Characteristics and treatment results of refractory and relapsed acute myeloid leukaemia in paediatric patients treated in Polish Paediatric Leukaemia/Lymphoma Study Group institutions according to the Protocol Acute Myeloid Leukaemia Berlin-Frankfurt-Munster 2012 and a review of novel treatment possibilities in paediatric acute myeloid leukaemia.

Introduction: This study aimed to present the clinical features and results of treatment of patients diagnosed with refractory or relapsed acute myeloid leukaemia (AML) in Polish Paediatric Leukaemia/Lymphoma Study Group (PPL/LSG) institutions, treated in accordance with the Protocol Acute Myeloid Leukaemia Berlin-Frankfurt-Munster 2012, as their first-line therapy. Material and methods: The outcome data of 10 patients with refractory AML (median age 9.5 years) and 30 with relapsed AML (median age 12 years) were analysed retrospectively. Re-induction was usually based on idarubicin, fludarabine, and cytarabine along with allogeneic haematopoietic stem cell transplant (allo-HSCT) in 5 patients with refractory AML and 7 relapsed AML children. Results: 37.5% (3/8) of refractory AML patients achieved second complete remission second complete remission (CRII). One of ten patients (1/10; 10%) was alive and stayed in complete remission for 34 months after the allo-HSCT. The probability of 3-year event-free survival (pEFS) in this group was 0.125 ±0.11. In the group of relapsed AML patients, the CRII was achieved in 9 patients (34%), and the probability of survival was: pEFS = 0.24 ±0.08; probability overall survival (pOS) = 0.34 ±0.09, with significantly better results achieved in patients who underwent allo-HSCT (pOS = 0.54 ±0.14 vs. 0.08 ±0.08, p < 0.0001). Conclusions: The prognosis of refractory AML and the first AML recurrence in children who were first-line treated in PPL/LSG centres according to Protocol Acute Myeloid Leukaemia Berlin-Frankfurt-Munster 2012 is poor. Failures of re-induction treatment particularly result from difficulties in achieving remission. Allogeneic HSCT improves prognosis in children with refractory and first recurrent AML, under the condition it is performed in complete remission. Novel therapeutic approaches are needed to increase the remission rate and improve the outcomes.

The Gene and Protein Expression of the Main Components of the Lipolytic System in Human Myocardium and Heart Perivascular Adipose Tissue. Effect of Coronary Atherosclerosis.

The aim of our study was to examine the regulation of triacylglycerols (TG) metabolism in myocardium and heart perivascular adipose tissue in coronary atherosclerosis. Adipose triglyceride lipase (ATGL) is the major TG-hydrolase. The enzyme is activated by a protein called comparative gene identification 58 (CGI-58) and inhibited by a protein called G0/G1 switch protein 2 (G0S2). Samples of the right atrial appendage and perivascular adipose tissue were obtained from two groups of patients: 1-with multivessel coronary artery disease qualified for coronary artery bypass grafting (CAD), 2-patients with no atherosclerosis qualified for a valve replacement (NCAD). The mRNA and protein analysis of ATGL, HSL, CGI-58, G0S2, FABP4, FAT/CD36, LPL, ß-HAD, CS, COX4/1, FAS, SREBP-1c, GPAT1, COX-2, 15-LO, and NFκß were determined by using real-time PCR and Western Blot. The level of lipids (i.e., TG, diacylglycerol (DG), and FFA) was examined by GLC. We demonstrated that in myocardium coronary atherosclerosis increases only the transcript level of G0S2 and FABP4. Most importantly, ATGL, ß-HAD, and COX4/1 protein expression was reduced and it was accompanied by over double the elevation in TG content in the CAD group. The fatty acid synthesis and their cellular uptake were stable in the myocardium of patients with CAD. Additionally, the expression of proteins contributing to inflammation was increased in the myocardium of patients with coronary stenosis. Finally, in the perivascular adipose tissue, the mRNA of G0S2 was elevated, whereas the protein content of FABP-4 was increased and for COX4/1 diminished. These data suggest that a reduction in ATGL protein expression leads to myocardial steatosis in patients with CAD.

In-hospital mortality as the side effect of missed care.

