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Medical Herald of the South of Russia

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No 2 (2010)
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REVIEW

4-9 293
Abstract

By present time safety problems of gene therapy are widely discussed among scientists of all world. In this review results of clinical researches are summarized; explanations concerning side effects of vectors integration are given; factors that can cause genotoxicity are discussed. Approaches which can save or increase clinical efficacy of gene therapy with use as targets hematopoietic stem cells, thus significantly reduced risk of leukemia development and other side effects related to vectors including in genome, are presented.

10-17 299
Abstract

Imatinib mesylate is a potent and high selective inhibitor of Bcr-Abl tyrosine kinase, which is established now as the standard of Philadelphia chromosome positive (Ph) chronic myeloid leukemia (CML) treatment. The treatment of patients with chronic phase of CML with imatinib has resulted in high rates of hematologic and cytogenetic responses. Nevertheless, primary and acquired resistance have been observed in few CML patients. The mechanisms of resistance to imatinib and its clinical significance were discussed in this review.

ORIGINAL ARTICLES

18-25 294
Abstract

The hypoxia is the powerful stressful factor, and, presumably, should result not only in rising of erythrocytes counts for the compensatory purpose, but also in rising of other blood cells number, as consequence of mobilization in the answer stress.
In article the influence assessment of pregnancy and delivery features, acute and chronic fetal hypoxia on cord blood cell composition of the full-term newborns is shown. Increase of leucocytes count at elongation of the second period of delivery, presence of chronic prenatal hypoxia or acute hypoxia in delivery is revealed. Differences of cord blood cell composition depending on newborn gender and body weight which consist in higher leucocytes count (all subpopulations including hematopoietic stem cells) at boys and at higher body weight are shown. Differences according to gender and to body weight are independent from each other. It is shown, that boys worse adapt for delivery that is expressed in higher concentration of mobilization cytokines and higher leucocytes and hemopoietic progenitors count in cord blood. It is shown, that mobilization cytokine concentration (IL-8, G-CSF, MMP-9) in cord blood of full-term newborns statistically significantly above, than in peripheral blood of healthy donors. Thus IL-8, G-CSF, MMP-9 concentration higher at boys in comparison with girls; at normal delivery in comparison with delivery by planned cesarean sections and at acute fetal hypoxia.

26-34 293
Abstract

Cord blood cell composition is a short-lasting delivery stress consequence and is similar on adult G-CSF-mobilized peripheral blood. G-SCF realizes biological effect by number of secondary messengers, many of which are also natural effectors of stress. In this research comparative characteristics of cell composition, including lymphocytes subpopulations, CD34+ and CD133+ cells, hematopoietic progenitor cells colony-forming activity and mobilizing cytokines concentration (IL-8, MMP-2, MMP-9), between term newborns cord blood and G-SCF-mobilized peripheral blood is shown.
Mobilized cytokines concentration ratio in combination with CD34+ cells count in cord blood and mobilized peripheral blood suggests that delivery stress possibly is not single cause of cord blood features.

35-37 297
Abstract

The development of progressive myocardial dysfunction is one of the side effects of polychemotherapy. Changes in diastolic filling of the left ventricular is the earliest marker that precedes the full-scaled clinical picture of heart failure.
Purpose: Assessment of left ventricle’s diastolic function and the study of the state of lipid peroxidation processes in children with acute leukemia at the stages of polychemotherapy.
Materials and Methods: We have examined 54 patients with acute lymphoblastic leukemia at the age ranging from 3 to 17 years old.
Results: Complex transthoracic Doppler echocardiography shows the occurrence of diastolic dysfunction at the time when the disease is diagnosed and its progression after the end of polychemotherapy. The development of cardiac complications is accompanied by an increase in the generation of reactive oxygen species, accumulation of oxydated lipoproteins, which suggests the free-radical genesis of myocardial damage.
Summary: Early detection of disorder of diastolic function of the left ventricle may allow to appoint cardiotropic therapy on the initial stage and decrease the rate of complications of polychemotherapy.

38-40 266
Abstract

Purpose: To investigate the effectiveness of recombinant VII activated coagulation factor in complex therapy of children with resistant coagulopathy caused by sepsis.
Materials and Methods: 38 children from 2 months to 17 years old were investigated with resistant coagulopathy caused by sepsis and using of recombinant VII activated coagulation factor. Patients were divided into 2 groups. The first group concluded the children before 1 year old; the second group concluded the children after 1 year old.
Results: The children of first group revealed the improvement of clinical and laboratory status after two intravenous applications (2 hours between using) of recombinant VII activated coagulation factor. The single use of recombinant VII activated coagulation factor was sufficient for children of second group. There was no effect in both groups of children with sepsis and multiorgan failure.
Summary: The application of recombinant VII activated coagulation factor is effective for therapy of children with resistant coagulopathy caused by severe sepsis.

