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INTRODUCTION The thermodynamic reference database THEREDA is maintained within the frame of a collaborative project aiming at the calculation of solubilities in high-saline solutions. The database is designed for applications in the context of the disposal of radioactive waste in rock salt formations and clay formations featuring solutions with a higher ionic strength. The project provides an internally consistent set of data, formatted for the use by several widely used geochemical codes. With the focus on high ionic strength systems, THEREDA complements other database projects focusing on low-saline solutions, such as THERMOCHIMIE or the PSI Chemical Thermodynamic Database. Users can access data and application examples, as well as download parameter files for their own use, from the website www.thereda.de. RECENT DEVELOPMENTS New project phase In 2020, the core project entered its 4th phase scheduled to run until 31st of January 2025. Parallel to the core project, coordinated and supported by it, other projects are running dedicated to the extension of THEREDA in specific fields. Maintenance Efforts are constantly taken to facilitate maintenance and future development of THEREDA. This includes databank structure, further development of graphical user interface to promote networking on the database, measures for knowledge preservation, and processes and quality management for future releases. Data release In 2022 new data were released. Apart from adding and updating datasets released before this was the first cumulative release where all system data sets were combined into one parameter file for each supported code. The following elements are part of the present release: • actinides and their chemical analogues: Th, U, Np, Pu, Am, Cm & Nd, • fission products: Se, Sr, Tc & Cs, • matrix elements: Na, K, Mg, Ca, Al, Si - Cl, SO4, CO3, PO4, • heavy metals: Pb. Supported codes After having abandoned the support for EQ3/6, TOUGH-REACT hast been added to the existing support of PHREEQC, ChemApp and Geochemist’s Workbench. PRESENT AND FUTURE DEVELOPMENTS Data release planned in 2023 A new data release is planned for the last quarter of this year. Updates and extensions will include: • Zn – Na, K, Mg, Ca – Cl, SO4, CO3 – H2O(l) (also alkaline systems), • U(+IV) hydrolysis in NaCl, MgCl2 and CaCl2 systems, • Solubility of carbonates up to 100°C, • Si, Al – Na, K, Mg, Ca – Cl, SO4, CO3 – H2O(l) (aqueous species only, polythermal, also alkaline systems), • Na, K, Mg, Ca – Cl, SO4 – H2O(l) (alkaline systems, T ≤ 40°C), • H2S(g), HS- – Na, K, Mg – Cl, SO4 – H2O(l) (partially polythermal), • Na, K, Mg, Ca – Cl, SO4, NO3 – H2O(l), • molar volumes for selected mineral phases. Application examples Altogether 505 application examples were created, on display on our website (www.thereda.de). They demonstrate the capabilities of THEREDA and indicate fields of future improvement. Two application example calculations of the data release planned in 2023 are shown in Figure 1 and Figure 2. Archiving and assessment of exp. data for the system of oceanic salts The polythermal dataset is based on the evaluation of about 16,000 solubility data points. Since the beginning of the 4th project phase their bibliographic origin is captured in the databank. Previous application examples are reproduced, or new ones created in the process. At present, binary subsystems are completed and ternary subsystems are in the process of revision. Altogether more than 400 primary data files and more than 100 application examples were created. ACKNOWLEDGEMENTS THEREDA is funded by the German “Bundesgesellschaft für Endlagerung (BGE)”, contract number 45181017.

Contains fulltext : 172671.pdf (Publisher’s version ) (Closed access) Since their discovery in patients with autosomal dominant (AD) chronic mucocutaneous candidiasis (CMC) in 2011, heterozygous STAT1 gain-of-function (GOF) mutations have increasingly been identified worldwide. The clinical spectrum associated with them needed to be delineated. We enrolled 274 patients from 167 kindreds originating from 40 countries from 5 continents. Demographic data, clinical features, immunological parameters, treatment, and outcome were recorded. The median age of the 274 patients was 22 years (range, 1-71 years); 98% of them had CMC, with a median age at onset of 1 year (range, 0-24 years). Patients often displayed bacterial (74%) infections, mostly because of Staphylococcus aureus (36%), including the respiratory tract and the skin in 47% and 28% of patients, respectively, and viral (38%) infections, mostly because of Herpesviridae (83%) and affecting the skin in 32% of patients. Invasive fungal infections (10%), mostly caused by Candida spp. (29%), and mycobacterial disease (6%) caused by Mycobacterium tuberculosis, environmental mycobacteria, or Bacille Calmette-Guerin vaccines were less common. Many patients had autoimmune manifestations (37%), including hypothyroidism (22%), type 1 diabetes (4%), blood cytopenia (4%), and systemic lupus erythematosus (2%). Invasive infections (25%), cerebral aneurysms (6%), and cancers (6%) were the strongest predictors of poor outcome. CMC persisted in 39% of the 202 patients receiving prolonged antifungal treatment. Circulating interleukin-17A-producing T-cell count was low for most (82%) but not all of the patients tested. STAT1 GOF mutations underlie AD CMC, as well as an unexpectedly wide range of other clinical features, including not only a variety of infectious and autoimmune diseases, but also cerebral aneurysms and carcinomas that confer a poor prognosis.