AIM: The aim of the study was to assess the influence of the number of hours of daily nursing care for NHPPD in medical departments on missed care and the correlation between NHPPD and in-hospital mortality. BACKGROUND: Patient mortality can be a consequence of missed care as it correlates with the nurse-patient ratio. One of the methods to measure missed care is the Nursing Hours per Patient Day rating. METHODS: The study sample included 44,809 patients including 971 deaths in 8 wards. The influence of nursing hours, nursing education, and the percentage of patients' classification on in-hospital mortality were evaluated with backward stepwise linear regression. RESULTS: One hour added to the average NHPPD in medical departments was related to a decrease in mortality rate by 6.8 per 1,000 patient days and a lower chance for the emergence of unplanned death by 36%. CONCLUSIONS: The number of NHPPD and the percentage of professional nurses are factors influencing missed care and in-hospital mortality. IMPLICATIONS FOR NURSING MANAGEMENT: The severe consequences of missed care, that is mortality, and the correlation between in-hospital mortality, nursing education and nursing-patient ratio, which are indicators of care quality, are arguments for maintaining adequate staffing levels to avoid missed care.

Outcome of refractory and relapsed acute myeloid leukemia in children treated during 2005-2011 - experience of the Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG).

AIM OF THE STUDY: Recent studies showed relatively better outcome for children with refractory (refAML) and relapsed acute myeloid leukemia (relAML). Treatment of these patients has not been unified within Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG) so far. The goal of this study is to analyze the results of this therapy performed between 2005-2011. MATERIAL AND METHODS: The outcome data of 16 patients with refAML and 62 with relAML were analyzed retrospectively. Reinduction was usually based on idarubicine, fludarabine and cytarabine with allogenic hematopoietic stem cell transplant (alloHSCT) in 5 refAML and 30 relAML children. RESULTS: Seventy seven percent relAML patients entered second complete remission (CR2). Five-year OS and disease-free survival (DFS) were estimated at 16% and 30%. The outcome for patients after alloHSCT in CR2 (63%) was better than that of those not transplanted (36%) with 5-year OS of 34% vs. 2-year of 7% and 5-year DFS of 40% vs. 12.5%. Second complete remission achievement and alloHSCT were the most significant predictors of better prognosis (p = 0.000 and p = 0.024). The outcome of refAML children was significantly worse than relAML with first remission (CR1) rate of 33%, OS and DFS of 25% at 3 years and 53% at 2 years, respectively. All survivors of refAML were treated with alloHSCT after CR1. CONCLUSIONS: The uniform reinduction regimen of the documented efficacy and subsequent alloHSCT in remission is needed to improve the outcome for ref/relAML children treated within PPLLSG. The focus should be on the future risk-directed both front and second line AML therapy.

Economic evaluation of the prevention of falls resulting from missed care in polish hospitals.

Objectives: Falls are associated with increased morbidity, mortality, prolonged hospitalization and an increase in the cost of treatment in hospitals. They contribute to the deterioration of fitness and quality of life, especially among older patients, thus posing a serious social and economic problem. They increase the risk of premature death. Falls are adverse, costly, and potentially preventable. The aim of the study was to analyze the cost-effectiveness of avoiding one fall by nurse care provided by the nurses with higher education, from the perspective of the health service provider. Methods: The economic analysis included and compared only the cost of nurse intervention measured by the hours of care provided with higher education in non-surgical departments (40.5%) with higher time spend by nurses with higher education level an increase in the number of hours by 10% (50.5%) to avoid one fall. The time horizon for the study is 1 year (2021). Cost-effectiveness and Cost-benefit analysis were performed. All registered falls of all hospitalized patients were included in the study. Results: In the analyzed was based on the case control study where, 7,305 patients were hospitalized, which amounted to 41,762 patient care days. Care was provided by 100 nurses, including 40 nurses with bachelor's degrees and nurses with Master of Science in Nursing. Increasing the hours number of high-educated nurses care by 10% in non-surgical departments decreased the chance for falls by 9%; however, this dependence was statistically insignificant (OR = 1.09; 95% CI: 0.72-1.65; p = 0.65). After the intervention (a 10% increase in Bachelor's Degrees/Master of Science in Nursing hours), the number of additional Bachelor's Degrees/Master of Science hours was 6100.5, and the cost was USD 7630.4. The intervention eliminated four falls. The cost of preventing one fall is CER = USD 1697.1. Conclusion: The results of these studies broaden the understanding of the relationship among nursing education, falls, and the economic outcomes of hospital care. According to the authors, the proposed intervention has an economic justification.

