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Children with Down syndrome (DS) are at a significantly higher risk of developing acute myeloid leukemia, also termed myeloid leukemia associated with DS (ML-DS). In contrast to the highly favorable prognosis of primary ML-DS, the limited data that are available for children who relapse or who have refractory ML-DS (r/r ML-DS) suggest a dismal prognosis. There are few clinical trials and no standardized treatment approach for this population. We conducted a retrospective analysis of international study groups and pediatric oncology centers and identified 62 patients who received treatment with curative intent for r/r ML-DS between 2000-2021. Median time from diagnosis to relapse was 6.8 (range 1.1 - 45.5) months. Three-year event-free (EFS) and overall survival (OS) were 20.9±5.3% and 22.1±5.4%, respectively. Survival was associated with receipt of hematopoietic stem cell transplantation (HSCT) (HR 0.28), duration of first complete remission (CR1) (HR 0.31 for > 12 months) and attainment of remission after relapse (HR 4.03). Patients who achieved CR prior to HSCT, had an improved OS and EFS of 56.0±11.8% and 50.5±11.9% respectively, compared to those who underwent HSCT without CR (3-year OS and EFS of 10.0±9.5%). Treatment failure after HSCT was predominantly due to disease recurrence (52%) followed by treatment related mortality (10%). The prognosis of r/r ML-DS remains dismal even in the current treatment period and serve as a reference point for current prognostication and future interventional studies. Clinical trials aimed at improving the survival of patients with r/r ML-DS are needed.

Súhrn: Bolesti brucha patria medzi pomerne časté symptómy u adolescentov. Príčiny, ktoré sa podieľajú na bolestiach brucha, sú pestré a tým aj diferenciálna dia­gnostika je pomerne rozsiahla a zložitá. To súvisí pravdepodobne s tým, že brucho zaberá pomerne veľkú oblasť, obsahuje množstvo orgánov, štruktúr, systémov vrátane lymfatických uzlín, ktoré generalizovane reagujú na infekciu, či nádorové ochorenia. V našej kazuistike opisujeme prípad 17-ročného pacienta s náhlymi bolesťami brucha, chudnutím, vracaním a ikterom. V rámci diferenciálnej dia­gnostiky sa realizovali laboratórne a zobrazovacie vyšetrenia so zameraním na pankreas a hepatobiliárny trakt, kde sa zobrazila zväčšená hlava a chvost pankreasu, peripankreatická lymfadenopatia s vedľajším nálezom drobných ložísk na obličkách. V úvode sa suponovala skôr hereditárna pankreatitída a pseudotumory na obličkách, vzhľadom na vek pacienta bol menej pravdepodobný tumor pankreasu a metastázy. Definitívna dia­gnóza bola stanovená odberom bio­ptických vzoriek pankreasu pomocou endosonografického vyšetrenia. Histologický a cytologický nález preukázal prítomnosť lymfocytárnych blastických buniek, charakteru CD20+ B-NHL, konštatovaný difúzny veľkobunkový B-lymfóm pankreasu (DLBCL – diffuse large B cell lymphoma). Staging ochorenia nepreukázal inflitráciu malígnych buniek v kostnej dreni a centrálnej nervovej sústave. Promptne stanovená dia­gnóza a podaná príslušná onkologická liečba (chemoterapia v kombinácii s monoklonálnou protilátkou) zabezpečila pacientovi návrat do každodenného života a je nevyhnutným predpokladom priaznivej prognózy pacienta. Kľúčové slová: bolesti brucha – žltačka – difúzny veľkobunkový B-lymfóm

OBJECTIVES The article focuses on main topics related to disease, death, and dying in communication between parents and their adolescent children with this diagnosis. METHODS We conducted qualitative research comprising 13 interviews with parents who lost their adolescent child to cancer. We used a semi-structured interview and interpretative phenomenological analysis. RESULTS Results introduced 6 basic topics: mutual protection, openness in the communication about cancer and death, making treatment decisions together, talks at the time of passing, hope, and spiritual experience. CONCLUSION Adolescents appreciate age-appropriate, open communication about their disease. Talking about the disease and its prognosis appears to be the way from mutual protection to open truthfulness. Openness also includes the participation of adolescents in further treatment. For some parents, it makes sense to constantly protect the child from the fact of death. Caregivers should support discussions about death between parents and their terminally ill adolescent children and accept individual decisions to talk about death (Tab. 1, Ref. 25).

