105 results match your criteria: "Regional Children's Hospital[Affiliation]"

The arylbipyridine platinum (II) complex increases the level of ROS and induces lipid peroxidation in glioblastoma cells.

Biometals

October 2024

Scientific, Educational and Innovative Center of Chemical and Pharmaceutical Technologies, Ural Federal University, 620002, Yekaterinburg, Russian Federation.

Here we present the biological properties of the arylbipyridine platinum (II) complex (arylbipy-Pt) and describe the potential mechanism of its antitumor action which differs from that of the well-known cisplatin. Leading to the oxidative stress and lipid peroxidation, the arylbipyridine platinum (II) complex showcases the significant cytotoxicity against the glioblastoma cells as shown by the MTT test. Using the 5-ethyl-2-deoxyuridine we study the proliferative activity of glioblastoma cells to affirm that arylbipyridine platinum (II) complex does not impede cell division or DNA replication.

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Article Synopsis
  • Head trauma significantly affects pediatric patients, and while guidelines recommend head CT scans (CTH), the need for dedicated facial CT scans (CTF) is less defined.
  • A study involving 2117 pediatric trauma patients from 2016 to 2021 found that those receiving both CTH and CTF were typically older, experienced high-velocity injuries, and had longer hospital stays, with a notable percentage having no fractures detected.
  • Although CTF can reveal additional fractures, most do not require treatment, leading researchers to recommend its use be limited to specific cases involving facial injuries.
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Arterial hypertension is a disease that significantly increases the risk of sudden death in different age groups. It is of high scientific interest to study the relationship of arterial hypertension manifestations with different weather conditions. The article provides a review of literature data on the variability of arterial hypertension course depending on meteorological conditions as a risk factor for sudden death.

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  • Nijmegen breakage syndrome (NBS) is an autosomal recessive disorder primarily affecting Slavic populations, particularly in Ukraine, and is marked by symptoms such as microcephaly, immunodeficiency, and impaired DNA repair.
  • A study of 84 NBS patients from Ukraine revealed that about 65.5% were alive, with a median age of 11 years; the prevalence of NBS diagnoses has shown a recent increase, especially from central and southeastern regions of the country.
  • Clinical findings indicated that most patients experienced delayed physical development and infections, with malignancies (especially lymphomas) being a significant cause of death; immunological analysis showed reduced CD4+, CD19+, and immunoglobulin levels in the
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Objectives: The aim of the study was to establish an international multicenter registry to collect data on patients with Multisystem Inflammatory Syndrome in Children (MIS-C), in order to highlight a relationship between clinical presentation, age of onset and geographical distribution on the clinical outcome.

Study Design: Multicenter retrospective study involving different international societies for rare immunological disorders.1009 patients diagnosed with MIS-C between March and September 2022, from 48 centers and 22 countries were collected.

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Background: Quantitative measurement of minimal residual disease (MRD) is the "gold standard" for estimating the response to therapy in childhood B-cell precursor acute lymphoblastic leukemia (BCP-ALL). Nevertheless, the speed of the MRD response differs for different cytogenetic subgroups. Here we present results of MRD measurement in children with BCP-ALL, in terms of genetic subgroups with relation to clinically defined risk groups.

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Hemophagocytic syndrome is a key point in the pathogenesis of severe forms of multisystem inflammatory syndrome associated with COVID-19 in children (MIS-C). The factors associated with hemophagocytosis in patients with MIS-C were assessed in the present study of 94 boys and 64 girls ranging in age from 4 months to 17 years, each of whose HScore was calculated. In accordance with a previous analysis, patients with HScore ≤ 91 ( = 79) and HScore > 91 ( = 79) were compared.

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Unlabelled: This report presents the results of the assessment of MRD response by multicolor flow cytometry (MFC) with regard to the randomized use of pegylated asparaginase (PEG). In this study, PEG was randomly administered at a dose of 1000 U/m on day 3 of induction therapy in children with B-lineage ALL.

Methods: Conventional induction therapy consisted of dexamethasone, vincristine, and daunorubicin.

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Article Synopsis
  • The study focuses on Multisystem Inflammatory Syndrome in Children (MIS-C) related to COVID-19, a severe condition often requiring ICU care.
  • It analyzed 166 patients, finding that older children and those exhibiting specific symptoms like rashes, swelling, and respiratory issues were more likely to experience severe MIS-C.
  • Key predictors for severe cases included significant increases in certain blood markers (CRP, troponin, D-dimer) and physical signs such as hepatomegaly and splenomegaly, which could help identify at-risk patients early on.
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The causative agent of the new coronavirus infection SARS-CoV-2 has unique properties causing hyperinflammatory syndrome and cytokine storm, as well as widespread endotheliitis and thrombotic microangiopathy, initially detected in the lungs of adult patients who died from a severe form of the disease. Venous and arterial thrombosis in adults were identified as common causes of severe complications and deaths in new coronavirus infections. There are very few reports of thrombotic events in children with COVID-19 in the literature.

