Tipping the balance in autoimmunity: are regulatory t cells the cause, the cure, or both?
AbstractRegulatory T cells (Tregs) are a specialized subgroup of T-cell lymphocytes that is crucial for maintaining immune homeostasis and preventing excessive immune responses. Depending on their differentiation route, Tregs can be subdivided into thymically derived Tregs (tTregs) and peripherally induced Tregs (pTregs), which originate from conventional T cells after extrathymic differentiation at peripheral sites. Although the regulatory attributes of tTregs and pTregs partially overlap, their modes of action, protein expression profiles, and functional stability exhibit specific characteristics unique to each subset. O...
Source: Molecular and Cellular Pediatrics - March 20, 2024 Category: Cytology Source Type: research
Adverse effects of remdesivir for the treatment of acute COVID-19 in the pediatric population: a retrospective observational study
ConclusionsOur data suggests that RDV is safe in children, with no reported serious AEs. However, the absence of a control group limits the extent to which conclusions can be drawn. RDV may contribute to clinical improvement, particularly in high-risk patients. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - February 21, 2024 Category: Cytology Source Type: research
The role of regulatory B cells in immune regulation and childhood allergic asthma
ConclusionKnowledge about the exact function of human Bregs in allergic asthma is still very limited. This review aims to summarize the current knowledge on Bregs. We discuss different human Breg subsets, several ways of Breg induction as well as the mechanisms through which they exert immunoregulatory functions, and their role in (childhood) allergic asthma. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - January 4, 2024 Category: Cytology Source Type: research
Phagocytic cell death leads to enhanced release of pro-inflammatory S100A12 in familial Mediterranean fever
ConclusionsWe are able to demonstrate that activation threshold of neutrophils from inactive FMF patients is decreased, most likely by pre-activated pyrin. FMF neutrophils present with intrinsically higher ROS production, when cultured ex vivo. This higher baseline ROS activity leads to increased GSDMD cleavage and subsequent release of, e.g., S100A12, and to increased cell death with features of NETosis and pyroptosis. We show for the first time that cell death pathways in neutrophils of inactive FMF patients are easily triggered and lead to ROS- and GSDMD-dependent activation mechanisms and possibly pathology. This could...
Source: Molecular and Cellular Pediatrics - December 13, 2023 Category: Cytology Source Type: research
Installation of the developing nephron in the fetal human kidney during advanced pregnancy
ConclusionsThe investigation depicts that the installation is an interactive process between the stages of nephron anlage and its structural neighbors. A special meaning has the interjacent interstitium. It is vital for the positioning, shaping, and physiological integration. Due to its special location, this is mainly exposed to noxae. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - November 28, 2023 Category: Cytology Source Type: research
Gene therapy-based strategies for spinal muscular atrophy —an Asia-Pacific perspective
AbstractOnasemnogene abeparvovec has been life-changing for children with spinal muscular atrophy (SMA), signifying the potential and progress occurring in gene- and cell-based therapies for rare genetic diseases. Hence, it is important that clinicians gain knowledge and understanding in gene therapy-based treatment strategies for SMA. In this review, we describe the development and translation of onasemnogene abeparvovec from clinical trials to healthcare practice and share knowledge on the facilitators and barriers to implementation. Rapid and accurate SMA diagnosis, awareness, and education to safely deliver gene therap...
Source: Molecular and Cellular Pediatrics - November 15, 2023 Category: Cytology Source Type: research
Relevance and consequence of chronic inflammation for obesity development
AbstractBackgroundIncreasing prevalence of morbid obesity accompanied by comorbidities like type 2 diabetes mellitus (T2DM) led to a demand for improving therapeutic strategies and pharmacological intervention options. Apart from genetics, inflammation processes have been hypothesized to be of importance for the development of obesity and related aspects like insulin resistance.Main textWithin this review, we provide an overview of the intricate interplay between chronic inflammation of the adipose tissue and the hypothalamus and the development of obesity. Further understanding of this relationship might improve the under...
