Assembly Conference Abstracts (p. 19441).

Eleven unique core samples from the National Radioactive Waste Repository of Hungary, Bataapati were studied in this work. The samples all cross the granite–concrete interface and have been drilled from around 275 m depth from the surface, 1–15 months after concrete injection. Phase analytical techniques, optical microscopy, SEM-EDS and Raman-spectroscopy were used for the analysis of interactions between granitic rock and cementitious building material. Newly formed phases, Ca-carbonates and titanite, were observed at the interface. Carbonation may reduce the porosity and permeability in the contact zone. The presence of titanite indicates the changing geochemical and thermodynamical constrains along the reaction front of granite–concrete, furthermore, it may help in the validation of future geochemical models. The cementitious material is seen to penetrate among the sheets of biotite mineral in granite which process is probable to cause the attachment of granite and concrete.

No abstract avalilable.

International data indicate that arterial, venous and microvascular thrombosis or disseminated intravascular coagulation occur in more than 30% of hospitalized patients with COVID-19. This condition is characterized by high levels of D-dimer and fibrinogen, prolonged prothrombin time and activated partial thromboplastin time.Blood samples from three COVID-19 patients treated in a Hungarian intensive care unit were collected and analyzed with ClotPro® tests. EX-tests, IN-test, FIB-tests, RVV-tests, and TPA-tests were performed. The results were interpreted with respect to the clinical condition of the patients.Procoagulation, hypercoagulation and either fibrinolysis or a "shut down" phenomenon of the fibrinolytic process were found with ClotPro®. The ClotPro® parameters were consistent with the conventional coagulation tests and corresponded with the criteria of non-overt disseminated intravascular coagulation.These findings encourage further investigations to elucidate the underlying pathophysiology of thromboembolic events in COVID-19 patients and may support the introduction of full dose anticoagulation with or without antiplatelet therapy. Interventional clinical trials may be helpful in defining the appropriate drug(s), for this purpose, the algorithms of administration, and the optimal duration of therapy. At present, the authorization of a clinical trial that attempts to answer these questions is in progress. Orv Hetil. 2020; 161(22): 899-907.