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The KMT2A recombinome of acute leukemias in 2023.

Leukemia

May 2023

DCAL/Institute of Pharm. Biology, Goethe-University, Frankfurt/Main, Germany.

Chromosomal rearrangements of the human KMT2A/MLL gene are associated with de novo as well as therapy-induced infant, pediatric, and adult acute leukemias. Here, we present the data obtained from 3401 acute leukemia patients that have been analyzed between 2003 and 2022. Genomic breakpoints within the KMT2A gene and the involved translocation partner genes (TPGs) and KMT2A-partial tandem duplications (PTDs) were determined.

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Purpose: Allogeneic hematopoietic stem cell transplantation (HSCT) is an established therapy for many inborn errors of immunity (IEI). The indications for HSCT have expanded over the last decade. The study aimed to collect and analyze the data on HSCT activity in IEI in Russia.

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  • This study investigates cases of vasculitis in children linked to COVID-19, highlighting a limited but concerning phenomenon.
  • Four pediatric cases showed diverse systemic involvement, primarily affecting the skin; while all patients presented with high inflammatory markers, none fully met criteria for multisystem inflammatory syndrome.
  • Treatment included systemic glucocorticosteroids, with additional therapies like DMARDs and biologics, underscoring the potential severity of COVID-19-related vasculitis and the need for more comprehensive research.
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Sequential monitoring of minimal residual disease (MRD) by molecular techniques or multicolor flow cytometry (MFC) has emerged over the past two decades as the primary tool to optimize treatment in pediatric B-cell precursor acute lymphoblastic leukemia (BCP-ALL). The aim of our study was to compare the prognostic power of repeated MFC-MRD measurement with single-point MRD assessment in children with BCP-ALL treated with the reduced-intensity protocol ALL-MB 2008. Data from consecutive MFC-MRD at day 15 and day 36 (end of induction, EOI) were available for 507 children with Philadelphia-negative BCP-ALL.

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  • The study analyzes infants with germline KMT2A-g B-cell precursor acute lymphoblastic leukemia (BCP-ALL) receiving treatment under the MLL-Baby protocol, highlighting outcomes and diagnostic features.
  • Out of 139 patients, 39 had KMT2A-g, who were generally older, had lower white blood cell counts, and better survival rates (6-year event-free survival of 74%).
  • The findings suggest that while moderate-intensity therapy is effective, patients showing slow response to treatment may benefit from more advanced therapies like targeted or immunotherapies.
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Hyperinsulinaemic hypoglycaemia (HH) is the most common cause of persistent hypoglycaemia in infants and children with incidence estimated at 1 per 50,000 live births. Congenital hyperinsulinism (CHI) is symptomatic mostly in early infancy and the neonatal period. Symptoms range from ones that are unspecific, such as poor feeding, lethargy, irritability, apnoea and hypothermia, to more serious symptoms, such as seizures and coma.

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Unlabelled: Antibacterial therapy in acute rhinosinusitis (ARS) is prescribed 4-9 times more often than recommended, while no >5 % of patients require such treatment. The main motive for the irrational antibiotic prescription is the presence of mucopurulent discharge and nasal congestion in combination with hyperthermia. The study objective was to determine the efficacy of hypertonic seawater solution in the technology of delayed antibiotic prescription in patients with ARS.

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Juvenile dermatomyositis (JDM) is a heterogeneous autoimmune inflammatory myositis with symmetrical proximal muscle weakness and a characteristic rash. Juvenile dermatomyositis is characterized by variable presentation and phenotypes. Detection of myositis autoantibodies is useful in improving JDM diagnosis and predicting the prognosis.

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Background:  Measurement of minimal residual disease (MRD) with multicolor flow cytometry (MFC) has become an important tool in childhood acute lymphoblastic leukemia (ALL), mainly to identify rapid responders and reduce their therapy intensity. Protocols of the Moscow-Berlin (MB) group use a comparatively low (for standard risk; SR) or moderate (for intermediate risk; ImR) treatment intensity from the onset, based on initial patient characteristics. Recently, we reported that 90% of SR patients-50% B cell precursor (BCP-ALL)-MFC-MRD negative at end of induction (EOI)-had 95% event-free survival (EFS).

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Phenylketonuria (PKU) is hyperphenylalaninemia that develops due to a deficiency of the phenylalanine hydroxylase enzyme (PAH). Identification of variants in the gene is necessary for verification of the diagnosis, choice of treatment tactics, detection of heterozygous carriers. The aim of the study was to analyze the effectiveness of identification of selected pathological variants in the gene during the newborn screening program.

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