Cost-Effectiveness Analysis of Improving Nurses' Education Level in the Context of In-Hospital Mortality.

(1) Background: an assessment of the cost-effectiveness of employing an increased number of nurses with higher education from the perspective of the service provider. (2) Methods: Based on a year-long study results and data collected from a large hospital, we conducted of the costs of preventing one death. The study involved intervention by 10% increase in the percentage of nursing care hours provided by nurses with higher education. The measure of health effects was the cost of avoiding one death (CER). The cost-effectiveness analysis (CEA) was used as the evaluation method. (3) Results: The cost of employing a larger percentage of nurses with higher education amounts to a total of amounts to a USD 11,730.62 an increase of 3.02% as compared to the base costs. The estimated number of deaths that could be prevented was 44 deaths. Mortality per 1000 patient days was 9.42, mortality after intervention was 8.41. The cost of preventing one death by the 10% increase in BSN/MSN NCH percentage in non-surgical wards USD 263.92. (4) Conclusions: increasing the percentage of care hours provided by nurses with tertiary education is a cost-effective method of reducing in-hospital mortality.

The Influence of Nurse Education Level on Hospital Readmissions-A Cost-Effectiveness Analysis.

Background: Readmissions are adverse, costly, and potentially preventable. The study aimed to evaluate the cost-effectiveness of reducing readmissions resulting from missed care, depending on the level of education of nurses, from the perspective of the service provider. Methods: We calculated missed care resulting in additional readmissions based on the longitudinal study conducted between 2012 and 2014, as well as readmissions that could have been potentially prevented by adding a 10% increase in hours of nursing care provided by BSN/MSc nurses for 2014. The cost-effectiveness analysis (CEA) was performed to calculate the cost-effectiveness of preventing one hospitalization in non-surgical and surgical wards by increasing the number of nursing hours provided by BSN/MSc nurses. Cost−benefit analysis (CBA) was performed, and the CBR (cost−benefit ratio) and BCR (benefit−cost ratio) were calculated. Results: Increasing the number of hours of nursing care (RN) by 10% decreased the chance for an unplanned readmission by 11%; (OR = 0.89; 95% CI: 0.78−1.01; p = 0.08) in non-surgical wards and 43% (OR = 0.57; 95% CI: 0.49−0.67; p < 0.001) in surgical wards. In non-surgical wards, the number of readmissions that were preventable with extra hours provided by BSN/MSc nurses was 52, and the cost-effectiveness ratio (CER) was USD 226.1. The number of preventable readmissions in surgical wards was 172, and the CER was USD 54.96. In non-surgical wards, the CBR was USD 0.07, while the BCR was USD 1.4. In surgical wards, the CBR was USD 0.02, and the BCR was USD 4.4. Conclusions: The results of these studies broaden the understanding of the relationship among nursing education, patient readmission, and the economic outcomes of hospital care. According to the authors, the proposed intervention has an economic justification. Hence, the authors recommend it for approval by the service provider.

[Complete autologous bone marrow recovery after allogeneic stem cell transplantation in a child with acute monoblastic leukemia]. / Pelna odnowa autologiczna szpiku kostnego po alloprzeszczepieniu u dziewczynki z ostra bialaczka monoblastyczna.

We present a case of autologous bone marrow recovery after allogeneic hematopoietic stem cell transplantation (HSCT) in a 7-year old girl who was treated due to acute myelocytic leukemia. First complete remission is lasting for 81 months after the allo-HSCT. Presented case constitutes an exceptional clinical situation and it indicates that diagnosis of leukemia relapse should be cautiously considered once the autologous bone marrow recovery is observed after allogeneic HSCT.

[Genotyping and minimal residual disease study in children with acute myeloid leukemia: preliminary results]. / Genotypowanie i oznaczania choroby resztkowej u dzieci z ostra bialaczka mieloblastycznq: wstepne wyniki.

