Introduction. The main features of bone marrow blasts cells in Burkitt lymphoma/leukemia (BL) are L3 morphology, mature immunophenotype of blasts with surface IgM expression, and presence of typical MYC gene rearrangements.The aim of the study was to show discrepancy examples in laboratory signs of BL.Patients and methods. 10 patients (8 boys and 2 girls) aged 1 to 18 years were included in the present study. The inclusion criterion was the identification of discrepancies between flow cytometric, morphological and cytogenetic data.Results. In 2 cases there were no rearrangements of the MYC gene. In 2 patients, the L2 morphological variant went against the presence of typical MYC gene rearrangements. In one case, undifferentiated blasts cells were described by morphology together with presence of surface IgM, and atypical genetics. In 8 patients, there was no expression of surface IgM. Of these, patients with absence of cytomorphological data cytometric and genetic data were controversial.Сonclusion. The cases presented in this study and the cases described in the literature demonstrate the importance of an attentive and comprehensive approach in evaluating the results of laboratory tests in the diagnosis of BL.

Introduction. Surface immunoglobulin expression is a main immunophenotypic criteria of mature subtype of B-lineage acute lymphoblastic leukemia. Although the majority of such cases represents Burkitt leukemia/lymphoma, it was shown for several times that membrane IgM could be detected in the absence of other mature lymphomas signs.The aim of the study was to evaluate heterogeneity of childhood acute lymphoblastic leukemia (ALL) with surface IgM expression and to assess correspondence of BIV EGIL ALL subtype with Burkitt lymphoma (BL) bone marrow dissemination.Materials and methods. Immunophenotypic, cytomorfologic and genetic data of 54 BIV-ALL cases were analyzed.Results. Among the studied patients 39 had BL, while others belonged to B-cell precursor ALL (BCP-ALL). All BL patients and none of BCP-ALL patients carried C-MYC rearrangement while in BCP-ALL group in 8 cases and in any BL cases KMT2A rearrangements were found. None of BCP-ALL children had L3 morphology according to FAB classification.Conclusions. B-lineage ALL with surface IgM expression is rather heterogeneous group of cases including typical BL and rare cases of BCP-ALL even with KMT2A-rearrangements. Combination of all available diagnostic technologies will allow precise split of these two different disease and select the appropriate treatment scheme.

Comparison of interpretation of acute lymphoblastic leukemia (ALL) flow cytometric diagnostics data was the aim of the study. Immunophenotyping data obtained from 10 patients with ALL were analysed separately in 26 laboratories from Russian Federation and Kazahstan. Results comparison showed four main type of discordance: B-lineage ALL diagnostics during heavy bone marrow regeneration, great variability of T-ALL interpretation, complexity of ambiguous lineage acute leukemia and, finally, very different report types, unique for each laboratory. All these problems are the serious obstacles for standardization of flow cytometric ALL diagnostics in multicenter setting. Continuation of similar QC rounds following by consecutive discussions with further development of consensus diagnostic algorithm could be the first step for standardization of ALL immunophenotyping in Russian Federation and CIS countries.

Aim of study was to evaluate functional state of central nervous system motoneurons in patients with inflammatory disseminated small lesions of the brain. Methods : 20 controls (11 boys, 9 girls, mean age 15 years) and 32 patients with ADEM (n = 17) and MS (n = 15) were enrolled. Diagnosis was established by MRI and thorough clinical investigation. Mean age of the group of children with ADEM and MS was 14 years, there were 14 boys & 18 girls. All patients underwent transcranial magnetic stimulation (TMS), single-pulse protocol. Results : TMS showed good tolerability in children with encephalomyelitis and multiple sclerosis. Central motor pathways in patients with relatively small disseminated (multiple sclerosis, ADEM) lesions demonstrated high durability and stable TMS parameters, perhaps due to neuroplasticity. In all groups MEP latencies and amplitudes were comparable with medical normative, there were no significant differences with the controls. But it have to be noted, that in children in both MS and ADEM groups elevation of MEP threshold was seen significantly more often, which may be interpreted as signs of abnormal lowered functional state of motoneurons. As non-invasive, sensitive and relatively safe method, TMS should be more often implemented into the diagnostic protocols in pediatrics.

In our review we present data on sleep disturbances in neuroinfections (encephalitis and meningitis), their clinical signs, neurochemistry and neurophysiology. These signs include lethargy, narcolepsy, sleep apnoe, sleep structure disruptions and may appear in acute period and as a sequelae of the disease. Reticular formation involvement seems to be the main reason for such sleep disorders. GABA and orexin systems disruption may play the key role in them. Neurophysiology evaluation of such disorders includes polysomnography, EEG-monitoring, brainstem acoustic evoked potentials and H-reflex investigation.