Not available.

Recently, we described B-cell precursor acute lymphoblastic leukemia (BCP-ALL) subtype with an early switch to the monocytic lineage and the loss of the B-cell immunophenotype, including CD19 expression. Thus far, the genetic background has remained unknown. Among 726 children consecutively diagnosed with BCP-ALL, 8% patients experienced a switch detectable by flow cytometry (FC). Using exome and RNA sequencing, the switch was found to positively correlate with three different genetic subtypes: PAX5-P80R mutation (five cases with switch of five), rearranged (DUX4r) (30 cases of 41) and rearranged (ZNF384r) (four cases of ten). Expression profiles or phenotypic patterns correlated with genotypes, but within each genotype no cases who subsequently switched could be indentified. If switching was not taken into account, the B-cell-oriented FC assessment underestimated the minimal residual disease level. For patients with PAX5-P80R, a discordance between FC-determined and polymerase chain reactiondetermined minimal residual disease was found on day 15, resulting from a rapid loss of the B-cell phenotype. Discordance on day 33 was observed in all the DUX4r, PAX5-P80R and ZNF384r subtypes. Importantly, despite the substantial phenotypic changes, possibly even challenging the appropriateness of BCP-ALL therapy, the monocytic switch was not associated with a higher incidence of relapse and poorer prognosis in patients undergoing standard ALL treatment.

An Abelian group on which every nonzero ring is isomorphic to the ring of endomorphisms of this group is called an RE-group. In the present paper, the RE-groups are described in some classes of Abelian groups, including periodic, divisible, unreduced, and torsion-free rank-1 groups. It is shown that there are no RE-groups in the class of completely decomposable torsion-free Abelian groups.

Абелева группа, всякое ненулевое кольцо на которой изоморфно кольцу ее эндоморфизмов, называется RE-группой. В настоящей работе RE-группы описаны в классах абелевых групп, таких как: периодические, делимые, нередуцированные, без кручения ранга один. Показано, что в классе вполне разложимых абелевых групп без кручения не существует RE-групп. Библиография: 4 названия.

INTRODUCTION: Since the beginning of COVID-19 pandemic, it is known that the severe course of the disease occurs mostly among the elderly, whereas it is rare among children and young adults. Comorbidities, in particular, diabetes and hypertension, clearly associated with age, besides obesity and smoke, are strongly associated with the need for intensive treatment and a dismal outcome. A weaker immunity of the elderly has been proposed as a possible explanation of this uneven age distribution. Thus, there is concern that children treated for cancer may allso be at risk for an unfavourable course of infection. Along the same line, anecdotal information from Wuhan, China, mentioned a severe course of COVID-19 in a child treated for leukaemia. AIM AND METHODS: We made a flash survey on COVID-19 incidence and severity among children on anticancer treatment. Respondents were asked by email to fill in a short Web-based survey. RESULTS: We received reports from 25 countries, where approximately 10,000 patients at risk are followed up. At the time of the survey, more than 200 of these children were tested, nine of whom were positive for COVID-19. Eight of the nine cases had asymptomatic to mild disease, and one was just diagnosed with COVID-19. We also discuss preventive measures that are in place or should be taken and treatment options in immunocompromised children with COVID-19. CONCLUSION: Thus, even children receiving anticancer chemotherapy may have a mild or asymptomatic course of COVID-19. While we should not underestimate the risk of developing a more severe course of COVID-19 than that observed here, the intensity of preventive measures should not cause delays or obstructions in oncological treatment.

An essential qualification requirement of the teachers´ profession is to handle the conflict situations effectively focusing on cultivation healthy kinds of relationship with other participants of the educational process, keeping discipline, communicative and good teaching atmosphere. In this contribution, we compare styles used in solving the conflict situations insight teaching profession objectively. Slovak elementary school teachers (N = 22) and secondary grammar school teachers (N = 42) were ask to complete a questionnaire consisting of 25 questions with 5 types of solving the conflict situations at school (avoiding, fighting, compromising, accommodating, collaborating). The occurrence and the use of different teachers´ approaches to solving the conflict situation were compared and analyzed. The Likert scaling and the method verified by professor Northouse were used for data evaluation. It is of high importance to pay attention to the dominant style (or the absent one) of solving the conflict situations in the teacher-student, teacher-teacher or teacher-superior interactions and to explore the differences of applying them.

