Outcomes of a 12-month course of early and late rituximab BCD020 biosimilar administration in juvenile systemic lupus erythematosus: A retrospective study.

Background: Juvenile systemic lupus erythematosus (SLE) is a severe, life-threatening disease. However, the role of rituximab in managing juvenile SLE remains undefined, although early biological intervention may improve disease outcomes.

Aim: To assess the differences in the outcomes of different types of rituximab administration (early and late).

Case Report: New phenotype of late-onset Stüve-Wiedemann syndrome due to a C-terminal variant in the gene.

An early diagnosis of Stüve-Wiedemann syndrome is crucial due to its high neonatal lethality and the potential for autonomic dysfunction in children. Herein, we describe a patient with a late-onset, arthrogryposis-like phenotype form of Stüve-Wiedemann syndrome. While most cases result in neonatal complications, our patient only presented with camptodactyly, ulnar deviation of the wrist, and minor facial features at birth, resembling an arthrogryposis-like phenotype.

The Wound-Healing Effect of a Novel Fibroblasts-Impregnated Hydroxyethylcellulose Gel in a Rat Full-Thickness Burn Model: A Preclinical Study.

The objective of this study was to assess the efficacy of a cell-containing wound dressing based on fibroblasts in hydroxyethylcellulose (HEC) gel for the local treatment of deep partial-thickness and/or full-thickness skin burns in an animal model. The rats (male Wistar, n = 100) were subjected to a full-thickness thermal burn (16 cm). Radical necrectomy was performed one day after the burn.

Specific Features of Juvenile Idiopathic Arthritis Patients' Chemokine Profile: The Data of Case-Control Study Analysis.

Background: Juvenile idiopathic arthritis pathogenesis involves a large number of different immune system cells, which are both sources and targets of chemokines, that affect not only their migration but also survival, proliferation, differentiation, production of all cytokine types, degranulation, and also directly stimulating or suppressing angiogenesis. Studyingthe contribution of chemokines to this disease pathogenesis will make it possible to identify new sensitive and specific markers for its diagnosis and subsequent dynamic monitoring of treatment effectiveness. The study aimed to identify a list of the most informative diagnostic markers from a wide range of juvenile idiopathic arthritis patients' blood plasma chemokines.

IFN-I Score and Rare Genetic Variants in Children with Systemic Lupus Erythematosus.

Interferon I (IFN I) signaling hyperactivation is considered one of the most important pathogenetic mechanisms in systemic lupus erythematosus (SLE). Early manifestation and more severe SLE courses in children suggest a stronger genetic influence in childhood-onset SLE (cSLE). To evaluate IFN-I score and SLE-associated genetic variants in cSLE.

Interferon type I signature associated with skin disease in juvenile dermatomyositis.

Background: Interferon type I (IFN-I) signaling system hyperactivation plays an important role in the pathogenesis of juvenile dermatomyositis (JDM).

Aim Of The Study: To analyze IFN-I score with disease activity in patients with JDM.

Materials And Methods: Clinical manifestations laboratory data, and treatment options were analyzed in 15 children with JDM.

Expression in Gliomas Is Dependent on Cell Metabolism.

The OCT4 transcription factor is necessary to maintain cell stemness in the early stages of embryogenesis and is involved in the formation of induced pluripotent stem cells, but its role in oncogenesis is not yet entirely clear. In this work, expression was investigated in malignant gliomas. Twenty glioma cell lines and a sample of normal adult brain tissue were used.

Less correction with minimally invasive surgery for adolescent idiopathic scoliosis compared to open surgical correction.

Purpose: In this study, we investigated the relationship between the results of thoracic curve correction using minimally invasive surgeries in 35 patients and open surgical correction in 47 patients with adolescent idiopathic scoliosis.

Methods: The correlations between the Cobb's angle of the primary and postoperative curves, angle of thoracic kyphosis and lumbar lordosis, correction percentage, derotation values, estimated blood loss, duration of surgery, and period of hospitalization after surgery were assessed by calculating the mean and standard deviation. Calculation and comparison were performed using Pearson correlation.

Severe Course of COVID-19 and Long-COVID-19 in Children: Difficulties in Diagnosis.

The question of COVID-19 and long-COVID-19 course in children remains unsolved. This infection in children, which is associated with COVID-19, can vary from asymptomatic to systemic damage of various systems. Multisystem inflammatory syndrome in children, associated with SARS-CoV-2 (MIS-C), is a serious condition in children and adolescents after experiencing COVID-19.

COVID-19 Heart Lesions in Children: Clinical, Diagnostic and Immunological Changes.

In the beginning of COVID-19, the proportion of confirmed cases in the pediatric population was relatively small and there was an opinion that children often had a mild or asymptomatic course of infection. Our understanding of the immune response, diagnosis and treatment of COVID-19 is highly oriented towards the adult population. At the same time, despite the fact that COVID-19 in children usually occurs in a mild form, there is an incomplete understanding of the course as an acute infection and its subsequent manifestations such as Long-COVID-19 or Post-COVID-19, PASC in the pediatric population, correlations with comorbidities and immunological changes.

Cardiac Involvement in Children Affected by COVID-19: Clinical Features and Diagnosis.

COVID-19 () in children is usually mild. However, multiple organ disorders associated with SARS-CoV-2 () have been detected with poor respiratory symptoms. Cardiac changes are noted in 17% to 75% of cases, which are associated with diagnostic difficulties in high-risk groups for the development of complications that are associated with myocardial damage by the SARS-CoV-2 virus.