Abstract. A new role of B-cells in pathogenesis of multiple sclerosis (MS), their relations with B-cells indevelopment of inflammation and probable mechanisms of advanced therapeutic intervention in order to reach B-cell depletion are subject to discussion in present article. Here we describe a clinical case of MS with a resistant clinical course, and results of anti B-cell treatment. To attain maximal clinical effect, a novel therapeutic regimen (a combination of rituximab and mitozantrone) was applied. Clinical, radiological and laboratory methods were used to substantiate the efficiency of treatment.

THEAIM. To determine changes of aKlotho protein kidney expression, circulating levels of fibroblast growth factor 23 (FGF23) and intact parathyroid hormone (PTH) and the parameters of inorganic phosphate (Pi) exchange in experimental modeling of early stages of chronic kidney disease. MATERIAL AND METHODS. The experimental models the chronic kidney injury were 3/4 or 5/6 nephrectomy (NE) in SHR rats while sham-operated SHR rats served as control groups. The duration of experiments was 1 or 2 months. The indices of Pi urinary excretion were determined as well as renal aKlotho protein expression by immunohistochemistry, serum concentrations of FGF23 and PTH (by enzyme-linked immunosorbent assay). RESULTS. The implemented models corresponded to 1C-3C stages of chronic kidney disease. Renal excretion of Pi was significantly increased in the groups of nephrectomized animals. No significant differences were observed in the serum concentration of FGF23 and PTH between control and experimental groups. FGF23 levels were significantly higher only in model of 5/6NE in compare to control groups. In contrary, the renal expression of aKlotho protein was significantly lower in all experimental models of 3/4NE, 5/6 NE compared to the control (sham-operated SHR, 1 month). Moreover, a significant reduction of aKlotho protein was identified at the earliest stage of kidney damage among models applied that was sham-operated SHR, 2 months (vs. sham-operated SHR, 1 month). CONCLUSION. Changes in FGF23/aKlotho system preceeds development of secondary hyperparathyroidism; in early stages of chronic kidney injury the reduction aKlotho in kidney occurs earlier than the systemic increase of FGF23; increase of relative and absolute phosphate excretion in the early stages of experimental CKD is independent from aKlotho, FGF23 and PTH.

Aim. Hereditary spherocytosis (HS) is the most commonly encountered erythrocyte membranopathy. Frequency of occurrence of the disease makes one case per 2000−5000 newborns. Hereditary spherocytosis often causes a complex of clinical signs, including hemolytic crises in patients. At the same time many patients have asymptomatic HS. Differential diagnosis of HS is quite complex and in modern workload conditions the clinical doctors need a simpler diagnostics procedure. Patients and methods. Participants included 13 adults with verified hereditary spheroсytosis and 42 children with identified hereditary spherocytosis, 311 adults without hematological disorders, 42 children without hematological disorders. Verification of hereditary spherocytosis diagnosis was carried out using flow cytometry test (eosine-5 maleimid-binding), Deich`s method of determination of erythrocyte osmotic resistance and Sodium Dodecyl Sulfate-Poly Acrylamide Gel Electrophoresis. In this study we have assessed diagnostic value of hematological parameters provided by the hematological analyzer Beckman Coulter Cellular Analysis System DxH800 for identifying the degree of erythropoiesis disorder in patients with hereditary spherocytosis at the stage of reticulocytes maturation. According to our data, the ratio RET/IRF and calculated parameter MCV-MSCV can be used as the screening tests for hereditary spherocytosis. Results. Evaluation of the erythrocytes and reticulocytes parameters at the hematological analyzer identified the significant difference in estimate index MCV-MSCV (p < 0,0001, sensitivity 100%, specificity 100%, area under the ROC-curve 1,0) and RET/IRF (p < 0,0001, sensitivity 96,3%, specificity 94,1, area under the ROC-curve 0,97) between group of patients with HS and control group. We also evaluated the usability of eosine-5 maleimide binding in flow cytometry for verification of this membranopathy. For unify the test results we offer to use estimate indicator S (sample`s MFI / control`s mean MFI), cut level for positive cases of hereditary spherocytosis S < 0,84 (p = 0,0001, sensitivity 98,2%, specificity 99,2%, area under the ROC-curve 0,99). Conclusion. We recommend the hematological analyzer evaluation as the screening option for the identification of HS in patients and determine the estimated parameters for the values of the patients MCV-MSCV and RET/IRF. The most informative verifying test to prove hereditary spherocytosis is the flow cytometry test using eosine-5 maleimid. It is the laboratory test that proves a high degree of sensitivity and specificity for hereditary spherocytosis. Sodium Dodecyl Sulfate-PolyAcrylamide Gel Electrophoresis of red blood cells membranes proteins is useful for specify molecular deficiency in each hereditary spherocytosis case.

