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Copyright of Czecho-Slovak Pediatrics / Česko-Slovenská Pediatrie is the property of Czech Medical Association of JE Purkyne and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The article is trying to provide a comprehensive view of the role of enteral nutrition in patients with inflammatory bowel disease (IBD). By influencing nutrition, we can not only induce remission of Crohn's disease, or supplement the nutritional deficiency in patients with IBD, but we can influence one of the key factors in the pathogenesis of this disease. The complex pathophysiological mechanisms involved in the development of IBD include interactions between genetic factors, diet, intestinal microbiom and mucosal immune system. Current knowledge suggests, that by modifying eating habits we can not only change the development of an already ongoing disease, but also influence the risk of developing IBD. [ABSTRACT FROM AUTHOR]

Copyright of Czecho-Slovak Pediatrics / Česko-Slovenská Pediatrie is the property of Czech Medical Association of JE Purkyne and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Unlabelled: Bouveret syndrome is a gastric outlet obstruction caused by impaction of a gallstone that passes through a cholecystoduodenal or cholecystogastric fistula. It is a rare disease, most common in elderly women with multiple comorbidities and high surgical risk. The diagnosis can be made either radiologically or endoscopically. Endoscopic extraction is the preferred therapeutic option. Surgical intervention is indicated when endoscopic methods fail. We describe a case of Bouveret syndrome in a 79 years old woman. The report is followed by a review of literature on the diagnostics and treatment of this rare syndrome., Key Words: gallstones bilioenteric fistula gallstone ileus duodenal obstruction Bouveret syndrome.

Patients with chronic lymphocytic leukemia with deletion of the short arm of chromosome 17 (17p -) or mutation of the TP53 gene have significantly worse prognosis with a higher risk of progression to symptomatic disease, worse and shorter responses to chemo immunotherapy, and more frequent occurrence of Richters syndrome. TP53 deletion/ mutation is currently the only genetic abnormality that independently predicts response to treatment and also affects the choice of therapeutic approach in chronic lymphocytic leukemia. This work summarizes treatment options available for this poor prognosis variant of chronic lymphocytic leukemia. Traditional chemo immunotherapy (e. g. FCR) does not offer longterm disease control, and patients with TP53 deletion/ mutation were usually considered to undergo allogeneic bone marrow transplantation. New molecules from the group of BCR inhibitors or BCL2 antagonists achieve excellent efficacy in chronic lymphocytic leukemia with del17p even in relapsed/ refractory (R/ R) cases, with a higher percentage of responses and prolonged survival without progression. Clinical trials are ongoing to determine optimal therapeutic approach and to induce longterm remission of the disease. The new molecules change algorithms for treatment of patients with TP53 aberration, including indication for allogeneic transplantation. Especially younger patients should be consulted in centers of intensive hematological care to consider their inclusion into clinical trials testing new molecules or to indicate allogeneic transplantation at the optimal time.

Primary myelofibrosis (PMF) belongs to Ph- myeloproliferative diseases. The only curative treatment is hematopoietic stem cell transplantation (HSCT). Conservative treatment options comprise supportive care, especially administration of red blood cell and platelet transfusions, and medication. Hydroxyurea, interferon α, anagrelide, corticosteroids, androgens, or inhibitors of angiogenesis (thalidomide, lenalidomide, pomalidomide) may be used for treatment of PMF, depending on the clinical stage and disease symptoms present. Also splenectomy or radiotherapy of enlarged spleen have palliative potential. JAK2 kinase inhibitors represent a novel class of drugs with a very dynamic development. Ruxolitinib, an oral selective inhibitor of JAK1 and JAK2 kinases, has shown high efficacy in patients with high-risk PMF (or with myelofibrosis following polycythemia vera or essential thrombocythemia) to ameliorate disease symptoms and to reduce splenomegaly in randomized trials COMFORT-I and COMFORT-II. Long-term monitoring of the enrolled patients demonstated prolongation of overall survival. The drug is well-tolerated, the most common side effects of treatment with ruxolitinib being deepening of thrombocytopenia and temporary worsening of anemia. The current review deals with the place of JAK2 inhibitors (and the only drug already approved for clinical use - ruxolitinib) in the management of PMF, as an addendum to the Summary of recommendations for the diagnosis and therapy of BCR/ABL-negative myeloproliferations of the Czech Hematological Societys CZEMP.

Czech Working Group for Ph-negative Myeloproliferative diseases (CZEMP) recommends anagrelid (Thromboreductin®) for the treatment of Ph-negative chronic myeloproliferative disease (MPO) with thrombocythemia accompanying. To evaluate the efficacy of this treatment, the patient registry with essential thrombocythemia and/or thrombocytosis accompanying other Ph-negative myeloproliferative diseases was established. The beginnings of data collection go back to 2001, registry itself is maintained from 2005 and the aim is to archive the medical records with detailed physical and laboratory examination, safety patient profile included. The longest follow-up monitors 150 months period. Registry database contained 1,325 patients in the end of 2013, with an annual increase of anagrelid therapy as a drug of first choice in accordance with CZEMP guidelines approved by the Czech Society of Hematology of Czech Medical Association of J. E. Purkyne. Indication criteria contribute to this trend as anagrelid is the first choice agent in 65 years old patients, instead previous 60 years of age. Often, we can observe the combined treatment, especially, in older patients and in patients with primary myelofibrosis and polycythemia vera. There have been founded 543 thrombotic events in 413 patients and 63 bleeding events in 58 patients of study group by the end of 2013. During treatment, thrombosis was diagnosed 225 times in 171 patients and bleeding was observed 139 times in 104 patients. The therapeutic response is achieved after 3 months in 77% and after 6 months in 83% of subjects, but after 12 months, the treatment still fails in 12,5% of patients. It might be caused by slow titration of Thromboreductin®. One of the most important indicators of treatment success is the effect on clinical symptoms presentation, especially the occurrence of thrombotic events. The proof of a good treatment efficacy is demonstrated by 1.8 fold decrease in arterial thrombosis, more than 1.5 fold decrease in microvascular thrombosis and even 6.2 fold decrease in venous thromboembolism events. Bleeding is observed in about double more patients in comparison to the period before inclusion in the systematic monitoring, but the bleedings are clinically insignificant.Key words: anagrelid (Thromboreductin®) - Ph-myeloproliferative diseases - registry - thrombosis.

