The paper concerns conceptual design of two-cylinder spark-ignition internal combustion engine for a hybrid car with power requirement at least 45 kW at 6000 rpm intended for use in HEV and PHEV ...vehicles. In the first part of the paper is presented the design of the engine and 3D CAD model, created with support of 3D CAD software. The main part shows the thermodynamic calculation of the main parameters of the combustion engine by means of special software.
Sequential therapy with tyrosine kinase inhibitors (TKIs), sunitinib and sorafenib, is a common treatment choice for patients with advanced/metastatic renal cell carcinoma (mRCC) despite lack of ...randomised trials. The aim of this retrospective registry-based study was to analyse the outcomes of RCC patients treated with sunitinib–sorafenib or sorafenib–sunitinib sequence.
The Czech database containing information on patients treated for mRCC using targeted agents was used as a source of data for retrospective analysis. There were 138 patients treated with sunitinib–sorafenib sequence and 122 patients treated with sorafenib–sunitinib sequence.
Progression-free survival (PFS) was 17.7 months for patients treated with sunitinib–sorafenib sequence and 18.8 months for those receiving sorafenib followed by sunitinib (P = 0.47). Overall survival (OS) at 1 year was 83% 95% confidence interval (CI) 77% to 90% for patients treated with sunitinib–sorafenib and 84% (95% CI 77% to 91%) for sorafenib–sunitinib patients (P = 0.99). Treatment toxic effects were predictable but a significant proportion of patients (up to 14%–25% for different lines of therapy and used TKI) switched between TKIs or discontinued TKI therapy because of toxicity.
In contrast to most of the previously published reports, we have not observed improved PFS or OS for mRCC patients treated with the sorafenib–sunitinib sequence as compared to the sunitinib-sorafenib sequence.
Identifying haemophilia patients with inhibitors for clinical trials is difficult due to the limited number of patients available. Registries are therefore being established as an additional means of ...data collection. The aim of this study was to investigate the effect of different recombinant activated factor VII (rFVIIa; NovoSeven®) dose ranges and dosing schedules on the incidence of re‐bleeding in haemophilia patients with inhibitors. In this retrospective, uncontrolled study, data on the bleeding patterns of adult haemophilia patients with high responding inhibitors were analysed. Only data from the Czech Republic, obtained by the HemoRec registry, were used. This study analysed ‘real‐life’ clinical data and focused on the collection of the same parameters in different patients: time from bleeding onset to first injection, effect of first injection, number of re‐bleedings, total number of injections and total amount of haemostatic drug used. Fifteen patients met the inclusion criteria and were included into the study (128 bleeding episodes). Patients treated within 2 h of bleeding onset experienced less re‐bleeding than patients treated after 2 h of bleeding onset (5.2% vs. 13.7%, respectively). In addition, patients who were treated after 2 h of bleeding onset experienced fewer re‐bleedings when high‐dose rFVIIa was used (15.8% and 0%; <120 μg kg−1 and >250 μg kg−1, respectively). Initial high‐dose rFVIIa was also associated with a decline in total rFVIIa consumption. This registry has provided a unique insight into the bleeding patterns of inhibitor patients, highlighting the importance of early treatment initiation and appropriate starting dose.
Abstract The patient registries are the key activities which can help us in planning of the effective health care, assessing standards of diagnosis and care, and answer the questions concerning of ...the prevalence of neuromuscular disorders. Preliminary survey in the nine neuromuscular centers and four genetic laboratories in Czech Republic (CR) revealed about 400 patients with myotonic disorders. The majority seems to be the patients with myotonic dystrophy type 2 (MD2), but the exact figures have not been available up to date. Therefore Czech neuromuscular society decided to establish the national myotonic registry which can answer these questions. The technological aspect of the project, the data collection, storage and backup and their analysis are provided by the Institute of Biostatistics and Analyses, Masaryk University, Brno, CR. On-line data collection is based on a TRIALDB system developed on Yale University, Connecticut, USA, which is widely used for this purpose. So it is not necessary to install any additional computer software. The database can be accessed only by authorized persons using their login and password. For each patient is generated a unique ID; all data transfer is encrypted and the system is designed to prevent their unauthorized use during data transfer. Laws and regulations in CR require having an informed consent from all patients whose data are used in the registry. All claims for personal data protection were met. Data are stored on the central server on Masaryk University in Brno in Oracle 9i database. The registry was launched in the June 2011 and up to February 2012 contains 235 records from eight Czech neuromuscular centers. The database include 76 patients with MD1, 116 patients with MD2, 25 peoples with ClCN1 mutations, and 14 persons with SCN4A mutations. The majority (85%) of records are from two centers (Prague and Brno), five centers have completed less than 10 records.
The patient registries belong to the core activities which can help us in planning of the effective health care, assessing standards of diagnosis and care, and answer the questions concerning on ...epidemiologic data. Besides of the local hospital-based databases and registries we can find in Czech Republic four national registries of hereditary neuromuscular disorders associated under unique name: ReaDy (registry of muscular dystrophy). Four registries are currently running: Duchenne/Becker muscular dystrophy (DMD/BMD), spinal muscular atrophy (SMA), myotonic disorders (MD), and facioscapulohumeral muscular dystrophy (FSHD). Each registry is independent and has its own curator. The registries are under the supervision of Czech neuromuscular society. The technology, the data collection, the storage, the backup, and analyses are provided by the Institute of Biostatistics and Analyses, Masaryk University, Brno, CR. On-line data collection is based on a TRIALDB system developed on Yale University, Connecticut, USA, which is widely used for this purpose. For each patient is generated a unique ID; all data transfer is encrypted and the system is designed to prevent their unauthorized use during data transfer. Laws and regulations in CR require having an informed consent from all patients whose data are used in the registry. All claims for personal data protection were met. Data are stored on the central server on Masaryk University in Brno in Oracle 9i database. Since 2011 to the March 2014 796 Czech patients were collected: 370 DM, 277 DMD/BMD, 89 FSHD, and 60 SMA. The majority (76%) of all records are from two centers (Prague and Brno). The average annual increase during last three years is 96 patients. The biggest acquisition reveal patients with myotonic disorders (about 45 per year), the smallest growth has the registry FSHD with approx. 11 patients per annum.