The aim of the paper is to present the initial results of molecular examination which was started in 2006 for children with acute myeloid leukemia. Better knowledge of biology of this disease, can result in establishing of new risk factors what allows more precise patient stratification to different therapeutic groups. Study was obtained patients until to 18 years of age treated according to AML-BFM 2004 INTERIM protocol in 14 centers of the Polish Pediatric Leukemia/Lymphoma Study Group. Mononuclear cells were collected from bone marrow on time points established according to the AML-BFM 2004 INTERIM protocol. Collected cells were isolated on Ficoll gradient, and RNA and DNA were isolated using TRIZOL reagent. To synthesize cDNA an amount of 1 mg of total RNA was used. To perform quantitative RT-PCR and RQ-PCR reactions 4 fusion gene transcripts (AML1-ETO, CBFb-MYH11, PML-RARA /subtype bcrl and bcr3/) were used according to the protocol established by Europe Against Cancer Program. An expression of WT1 gene was tested additionally. An analysis of ABL control gene was used to normalize of achieved results. Determination of duplication of FLT3 gene in DNA sample was performed with starters complementary to JM region. Genotyping was performed in 75 patients with acute myeloid leukemia so far. AML1-ETO fusion gene transcript was found in 14 patients (19%). PML-RARA (subtype bcr3) and CBFB-MYH11 gene transcripts were detected in 3 (4%) and 3 (4%) patients, respectively. Duplication of FLT3 gene was found in 4 (5.3%) cases. Between 67 tested children over expression of WT1 was present in 51 patients (76%). Analysis of MRD level in subsequent time points showed systematic decrease of number of fusion gene transcript copies and gene WT1 expression. To establish the rate of molecular marker presence in AML in children and the influence of the presence of MRD on the treatment results as well, the study has to be conducted on a larger group of patients with longer follow-up.

[Treatment failure in children with acute myelocytic leukemia: over 25-year experience of Polish Pediatric Leukemia/Lymphoma Study Group with four consecutive unified treatment protocols for childhood acute myelocytic leukemia]. / Niepowodzenia leczenia w ostrej bialaczce szpikowej u dzieci: ponad 25-letnie doswiadczenia Polskiej Pediatrycznej Grupy ds. Leczenia Bialaczek i Chloniaków.

Four consecutive intensive unified regimens (BFM-AML-83, PGP-AML 94, PGP-AML 98 AML-BFM 2004 Interim) for acute myelocytic leukemia (AML) have been conducted by the Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG) since 1983. The last one, introduced four years ago is still active, and only preliminary result may be presented. There were 726 children with AML diagnosed (226, 102, 247 and 151 in the I, II , III and IV periods, respectively), and 603 of them were eligible for evaluation (208, 83, 195 and 117, respectively). Complete remission rates were: 71.4%, 67.5%, 81.4% and 87% in consecutive periods, respectively. Five-year overall survival (OS) and event-free survival (EFS) rates were: 33% and 32% for PGP-AML 83 regimen, 38% and 36% for PGP-AML 94 regimen, and 53% and 46% for PGP-AML 98 regimen, respectively. For AML-BFM Interim 2004 the 3-year OS and EFS were 57% and 57%, respectively. Despite continuous improvement of the treatment results, the number of failures have remained too high, but the pattern have changed in the following way: Early deaths (from diagnosis to 15 day of treatment) decreased only in the fourth period to 3%. "Aplasia deaths" (between day 15 and 42) decreased gradually from 16% in the first period to 1.5% and 2.2% in the third and in the fourth period, respectively. Deaths in remission decreased from 10% in first and second period to 3.5% at present. Number of non responders increased between first and second period from 6% to 18%, later decreased to 8.2% at present. These trends e.g. decrease of early death and treatment related mortality reflect both the better efficacy of antileukemic treatment and the improvement of supportive care.

HLA-inferred extended haplotype disparity level is more relevant than the level of HLA mismatch alone for the patients survival and GvHD in T cell-replate hematopoietic stem cell transplantation from unrelated donor.