In the Czech Republic, anagrelide (Thromboreductin®) [29] is used according to the recommendations of the Czech Working Group on Myeloproliferative Disorders (CZEMP) for treatment of thrombocythemia associated with Ph negative myeloproliferative disorders (MPDs). The patient data are collected in the Registry of patients with essential thrombocythemia (ET) and thrombocythemia associated with other MPDs treated with Thromboreductin®. At the end of 2012, the Registry contained data on 1,161 patients. Out of these, 1,159 patients with the dia-gnosis of a Ph negative MPD were evaluated. In 844 patients, precise WHO based dia-gnosis was known at start of therapy: 442 (52.4%) had ET, 108 (12.8%) had polycythaemia vera (PV) and 243 had primary myelofibrosis (PMF). The median age was 51 years at the time of diagnosis. At the time of the evaluation of the population, the median was 59 years. Every year, the proportion of patients newly treated with anagrelide as a firstline treatment in accordance with the CZEMP guidelines has been increasing. A growing proportion of patients has been treated with an additional cytoreducing drug, such as hydroxyurea and interferon. The majority of the patients received also an antiaggregant (or anticoagulant). More than a half of patients harbors the JAK2 mutation. A prompt decrease of platelet counts (as the response to Thromboreductin® treatment) was documented in most of the patients. After one year, 86.9% of patients had a full or partial response. In poorer responders, combination cytoreductive treatment was administered rather then the escalation of the Thromboreductin® dosage. There were 461 thrombotic manifestations in 363 patients and 61 haemorrhagic events in 57 patients recorded in the patients history. In the course of treatment (followup; F U), thrombosis was diagnosed only 179-times in 136 patients. There were more haemorrhagic events during F U: 109 events in 83 patients. Upon comparison of the number of events during F U to their numbers in history, we found a twofold decrease in arterial thrombosis, an almost twofold decrease in microvascular thrombosis and even a 6.6- fold decrease in venous thromboembolism events. Bleeding episodes increased 1.8-fold during F U. However, the vast majority of these hemorrhagic events were clinically insignificant. In conclusion, the treatment strategy according to the CZEMP guidelines incorporating anagrelide is highly effective in reducing the platelet counts, strongly prevents venous events, reduces arterial events, and leads to an increase of minor hemorrhages.

Introduction: Lymphadenectomy is an essential part of surgical treatment of gastric cancer. In the part of hematoxylin-eosin negative nodes, small foci of tumour cells (micrometastastasis and isolated tumour cells) can be found using immunostaing or RT-PCR. The aim of this study is to asses clinical and prognostic relevance of these findings., Material and Methods: Multicenter, prospective, non-randomised clinical trial running in four Czech centres. All lymphatic nodes from patients after radical resection are stained using standard hematoxylin-eosin technique, all negative nodes are further processed with immunostaining employing cytokeratin antibody., Results: In the period of two years (1st January 2009 - 31st December 2010), 73 patients (100%) were included into the study from four Czech centers. All patients underwent radical resection for gastric cancer. Subtotal resection was performed in 33 patients (45%), total gastrectomy in the remaining 40 patients (55%). Total number of acquired lymphatic nodes (LN) reached 1245, average number of nodes per one patient was 17.3. H-E metastasis were disclosed in 364 LN (29%). All H-E negative nodes were further processed using immunohistochemical staining. Lymph node micrometastasis (MM) were discovered in 35 LN (3%), isolated tumour cells (ITC) in another 72 LN (6%). Clinical and prognostic relevance of lymph node MM and ITC was assessed based on the patients' survival data., Conclusion: Preliminary results of this study indicate that presence of the lymph node MM and ITC in gastric cancer patients is not linked to worse oncological outcome. Based on our results we can conclude, that expensive, time consuming and technically demanding immunostaining technique could not yet be recommended as a routine part of histological investigation of lymphatic nodes.