The project IP EUROTRANS, domain DEMETRA, is primary focused on the study of the technology of the interaction between steels and heavy liquid metals. The characterization of the metal response to ...sudden changes, simulating accidental conditions in liquid lead–bismuth eutectic was carried out. This paper reports the results of two hot-spot simulations with two different oxygen concentrations (10
−8
wt%, 10
−6
wt%). Each experiment was divided in two main periods: the initial, long period at the standard operating temperature 550
°C; the second, short period, at higher temperature, 650
°C. The damage that occurs on the austenitic steel AISI 316L and the ferritic–martensitic steel T91 was investigated. The amount of damage for both steels was higher at lower oxygen contents and the short, hot spot simulation, markedly affected the T91. At higher oxygen content the amount of damage decreased. A few, localized pits, were observed; however, there was no visible increment in the amount of damage after the hot spot simulation.
We present data collected in HemoRec, an Internet‐based platform implemented in 2006 in 15 haemophilia treatment centres in Poland and compare them with the national registry of inherited bleeding ...disorders established since 1991 at the Institute of Haematology and Blood Transfusion in Warsaw. We also analyse the current status of haemophilia treatment in Poland as well as future perspectives. Data on 1102 patients registered in HemoRec were analysed and compared with 4294 patients in the national registry (status as at 17.08.2009). The number of patients with severe haemophilia, mild/moderate haemophilia and von Willebrand in HemoRec is 530, 328 and 54 (respectively), compared with 1199, 1167 and 1128 in the national registry. The mean age of all haemophilic patients registered in HemoRec is 26.2 years, compared with 37.3 years in the general Polish male population in 2008. The number of haemophilic patients with inhibitor registered in HemoRec is 102 compared with 155 in the national registry (resulting in a prevalence of 14.9% of all severe haemophilia A and 1.6% of all severe haemophilia B patients). HemoRec includes data on a representative group of Polish haemophilic patients, mostly with haemophilia and haemophilia with inhibitor. von Willebrand’s disease is largely under‐registered in Poland. The survival of Polish haemophilic patients is shorter than that in the general population. The number of inhibitor patients in Poland is relatively large and should be decreased by wider availability of immunotolerance induction in 2010.
FEIBA® (factor eight inhibitor by‐passing activity) is used to achieve haemostasis in haemophiliacs with inhibitor. The aim of this study was to evaluate efficacy and consumption of the product in ...treatment of haemorrhages in haemophiliacs with factor VIII inhibitor, and determine factors that can influence the results of treatment. We used data from our haemophilia centre from years 2000–2008. Six haemophiliacs with factor VIII inhibitor were treated on demand with FEIBA® for 61 bleeding episodes (45 haemarthroses, six muscle bleeds, six other sites bleeds and four multiple sites bleeds). The median cumulative dose of FEIBA® per bleeding episode was 205 U kg−1. Bleeding was stopped in 96.7% (59 of 61) of events but re‐bleeding occurred in 3 events (4.9%) within 48 h after cessation of bleeding. In home treatment (20 of 61) bleeding stopped in 90% (18 of 20) without recurrence and the median consumption per event was reduced to 153 U kg−1. Without the use of home treatment the median consumption was 250 U kg−1 per event and bleeding ceased definitely in 92.7% (38 of 41) of cases. The cumulative dose of FEIBA® was lower for three episodes with re‐bleeding: median 96 U kg−1 but not in the two cases of ineffective treatment: 361 U kg−1. FEIBA® in management of bleeding episodes completely resolved the haemorrhage in 91.8% of events and in a further 4.9% if treatment was restarted. Using home treatment saved expenditure due to the lower cumulative dose needed for treatment of haemorrhage.
A retrospective analysis of consecutive patients (183 in total, of which 105 were males and 78 females) with gastrointestinal stromal tumour (GIST) was performed. The mean age was 61 years, median ...age 64 years. The most frequent localization of the tumour was stomach in 74 patients (40.4 %) and the small intestine in 46 patients (25.1 %). Two or more different synchronous or metachronous cancers occurred in 34 (18.6 %) patients with histologically confirmed GIST. Ninety-six patients were treated with imatinib mesylate in palliative setting during the course of their disease. The therapy was finished in 60 patients and 36 patients have been treated so far. The median progression-free survival reached 32.9 months in the group of 96 patients treated with imatinib. The median overall survival in the group of 96 patients treated for metastatic disease reached 77 months. Two-year and 5-year survival was 85.2 % and 63.1 %, respectively. The second-line therapy with sunitinib malate was administered in 37 patients, of which 31 finished and 6 continued in the therapy. The median progression free survival and median survival since the sunitinib therapy initiation reached 8.4 and 22.1 months, respectively (Tab. 2, Fig. 2, Ref. 16).
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