Examining the Relationship between Systemic Immune-Inflammation Index and Disease Severity in Juvenile Idiopathic Arthritis.

Juvenile Idiopathic Arthritis (JIA), the leading childhood rheumatic condition, has a chronic course in which persistent disease activity leads to long-term consequences. In the era of biologic therapy and tailored treatment, precise disease activity assessment and aggressive intervention for high disease activity are crucial for improved outcomes. As inflammation is a fundamental aspect of JIA, evaluating it reflects disease severity. Recently, there has been growing interest in investigating cellular immune inflammation indices such as the neutrophil-to-lymphocyte ratio (NLR) and systemic immune inflammation index (SII) as measures of disease severity. The aim of this retrospective study was to explore the potential of the SII in reflecting both inflammation and disease severity in children with JIA. The study comprised 74 JIA patients and 50 healthy controls. The results reveal a notable increase in median SII values corresponding to disease severity, exhibiting strong correlations with traditional inflammatory markers, including CRP and ESR (ρ = 0.714, ρ = 0.661), as well as the JADAS10 score (ρ = 0.690). Multiple regression analysis revealed the SII to be independently associated with JADAS10. Furthermore, the SII accurately distinguished patients with high disease activity from other severity groups (AUC = 0.827, sensitivity 81.5%, specificity 66%). These findings suggest that integrating the SII as an additional measure holds potential for assessing disease activity in JIA.

Factors Impacting the Reduction in Neophobia Prevalence in Phenylketonuria Patients.

Food neophobia (FN), the fear of sampling new foods, can have a significant impact on children's eating habits. Children with phenylketonuria (PKU), a hereditary condition that inhibits the body's capacity to metabolize phenylalanine, should take this attitude with caution. Patients with PKU must follow a rigorous phenylalanine (Phe)-restricted diet to avoid brain malfunction that can include intellectual disability, seizures, and behavioral difficulties. The novelty of our work stems from the fact that we explored the origins of this incorrect intake pattern, which exacerbates PKU patients' already fragile health. We conducted a cross-sectional study on 34 previously diagnosed phenylketonuria patients and a control group ranging in age from 7 months to 40 years, with a sex ratio of M/F 2:1. The Food Neophobia Scale (FNS) was used to determine neophobia. We used JASP (version 0.18.1) statistical analysis to examine the relationship between neophobia and PKU condition, age and nutritional status at the time of study, diet compliance, parental educational level, period from birth to PKU diagnosis, and environmental (rural/urban) provenience of PKU patients. According to the data, 61.76% of patients with PKU were neophobic, as were 70.57% of the control group. Food neophobia was associated with PKU patients' present age, the period from birth to PKU diagnosis, and parental educational level.

The Role of Intestinal Microbiota in Celiac Disease and Further Therapeutic Perspectives.

Celiac disease (CD) is an immune-mediated enteropathy caused by exposure to gluten and related prolamins in genetically susceptible individuals. It is a complex genetic disorder with multiple contributing genes. Linkage studies have identified several genomic regions that probably contain CD susceptibility genes. The most important genetic factors are HLA-DQ2 and DQ8. Several known environmental triggers promote the onset of CD at any age after gluten introduction in individuals with a genetic background, such as viral infections and intestinal dysbiosis. Recent publications have described the interference of the intestinal microbiome in gluten metabolism, modulation of local immune reactions, and in maintaining normal gut permeability. These results have promoted further lines of research on the benefit of probiotic administration to prevent disease onset or alleviate clinical symptoms along with a gluten-free diet (GFD). The relationship between gut microbiome changes and the onset of CD is incompletely understood, still being the subject of current research. This narrative review analyzes the interplay between environmental factors, intestinal microbiome alterations, and the course of CD. Furthermore, this review sets out to discuss if modulation of intestinal microflora with pre- and probiotics along with a GFD could represent a reliable therapeutic target for celiac patients.

Assessing the Relationship between Systemic Immune-Inflammation Index and Metabolic Syndrome in Children with Obesity.