In 2009, the recommendations of the Czech Collaborative Group for Ph- Myeloproliferative Diseases (CZEMP) for diagnosis and treatment of BCR/ABL-negative myeloproliferative diseases (MPD), i.e., essential thrombocythemia (ET), polycythaemia vera (PV) and primary myelofibrosis (PMF) were updated and extended. The present article gives the rationale of the recommendations in full detail. The CZEMP diagnostic criteria for ET and PMF are based on histopathological (HP) findings, which must unconditionally be in line with the given clinical and laboratory characteristics of ET or of a certain stage of PMF, respectively. The platelet count is not decisive for diagnosis. In cases lacking an adequately taken and read HP finding, the Polycythemia Vera Study Group (PVSG) criteria are recommended. The diagnosis of typical PV is based on demonstration of the V617F mutation of the JAK2 gene along with a significant increase of red cell parameters. If these are close to borderline, the demonstration of increased total red cell mass (RCM) is required. In atypical cases lacking polyglobulia or elevated RCM, the HP picture of PV (in accordance with WHO description) plus JAK2 V617F mutation is satisfactory for diagnosis, or, in cases lacking JAK2 V617F mutation, the HP picture of PV along with polyglobulia (or increased RCM) is sufficient. The treatment principles of ET and other MPDs with thrombocythemia (MPD-T; i.e., the early stages of PMF and PV) are identical. The patients are stratified by their thrombotic risk (preceding thrombosis, another thrombophilic state, jAK2 mutation), presence of disease symptoms (mainly microcirculatory), platelet count and age. Only patients up to 65 years lacking the above mentioned risks with a platelet count < 1000 x 10(9)/l are considered as low-risk and do not demand cytoreducing therapy. The others are high-risk ones and have an indication for thromboreduction. In patients older than 65 years, the potentially leukemogenic drug hydroxyurea (HU) may be used. In the younger ones, the choice is between anagrelide (ANG) or interferon-alpha (IFN). In high-risk patients, the treatment goal is to maintain platelet counts below 400, and in low-risk ones, below 600 x 10(9)/l. In PV, polycythemia itself is another thrombotic risk factor. The condition is treated by bloodletting or erythrocytaphereses. If hematocrit levels < or =45 are not achieved, cytoreductive therapy using HU in patients over 65 years, or IFN in younger individuals is required. All patients with thrombocythemia in PV are high-risk and have an indication for cytoreduction. Acetylsalicylic acid is given to all patients with MPD-T with platelets < 1000 x 10(9)/l (at higher counts, hemorrhage is imminent), and to all individuals with PV, unless contraindication is present. In case of platelet count normalization, it may be withdrawn in cases of low-risk ET or PMF, not in JAK2+ PV. The treatment of advanced stages of PMF is symptomatic, with substitution of blood derivatives being the basis. The only curative treatment is allogeneic stem cell transplantation, which should not be indicated too early seeing to its risks, but also not too late--we must not allow transition into acute leukemia, which is heralded by blasts in the blood picture. The indication is the presence of any of the following criteria: values of hemoglobin < 10 g/dl, WBC < 4 x 10(9)/l and platelets < 100 x 10(9)/l, any percentage of blasts or > or = 10% immature granulocytes in the differential picture, >1 erythroblast per 100 cells--all at repeated examinations within at least a 2-month interval, and in addition, rapid progression of hepato-/splenomegaly, presence of general symptoms of the disease, portal hypertension and extensive swellings.

In the Czech Republic, anagrelid is used according to the recommendations of the Czech Working Group on Myeloproliferative Disorders for treatment ofthrombocythaemias associated with chronic myeloproliferative disorders--mainly essential thrombocythaemia and, regularly, reports are being presented from the Register of Patients Treated with Thromboreductin, most recently last year (Vnitr Lék 2009; 55: I-XII). The Register commenced in 2005 and from then it aims to determine detailed clinical and laboratory profiles of the patients. The structure of the Register has changed significantly in the course of its existence, reflecting the reports from each of the analyses conducted so far. Also, the data entry in the database improves every year and it reaches 97% on some of the items. The longest evaluation period in some of the patients is 108 months. By April 2010, the Register database contained data on 717 patients. Of these, 672 patients with the diagnosis of a Ph-negative chronic myeloproliferative disorder were evaluated. This year's analysis included the patients with essential thrombocythaemia, polycythaemia vera and primary myelofibrosis only. The analysis included 418 women and 254 men with median age of50 years. Unlike the first years, 2/3 of the current sample are non pretreated patients, meaning that the patients reach the specialized centres early in their treatment. Also, patients, and the older patients in particular, are more frequently treated with combined regimens including Thromboreductin. We increasingly observe hypertension as one of the monitored risk factors preceding the disease and laboratory parameters showJAK2 mutation in more than a half of patients while some form ofthrombotic diathesis is found in the anamnesis of 7-10% of patients. Some bleeding is observed in 1-5% of the registered patients. In comparison to the previous years, this is a decrease in the prevalence of clinical symptoms prior to the disease onset; this is very likely associated with an earlier patient diagnosis within the asymptomatic phase of the disease. Therapeutically, we achieve a fast treatment response but there still are 16.3% of sufficient afterone year of treatment. Thromboreductin dose is increasing but even in this group it does not exceeds the mean of 2.38 mg per 24 hours. Complications are observed in 6.2% of patients in the first year of therapy, and ofthese, thrombotic events in about 2.5% and (small) bleeding complications in 4% of patients. The data suggest that we still do not reach treatment response in a certain proportion of patients after a year of their therapy. Even though the care results from the analysed data improve every year, the Register helps to uncover some issues that still remain, such as treatment intensification and other treatment modifications.

The registry of patients treated with Thromboreductin (anagrelide) in the Czech Republic contains data concerning patients that have been treated using this drug since 2004. As of June 2009, the total number of patients was 549. The current analysis focused mainly on evaluation of anagrelide dosage needed to achieve a complete response in high-risk patients: reduction in platelet count to below 400 x 10(9)/l, which was also considered as reaching the therapeutic goal. The outcomes of the registry confirm that although anagrelide (Thromboreductin) is a very effective platelet-reducing agent, the administration of which is related to a low incidence of adverse effects and complications, the therapeutic goal is not achieved in all cases and or despite a quick treatment response, the therapeutic goal is achieved more slowly.