Razvoj procedura za dijagnozu kardiovaskularnih bolesti (KVB) kao i uvođenje lekova iz grupe statina u terapiju pacijenata koji pate od KVB, krajem dvadesetog veka u stručnoj javnosti doveo je do optimističkih procena da će se visoka učestalost mortaliteta uzrokovan komplikacijama KVB značajno umanjiti. Međutim, poslednji podaci Svetske Zdravstvene Organizacije (SZO) svedoče da su bolesti srca i krvnih sudova i dalje vodeći uzrok smrtnosti u srednje i visoko razvijenim zemljama. Navedeni poražavajući podaci ukazuju na potrebu u ispitivanja novih markera koji bi doprineli boljem razumevanju nastanka i razvoja KVB kao i ranom otkrivanju, efikasnijoj terapiji i pouzdanijoj prognozi ishoda bolesti. KVB se često javljaju kao posledica metaboličkih promena koje se dešavaju u sklopu neke druge bolesti. Jedno od takvih stanja je hronična bubrežna insuficijencija (HBI) koja je praćena kompleksnim metaboličkim promenama koji omogućavaju progresivan tok ateroskleroze, pa su pacijenti sa HBI svrstani u grupu sa najvećim rizikom za razvoj KVB. Gojaznost je jedan od tradicionalnih faktora rizika za razvoj KVB. Masno tkivo predstavlja vrlo moćan sekretorni organ koji oslobađa veliki broj molekula sa endokrinim potencijalom utičući tako na različite fiziološke procese. Prekomerna akumulacija masnog tkiva praćena je promenom u funkciji adipocita koji stvarajući proinflamatornu sredinu olakšavaju razvoj ateroskleroze koja leži u osnovi svih KVB. Adiponektin je jedan od najznačajnijih molekula koje oslobađaju adipociti i za razliku od većine drugih pokazuje antiinflamatorno, antiapoptotsko, antiaterosklerotsko i kardioproktektivno dejstvo. Navedeni efekti ostvaruju se aktivacijom adiponektinskih receptora, označenih kao AdipoR1 i AdipoR2. Uprkos njegovim zaštitnim efektima utvrđeno je da su povišene vrednosti adiponektina u koronarnoj srčanoj bolesti (KSB) i HBI povezane sa povećanim rizikom od smrtnosti. Smatra se da bi jedan od mogućih uzroka ovog paradoksa mogao biti poremećaj na nivou ose ligand/receptor koji dovodi do kompenzatornog porasta koncentracije adiponektina. Cilj našeg istraživanja je bio da se ispitaju koncentracije cirkulišućeg adiponektina i nivoi genske ekspresije adiponektinskih receptora AdipoR1 i AdipoR2 u mononuklearnim ćelijama periferne krvi u grupi zdravih ispitanika, pacijenata sa KSB i pacijenata sa HBI na hemodijalizi... Development of powerful procedures for diagnosis of cardiovascular diseases (CVDs) as well as introduction of statin therapy to patients with various CVD has led scientific community to an optimistic assessment that by the end of 1990s prevalence of CVD related mortality will be dramatically decreased. However, latest World Health Organization reports show that CVD are still leading cause of mortality worldwide indicating a need for constant search for new biomarkers which would contribute to better understanding of CVD development and progression, and enable early detection, more efficient therapy and more reliable prognosis. CVD are often consequence of metabolic rearrangements associated with several other diseases. One good example for such scenario is chronic kidney disease (CKD) which is associated with complex metabolic changes and progressive atherosclerosis. CKD patients are considered to be at very high risk of CVD mortality. Obesity is considered to be one of the traditional risk factors for CVD development. Adipose tissue is very powerful secretory organ which synthesize and secrete a vast number of molecules with endocrine potential thus influencing different physiological processes. Accumulation of fat is followed by adipocytes hypertrophy and over secretion of proinflammatory molecules which facilitate atherosclerosis progression. Adiponectin is most abundant protein produced by adipose tissue, and unlike most other adipokines has antiinflammatory, anti-apoptotic, anti-atherosclerotic and cardio-protective properties. These effects are mainly achieved by activation of two adiponectin receptors, AdipoR1 and AdipoR2. However, despite its effects it has been shown that increased, rather than decreased, levels of adiponectin are associated with higher mortality in patients with CVD or CKD. Previous studies suggested that one of the reasons for this paradox is dysfunction of ligand/receptor axis which leads to compensatory rise of adiponectin. In our study we sought to investigate if circulating levels of adiponectin and gene expression of adiponectin receptors AdipoR1 and AdipoR2 in peripheral blood mononuclear cells (PBMC) are changed in patients with coronary artery disease (CAD) and patients with CKD on hemodialysis compared to healthy subjects. In order to investigate if development of CAD is associated with changes in adiponectin levels and gene expression of adiponectin receptors we have divided CAD patients according to coronary angiography to patients without and patients with significant stenosis...

Defined by each country separately, according to real options, circumstances and traditions, the services of general economic interest have an objective purpose in ensuring protection and security for population. The services of general economic interest involve both public and economic services and show characteristics of both fields, reflecting the capabilities of communities to organize, regulate and provide them. Considering the accessibility to the essential service of general economic interest of providing household heating, as an undeniable condition of consumer protection, an analysis has been made in this field, with reference to the concrete manner of providing these services. The goal of this endeavor was to emphasize the actual conditionalities induced by the budget constraints of households while ensuring the universality of the access to the essential heating service. The empirical study is based on a survey of 55 households in sector 2 of Bucharest that have access to gas heating systems, while they have different revenues and equipments. The processing of the gathered data allowed the procurement of certain indicators that explain how household revenues determine the access to the heating services and how the deficiencies of the insurance system of these services deepen the social polarization and increase the weightings of those living at the limit of subsistence.