В.А. Добронравов1, Е.О. Богданова1, Н.Ю. Семенова5, О.Н. Береснева1, М.М. Парастаева1, О.В. Галкина1, И.М. Зубина1, Е.Е. Зуева3, Г.Т. Иванова1, И.Г. Каюков1, Т.Л. Коваленко3, Л.В. Котенко3, Г.М. Нутфуллина2, В.Г. Сиповский1, В.А. Цинзерлинг4, А.В. Смирнов1 ПОЧЕЧНАЯ ЭКСПРЕССИЯ БЕЛКА αKLOTHO, ФАКТОР РОСТА ФИБРОБЛАСТОВ 23 И ПАРАТИРЕОИДНЫЙ ГОРМОН ПРИ ЭКСПЕРИМЕНТАЛЬНОМ МОДЕЛИРОВАНИИ РАННИХ СТАДИЙ ХРОНИЧЕСКОГО ПОВРЕЖДЕНИЯ ПОЧЕК

Treatment results in children with acute lymphoblastic leukemia (ALL) treating in St.-Petersburg hospitals according to two modified version of German protocol CO ALL-92 from 01.01.1993 to 01.01.2007 are presented. 438 primary A LL patients aged from 4 months to 17 year s have been included in the study . ALL diagnosed according to international criteria. Based on prognostic factors patients were a llocated to one of two risk groups, which determined therapy intensity . The total treatment duration in both groups w as 2 years and consist ed of 5.5–8 months intensive phase with subsequent maintenance therapy . A comparative treatment results analysis in children with A LL according to two modified versions of COALL-92 is presented.

The aim of the study was to reveal specificity of the anemnesis and of the immune system condition in children with primary peritinitis who had undergone a cavitory operation that failed to reveal the cause of the inflammation and to improve their health. There was some increase of the relative content of activated monocytes with the classic phenotype CD14 bright CD16 - HLA-DR + versus the total number of monocytes with a phenotype CD14 bright.

The technique of binding eosin-5 maleimid fluorescent dye with lysine-430 of first extracellular protein bulge of band 3 of erythrocytes' membranes makes it possible to detect the defects of cytoskeleton of erythrocytes as a biological foundation of pathogenesis of hereditary spherocytosis. The samples of peripheral blood from 125 adult persons and 18 children with established absence of hematologic disorders were analyzed. The samples of peripheral blood from 19 patients with verified hereditary spherocytosis were analyzed too. The method of flow cytometry was applied to register the average intensity of fluorescence of eosin-5 maleimid. The decrease of average intensity of fluorescence of eosin-5 maleimid of erythrocytes of patients with hereditary spherocytosis as compared with data from comparison groups was established in all cases.

AIM: to estimate level of fibroblast growth factor 23 (FGF23) in renal allograft recipients and to evaluate interaction between FGF23 level and some clinical laboratory factors in early and long date after cadaver renal allografting (CRA). PATIENTS AND METHODS. Research included 46 renal allograft recipients, where 21 patient’s postoperative period was less than 24 months on the research period (group 1) and 26 patients with postoperative period more than 24 months (group 2). All patients were performed laboratory research complex, renal allograft ultrasound, serum FGF23 level detection by enzyme-linked immunoelectrodiffusion essay. RESULTS. In group 1 revealed statistically significant correlations between serum FGF23 level and clinical laboratory factors. FGF23 level directly correlate with age of patients (r=0,472; P=0,031), renal replacement therapy (RRT) duration before CRA (r=0,474; P=0,030), systolic blood pressure (BPs) level (r=0,482; P=0,027), erythrocyte sedimentation rate (ESR) (r=0,753; P<0,0001), creatinine (r=0,523; P=0,015), urea (r=0,483; P=0,026), sodium (r=0,634; P=0,002), uric acid (r=0,712; P<0,0001), triglycerides (r=0,476; P=0,029), glucose (r=0494; P=0,023), serum alkaline phosphatase (r=0,506; P=0,019) and proteinuria (r=0,615; P=0,003). High levels of FGF23 were noticed in patients with lower GFR values (r=-0,493; P=0,023). FGF23 feedback with phosphor level was noticed (r=-0,439; P=0,046). In second group FGF23 authentically increased in proportion to allograft function deterioration: direct correlation with serum creatinine (r=0,430; P=0,031) and invert correlation with GFR (r=-0,542, P=0,005). Proteinuria augmentation was involved with high level of FGF23 (r=0,637, P=0,001). In second group serum phosphor directly correlated with FGF23 (r=0,413, P=0,04) (fig. 2). CONCLUSION. In early periods after CRA noticed FGF23 level decreasing in proportion to phospho-calcium homeostasis factors normalization, when in the following in proportion to nephropathy progression in transplant, phospho-calcium metabolic imbalances increase, leading to increased FGF23 synthesis. Maybe in future FGF23 will become new therapeutic target for results improvement in postoperative period.