We have retrospectively evaluated a cohort of 144 patients (including 17 pediatric ones) with de novo acute promyelocytic leukemia registered in databases of institutions cooperating within the CELL group (The Czech Leukemia Study Group for Life). The patients were diagnosed according to WHO criteria from 1989 until 2006. The aim was to check how well fared the patients, the majority of whom was not included into clinical trials, in real life. Of 140 evaluable patients, 97 (69.3%) attained complete remission (CR). The projected overall survival (OS) 4 years after diagnosis was 58.9%, and 55.3% at 6 years. In 8 patients (6.0%), no antileukemic therapy at all was given (either they died shortly after admission to the ward or therapy was not feasible due to their clinical status). Of 125 patients with documented commencement of some kind of therapy, 96 (76.8%) achieved CR. Of 102 patients with induction treatment with a combination of anthracycline and tretinoin (ATRA), 84 individuals (82.4%) attained CR (typically, this cohort might have been subjected to clinical trials). This result was better than that of patients treated by chemotherapy only (n = 15; CR 46.7%; P = 0.003) or by ATRA monotherapy (n = 13; CR 62.5%; P = 0.17). Another parameter with a significant impact on attaining CR was the leukocyte (WBC) count at diagnosis: its median values in patients achieving and not achieving CR were 2.1 and 24.0 x 10(9)/l, respectively (P < 0.0001). The WBC counts affected OS as well (P = 0.0001). However, when only patients after attaining CR were evaluated, the initial WBC counts no longer affected OS (P = 0.18). Achieving CR was also influenced by the performance status (PS) 0-1 (P = 0.005), which was in turn closely correlated to WBC counts (P = 0.0006). Additional factors (most likely connected with leukocytosis) influenced attaining CR with borderline statistical significance: e.g. FAB M3v morphology, LDH serum level, fibrinogen level, presence of internal tandem duplication (ITD) of the FLT3 gene (which was strongly associated with leukocytosis and also with the short PML/RARalpha transcript resulting from the bcr3 break in the PML gene). It may be speculated that FLT3-ITD is just one of the possible factors that lead to leukocytosis. The platelet counts at diagnosis had no impact on entering CR. Thus, we have not validated the current PETHEMA risk stratification in distinguishing intertermediate and low risk patients. Our study points to a significant difference of the results obtained in real life and of the results that could be achieved in patients who were fit to enter clinical trials. Among the prognostic factors, the most important one was the WBC count, the PS (which is highly affected by the WBC count), and feasibility of administration of the most potent induction therapy with anthracyclines and ATRA.

A review of diagnosis of acute promyelocytic leukemia (APL) is presented. There are still many patients with progressive disease with leukocytosis at presentation. These are at greater risk of early death due to bleeding (often intracranial), or, less frequently, due to thrombotic complications. In Czechia, we have, in some instances, noted an unacceptably long time from the first symptoms to diagnosis and to administration of the highly specific differentiation therapy with tretinoin (ATRA) along with anthracycline chemotherapy. This combination is highly efficient--cures are seen in some 70% of patients. Therefore, we present a diagnostic minimum for each and every internist, and even better for every general practician, to get acquainted with. All cases of pancytopenia and consumption coagulopathy should be suspected of APL and referred to a specialized hematologist without any delay. In the following more detailed review of diagnostic measures, much attention is given to APL morphology, which is the first clue leading to diagnosis. The finding of the typical hypergranular FAB M3 morphology and of cells with bundles of Auer rods ("faggot cells"), along with the HLA-DR, CD33+ immunophenotype, is highly (but not absolutely) specific for APL. In cases of the micro-/hypo-granular variant FAB M3v Form, and whenever APL cannot be ruled out with certainty, a test to prove the presence of the PML/RARalpha fusion gene is indicated, using either RT-PCR or, eventually, immunological demonstration of the specific distribution of the PML protein in the cell nucleus. Given that morphology of APL cases, as defined according to WHO criteria (95% of which carry the PML/RARalpha fusion gene), admits extremely divergent morphological pictures ofthe variant forms, we recommend these investigations to be performed in every case of de novo acute myeloid leukemia. A review of the less frequent morphological, as well as genetic variants is given, and the principles of immunophenotypic, cytogenetic and molecular diagnostics are also reviewed.

The registry of patients treated with Thromboreductine (anagrelid) in the contributing centres in the Czech Republic has been updated with data on the patients receiving this medication since 2004. The original purpose of the registry was to record responses to Thromboreductine therapy and adverse drug reactions in patients with essential thrombocytopenia. However, data on additional Ph negative myeloproliferations, as well as data on cytoreductive therapies other than exclusively that using Thromboreductine has also been recorded in the course of its compilation, including data on combined regimes. At present, the database contains data on 421 patients, and valid conclusions can be drawn if the level of data filling is enhanced. Evaluation has been currently focused on the analysis of the risk of development of clinical symptoms of thrombosis and on the standards of treatment from the viewpoint of the achieved treatment response. Analyses of data from the registry corroborate the special importance of the proof of JAK2 mutation, and of the test for factor V Leiden mutation, and of protein of S for the assessment of the risk of thromboembolic complications. The output of the analysis confirms that anagrelid is a very efficient thromboreductive agent the administration of which is associated with a low incidence of non-serious adverse effects (10.9%). However, in spite of a fast response to therapy, the therapeutic goal consisting in the reduction of the platelet count below 400 (or below 600) x 10(9)/l, i.e. the complete (or partial) treatment response, is relatively slow to achieve. This is likely to be due to lack of radical corrections in the dosage of the drug for different reasons.