Clinical and Genetic Characteristics of Multiple Epiphyseal Dysplasia Type 4.

Multiple epiphyseal dysplasias (MED) are a clinically and genetically heterogeneous group of skeletal dysplasias with a predominant lesion in the epiphyses of tubular bones. Variants in the SLC26A2 gene cause their autosomal recessive form (rMED or MED type 4). The accumulation of data regarding the genotype−phenotype correlation can help in the diagnosis and proper management of these patients.

Simultaneous Onset of Pediatric Systemic Lupus Erythematosus in Twin Brothers: Case Report.

Unlabelled: There are hundreds of twin adult patients with systemic lupus erythematosus (SLE), but male children with SLE are rarely affected. Two monozygotic twin brothers developed SLE at the age of 11 years during 1 month. The index brother manifested with Henoch-Shonlein purpura, accompanied by ANA positivity, and later developed critical left femoral arterial stenosis with high levels of anti-dsDNA, antiphospholipid antibodies, hypocomplementemia, and Coombs-positive hemolytic anemia.

A Comparative Study of Surgical Correction of Idiopathic Scoliosis With Spinal Transpedicular Metal Structures in Children.

A comparative study of surgical correction of idiopathic thoracic scoliosis using transpedicular spinal systems in children was performed. The study showed that using the transpedicular supporting elements along the entire length of the deformation (concave and convex sides) using the VCM (vertebral column manipulation) system, the correction was significantly better ( ≤ 0.05) than for the patients for whom the screws were not installed over two or more vertebrae from the concave side of the curvature, regardless of the magnitude of the spinal deformity.

Updated Understanding of the Causes of Cancer, and a New Theoretical Perspective of Combinational Cancer Therapies, a Hypothesis.

We present an integrative understanding of cancer as a metabolic multifactorial, multistage disease. We focus on underlying genetics-environmental interactions, evidenced by telomere changes. A range of genetic and epigenetic factors, including physical agents and predisposing factors such as diet and lifestyle are included.

Addressing the need for patient-friendly medical communications: adaptation of the 2019 recommendations for the management of MPS VI and MPS IVA.

Background: Patients are the most important stakeholders in the care of any disease and have an educational need to learn about their condition and the treatment they should receive. Considering this need for patient-focused materials, we present a directed approach for mucopolysaccharidosis (MPS) VI and MPS IVA, a pair of rare, inherited diseases that affects multiple organs and parts of the body. Independent guidelines on the treatment of these diseases were recently published, providing evidence- and expertise-driven recommendations to optimize patient management.

The classification of scoliosis braces developed by SOSORT with SRS, ISPO, and POSNA and approved by ESPRM.

Purpose: Studies have shown that bracing is an effective treatment for patients with idiopathic scoliosis. According to the current classification, almost all braces fall in the thoracolumbosacral orthosis (TLSO) category. Consequently, the generalization of scientific results is either impossible or misleading.

Bacterial Cellulose-Based Nanocomposites Containing Ceria and Their Use in the Process of Stem Cell Proliferation.

A technique for the fabrication of bacterial cellulose-based films with CeO nanofiller has been developed. The structural and morphological characteristics of the materials have been studied, their thermal and mechanical properties in dry and swollen states having been determined. The preparation methodology makes it possible to obtain composites with a uniform distribution of nanoparticles.

The presence of polymorphisms in genes controlling neurotransmitter metabolism and disease prognosis in patients with prostate cancer: a possible link with schizophrenia.

Polymorphisms of neurotransmitter metabolism genes were studied in patients with prostate cancer (PC) characterized by either reduced or extended serum prostate-specific antigen doubling time (PSADT) corresponding to unfavorable and favorable disease prognosis respectively. The 'unfavorable prognosis' group (40 cases) was defined by PSADT ≤ 2 months, whereas patients in the 'favorable prognosis' group (67 cases) had PSADT ≥ 30 months. The following gene polymorphisms known to be associated with neuropsychiatric disorders were investigated: a) the STin2 VNTR in the serotonin transporter gene; b) the 30-bp VNTR in the monoamine oxidase A gene; c) the Val158Met polymorphism in the catechol-ortho-methyltransferase gene; d) the promoter region C-521T polymorphism and the 48 VNTR in the third exon of the dopamine receptor gene.

[Compensatory and adaptive mechanisms in children with congenital multiple arthrogryposis in the absence of active flexion in the elbow joint].

Unlabelled: Arthrogryposis is one of the most severe congenital orthopedic diseases, characterized by multiple joint contractures, hypoplasia or aplasia of the skeletal muscles of the extremities, as well as severe motor disorders. The use of compensatory strategies allows patients to improve the ability to self-care.

Objective: To study the compensatory strategies in children with arthrogryposis with absent of active flexion in the elbow joint and estimate their dynamic after surgical treatment.

A new family with epiphyseal chondrodysplasia type Miura.

Epiphyseal chondrodysplasia, Miura type (ECDM) is a skeletal dysplasia with tall stature and distinctive skeletal features caused by heterozygous NPR2 pathogenic variants. Only four families have been reported. We present a family with five affected individuals (mother, three sons, and daughter).

Chondrogenic differentiation followed IGFBP3 loss in human endometrial mesenchymal stem cells.

Insulin-like growth factor binding protein 3 (IGFBP3) is a multifunctional protein, able either to stimulate the cell growth or to promote apoptosis. In particular, IGFBP3 plays significant role in propagation of stress-induced senescence in human endometrium-derived mesenchymal stem cells (MESCs) (Vassilieva et al., 2020).