Serious risks in unrelated hematopoietic stem cell transplantation (HSCT) including graft versus host disease (GvHD) and mortality are associated with HLA disparity between donor and recipient. The increased risks might be dependent on disparity in not-routinely-tested multiple polymorphisms in genetically dense MHC region, being organized in combinations of two extended MHC haplotypes (Ehp). We assessed the clinical role of donor-recipient Ehp disparity levels in N = 889 patients by the population-based detection of HLA allele phase mismatch. We found increased GvHD incidences and mortality rates with increasing Ehp mismatch level even with the same HLA mismatch level. In multivariate analysis HLA mismatch levels were excluded from models and Ehp disparity level remained independent prognostic factor for high grade acute GvHD (p = 0.000037, HR = 10.68, 95%CI 5.50-32.5) and extended chronic GvHD (p < 0.000001, HR = 15.51, CI95% 5.36-44.8). In group with single HLA mismatch, patients with double Ehp disparity had worse 5-year overall survival (45% vs. 56%, p = 0.00065, HR = 4.05, CI95% 1.69-9.71) and non-relapse mortality (40% vs. 31%, p = 0.00037, HR = 5.63, CI95% 2.04-15.5) than patients with single Ehp disparity. We conclude that Ehp-linked factors contribute to the high morbidity and mortality in recipients given HLA-mismatched unrelated transplant and Ehp matching should be considered in clinical HSCT.

Fludarabine, treosulfan and etoposide sensitivity and the outcome of hematopoietic stem cell transplantation in childhood acute myeloid leukemia.

BACKGROUND: The prognostic role of the ex vivo drug resistance profile has not yet been proved in childhood acute myeloid leukemia (AML). The aim of the study was the analysis of the impact of the ex vivo drug resistance profile in a cohort of 44 children with AML undergoing hematopoietic stem cell transplantation (HSCT). PATIENTS AND METHODS: Myeloblasts for drug resistance testing were obtained from the bone marrow either on diagnosis or at relapse, before the HSCT procedure and were tested by the MTT assay. RESULTS: Children who relapsed after transplantation showed higher ex vivo resistance of the leukemic blasts to etoposide, mercaptopurine, thioguanine, fludarabine, mitoxantrone and treosulfan than those who stayed in remission. Despite being nondiscriminative, the combined ex vivo drug resistance profile to fludarabine, treosulfan and etoposide (FTE score) was the strongest prognostic factor by multivariate analysis. CONCLUSION: The combined drug resistance profile to fludarabine, treosulfan and etoposide may be useful for better stratification of children with AML undergoing stem cell transplantation or to indicate the necessity for additional post-transplant therapy.

Effect of plasma free fatty acid supply on the rate of ceramide synthesis in different muscle types in the rat.

Ceramide is a key compound in sphingolipid metabolism. Dynamics of ceramide synthesis is important in the several biological processes, such as induction of apoptosis or insulin resistance. So far, its de novo synthesis rate was evaluated indirectly, based on the content of the compound, its intermediates and the activity of respective enzymes. The aim of the present study was to directly measure ceramide synthesis rate (FSR) in different muscle types under varied plasma FFA supply in rat with the use of [U-13C] palmitate tracer and LC/MS/MS. The experiments were carried out on male Wistar rats, divided into three groups: 1-control, 2-with elevated plasma free fatty acid (FFA) concentration by means of intralipid and heparin, 3-with reduced plasma FFA concentration by means of nicotinic acid. The stable plasma FFA concentration and plasma [U-13C] palmitate enrichment was maintained for two hours by simultaneous infusion of the tracer and the respective compounds. At the end of the experiment, samples of blood from the abdominal aorta, the heart, diaphragm, soleus and white section of the gastrocnemius were taken. Muscle sphinganine, sphingosine and ceramide content and enrichment and plasma palmitate enrichment was measured with the use of LC/MS/MS. Plasma FFA concentration and composition was measured by means of gas-liquid chromatography. Under basal conditions ceramide FSR in the heart and the diaphragm was higher than in the soleus and the white gastrocnemius. Elevation in the plasma FFA concentration increased the FSR and ceramide content in each muscle, which correlated with increased HOMA-IR. The highest FSR was noted in the heart. Reduction in the plasma FFA concentration decreased ceramide FSR in each muscle type, which was accompanied by marked reduction in HOMA-IR. It is concluded that ceramide FSR depends on both the muscle type and the plasma FFA supply and is correlated with whole body insulin sensitivity under varying plasma FFA supply.