PURPOSE Off-label use of vemurafenib (VMF) to treat BRAFV600E mutation–positive, refractory, childhood Langerhans cell histiocytosis (LCH) was evaluated. PATIENTS AND METHODS Fifty-four patients from 12 countries took VMF 20 mg/kg/d. They were classified according to risk organ involvement: liver, spleen, and/or blood cytopenia. The main evaluation criteria were adverse events (Common Terminology Criteria for Adverse Events [version 4.3]) and therapeutic responses according to Disease Activity Score. RESULTS LCH extent was distributed as follows: 44 with positive and 10 with negative risk organ involvement. Median age at diagnosis was 0.9 years (range, 0.1 to 6.5 years). Median age at VMF initiation was 1.8 years (range, 0.18 to 14 years), with a median follow-up of 22 months (range, 4.3 to 57 months), whereas median treatment duration was 13.9 months (for 855 patient-months). At 8 weeks, 38 complete responses and 16 partial responses had been achieved, with the median Disease Activity Score decreasing from 7 at diagnosis to 0 ( P < .001). Skin rash, the most frequent adverse event, affected 74% of patients. No secondary skin cancer was observed. Therapeutic plasma VMF concentrations (range, 10 to 20 mg/L) seemed to be safe and effective. VMF discontinuation for 30 patients led to 24 LCH reactivations. The blood BRAFV600E allele load, assessed as circulating cell-free DNA, decreased after starting VMF but remained positive (median, 3.6% at diagnosis, and 1.6% during VMF treatment; P < .001) and was associated with a higher risk of reactivation at VMF discontinuation. None of the various empirical therapies (hematopoietic stem-cell transplantation, cladribine and cytarabine, anti-MEK agent, vinblastine, etc) used for maintenance could eradicate the BRAFV600E clone. CONCLUSION VMF seemed safe and effective in children with refractory BRAFV600E-positive LCH. Additional studies are needed to find effective maintenance therapy approaches.

INTRODUCTION: Central nervous system germ cell tumors (CNS GCTs) are very heterogenous group of tumors, which are divided into two main subgroups - germinomas and non- germinomatous germ cell tumors (NGGCTs). NGGCTs consist of embryonal carcinomas, yolk sac tumors, choriocarcinomas, and teratomas - mature, immature, or with malignant transformation, as well as mixed types. CNS GCTs are rare tumors, accounting approximately for 4-5% of all brain tumors in children, with a peak of incidence between the ages of 10 and 15 years, affecting predominantly boys. METHODS: Retrospective study of a cohort of 34 patients, age 0-18 years old, with CNS germ cell tumors diagnosed and treated in one of three Slovak pediatric oncology centers over a 21-year period from 01/2000 to 12/2020. Treatment was done according to the SIOP strategy. RESULTS: Out of 34 patients 15 patients were treated in the largest center, in Bratislava and 19 patients in the remaining 2 centers. Germinomas occurred in 16 (47.1%) and non-germinomatous germ cell tumors in 18 (52.9%) patients, 7 of whom had teratomas. In our cohort, 24 patients (70.6%) survived with an average follow-up age of 8.5 years. Twenty two patients remain in the 1st remission of the disease, 2 patients got through after disease recurrence. Ten patients (29.4%) died, 9 patients due to disease progression and 1 patient due to treatment complications. CONCLUSION: The overall survival of children with CNS GCT is most dependent on the histologic subtype of the tumor. Overall survival in children with germinomas reaches 90% and with NGGCTs around 60-70%, depending on the extent of the disease. In our cohort, we have achieved 70.6% 5 year overall survival of patients.