В статье представлены результаты лечения детей с острым лимфобластным лейкозом (ОЛЛ) в Санкт-Петербурге за период с 01.01.1993 по 01.01.2007. В качестве терапевтической программы использовались две модифицированные версии немецкой программы COALL-92: протоколы PECO-92 и COALL-С-Петербург-92, основанные на применении интенсивной химиотерапии. В исследование было включено 438 первичных пациентов с ОЛЛ в возрасте до 18 лет, проживающих в Санкт-Петербурге и Ленинградской области. Диагноз острого лимфобластного лейкоза устанавливали на основании международных критериев, с последующей стратификацией пациентов на 2 группы риска. Приводится сравнительный анализ результатов лечения в соответствии с двумя версиями немецкого протокола COALL 92. Обсуждаются причины различной терапевтической эффективности протоколов PECO-92 и COALL-С-Петербург-92 и пути дальнейшей оптимизации терапии ОЛЛ у детей.

Regardless the success gained in treatment of acute lymphoblastic leukaemia, several problems still remain to be solved, such as: overcoming primary drug resistance and minimizing the amount of relapses as well as decreasing of chemotherapy toxicity without detriment to the final outcome of the treatment. Development of an optimal chemotherapeutical strategy still remains a hot issue. Objective: to evaluate an efficacy of two modifications of German protocol COALL-92 in treatment of ALL in children in St.-Petersburg. Methods: the retrospective analysis of results of treatment in patients under 18 years old with ALL was performed. The diagnosis was confirmed according to international criteria. The treatment was performed via protocols PECO-92 and COALL-St.-Petersburg-92. Results: 438 initial patients with ALL were treated in St.-Petersburg clinics during the period from 01.01.1993 to 01.01.2007. At the time of analysis the probability of event-free survival (pEFS) was 60% in group of PECO-92 protocol and 70% — in COALL group (plog-rank = 0,048), probability of relapse-free survival (рRFS) was 65 and 74% (plog-rank = 0,002), probability of overall survival was (pOS) 78 and 70%, correspondingly (plog-rank = 0,079). Conclusion: inclusion of protocol treatment in practice of St.-Petersburg hospitals resulted in significant improvement of treatment results in children with ALL. The problem of both versions of COALL protocol is high rate of postremission mortality due to high toxicity of intensive stage if chemotherapy. Key words: children, acute lymphoblastic leukemia, intensive chemotherapy. (Voprosy sovremennoi pediatrii — Current Pediatrics. 2011; 10 (3): 33–42)

Regardless the success gained in treatment of acute lymphoblastic leukaemia, several problems still remain to be solved, such as: overcoming primary drug resistance and minimizing the amount of relapses as well as decreasing of chemotherapy toxicity without detriment to the final outcome of the treatment. Development of an optimal chemotherapeutical strategy still remains a hot issue. Objective: to evaluate an efficacy of two modifications of German protocol COALL-92 in treatment of ALL in children in St.-Petersburg. Methods: the retrospective analysis of results of treatment in patients under 18 years old with ALL was performed. The diagnosis was confirmed according to international criteria. The treatment was performed via protocols PECO-92 and COALL-St.-Petersburg-92. Results: 438 initial patients with ALL were treated in St.-Petersburg clinics during the period from 01.01.1993 to 01.01.2007. At the time of analysis the probability of event-free survival (pEFS) was 60% in group of PECO-92 protocol and 70% — in COALL group (plog-rank = 0,048), probability of relapse-free survival (рRFS) was 65 and 74% (plog-rank = 0,002), probability of overall survival was (pOS) 78 and 70%, correspondingly (plog-rank = 0,079). Conclusion: inclusion of protocol treatment in practice of St.-Petersburg hospitals resulted in significant improvement of treatment results in children with ALL. The problem of both versions of COALL protocol is high rate of postremission mortality due to high toxicity of intensive stage if chemotherapy.Key words: children, acute lymphoblastic leukemia, intensive chemotherapy.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2011; 10 (3): 33–42)