Childhood obesity represents a worldwide concern as many countries have reported an increase in its incidence, with possible cardiovascular long-term implications. The mechanism that links cardiovascular disease to obesity is related to low-grade inflammation. We designed this study to investigate the diagnostic utility of inflammatory indices (NLR, neutrophil-to-lymphocyte ratio; PLR, platelet-to-lymphocyte ratio; SII, systemic immune-inflammation index; SIRI, systemic inflammation response index) in obese children with metabolic syndrome (MetS) and their relationship with cardiometabolic risk biomarkers, such as the Homeostasis Model Assessment of Insulin Resistance (HOMA-IR), triglyceride-to-high-density lipoprotein cholesterol (TG:HDL-C), and non-high-density lipoprotein cholesterol (non-HDL-C). A total of 191 obese children from one large Romanian reference center was included in the study. Patients were classified in two groups according to the presence (MetS group) or absence (non-MetS group) of metabolic syndrome. According to our results, the SII index proved to have diagnostic value in distinguishing MetS patients among children with obesity (AUC = 0.843, a sensitivity of 0.83, and a specificity of 0.63). Furthermore, the SII was positively associated with cardiometabolic risk biomarkers (HOMA-IR, p < 0.001; TG:HDL-C, p = 0.002; non-HDL-C, p = 0.021), highlighting its possible role as an additional measure of cardiometabolic instability in obese children.

Central precocious puberty in Prader-Willi syndrome: a narrative review.

Prader-Willi syndrome (PWS, OMIM176270) is a rare genetic disorder with recognizable dysmorphic features and multisystemic consequences such as endocrine, neurocognitive and metabolic ones. Although most patients with Prader-Willi syndrome exhibit hypogonadotropic hypogonadism, there is variability regarding sexual maturation, with precocious puberty occurring in rare cases. Our aim is to elaborate a thorough review of Prader-Willi patients with central precocious puberty, in order to raise awareness of such cases and to enhance our knowledge regarding the diagnosis and prompt treatment of this particular PWS patients.

Evaluating the Diagnostic Performance of Systemic Immune-Inflammation Index in Childhood Inflammatory Arthritis: A Focus on Differentiating Juvenile Idiopathic Arthritis from Reactive Arthritis.

In pediatric care, the range of potential diagnoses for arthritis can be relatively extensive, primarily involving infectious and inflammatory causes and, to a lesser extent, oncological conditions. Specifically, when addressing inflammatory causes, differentiating between Juvenile Idiopathic Arthritis (JIA) and Reactive Arthritis (ReA) can prove to be challenging during the first weeks, owing to the lack of specific antibodies in several JIA subtypes. This single-center retrospective study of 108 children with arthritis aimed to evaluate in greater detail the complete blood count (CBC) profiles of children with JIA and ReA in greater detail. The most significant differences were noted in terms of the Systemic Immune-Inflammation Index (SII), with higher values in the JIA group. Moreover, within the JIA group, SII displayed a significant positive correlation with conventional inflammatory biomarkers, specifically C-reactive protein (ρ = 0.579) and Erythrocyte Sedimentation Rate (ρ = 0.430). It was the only independent factor associated with the presence of JIA after adjusting for age (p = 0.030). Also, even with the moderate diagnostic value, the discriminating capacity of SII was superior to those of each of its component CBC parameters according to receiver operating characteristic (ROC) analysis. In summary, this study identified elevated SII values in the JIA group compared to the ReA group, indicating the potential utility of SII as an adjuvant discriminatory marker between these two arthritis forms.

Conducting and Magnetic Hybrid Polypyrrole/Nickel Composites and Their Application in Magnetorheology.