41-45 346
Abstract

Purpose: To create the algorithm of hepatitis B vaccinal prevention at children with acute lymphoblastic leukemia.
Materials and Methods: The data of 181 children with acute lymphoblastic leukemia, from which 99 patients received specific hepatitis B prevention with vaccine «Engerix B», have been investigated. The authors have assessed correlations between efficiency of offered vaccinal prevention and leuco/neutropenia degrees at patients in days of vaccinations. The analysis of hepatitis B infect level at stages of vaccination was carried out.
Results: The algorithm of hepatitis B vaccinal prevention at children with acute lymphoblastic leukemia is offered.

46-49 310
Abstract

In our days the availability of neurovisualization methods don’t solve the problems of the early diagnostics of brain tumors in childhood. The comparative study was carried out in 57 patients aged to 18 years with brain tumors. By the discriminant analysis method the risk factors were defined, the model of the individual risk of brain tumor development in childhood was constructed.

50-53 304
Abstract

Purpose: Definition of immunological criteria of purpose of rhGCSF became research aim newborns with respiratory distress syndrome, being on PPV.
Materials and Methods: At 64 newborns determined at receipt: G-CSF, FRF, sFas, IL-6, IL-8, IL-4, IL-1, TNF (IFA), subpopulational structure of lymphocytes and level of lymphocytes in early and late apoptosis (flow cytometry with colouring annexin and propidium iodide; Beckman Coulter Epics, USA).
Summary: Criteria for prescription of rhGCSF are decrease of plasma level of endogenous hr-GCSF low 1556 pg/ml and increase of lymphocytes, being in early and late apoptosis, above 9,5% and 0,56% (accordingly). Realization of randomized studies use rh-GCSF at newborns to development of neutropenic is necessary.

54-56 388
Abstract

Urgency: The main clinical symptoms of hemophilia are depended on methods therapy.
Purpose: Hemophilia clinical characteristics observed in children who was received therapy by concentrates of coagulability factors.
Materials and Methods: Analysis of 145 history cases children with hemophilia and evaluation of the basic symptom occurrence depending on methods applied was carried out. The inhibitory forms of hemophilia clinical manifestations was described.
Results: Substitute therapy efficacy by concentrates of coagulability factors was demonstrated. The latter testifies the reduction of spontaneous hemorrhage, post-hemorrhage anemia and absence of articular contraction in the clinics.

57-61 301
Abstract

Purpose: Analysis of clinical material in the group of patients with neuroblastoma for the period 2001-2009, summary of the material, assessment of treatment results.
Materials and Methods: 79 patients with neuroectodermal tumours.
Results: Patients have been analyzed depending on sex, age, stage of the disease, prognostic factors, site of the primary focus and secondary affection. We analyzed immediate and long-term results of neuroblastoma treatment with various regimes of complex treatment including administration of cytostatics on automedia of organism (autoblood, autoplasma): AHCT - autohemochemotherapy, APCT – autoplasmochemotherapy, as well as use of biotherapy (chemicals of transretinoic acid).
Summary: Most patients at the time of diagnosis are III and IV stages of cancer (85-90%); 50% of affected children under three years old. When localized stage 100% of the patients are alive with different period of observations. Child survival with III and IV stage disease should not exceed 20%. Treatment of this category of patients remained difficult challenges. Effectiveness requires further study of biotherapy.

62-66 458
Abstract

Chronic Epstein-Barr virus infection (CEBVI) is the frequent infectious cause of lymphoproliferative syndrome (LPS) in children.
Purpose: To characterize the most informative CEBVI diagnostic criteria in children according to age and disease form.
Materials and Methods: 168 children in age from 3 to 7 (116) and from 7 to 14 years old (52) with CEBVI (70) and chlamydiosis mixed CEBVI (98) were examined.
Results: Antenatal, intranatal and postnatal risk groups in all patients were exposed. LPS was detected in 100% children and characterized by combination of generalized lymphadenopathy, hepatomegaly, splenomegaly with palatine and pharyngeal tonsil hypertrophy. Except LPS, CEBVI syndromal model was included intoxication syndrome, respiratory and gastrointestinal tracts, central nervous system (CNS), cardiovascular system affection and arthralgia syndrome. CEBI in young children was characterized by frequent risk groups, palatine tonsil hypertrophy, hepatomegaly, pharyngotonsillitis and CNS affection. In patients with mixed CEBVI, high frequency of risk groups, pharyngeal tonsil hypertrophy, hepatomegaly, splenomegaly, respiratory and gastrointestinal tract, CNS pathology was detected.