Changes in the Diaphragm Lipid Content after Administration of Streptozotocin and High-Fat Diet Regime.

The diaphragm is a dome-shaped skeletal muscle indispensable for breathing. Its activity contributes up to 70% of the total ventilatory function at rest. In comparison to other skeletal muscles, it is distinguished by an oxidative phenotype and uninterrupted cyclic contraction pattern. Surprisingly, the research regarding diaphragm diabetic phenotype particularly in the light of lipid-induced insulin resistance is virtually nonexistent. Male Wistar rats were randomly allocated into 3 groups: control, streptozotocin-induced (STZ) type-1 diabetes, and rodents fed with high-fat diet (HFD). Additionally, half of the animals from each group were administered with myriocin, a robust, selective inhibitor of ceramide synthesis and, therefore, a potent agent ameliorating insulin resistance. Diaphragm lipid contents were evaluated using chromatography. Fatty acid transporter expression was determined by Western blot. The STZ and HFD rats had increased concentration of lipids, namely, ceramides (CER) and diacylglycerols (DAG). Interestingly, this coincided with an increased concentration of long-chain (C ≥ 16) saturated fatty acid species present in both the aforementioned lipid fractions. The CER/DAG accumulation was accompanied by an elevated fatty acid transporter expression (FATP-1 in HFD and FATP-4 in STZ). Surprisingly, we observed a significantly decreased triacylglycerol content in the diaphragms of STZ-treated rats.

Reduction of ceramide de novo synthesis in solid tissues changes sphingolipid levels in rat plasma, erythrocytes and platelets.

PURPOSE: De novo sphingolipid synthesis does not occur in plasma, erythrocytes and platelets. The purpose of the study was to examine the effect of inhibition of sphingolipid synthesis in solid tissues on the level of the following bioactive sphingolipids: sphinganine, ceramide, sphingosine and sphingosine 1-phosphate in plasma, erythrocytes and platelets. MATERIAL/METHODS: The experiments were carried out on male Wistar rats. Myriocin was used to inhibit serine palmitoyltransferase activity (the enzyme catalyzes the first step of ceramide de novo synthesis) and nicotinic acid was used to reduce the concentration of plasma free fatty acids (a substrate for the de novo ceramide synthesis). The sphingolipids were quantified by means of liquid chromatography/mass spectrometry. RESULTS: Myriocin reduced the level of each compound in plasma. It reduced the level of sphinganine, sphingosine-1-phosphate and total ceramide and elevated the level of sphingosine in erythrocytes. In platelets, myriocin reduced the total level of ceramide. Nicotinic acid reduced the plasma level of sphinganine, sphingosine and total ceramide. It increased the level of sphingosine-1-phosphate in erythrocytes. In platelets, nicotinioc acid increased the level of sphinganine and sphingosine and reduced the level of sphingosine-1-phosphate and total ceramide. CONCLUSIONS: Inhibition of serine palmitoyltransferase activity in solid tissues and reduction in plasma free fatty acids concentration affects sphingolipid level in plasma, erythrocytes and platelets. The changes in erythrocytes and platelets depend both on the cell type and the sphingolipid studied and only partially follow the changes in the plasma.

[Autologous stem-cell transplantations in children with non-Hodgkin lymphomas]. / Autologiczne transplantacje komórek hematopoetycznych u dzieci z chloniakami nieziarniczymi.