Pleuropulmonary blastoma (PPB) is a rare cancer occurring mainly during early childhood and often associated with germline DICER1 mutations. It is classified by the macroscopic appearance into three interrelated clinico-pathologic entities on a developmental continuum. Complete tumor resection is a main prognostic factor and can be performed at diagnosis or after neoadjuvant treatment that includes chemotherapy and in some cases radiotherapy. Optimal modalities of neo- or adjuvant treatments can be challenging taking into account potential long-term toxicities in this young population. This paper presents the recommendations for diagnosis and treatment of children and adolescents with PPB elaborated by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) within the European Union-funded project PARTNER (Paediatric Rare Tumours Network - European Registry).

Pancreatoblastoma (PBL) is a rare malignant epithelial neoplasm affecting typically young children. Signs related to advanced upper-abdominal tumor accompanied by elevated serum α-fetoprotein levels in a young child suggest PBL, however histopathological examination is required for diagnosis. The mainstay of treatment is a complete surgical resection. Inoperable and/or metastatic PBL may become amenable to complete, delayed surgery after neo-adjuvant chemotherapy. This manuscript presents the internationally consensus recommendations for the diagnosis and treatment of children with PBL, established by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) within the EU-funded PARTNER (Paediatric Rare Tumors Network – European Registry) project.

Nowadays, the Internet has a serious impact on the human psyche, and this problem is only gaining momentum. During the COVID-19 pandemic, as part of a forced necessity, the whole world moved to the global information space. There are both pros and cons to such absolute digitalization. We, as psychologists, will be interested in the other side of the coin. On the basis of this, we conducted an empirical study dedicated to the study of the psychological characteristics of the personality of students with varying degrees of severity of Internet addiction. This article will present the results of a study involving 250 students from various universities in Russia. In the course of empirical research, we used the following methods: an author's questionnaire to determine the amount and quality of time spent by respondents on the Internet;Diagnostics of the tendency to 13 types of addictions (G.V. Lozovaya);Test Self-assessment of mental states (G.Yu. Eysenck);Methodology Diagnostics of typologies of psychological defense (R. Plutchikas adapted by L.I. Wasserman, O.F. Eryshev, E.B. Klubova, etc.);Methodology for «Determining stress resistance and social adaptation of Holmes and Rage;Kimberly Young «Internet Addiction Test. The results obtained in the course of the research can be used by practicing psychologists, psychotherapists, clinical psychologists when working with addicts and co-addicts. The presented results can be applied in the practice of the educational process as prevention of Internet addiction. © The Authors, published by EDP Sciences, 2021.

The article is devoted to the problem of systemic value orientations of the personality of girls who are officially and informally married. In the modern world, the values of the family are very strongly influenced by negative trends, non-observance of traditions, unstable marriages, and the collapse of moral norms. Creating a family and fostering values for modern society is a difficult task. The purpose of the study: to identify the value orientations of young girls who are in an informal and official marriage. The paper consistently solves the following problems: the concept of value orientations is considered; the position of the family in the system of value orientations of girls who are in an informal and official marriage is revealed; domestic and foreign approaches to the study of value orientations are compared. Research methods: theoretical and empirical methods were used to solve the tasks set. As a result of the conducted empirical research, the formulated hypothesis is proved, the following tasks are solved: the value orientations of girls who are in an informal and official marriage are compared; the meaning of girls ' lives is investigated; the degree of satisfaction with marriage is revealed.

Background Neuroblastoma is the most common extracranial solid tumour of childhood with extremely heterogeneous bio-logical and clinical behaviour. Despite advances in its treatment, the long-term prognosis of patients with a high-risk and relapsed neuroblastoma remains poor. The implementation of immunotherapy into the treatment protocols has the potential to improve it. Dinutuximab, a chimeric monoclonal antibody, leads to the apoptosis of tumour cells through binding to the GD2 receptor. The article aim is to present the first experience of our centre with dinutuximab treatment. Patients and methods In 2018-2019, we administered 31 cycles of dinutuximab to seven patients. Five patients with high-risk neuroblastoma received dinutuximab in the first line, in two patients with relapse, dinutuximab was administered in the second line of treatment. To evaluate the toxicity of the treatment, the nursing records of patients during immunotherapy were retrospectively analysed. Results Two patients treated with dinutuximab in the first line are in complete remission, three patients achieved a partial response. Both patients with relapsed neuroblastoma were dia-gnosed with a second relapse after immunotherapy and died of disease progression. The treatment tolerance was acceptable in most patients - in six patients adverse events were managed with adequate supportive care. These were mainly symptoms of capillary leak syndrome, pain and hypersensitivity reactions. In one patient, the treatment was discontinued due to severe neurotoxicity. Conclusion Dinutuximab has a proven benefit in the eradication of the minimal residual disease in the treatment of neuroblastoma. Immunotherapy is currently the standard for first-line treatment of high-risk neuroblastoma. Its role in the treatment of relapsed neuroblastoma is a subject of several ongoing studies as well as the optimization of therapeutic regimens. Dinutuximab administration is associated with a considerable risk of severe adverse reactions, so the treatment belongs to the hands of an experienced paediatric oncology centre.