Summary: Risk groups and clinical symptoms of CEBVI are the indication for ELISA and PCR examination.

67-70 309
Abstract

Purpose: To study toxicity of multi-course chemotherapy on autoplasma obtained by discreet hardware membrane
plasmopheresis performed to children with malignant bone tumours.
Materials and Methods: 20 patients at the age of 8-20 years with osteosarcoma and Ewing’s sarcoma.
Results: High-dose multi-course autoplasmochemotherapy does not violate functional activity of the main transport protein of blood plasma.
Summary: Multi-course high-dose autoplasmochemotherapy performed on plasma after its preliminary extracorporal detoxication by the method of discreet hardware membrane plasmopheresis in children with osteosarcoma and Ewing’s sarcoma doesn’t cause blockade of the body detoxication systems, contributing to normalization of the studied parameters of endogenous intoxication.

71-73 295
Abstract

The main link in the pathogenesis of ITP is an excessive elimination of platelets in venous sinuses of the spleen. It justifies the splenectomy in this pathology, which increases the immunosuppression, which is caused by earlier courses prednizolonotherapy. A violation of proteinsynthetical detoxifying and liver function plays an essential link in the pathogenesis of postsplenectomic syndrome. It exacerbates immunosuppression. The composition allows saving all the structural elements of viable lymphoid tissue in the spleen for 7 days. Delayed autotransplantation of preserved splenic tissue gives the body a full lymphoid tissue without signs of hyperplasia. Two-moment autotransplantation of preserved splenic tissue in ITP allows to cut short the phenomenon postsplenectomic immunosuppression with normalization of platelet counts in peripheral blood and to avoid disability. The developed medical complex, including splenectomy with followed by a delayed autotransplantation of splenic tissue is technically simple, requires nonexpensive equipment and can be applied in practical public health services in the treatment of children’s ITP

СЛУЧАЙ ИЗ ПРАКТИКИ

74-76 385
Abstract

Primary T-cell cutaneus lymphoma presents in 10-15% of children with malignant tumour. This disease is results of the neoplastic proliferation T-lymphocytes in skin. Diagnostics has based on the histology, immunology and immunohistochemistry methods. We describe a clinical case of three-month-old child with Primary T-cell cutaneus lymphoma of skin.

77-79 335
Abstract

In work the case of visceral leishmaniasis at the child of early age with development of a getere anemia and hepatosplenomegalia is given. Diagnostic difficulties which practical doctors have faced with non-typical for this region leishmaniasis are shown. This clinical example convincingly proves necessity of well-timed research of marrow aspirate for diagnostics leishmaniasis with visceral defeats at children of early age.

EXPERIENCE EXCHANGE

80-81 290
Abstract

Cancer of nasopharynx is a rather rare pediatric disease. That is why the results of treatment of children with this disease presented in literature are scanty. We performed treatment analysis of children with nasopharyngeal cancer observed at Pediatric Oncology Department of Rostov Cancer Research Institute. The study includes 17 patients. The paper presents stages of the disease, morphologic distribution of nasopharyngeal cancer in the studied group, results of the performed treatment with a certain scheme of chemo-radiotherapy.

82-84 290
Abstract

This paper presents the results of work of Children’s center of oncology and hematology during 18 years. There are presented results of event free survival of patients, treated in accordance with the modern high-technological protocols of the chemotherapy and using the alternative methods of the treatment. We also present our experience of the psychosocial service organization and the rehabilitation of children with the oncology diseases.

85-86 338
Abstract

The authors of the article note the high rate of pediatric disabilities in Russia and describe the experience of Russia’s first organization actively acting in the field of pediatric social care and palliation under the auspices of the Russian Orthodox Church since 2003, “Pediatric hospice”. The article describe in detail the goals and functions of the pediatric hospice, its structure, actual experience of its different branches and departments. Coordinated work of inpatient department and home care teams allows increasing the quality of life of children with disabilities.