From January 1st 1995 to March 31st 2003 a total of 51 autologous stem-cell transplantations (auto-HSCT) in 49 children with non Hodgkin lymphomas (NHL) were carried out in the transplantation centres of the Polish Pediatric Group for Treating Leukemias and Lymphomas (PPGLBC). In 2 patients the transplantations were carried out twice. The age of children at the moment of the transplantation ranged from 2.8 to 17.3 years (median 10.0), with higher representation of boys than girls. Twenty eight of the procedures were carried out in children with the diagnosis of B-cell non Hodgkins lymphoma (B-NHL), eight--in children with non B-cell non Hodgkins lymphoma (NB-NHL) and thirteen--in patients with anaplastic large cell lymphoma (LCAL). 16 procedures were performed in children who at the moment of transplantation were in their first complete remission (CR), another 16 were carried out while in their 2nd and 3rd CRs, and 17 transplants were performed at partial remission (PR). In addition, two children received transplants in the phase of the disease relapse. In most cases (44) the BEAM protocol was applied as megachemotherapy. In 49 procedures peripheral blood was the source of stem-cells, in one--bone marrow, in one--bone marrow + peripheral blood. The number of CD34/kg cells transplanted ranged from 1.2 x 10(6) to 8.0 x 10(6) (median 4.2 x 10(6)). Hematologic reconstitution occurred in all but one patient who died on the 10th day from HSCT. 42 out of the 49 children (87%) survived with the observation time ranging from 1 to 94 months (median 47 months). During the observation time, 34 of children were in CR. In 15, disease relapses or progression were noted within the time ranging from 3 to 22 months from HSCT (median 6 months). Seven children died (14%) including 5 due to relapse. Expected overall survival (OS) at 5 years from transplantation for the whole group of patients was 0.85 and varied only slightly for individual categories of NHL. The probability of 5-year disease-free survival (DFS) for the whole group was 0.67 and was the highest in B-NHL (0.84) and was much lower in LCAL and in NB-NHL, 0.5 and 0.47 respectively. Our results suggest that BEAM megachemotherapy with autologous transplantation in children with NHL is a safe procedure, which at the same time improves the results of standard treatment, especially in children with NHL primary resistant to chemotherapy.

[Evaluation of systolic and diastolic function of the left ventricle in children with acute lymphoblastic leukaemia before treatment]. / Ocena czynnosci skurczowej i rozkurczowej lewej komory serca u dzieci z ostra bialaczka limfoblastyczna przed rozpoczeciem leczenia.

Between 1995 and 2001 echo-cardiography was performed in 244 children (128 boys, 116 girls) with acute lymphoblastic leukaemia (ALL) before the beginning of therapy with anthracyclines (medium 5.4 days after the diagnosis). The mean age at diagnosis was 5.4 years (range 9 months to 17.7 years). 189 children (97 boys and 92 girls) were included into the standard and medium risk groups and 55 (31 boys and 24 girls) into the high risk group. 29% of ALL children had disturbances in ECG. Changes in the thickness of the intraventricular septum (%IVSTh) and left ventricular posterior wall (%LVPWTh) were statistically lower, especially in children under 7 years of age. Some children showed lowering of shortening fraction (%FS - 8.6%), ejection fraction (%EF - 10.2%) and corrected velocity of fibber-shortening (Vcfc - 25.8%). Children with decreased shortening fraction (%FS) had left ventricular posterior wall thickness (%LVPWTh) impairment. Changes in diastolic function indicate impaired relaxation and compliance of the left ventricle. Decreased peak early filling velocity (E) was found. There were also longer deceleration time (EDecT) and decreased deceleration from peak E velocity (E/Dec) and longer isovolumetric relaxation time in children in standard and medium risk groups. Shorter acceleration time (EAccT) was seen in the high risk group. Evaluation of cardiac function before anthracycline chemotherapy will allow to select patients with pre-existing cardiac impairment for whom cardioprotective treatment is absolutely necessary.

Development of treatment and clinical results in childhood acute myeloid leukemia in Poland.