Source: Molecular and Cellular Pediatrics - November 14, 2023 Category: Cytology Source Type: research
B cell phenotype and serum levels of interferons, BAFF, and APRIL in multisystem inflammatory syndrome in children associated with COVID-19 (MIS-C)
ConclusionsOur work shows the involvement of humoral immunity in MIS-C and hints at parallels with the pathophysiology of SLE, with autoreactive B cells driven towards autoantibody production by elevated BAFF.Graphical Abstract (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - October 28, 2023 Category: Cytology Source Type: research
A novel serum calprotectin (MRP8/14) particle-enhanced immuno-turbidimetric assay (sCAL turbo) helps to differentiate systemic juvenile idiopathic arthritis from other diseases in routine clinical laboratory settings
ConclusionsSerum calprotectin analyses are a helpful tool supporting the diagnosis of SJIA in children with prolonged fever or inflammatory disease. Here, we show that an immuno-turbidimetric assay for detection of serum calprotectin on an automated laboratory instrument can be implemented in clinical laboratory settings to facilitate its use as a diagnostic routine test in clinical practice. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - October 25, 2023 Category: Cytology Source Type: research
What do we know about the sleep effects of caffeine used to treat apnoea of prematurity? A systematic review of the literature
Conclusions and implicationsSleep quality is an important determinant of health, and better investments in research with adequate sleep assessment tools are necessary to guarantee the ideal management of children who were born preterm. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - September 18, 2023 Category: Cytology Source Type: research
Retinopathy of prematurity: from oxygen management to molecular manipulation
AbstractIntroductionRetinopathy of prematurity (ROP) is a vasoproliferative disorder of the premature retina with the potential to progress to extraretinal neovascularisation. This review serves as an introduction to retinopathy of prematurity (ROP), outlining key parts of ROP pathophysiology, diagnosis and treatment. ROP is traditionally diagnosed by indirect ophthalmoscopy and classified using anatomical zones, stages of disease, and the presence or absence of “plus disease” (dilation and tortuosity of the major retinal arterioles and venules). ROP has a bi-phasic pathophysiology: initial hyperoxia causes reduced ret...
Source: Molecular and Cellular Pediatrics - September 15, 2023 Category: Cytology Source Type: research
Autoimmune lymphoproliferative immunodeficiencies (ALPID) in childhood: breakdown of immune homeostasis and immune dysregulation
AbstractMany inborn errors of immunity (IEI) manifest with hallmarks of both immunodeficiency and immune dysregulation due to uncontrolled immune responses and impaired immune homeostasis. A subgroup of these disorders frequently presents with autoimmunity and lymphoproliferation (ALPID phenotype). After the initial description of the genetic basis of autoimmune lymphoproliferative syndrome (ALPS) more than 20 years ago, progress in genetics has helped to identify many more genetic conditions underlying this ALPID phenotype. Among these, the majority is caused by a group of autosomal-dominant conditions including CTLA-4 ha...
Source: Molecular and Cellular Pediatrics - September 13, 2023 Category: Cytology Source Type: research
Characterization of adolescents with functional respiratory disorders and prior history of SARS-CoV-2
ConclusionsFunctional respiratory disorders are important differential diagnoses in persisting post-SARS-CoV-2 dyspnea in adolescents. A combination of clinical history, detailed examination of breathing patterns, and pulmonary function tests are helpful to correctly diagnose these conditions. Reassurance and rebreathing training are the mainstay of the therapy. The clinical course is favorable. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - September 12, 2023 Category: Cytology Source Type: research
Childhood asthma phenotypes and endotypes: a glance into the mosaic
ConclusionA better definition and comprehension of childhood asthma heterogeneity is key for improving diagnosis and treatment. This review aims at summarizing the current knowledge on this topic and discusses some limitations in their application as well as recommendations for future studies. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - August 30, 2023 Category: Cytology Source Type: research
Sex differences in long-term kidney fibrosis following neonatal nephron loss during ongoing nephrogenesis
ConclusionsBased on these observations we conclude that female sex is protective in the long-term response of the kidney to acute nephron loss under active nephrogenesis. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - August 25, 2023 Category: Cytology Source Type: research