87-89 503
Abstract

Purpose: to assess indices of peripheral blood and marrow of patients with funicular myelosis in comparison with anamnesis data in order to establish the reason of mielinopathy development to prescribe early pathogenic therapy.
Material and Methods: 25 patients aged 42-79, with verified funicular myelosis, having various possible reasons of myelinopathy development in anamnesis. The received results: the assessment of indices of peripheral blood and marrow revealed changes characteristic of В12 deficiency anaemia. Early diagnostics of В-12 deficiency anaemia and well-timed beginning of treatment have been shown to result in complete regression of clinical symptoms of funicular myelosis in all patients, whereas in cases with prolongation of the disease for more than 6 months even adequate therapy resulted only in partial regression of neurologic symptomatology.
Summary: presence of clinical sings of funicular myelosis, even at absence of changes of laboratory blood indices, requires more profound examination and more scrupulous anamnesis taking.

90-93 260
Abstract

The aim. To estimate heat capacity (C) measurements for individual dietary support of pediatric cancer patients during chemotherapy.
Material and methods. 43 cancer children are examined. Group 1: 15 children, 5 children receiving polychemotherapy, 10 children – in post-BMT period (5 of them were re-examined on clinical indications). Thus we got 21 resting heat capacity measures. Group 2 includes 14 children with cancer after completing chemotherapy. Group 3 (control group) – 14 children with gastroenterological pathology. (C) is determined by indirect calorimetry, then basal metabolic rate (BMR) is calculated by formula BMR=90%C and compared with (Cf), calculated by formula WHO (1985).
The results. In group 1 the average values of BMR and Еоф considerably differ: 986,5 and 1285,9 kcal accordingly (p<0,05). In group 2 BMR value is lower than Cf, however the difference is inconsiderable. The amount of children with decreased BMR in group 1 is considerably higher than in groups 2 and 3 – 76,2%; 42,9% and 28,6% accordingly (p<0,05). On the contrary group 3 includes more children with increased BMR, than group 1 (p<0,01). The marked decrease of heat capacity (C decrease) in group 1 is mainly associated with toxic effects of polychemotherapy and BMT on metabolic processes.
Conclusion. The findings justify the need to monitor the pediatric cancer patients during special treatment to estimate their heat capacity to work out individual programs of dietary support with further assessment of effectiveness and correction.

94-99 318
Abstract

Purpose: The research studies behavior of families having children with cancer, in a stressful situation and the role of various factors influencing reproductive strategy of this group of families.

Materials and methods. Questioning of 798 mothers from the different regions of the Russian Federation having disabled children with cancer, ages from 5 till 18 years, in the remission.
Results. Child cancer changes the system of parents’ vital values towards a pro-family orientation –family, children, health become their priorities. The analysis of average ideal (2.5), average desirable (2.6) and average expected (2.1) indicators showed high reproductive potential of respondents, which is more if compared with similar indicators in other domestic sociological researches. 40,8% of respondents proved the influence of child cancer on their family planning behavior. The article gives a detailed analysis of the factors determining these families reproduction strategy: socio-economic, socio-psychological and sociocultural, personal features of respondents, medical characteristics of the disease and the therapy methods.
Summary. The research proves that the majority of the families with cancer children consolidates to overcome the difficulties connected with cancer treatment, becomes more patriarchal and close-knit. The role of family values, paternity, motherhood, and, accordingly, the need in more children increases in this group of families. The families need psychological and medico-social support taking into account the complex of their problems.

100-103 299
Abstract

Purpose: to confirm the prevalence, identify early markers of metabolic Syndrome (MS) amongst college participants between the ages 16-18 years.
Materials and methods: 240 young men and 452 young women were observed, Body Mass Index (BMI), percentage fat composition in the organism of (OMRONBF-306 и BF-400), level of Arterial Blood Pressure (BP) (OMRONM3 Expert). We selected 30 young women with hereditary predisposition to metabolic syndrome. MS and normal body mass index BMI. For the purposes of identification of early markers of metabolic syndrome, further investigations on the blood glucose level, C-Peptide, lipidogram and volume of visceral fats using Computer Tomography (CT) was done.
Results: overwheight and obesity was found in 20.3% of participants, they all had an elevated level of fat composition. It was noted that 45.8% of the participants had an elevated level of fat composition but normal body mass index. Among this group with elevated fat composition, 98% was found to have visceral fat content 2-3 times higher than the control figures,72% had disorder of the lipid spectre.
Summary: the increasement of volume of BMI, found by CT of abdominal ares is the first objective sign of formation of MS in young people.



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ISSN 2219-8075 (Print)
ISSN 2618-7876 (Online)