The purpose is to analyze the structure of shareholders existing in consumer cooperation and their interests. The work uses materials from a comprehensive survey of shareholders conducted in 1988–1990. Also, the authors review the existing literature in the development of cooperative values, as well as the current data on changes in the number of shareholders, their composition, and current interests. The analysis shows a constant decrease in the number of members of consumer societies, the existence of a problem in enhancing the involvement of various categories of the population, including youth, as member-shareholders. The paper proposes several measures to enhance the participation of shareholders in the activities of a consumer cooperative and to motivate the members of the served population. The authors argue that the issues of motivating membership, maintaining, and developing the interest of shareholders in the activities of consumer cooperation plan are not developed enough scientifically.

Background: Based on previous retrospective results, we investigated the association of coagulation FXIII subunit A (FXIII-A) expression pattern on survival and correlations with known prognostic factors of B-cell progenitor (BCP) childhood acute lymphoblastic leukemia (ALL) as a pilot study of the prospective multi-center BFM ALL-IC 2009 clinical trial. Methods: The study included four national centers (n = 408). Immunophenotyping by flow cytometry and cytogenetic analysis were performed by standard methods. Copy number alteration was studied in a subset of patients (n = 59). Survival rates were estimated by Kaplan-Meier analysis. Correlations between FXIII-A expression patterns and risk factors were investigated with Cox and logistic regression models. Results: Three different patterns of FXIII-A expression were observed: negative (&lt, 20%), dim (20&ndash, 79%), and bright (&ge, 80%). The FXIII-A dim expression group had significantly higher 5-year event-free survival (EFS) (93%) than the FXIII-A negative (70%) and FXIII-A bright (61%) groups. Distribution of intermediate genetic risk categories and the &ldquo, B-other&rdquo, genetic subgroup differed significantly between the FXIII-A positive and negative groups. Multivariate logistic regression confirmed independent association between the FXIII-A negative expression characteristics and the prevalence of intermediate genetic risk group. Conclusions: FXIII-A negativity is associated with dismal survival in children with BCP-ALL and is an indicator for the presence of unfavorable genetic alterations.

Background Invasive fungal infections are a life-threatening complication of cancer treatments, especially in hemato-oncological patients. Mucormycosis is the third leading cause of invasive fungal infections after Aspergillus and Candida infections. The first clinical symptoms are usually non-specific, which can lead to a late diagnosis and delayed therapy. Purpose The objective of this report is to summarize data in the literature about mucormycosis and to present a case report of a patient with acute lymphoblastic leukemia, who developed this infection at our center. Risk factors for the development of mucormycosis, clinical symptoms, radiology, laboratory results, and outcome were retrospectively evaluated. Case We describe a 6-years-old female patient with acute lymphoblastic leukemia. During the induction phase of therapy, the patient developed febrile neutropenia and did not respond to therapy with a combination of antibiotics and supportive treatment. Pansinusitis and orbitocellulitis developed. Examination of the biological material revealed that the etiological agent was a Rhizopus sp. The patient was treated with a combination of antimycotic drugs, but the infection disseminated to the central nervous system. She underwent radical surgical resection of the affected tissue. At this time, she is still under treatment with antimycotic and oncology agents, but is in remission of the main diagnosis and in good clinical condition. Conclusion Mucormycosis is an invasive fungal infection with high morbidity and mortality. Early diagnosis and initiation of effective therapy using a combination of amphotericin B administration and surgery are necessary to obtain a favorable outcome. The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers.

