Long term experience of patients with unresectable or metastatic KIT positive gastrointestinal stromal tumours.

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).

[Epidemiology of screening-targeted cancers according to new data of the Czech National Cancer Registry]. / Epidemiologie zhoubných nádoru cílených screeningem dle nových dat Národního onkologického registru Ceské republiky.

The Czech Society for Oncology has developed an information system which combines the population-based Czech National Cancer Registry with clinical databases in order to cover the main areas of health care assessment - monitoring of the population burden, prediction of the number of cancer patients, diagnostic and treatment results. The presented data demonstrate a high cancer burden within the Czech population - each year there are approximately 8,000 new cases of colorectal cancer, 6,500 new cases of breast cancer, and 1,000 new cases of cervical cancer. And each year, about 4,000 people die from colorectal cancer, around 2,000 women die from breast cancer, and approximately 400 women die from cervical cancer in the Czech Republic. Population-based screening programmes focus on all of the above-mentioned groups of malignant tumours; therefore, it is essential to monitor epidemiological trends in order to assess the screening impact. Despite the high incidence rates of all three cancer types, the trend in mortality rates has been stable or has even decreased in the long term, which has inevitably led to a significant increase in the total prevalence of cancer patients. In 2011, the prevalence of colorectal cancer, breast cancer and cervical cancer amounted to 51,064 people, 67,261 women and 17,398 women, respectively. When compared with the year 2001, there was a 59%, 69% and 25% increase in the prevalence of colorectal cancer, breast cancer, and cervical cancer, respectively. Undoubtedly, taking care of high numbers of cancer patients will continue to require significant financial resources in the near future. As the epidemiological burden is still on the increase, preventive programmes need to be further promoted, including secondary prevention, which is provided through organised screening programmes. Although effective methods exist for timely diagnosis of all three of the above-mentioned cancer types, the epidemiological situation in the Czech Republic is being steadily worsened by a relatively high proportion of primary cancers being diagnosed too late. Each year, more than 50% of new colorectal cancer cases are diagnosed in clinical stage III or higher; in cervical cancer, this proportion is nearly 35%. By contrast, the well-promoted breast cancer screening programme has led to more than 75% of new cases of breast cancer being diagnosed in stages I or II, when the chance of successful treatment is significantly higher.

[State and development of background information for the evaluation of Czech cancer screening programmes]. / Stav a vývoj informacní základny pro hodnocení onkologických screeningových programu v Ceské republice.

Czech cancer screening programmes feature a comprehensive multimodal information system which covers all the levels of assessment needed - population-based monitoring (Czech National Cancer Registry), monitoring of results in the diagnostic databases of centres, as well as the quantification of coverage and outputs of primary care according to the administrative data of health care payers. A system of personalised invitations to cancer screening programmes was launched in 2014, based on a stand-alone component of the information system which makes it possible to identify eligible clients in health care payers databases. The system was fully standardised and uniformly implemented in all health insurance companies; its functionalities also involve both continuous and retrospective assessment of the results of personalised invitation. The legislative framework in force will have to be applied and implemented for a more comprehensive and integrated employment of all involved data sources, i.e. cancer registries, screening registries, and administrative data. The system must be able to analyse de-identified individual records on clients participation in screening programmes, and thus to ensure an adequate analysis of performance indicators in compliance with international recommendations.

Sunitinib followed by sorafenib or vice versa for metastatic renal cell carcinoma--data from the Czech registry.

BACKGROUND: 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. PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

[Clinical registers as a necessary support for personalized medicine]. / Klinické registry jako nezbytná podpora personalizované medicíny.

The article is an overview of major trends in the development of diagnostic and clinical registries which constitute a significant component of the informational background of personalized medicine. The rapid development of diagnostic methods has allowed data analysis to enter into the decisions about diagnosis and treatment of many diseases. However, practical application of the analyses still has serious limits related to the availability of complex, quality data. The basic trends of research in this field are the increasing standardization of content and structure of clinical databases and their association with clinical patient's records on an individual basis and ensuring the development of modern analytical tools.

Registry of inherited bleeding disorders in Poland--current status and potential role of the HemoRec database.

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.

[Czech Ophthalmologic Society's data collecting system for the monitoring and evaluating of the exsudative age-related macular degeneration treatment--the nation-wide project "AMADEUS"]. / Informacní zázemí Ceské oftalmologické spolecnosti CLS JEP pro monitoring a hodnocení lécby vlhké formy vekem podmínené makulárni degenerace-národní projekt AMADEUS.

The Czech Ophthalmologic Society (member of the Czech Medical Society of Jan Evangelista Purkyne) in collaboration with the Institute of Biostatistics and Analysis of the Masaryk University in Brno established a nation-wide information system for the follow-up of the epidemiology and treatment of the exsudative age-related macular degeneration (ARMD). The project named AMADEUS is a multi-centric, non-invasive, observation prospective study with the aim to improve the patients' treatment and to unify the criteria for the follow-up of the ARMD in the real clinical practice in the Czech Republic. The basic instrument of the whole project is a clinical register collecting parametric data from 9 specialized centers. Especially the project monitors the photodynamic therapy with Verteporfyrine (Visudyne) and treatment by anti-VEGF agents, or Pegaptanib (Macugen) and Ranibizumab (Lucentis). The project's protocol is trying to collect representative data about ARMD diagnosis and treatment; during regularly controls, the efficacy and safety of the treatment are followed-up.The data collecting does not influence the treatment strategy or the availability of chosen treatment for the patient. Since October 2008 until the end of the year 2009, the registry collected complex information about more than 1402 newly treated ARMD patients; out of them, in 582 cases the one-year follow-up period was regularly terminated. The available data shows well-considered indication of drugs indications by the doctors; the treated patients fulfill the indications criteria set by the scientific society. According to the data from the registry, in some treating centers the application schedule is not followed-up in some drugs (especially Pegaptanib); there is the possibility to improve the treatment. All the clinical centers participating in the AMADEUS registry will continue in the data collecting, which after a longer period of time will facilitate further treatment optimization and eliminating of the established imperfections. Further information about the project may be found at the web site: www.amadeus.registry.cz.