BACKGROUND: Since 1983 four consecutive unified regimens: acute myeloid leukemia-Polish pediatric leukemia/lymphoma study group (AML-PPLLSG) 83, AML-PPLLSG 94, AML-PPLLSG 98 and AML-BFM 2004 Interim, for AML have been conducted by the Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG). In this paper, we review four successive studies on the basis of acute myeloid leukemia-Berlin-Frankfurt-Munster (AML-BFM) protocol, in which a stepwise improvement of treatment outcome was observed. Treatment results of the last protocol AML-BFM 2004 Interim are presented in detail. METHODS: Three hundred and three patients with de novo AML were treated according to the AML-BFM 2004 Interim at 15 Polish centers from January 1, 2005 to June 30, 2011. A confrontation with previous treatment periods was based upon historical, already published data. RESULTS: In four consecutive periods, 723 children were eligible for evaluation (208, 83, 195, and 237, respectively). Complete remission rates in consecutive periods were: 71, 68, 81 and 87 %, respectively. The 5-year overall survival rates, event-free survival rates, and relapse-free survival rates were 33, 32, and 45%, respectively for AML-PPLLSG 83 regimen; 38, 36, and 53 % respectively for AML-PPLLSG 94 regimen; 53, 46, and 65 % respectively for AML-PPLLSG 98 regimen, and 63, 52, and 64 % for AML-BFM Interim 2004, respectively. Incidence of early deaths and that due to complications (mainly infections) in the first remission decreased over time from 22 to 4.6 % and from 10 to 5.9 %, respectively. CONCLUSIONS: Despite continuous improvement in the treatment outcome, the number of failures still remains too high. Further progress seemed to be possible due to continued cooperation of oncology centers within large international study groups.

A comparison of effectiveness of fascial relaxation and classic model of patients rehabilitation after hip joint endoprosthetics.

BACKGROUND: Degenerative joint disease usually leads to functional failure and pain in the affected parts of the musculoskeletal system. The aim of this study was to evaluate the effectiveness of fascial relaxation for tense muscles in the affected hip joint by comparing this method and classic physiotherapeutic rehabilitation in patients after hip arthroplasty with regard to the range of motion, presence of pain and quality of life. MATERIAL AND METHODS: The study involved 35 females qualified for hip arthroplasty. A control group consisted of 10 patients aged 47 to 77 years who received classic kinesiotherapy, including antithrombotic prophylaxis and isometric muscle exercises for the operated lower limb followed by active exercises for supported flexion in the hip and knee joints, active decompression exercises, assuming the upright position, and gait training. The experimental group consisted of 25 women aged 45 to 75 years who, along with conventional therapeutic exercises, were treated by fascial relaxation of the affected hip joint. The arthroplasty procedure was carried out from a posterior approach in all patients, in accordance with the technique recommended by Swanson. RESULTS: The results indicate that the group of patients who additionally received fascial relaxation demonstrated a significantly increased range of motion in the operated hip joint. The experimental group also reported considerable pain reduction and improved daily activity in comparison to the control group. CONCLUSIONS: The techniques of fascial relaxation significantly reduced recovery time and liminated muscle tensing in the operated hip joint, thus contributing to improving the range of motion.

[Asymptomatic thrombo-embolic venous disease before total hip replacement--when the risk is important?]. / Bezobjawowa zylna choroba zakrzepowa przed operacja protezoplastyki stawu biodrowego--kiedy ryzyko jest istotne?

In spite of widely applied prophylaxis against thrombo-embolic venous disease its future complications still present important clinical issue. According to rough statistics this disease occurs in 71/100 000 persons per year and reveals in 1/3 as pulmonary embolism and in 2/3 as a pure thrombotic venous disease. Massive pulmonary embolism is at present a major cause of death in hospitalized subjects. In the USA mortality due to diagnosed pulmonary embolism is reported as approximately 25 000 cases per year. The aim of this paper is to draw attention to the asymptomatic cases of the thrombotic venous disease in the patients before hip arthroplasty. A retrospective clinical analysis of 210 total hip replacements (years 2005-2008) was performed. Standard perioperative routine antithrombotic prophylaxis with low-molecule heparins was introduced in each case. Special attention was paid to the occurrence of the risk factors or the presence of venous thrombosis in the lower extremities perioperatively. In the analyzed group preoperative ultrasound revealed in 5 (2.38%) cases asymptomatic venous thrombi reaching above the knee region and this was the cause of postponing of the procedure. In the early postoperative period pulmonary embolism was evidenced in 4 cases (1.9%). The authors suggested that preoperative ultrasound investigation of lower extremities venous system is important issue together with the assessment of d-dimers in the blood in all patients admitted to total hip arthroplasty. In any suspicion of the pulmonary embolism in these patients an angio-CT should be performed as an emergency.