The progressing knowledge on chloroma and chloroleukemia is reviewed. However, it is uneasy to identify with certainty the cases of acute promyelocytic leukemia (APL) among the historical descriptions ofchloroma since 1823. In part, this is due to confusion produced by the historical cytological nomenclature--in practice, the leukemic promyelocytes were regarded, quite inaccurately, as a subtype of paramyeloblasts until early 1960's. The term promyelocytic leukemia first appeared in Naegeli's text-book in 1931. However, no clinical associations of the morphological description were then given. As a clinico-pathological entity, APLwas defined by Hillestad in Norway in 1957 and on a more detailed level, by Bernard et al in France in 1959. However, the descriptions of chloroma by Butterfield in 1909 and of leukemia with panmyelophthisis and defibrination by Risak in 1935 in the German-written literature and the case of chloroma described by Libánský in Czech in 1939 were cases of APL with high probability. Further on, experimental studies leading to the postulation of possible differentiation-inducing therapy of leukemia are reviewed--the importance of introducing of clonal culture of hematopoietic cells in the 1960's and the proof of the possibility of bypassing the maturation block in the works of Sachs's group in Rehovot, Israel, is stressed, as well as the later experiments with differentiation inducers in the cell line models in the 1970's and 1980's. The first trials with retinoids in therapy of APL are mentioned, along with the interesting background of the French-Chinese collaboration (under the auspices of professors Degos and Wang) in the introduction of retinoids into practice in the second half of the 1980's. It is discussed how the success of the clinical trials led to enormous progression in the field of molecular genetics of APL, which, in turn, led to development of a new generation of remedies, i.e. to the renaissance of arsenic in the treatment of APL in the 1990's, among others thanks to the current Chinese minister of health, Chen Zhu.

Authors present cases which illustrate a various initial symptoms and courses of patients between the first symptoms and the diagnosis of acute promyelocytic leukemia. Symptoms indicated possible serious hematological diseases are well-known: unusual bleeding, recurrent infections or infections not responded to antibiotics, fatigue, anemia symptoms. The disease progression was sometimes very rapid, however, in certain patients the symptoms were disappreciated or even minimized and the approaches were not adequate to risks resulted from newly diagnosed acute promyelocytic leukemia.

Since 2005, registers of patients treated with Thromboreductin (anagrelid) kept by some centres in the Czech Republic have been supplied with data concerning patients whose treatment with this preparation started in 2004. The purpose of the register is to record responses to therapy by Thromboreductin and adverse events in patients with essential thrombocytemia and other myeloproliferations, and to subsequently analyse the data. Another objective is to detect predisposition to clinical symptomatology and disease complications. Apart from thrombocyte count, additional risk factors are monitored. The database currently contains data for 336 patients. Initial analyses of data from the register point to the fact that anagrelid is a highly effective thromboreductive agent the administration of which is associated with relatively low incidence of adverse events (11.8 %) of mild and usually transitory nature. The therapeutic objective is attained at a relatively slow rate (according to overall stratification under 400 or under 600 x 10(9)/l thrombocytes), which is probably due to insufficient dose adjustment.

Introduction: Choledocholithiasis is nowadays managed endoscopicaly in the majority of patients. Open surgery with CBD exploration remains important, when endoscopy failed. The completeness of stones' extraction may be confirmed with choledochoscopy., Method: The retrospective analysis of patients operated on choledocholithiasis within the period of seven years was performed. Two groups of patients were studied. First group was managed by choledocholithotomy followed by choledochoscopy. Second group underwent exploration of the common bile duct without choledochoscopy. Frequency of the residual stones in both groups was studied. Statistical evaluation was done using alpha2 test. The value of p < 0.05 was settled as statistically significant., Results: In the study period of seven years 46 patients were operated on CBD stones. Choledocholithotomy followed by intra-operative choledochoscopy was performed in 21 patients. No remnant stones were recorded in this group. Exploration of the CBD with stone extraction without choledochoscopy was done in 25 patients. In this group residual stones occurred in 3 patients. The result was not statistically significant, p = 0.10., Conclusions: According to our experience and literature, intra-operative choledochoscopy can reduce frequency of the residual CBD stones. Peroperative choledochoscopy can thus be recommended as an accessory procedure to CBD exploration to prevent stones oversight.

Anagrelide hydrochloride is an effective drug used in patients with ET and other myeloproliferative disorders with thrombocythemia to selectively decrease the number of thrombocytes. Indications for use of anagrelide were described in detail in Czech medical literature. Since 2005 data concerning treatment with anagrelide in some medical clinics have been collected in patient register showing course of treatment from 2004, when the medicament obtained marketing authorization from State Institute for Drug Control to be used in the treatment of thrombocythemia in myeloproliferative disorders. Aim of patient register is to monitor medical effect of anagrelide therapy and incidence of adverse effects in patients with ET and other myeloproliferative disorders and subsequent analysis of collected data. At the moment patient register contains data from 154 patients.