Hybrid organic/inorganic conducting and magnetic composites of core-shell type have been prepared by in-situ coating of nickel microparticles with polypyrrole. Three series of syntheses have been made. In the first, pyrrole was oxidised with ammonium peroxydisulfate in water in the presence of various amounts of nickel and the composites contained up to 83 wt% of this metal. The second series used 0.1 M sulfuric acid as a reaction medium. Finally, the composites with polypyrrole nanotubes were prepared in water in the presence of structure-guiding methyl orange dye. The nanotubes have always been accompanied by the globular morphology. FTIR and Raman spectroscopies confirmed the formation of polypyrrole. The resistivity of composite powders of the order of tens to hundreds Ω cm was monitored as a function of pressure up to 10 MPa. The resistivity of composites slightly increased with increasing content of nickel. This apparent paradox is explained by the coating of nickel particles with polypyrrole, which prevents their contact and subsequent generation of metallic conducting pathways. Electrical properties were practically independent of the way of composite preparation or nickel content and were controlled by the polypyrrole phase. On the contrary, magnetic properties were determined exclusively by nickel content. The composites were used as a solid phase to prepare a magnetorheological fluid. The test showed better performance when compared with a different nickel system reported earlier.

Nanofiltration Composite Membranes Based on KIT-6 and Functionalized KIT-6 Nanoparticles in a Polymeric Matrix with Enhanced Performances.

The nanofiltration composite membranes were obtained by incorporation of KIT-6 ordered mesoporous silica, before and after its functionalization with amine groups, into polyphenylene-ether-ether-sulfone (PPEES) matrix. The incorporation of silica nanoparticles into PPEES polymer matrix was evidenced by FTIR and UV-VIS spectroscopy. SEM images of the membranes cross-section and their surface topology, evidenced by AFM, showed a low effect of KIT-6 silica nanoparticles loading and functionalization. The performances of the obtained membranes were appraised in permeation of Chaenomeles japonica fruit extracts and the selective separation of phenolic acids and flavonoids. The obtained results proved that the PPEES with functionalized KIT-6 nanofiltration membrane, we have prepared, is suitable for the polyphenolic compound's concentration from the natural extracts.

Basic biochemical and hematological parameters in perinatal asphyxia and their correlation with hypoxic ischemic encephalopathy.

Perinatal hypoxic-ischemic encephalopathy (HIE) represents a major cause of neonatal death or long-term disability. Inflammation plays an important role in mediating brain damage induced by neonatal hypoxic-ischemic encephalopathy. The mechanisms underlying the inflammatory response in hypoxia and ischemia are complex and are still being extensively researched. The objective of this study was to determine the predictive value of peak lactate dehydrogenase (LDH), C-reactive protein (CRP), procalcitonin (PCT) and of the evolution of leukocytes, neutrophils and lymphocytes in the first 96 h after birth for the grade of encephalopathy and neurodevelopmental outcome in newborns with HIE. In order to reveal this relationship we used comparisons between the above mention parameters. The observed hematological changes were nonspecific. The vast majority of the 78 newborns included in the study had PCT values above normal in the first 24 h, contrasting with CRP values that were positive in only 15.8% of the patients. A total of 76.9% of the patients had LDH values higher than the upper limit of normal values. The mean LDH values in patients with an unfavorable prognosis were 1,235 U/l. We can conclude that LDH is a good predictor of HIE in the first 12/24 h after birth.

Primary autoimmune neutropenia of infancy and childhood in a cohort of patients from western Romania.