[Recombinant activated factor VII in the treatment of life threatening post-partum haemorrhage; registry UniSeven in the Czech Republic]. / Rekombinantní aktivovaný faktor VII (rFVIIa) v lécbe závazného poporodního krvácení data z registru UniSeven v Ceské republice.

OBJECTIVE: To analyze the data related to the treatment of 80 Czech patients with life threatening postpartum haemorrhage recorded in the clinical registry UniSeven during years 2004-2009. DESIGN: Retrospective, observational, multicentre study. SETTINGS: ICU and Obstetrics departments of University and Regional hospitals in Czech Republic. MATERIAL AND METHODS: UniSeven is an international academic project of Masaryk University in Brno, Czech Republic, focused on recording of clinical data related to "off-label" use of rFVIIa (Novo Seven) in life threatening bleeding. Data of 80 case reports of post partum haemorrhage from the registry was analysed from the clinical (efficacy and safety) as well as laboratory aspects. RESULTS: In 97.5% of our patients the treatment with rFVIIa was able to control the bleeding. In 53 women (66.3%) only one dose of rFVIIa was sufficient to control the bleeding. The rest of the patient received two or more rFVIIa doses. First dose of rFVIIa given to patients who needed more than one dose was significantly lower (96.6 microg/kg) compared to patient succesfully treated with one dose only (110.6 microg/kg; p = 0.048). The mortality rate in our cohort of patients was 2.5%. We have not recorded any thrombembolic event as and adverse event related to the rFVIIa treatment. In 74.3% of patients where rFVIIa was administered before considering the hysterectomy, it was able to avoid hysterectomy what we consider to be a significant improvement of the patients' quality of life. CONCLUSIONS: Our data were also considered during the work up of national guidelines for the treatment of life threatening post-partum haemorrhage in the Czech Republic.

[Experience in data management of the clinical retrospective project in Czech and Slovak oncology centres (IKARUS Project)]. / Zkusenosti s managementem dat retrospektivního klinického projektu v podmínkách ceské a slovenské onkologie (projekt IKARUS).

BACKGROUND: The retrospective part of the IKARUS Project (Incidence of Skeletal Related Events in Breast Cancer) was focused on monitoring the incidence of skeletal related events in patients with metastatic breast cancer treated in the Czech and Slovak Republics.The aim was to describe the experience with data collection management in the conditions of the Czech and Slovak Republics. SUBJECTS AND METHODS: Retrospective collection of data in multi-centre, non-interventional, epidemiological and explorative studies. Female patients diagnosed since 2000 were involved in the project in order to respect the five-year period of monitoring and to describe the treatment of the period. RESULTS: During the initiation phase of the retrospective study each of the 18 Complex Cancer Centres in the Czech Republic (see www.linkos.cz) and 18 chosen oncology centres in the Slovak Republic were addressed. In the end, data were collected from 13 oncology centres in the Czech Republic and 12 oncology centres in the Slovak Republic. The initial plan to enrol 650 patients was not completed; data on 254 patients from the Czech Republic and 125 patients from the Slovak Republic were finally analysed.The effectiveness of retrospective data collection in the conditions of Czech and Slovak oncology corresponded with the possibilities of access to data of formerly diagnosed and treated patients. In searching for retrospective data the present hospital information systems could not be used in most oncology centres.Therefore, the cost of retrospective data collection was estimated and was shown to be relatively high. CONCLUSION: The binding methodical conclusion is that unless a systemic change is made in the functionality of hospital information systems and standardised electronic documentation is introduced, the retrospective collection of clinical data in our conditions will be associated with high costs and a relatively low recovery factor.

A whole population study of gastrointestinal stromal tumors in the Czech Republic and Slovakia.

Due to problems with identification and an incomplete understanding on the gastrointestinal stromal tumors (GIST) before 2001, there has been a lack of comprehensive long-term population-based studies on GIST epidemiology at present date. We used data from the online registry of Czech and Slovak GIST patients (http://gist.registry.cz/), which has been compiled and maintained since 2006 and involves patients diagnosed from the year 2000. 278 patients were included in this study. Most of the tumors fell into the high-risk category (58.7%), followed by the intermediate (21.4%), low (16.6%) and very low (3.3%) categories. Locations other than the small intestine and stomach had significantly higher contribution of high-risk tumors. The median time of overall survival was 93.2 months, 5-year relative survival was 78.3% overall, 71.9% for patients with high-risk tumors, 91.1% for intermediate patients, and 91.9% for patients from the low- and very low-risk category. The annual crude incidence between the years 2001-2005 was 0.52 cases per 100,000 inhabitants. The annual European ASR and World ASR were 0.44 and 0.31 per 100,000 inhabitants, respectively. Presented data generally correspond to the whole-population studies recently published, including actual data on epidemiology, clinical characteristics, and survival of patients. The registry helps in improving GIST diagnostics, knowledge about the properties and behaviour of tumors, communication among physicians, and, last but not least, therapeutical options and results.

FEIBA in treatment of acute bleeding episodes in patients with haemophilia A and factor VIII inhibitors: a retrospective survey in regional haemophilia centre.

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.

Effect of rFVIIa dose and time to treatment on patients with haemophilia and inhibitors: analysis of HemoRec registry data from the Czech Republic.

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 microg kg(-1) and >250 microg 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.