Dynamism in culture and personal value orientations leave their mark on such a socio-cultural phenomenon as success. It is classified as one of those concepts, the life meaning of which is clearly understood by everyday consciousness, but its psychological essence is sometimes not obvious due to the multifaceted nature of its meanings and relationships, expressed in its semantics. It was proved that codependent relationships affect the value-semantic sphere of the codependent, the content characteristics of the value-semantic sphere of the codependent change. Among other things, ideas about one's own life, one's future may change, the image of the I and the image of the world, the idea of success, change. The study involved 167 participants - women in codependent relationships. Research methods: methodology "Research of ideas about successful professional activity" (O. Yu. Klochkova); methodology "Motivation for success and fear of failure" (A. A. Rean); methodology "Self-assessment of manifestations of fear of success" (G.V. Turetskaya); scale of measuring codependency in relationships (L. Spann-D. Fisher, adapted by V.D. Moskalenko). Statistical analysis and data processing were performed using the «Microsoft Excel 7.0» и «SPSS» (22.0). Our comparative analysis of the data made it possible to reveal that there are significant differences in the indicators of ideas about success among women with a high and normal level of codependency, and there is also a relationship between the level of codependency and ideas about success.

Additional file 2: Supplementary Table S2. List of primary antibodies.

In the digital age of a transitive society, interaction between people is undergoing significant changes. Many researchers talk about the virtualization of modern society, in which visual communication comes to the fore. However, despite the prevalence of the above-mentioned processes, questions remain about strategies for presenting themselves to society using social networks, and how people position themselves when publicly presenting themselves in an online environment. As part of the empirical study, the object was 30 boys and 30 girls, representatives of modern youth who actively use social networks. The age of the subjects is from 14 to 17 years. We used four test methods: the "Who am I" Method of Kuhn and McPortland; the "Scale of self-presentation tactics" by S.J. Li in the adaptation of Pikuleva O.A.; "Scale of perfectionist self-presentation" By P. Hewitt in the adaptation of Zolotareva A.A.; "Test of self-confidence" by V.G. Romek. The theoretical and practical significance of the research lies in the development of ideas about the specifics of self-presentation of modern youth who actively use social networks. The results of the study can be used in the practice of family and individual counseling of parents and adolescents, as well as in teachers and psychologists of various educational institutions.

The modern social environment in general, and educational in particular, imposes a number of requirements on the personality of the student as a future representative of the professional community. This manifests itself in the need to master professional knowledge and skills in the conditions of constantly changing requirements, planning their activities for the short and / or long term, as well as multiple flexibility of behavior. The object of the research was 100 students of various faculties of DSTU (technical and humanitarian orientation). In the course of the empirical study, we used diagnostic methods for identifying self-regulation mechanisms in the student environment "USK" by J. Rotter; a questionnaire for the diagnosis of individual stylistic features of self-regulation (V. I. Morosanova), the methodology “Research of volitional self-regulation” (A. V. Zverkova, E. V. Eydman). The practical significance of the work lies in the subsequent use of the data obtained in the framework of psychocorrectional work with students of the organization of secondary vocational education.

In any branch of the agro-industrial complex, a special role is played by the management team. The success of an agro-industrial enterprise depends largely on the level of motivation of its head, as he forms and directs the work of subordinate personnel. Despite extensive theoretical and practical research in the field of labor motivation, these studies lack an industry-specific approach that takes into account the specifics of the labor activity of managers of the agro-industrial sector of production. The object of the study was 30 managers of various experimental production farms in the Rostov region. During the empirical study, two test methods were used: “career Anchors” (E. Shane) and “Motivational profile” (S. Ritchie and P. Martin), as well as the author’s projective questionnaire. The practical significance of the work consists in scientifically-based recommendations developed based on the analysis of the results of the study. These recommendations can be taken into account by managers and employees of personnel services of enterprises in the agro-industrial area when forming systems of motivation and development of higher needs for employees who hold senior positions in modern market conditions.

Additional file 1: Supplementary Table S1. Genetic characterization of leukemia cell lines.