Background: Despite a considerable effort, the majority of acute myeloid leukaemia (AML) patients do not have a suitable specific molecular marker for monitoring minimal residual disease (MRD). The results of some studies suggest the Wilms tumour gene (WT1) as a possible molecular marker of MRD., Methods and Results: We measured the expression of WT1 at diagnosis and during treatment of the acute myeloid leukaemia (AML) patients. The expression of WT1 was measured by the quantitative real-time RT-PCR in peripheral leukocytes from 56 AML at diagnosis and 7 patients with AML transformed from myelodysplastic syndromes (MDS). The WT1 expression was significantly elevated (up to 3 orders of magnitude) in peripheral blood samples (PB) of AML patients at diagnosis compared to PB samples of healthy donors (P < 0.0001). The level of WT1 expression depends particularly on FAB AML subtype, with the highest being found in AML patients with subtypes M4, M1, M3 and AML transformed from MDS. Conversely, AML patients with M2 and with the presence of AML1/ET0 at presentation showed a significantly lower expression of the WT1 gene compared to the remaining AML patients at presentation (P = 0,005). Further, sequence samples of 12 AML patients under long-term surveillance were tested for the WT1 expression in parallel with the expression of specific MRD markers--fusion genes: AMLI/ETO, PML/RARalpha and CBFB/MYH11. The levels of WT1 gene expression and the above specific fusion genes significantly correlated. Moreover, 14 patients without the specific MRD marker were tested for the WT1 expression. The results show that haematological relapses were associated with the rise of expression of the specific fusion genes and with the WT1 gene expression. The rise of WT1 expression above the level seen in leucocytes from peripheral blood and/or bone marrow of healthy donors--in four patients under long-term surveillance the "molecular relapse" predicted ongoing haematological relapses as early as 2 months in advance., Conclusions: Our results, in accordance with some of the previously published ones, show that WT1 expression seems to be a suitable marker of minimal residual disease in AML patients.

Lymphadenectomy in gastric cancer became vital and obligatory part of staging procedure. Therapeutic effect of lymphadenectomy with patient's prognosis improvement still remains uncertain. In subgroups, extensive lymphadenectomy enhance radicality of the procedure, enables to reach R0 resection in the area of tumour bed and hopefully improves survival. D2 lymphadenectomy is now becoming as a standard also in our country. Technique and possible complications of D2 lymphadenectomy are the matter of this article.

The diagnosis of acute appendicitis remains a difficult task for even the experienced surgeon. For this reason every supplementary examination affords help for the surgeon its diagnosis. The goal of our study was to evaluate the significance of ultrasonography (USG) in diagnosing right lower quadrant abdominal pain. 204 patients admitted for suspect acute appendicitis were evaluated retrospectively. The accuracy of USG was studied by comparing this finding with preoperative, histopathological findings respectively. In comparison leucocytosis was also studied. In our studied cohort, USG examination had a sensitivity of 88.4% and specificity of 93.0%. Leucocytosis for diagnosis gave a sensitivity of 88.6% and specificity of 60.0%.

The only hope for long time survival in gastric cancer is radical surgery with complete tumour removal. Lymphadenectomy as a part of surgical procedure helps to remove tumour completely. The value of lymphadenectomy for staging is clear and surgeons have no doubt about it. Therapeutic effect of lymphadenectomy with improvement of the prognosis remains uncertain. In this work we would like to discuss current trends in lymphadenectomy in gastric cancer, especially evaluating the value and extent of the procedure.

Background: The B-chronic lymphocytic leukemia (B-CLL) has highly variable prognosis. Possibility of more relevant prognosis has a great impact for the beginning and mode of therapy., Methods and Results: One hundred patients diagnosed as having B-CLL were included into the study. Beside usual examinations necessary to establish the diagnosis, cytogenetic examination for the detection of deletion 13q14, 17p13, 11q23 and trisomy 12 and immunophenotyping was done. The mean age of the patients was 58.2 years, there were 62 men and 38 women. 91 evaluated patients were divided into two groups--those with the steady disease (55 pts) and those with progressive disease (36 pts). No relation between the number of cytogenetic abnormalities and the Rai stage of the disease was found. We identified a relation between the bone marrow infiltration pattern (diffuse) and the number of cytogenetic abnormalities and the del 13q14 (p.05). Using the immunophenotyping of the lymphocytes we found a relation between the expression of CD38 and CD11c and the disease progression (p < 0.05). Neither of the method (FISH and immunophenotyping) revealed differences between results from bone marrow samples and those from peripheral blood., Conclusions: Though the cytogenetic (FISH) and immunophenotype evaluation at the time of diagnosis did not improve the ability to define better the clinical course of the B-CLL, we suggest to use both methods routinely as an important tool to identify patients who would develop the progressive disease.

Background: Leukemias develop due to defects in proliferation, differentiation and apoptosis, which take place in stem cells or progenitors of hematopoiesis. These processes have several crossing points, one of them is the role of inhibitors of cyclin-dependent kinases. The aim of this study was to study the expression of cyclin-dependent kinases inhibitors p21 Cip and p27 Kip and expression of proliferative antigen Ki-67 in leukocytes of human leukemia., Methods and Results: The expression of cyclin-dependent kinases inhibitors was detected at mRNA level mainly by comparative reverse-transcription polymerase chain reaction and in selected samples also by the real-time polymerase chain reaction. While p27 Kip expression in leukocytes of leukemic patients and healthy persons was universal, large differences in expression of p21 Cip were found both among individual patients of the same type of leukemia and between different types of leukemias and healthy persons. The p21 Cip expression was significantly higher in acute leukemias than in chronic ones and healthy persons. A comparison of p21 Cip expression with the clinical outcome of the leukemic patients showed that the group of 14 acute leukemia patients surviving more than 30 months had a significantly lower expression of p21 Cip than 12 patients of this type of leukemia who died within this time limit. Moreover, the results obtained on a smaller set of acute promyelocytic leukemia patients indicated that the lower p21 expression is connected with a better prognosis., Conclusion: Our results pointed out the importance of the cyclin-dependent kinase inhibitor p21 Cip in human leukemias and indicated that the lower p21 Cip expression might be a positive prognostic factor in acute myeloid leukemia patients.