Neutropenia is commonly diagnosed in pediatric clinics. Due to the special vulnerability of neutropenic patients, the assessment of the etiopathogenic background of neutropenia is mandatory. In this retrospective cross-sectional cohort study, we aimed to establish the status of primary autoimmune neutropenia (AIN) from the point of view of its clinical and biological features and its outcome in a cohort of pediatric patients. We recorded all of the 3,488 cases consecutively admitted to our hospital for different diagnoses but presenting neutropenia, during a period of 3 years (January 2016 to December 2018). We had to exclude 224 patients from the analysis due to incomplete data. Our study focused on patients with AIN or chronic benign neutropenia of infancy and childhood. In these patients, a granulocyte antibody screening by granulocyte immunofluorescence test (GIFT) and the granulocyte agglutination test (GAT) were performed. Regarding their pathogenic background, 0.1% of the patients presenting neutropenia were congenital forms, the rest being acquired forms. Primary AIN was encountered in 18 cases, representing approximately 0.5%. The median age at onset for primary AIN was 7.5 months. Male/female ratio in AIN was 1.94. In 72% of the patients with AIN, neutropenia was severe during the course of disease. In 3 patients, both GIFT and GAT were positive and in 8 patients, only GIFT was positive. For the remaining 7 patients (39%), both GIFT and GAT revealed negative results. 50% of the patients needed hospitalization, but only 3 patients presented severe infections. On-demand G-CSF was administered in 22% of the patients. Our study provides insight with regard to neutropenia, showing the high frequency and etiological diversity in childhood. Primary AIN is usually diagnosed by exclusion of the other causes of neutropenia. GIFT and GAT are useful, but rarely available diagnostic tools for the confirmation of primary AIN.

Pulsed Laser Deposition Derived Bioactive Glass-Ceramic Coatings for Enhancing the Biocompatibility of Scaffolding Materials.

The purpose of this work was to propose and evaluate a new composition for a bioactive glass-ceramic starting from the well-known 45S5 commercial product. Thus, we developed a modified version, including MgO, an oxide that turned out to induce superior mechanical properties and improved biological response. This had the following molar percentages: 46.1% SiO2, 2.6% P2O5, 16.9% CaO, 10.0% MgO, and 24.4% Na2O. The precursor alkoxides and nitrates were processed by a standard sol-gel technique, resulting in a glass-ceramic target, suitable for laser ablation experiments. Combeite (Na2Ca2Si3O9) was identified as a main crystalline phase within the calcined sol-gel powder, as well as in the case of the target sintered at 900 °C. The thin films were deposited on silicon substrates, at room temperature or 300 °C, being subsequently characterized from the material point of view, as well as in terms of bioactivity in simulated conditions and biocompatibility in relation to human fibroblast BJ cells. The investigations revealed the deposition of nanostructured glassy layers with a low proportion of crystalline domains; it was shown that a higher substrate temperature promoted the formation of surfaces with less irregularities, as a consequence of material arrangement into a shell with better morphological homogeneity. The complex elemental composition of the target was successfully transferred to the coatings, which ensured pronounced mineralization and a stimulating environment for the cell cultures. Thereby, both samples were covered with a thick layer of apatite after immersion in simulated body fluid for 28 days, and the one processed at room temperature was qualified to be the best in relation to the cells.

The immunomodulatory protein B7-H4 is overexpressed in breast and ovarian cancers and promotes epithelial cell transformation.

B7-H4 protein is expressed on the surface of a variety of immune cells and functions as a negative regulator of T cell responses. We independently identified B7-H4 (DD-O110) through a genomic effort to discover genes upregulated in tumors and here we describe a new functional role for B7-H4 protein in cancer. We show that B7-H4 mRNA and protein are overexpressed in human serous ovarian cancers and breast cancers with relatively little or no expression in normal tissues. B7-H4 protein is extensively glycosylated and displayed on the surface of tumor cells and we provide the first demonstration of a direct role for B7-H4 in promoting malignant transformation of epithelial cells. Overexpression of B7-H4 in a human ovarian cancer cell line with little endogenous B7-H4 expression increased tumor formation in SCID mice. Whereas overexpression of B7-H4 protected epithelial cells from anoikis, siRNA-mediated knockdown of B7-H4 mRNA and protein expression in a breast cancer cell line increased caspase activity and apoptosis. The restricted normal tissue distribution of B7-H4, its overexpression in a majority of breast and ovarian cancers and functional activity in transformation validate this cell surface protein as a new target for therapeutic intervention. A therapeutic antibody strategy aimed at B7-H4 could offer an exciting opportunity to inhibit the growth and progression of human ovarian and breast cancers.