Background: Premier Platinum HpSA EIA is an enzyme immunoassay developed for diagnosis of H. pylori infection using polyclonal antibodies against H. pylori in human stool. A new H. pylori stool antigen test, based on monoclonal antibodies, has been developed. Our aim was to evaluate prospectively the accuracy of the novel antigen stool test (DAKO HpStAR) using monoclonal antibodies for detection of H. pylori infection in children., Methods and Results: Total of 93 children undergoing upper gastrointestinal endoscopy were included in the study. Biopsy specimens were sampled from the gastric antrum and from the corpus. Patients were classified as H. pylori positive if histology and urease test were positive. All children provided a stool sample within 3 days after gastroscopy. IgG serology against H. pylori was also employed. HpStAR test was performed according to the manufacturers protocol. Results were read at 450/630 nm by spectrophotometry (cut-off point 0.150). Of the 93 children, 26 were H. pylori positive (13.1 +/- 3.2 yr), and 67 patients were H. pylori negative (12.8 +/- 4.7 yr). Only 2 children were misclassified (1 false negative, and 1 false positive). Sensitivity was 96.1%, specificity 98.5%, the positive and negative predicting values were 96.1% and 98.5%, respectively. Serology showed sensitivity 88.5%, specificity 70.2%; the positive and negative predicting values were 53.5% and 94% respectively., Conclusion: HpStAR test based on monoclonal antibodies can be considered an accurate, non-invasive, reliable method for the diagnosis of H. pylori infection in children.

Although the effectivity of an urgent ERCP was confirmed, some aspects relating to this method are still discussed. Timing of this procedure has not been established yet. Whereas there is no doubt about indication with decompression of the common bile duct in acute cholangitis, views on the effectiveness of examination incl. papillotomy in case of acute pancreatitis are not uniform. The authors demonstrate a group of 538 patients who had urgent ERCP within the last three years with a successful cannulation rate of 93%. The experience of large centres is confirmed as the great efficiency and effectivity of this procedure when the standard conditions and processes are respected.

Gastric cancer is a serious malignant disease. The only hope for long time survival is radical surgery. It consists of gastrectomy and also resection of adjacent lymphatic tissues. The extent of lymphadenectomy is one of the currently discussed questions. D2 lymphadenectomy is now becoming standard also in our country. This extensive procedure can be quite difficult and needs an experienced surgeon. It is also connected with prolonged operation time and higher costs. According to some authors, this procedure is also associated with a higher morbidity. The question, whether D2 lymphadenectomy should be performed, could be answered by the sentinel node concept, which is the topic of our work.

Background: B-chronic lymphocytic leukemia (B-CLL) is the most common adult leukemia in the Western countries. Any routinely used staging system does not distinguish exactly the probable course of the disease at the time of diagnosis. Therefore the new prognostic factors, which help to assess the optimal therapeutic plan of patients, are searched intensively., Methods and Results: We evaluated 154 patients with the B-CLL at the time of diagnosis--133 of them were retrospectively divided into two groups--one with stable form and the other with progressive form of the disease. We compared these two groups of patients with some of the prognostic factors (absolute lymphocyte count, the level of C-reactive protein, lactatdehydrogenase, beta-2-microglobulin, tumour necrosis factor, the immunoglobulin levels, the expression of CD38, FMC7, surface immunoglobulins, the type of bone marrow infiltration, and the cytogenetic abnormalities (trisomy 12, del(13)(q14), del(17)(p13) a del(11)(q23)). We found higher absolute lymphocyte count, level of beta-2-mikroglobulin, tumour necrosis factor, expression of CD38, light chains lambda, lower expression of FMC7 and less frequent nodular type of bone marrow infiltration by the patients with progressive disease. The correlation of the cytogenetic abnormalities and the course of the disease or stage according to the RAI et al. [27] staging system were not significant may be due to the small number of evaluated patients and short period of follow up., Conclusion: The routine evaluation of some risk factors in patients with B-chronic lymphocytic leukemia at the time of diagnosis helps to distinguish those with the probable more aggressive course of the disease and have the implication for the design of risk-adapted treatment strategies. The prognostic impact of the cytogenetic abnormalities and other risk factors has to be evaluated on larger group of patients during longer follow up period and repeated evaluations.

Intestinal duplications are described as uncommon congenital anomalies rarely diagnosed in adults. A case of young woman with obscure abdominal symptomatology is demonstrated. After investigation the cause of it was disclosed surgically. During operation a cystic ileal duplication was exposed and resected. Despite of difficulties calling for another operation, the case was successfully solved. Based on the author's experience some aspects of these diseases--etiopatogenesis, symptoms, possibilities of examination and complications are discussed and surgery as the main treatment is emphasized.

Background: Trisomy 12 was found to be the most frequent chromosomal aberration identified by conventional cytogenetic studies of bone marrow cells and peripheral lymphocytes of patients with CLL. Molecular-cytogenetic techniques which enable examination of dividing and/or non-diving interphase nuclei (I-FISH), proved existence of other chromosomal abnormalities, mainly deletions, which could have in CLL patients relation to the origin, course and prognosis of the disease., Methods and Results: During the last two years bone marrow chromosomes of all patients with CLL were examined by G-banding and by I-FISH. The numerical changes of chromosome 12 were followed by centromeric DNA probe in dividing and non-dividing cells. The small deletions were ascertained by locus specific probes for 13q14 (Rb gene), 17p13 (p53 protein) and 11q23 (MLL gene). These genes are responsible for cell division and their function is probably in connection with neoplastic process. It is of interest whether numerical and structural chromosomal rearrangements are primary or secondary changes and what is their impact on etiology of CLL. 93 patients were examined by DNA prove CEP12 and trisomy 12 was found in 24 of them (25.8%), the range of the clone was 2.5-75.5% of the screened cells. Deletion del(13)(q14) was examined by probe D13S319 in 73 patients and proved in 24 of them (32.8%), pathological clone ranged 2.5-80.0% of the cells. Deletion del(17)(p13) was found in 14 patients out of 61 examined by probe LSI p53 (22.9%). The extent of the clone was 2.5-34.0% of examined cells. Deletion 11q23 was not ascertained in any of 11 patients by means of probe LSI 11q23 (MLL). All probes used for FISH were manufactured by VYSIS., Conclusions: FISH is very sensitive method, suitable for molecular-cytogenetic examination of leukemic patients. With I-FISH the deletion of 13q14 was ascertained as the most frequent chromosomal aberration in series of 73 patients with CLL. We continue to increase the number of patients screened by I-FISH with all eligible DNA probes and start the prospective study on patients with chromosomal pathology. We will correlate the immunophenotype, morphology, clinical course and prognosis with karyotypic findings.

Acute hospital care is the most expensive form of medical care. Some of patients hospitalized in acute hospitals could be cared for in alternative settings without compromising quality of care. The Appropriateness Evaluation Protocol (AEP) is the most widely used tool for evaluation of appropriateness of hospitalisation in acute care hospitals. We applied AEP to 189 patients admitted within 20 days to department of surgery of a teaching hospital. We have evaluated appropriateness of admission and each following day of stay up till discharge or 15th day of hospitalization. Reasons of inappropriate admissions and delayed discharges were classified and analyzed. Results of the evaluation of selected patients were subject to control by a committee of fully specialized hospital physicians. 16 (8%) of 189 admissions and 306 (27.5%) of 1114 evaluated days of stay failed the AEP criteria. These patients could be well served by lower treatment intensity in outpatient clinics, nursery homes or their own homes. Such a shift in pattern of provided care requires profound organizational changes many of which are out of reach of individual acute hospitals. Despite some limitations we find AEP a useful tool for internal utilization review. External application of AEP in a representative sample of acute care hospitals could provide important data for future development of the Czech health care system.

The article has discoursed of a diagnosis and a therapy of bleeding peptic ulcer. There has been used the group of the patients admitted at The Surgical Unit of The Faculty Hospital Motol in Prague during two and half years. Urgent endoscopy and endoscopic treatment of indicated findings have been performed as a common procedures. The surgical therapy has been necessary for the part of this patients. Operation's indications, types of the operations and their timing have been discussed.

Acute hypergranular promyelocytic leukemia (AML M3) belongs to malignant diseases leading very rapidly to death. Immediate treatment based on early diagnosis may cure one third of patients. The typical finding in peripheral blood of patients is pancytopenia with or without atypical promyelocytes. In published studies only 15-25% patients exhibit leukocyte counts above 10 x 10(9)/l. Five of our ten patients studied had leukocyte count above 10 x 10(9)/l. The difference might be in connection with late and slow diagnosis of AML M3. AML is not taken into consideration during medical examination even if the disease occurs in medical family. Thus we describe clinical signs of AML M3 that could be divided into three main groups: bleeding, infections and anemia. In patients with bleeding or anemia or with infections repeating within a short period or with an infection and concurrent signs of bleeding or anemia the complete blood cell count should be examined immediately. If blood cell count abnormalities are found the patient should be sent immediately to hematology unit for further examination and treatment. Early diagnosis enables to start "differentiation therapy" with all-trans retinoic acid that could be administered as monotherapy only in patients with leukocytes below 5 x 10(9)/l. Early diagnosis of AML M3 might ameliorate the fate of patients, since four of our five patients referred to us with elevated leukocyte counts expired in the first five days.

The authors describe a case of extrapulmonary tuberculosis with affection of the reticuloendothelial system in a 59-year-old female patient. After successful treatment the findings in the spleen, bone marrow and the laboratory results had normal levels. The authors draw attention to the diagnostic difficulties. They were able to confirm the diagnosis only by histological examination of biopsy from the pelvic bone. An interesting finding was eruption of lupus vulgaris on the legs in the course of treatment. The authors discuss some aspects of the disease and its differential diagnosis.

A 57-year-old man, who for many years was treated for a pleomorphic lymphoma with cytotoxic drugs and x-ray therapy, died from the generalized tumor process. At autopsy, an extraordinary finding was found in form of acute pulmonary mycosts, which histologically appears to be histoplasmosis. Culture was not attempted, but the diagnosis is based on morphology, both of the organism proper and its intracellular location, and on results obtained by immunofluorescence studies. In numerous pulmonary vessels, endothelial pillows of histiocytes with yeast cells are found, typical for histoplasmosis, but no generalization was found in other organs. The source of the infection is not clear.