[Predictive value of peripheral blood lymphocyte subsets for children with intravenous immunoglobulin-resistant Kawasaki disease]. / å¤–å‘¨è¡€æ·‹å·´ç»†èƒžäºšç¾¤å¯¹é™è„‰æ³¨å°„å ç–«çƒè›‹ç™½æ— ååº”åž‹å·å´Žç— æ‚£å„¿çš„é¢„æµ‹ä»·å€¼.

OBJECTIVES: Based on peripheral blood lymphocyte subsets and common laboratory test indexes, this study aimed to construct a predictive scoring system for intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD). METHODS: Children hospitalized in Tianjin Children's Hospital from January 2021 to March 2023 were included in the study (185 cases of IVIG-sensitive KD and 41 cases of IVIG -resistant KD). Forty-six healthy children matched for age and gender were selected as controls. The relative percentage and absolute counts of peripheral lymphocyte subsets were measured by flow cytometry. Multivariate logistic regression was used to identify the predictive factors for IVIG-resistant KD and to construct a predictive scoring system for predicting IVIG-resistant KD. RESULTS: The multivariate logistic regression analysis showed that CD4+ T cell absolute count, natural killer cell absolute count, serum sodium level, globulin level, and total bilirubin level were identified as predictive factors for IVIG-resistant KD (P<0.05). The predictive scoring system based on these factors achieved a sensitivity of 70.7% and a specificity of 83.8% in predicting IVIG-resistant KD. CONCLUSIONS: Peripheral blood lymphocyte subsets can serve as predictive indicators for IVIG-resistant KD in children. The introduction of this indicator and the establishment of a scoring system based on it can provide a higher accuracy in predicting IVIG-resistant KD in children.

Purification, Structural Characterization, and Antitumor Activity of a Polysaccharide from Perilla Seeds.

A previous study found that a crude Perilla seed polysaccharide (PFSP) fraction exhibited obviously antitumor activity; however, the structural characterization and antitumor properties of this polysaccharide remain unclear. In this study, the PFSP was extracted and purified via combined column chromatography, and the structure of a single polysaccharide fraction was characterized by methylation, IC, GC-MS, NMR, and AFM. The results demonstrated that the efficient antitumor polysaccharide fraction PFSP-2-1 was screened from PFSP with a relative molecular weight of 8.81 × 106 Da. The primary structure of the PFSP main chain was →1)-Araf-(5→, →1,3)-Galp-(6→, →1)-Galp-(6→, →1,3)-Araf-(5→ and →1)-Xylp-(4→, and that of the side chains was →1)-Arap, →1,3)-Galp-(6→, →1)-Araf and →1)-Glcp-(4→, →1)-Galp-(3→ and →1)-Glcp, leading to a three-dimensional helical structure. CCK-8 experiments revealed that PFSP-2-1 significantly inhibited the growth of human hepatocellular carcinoma cells in vitro (p < 0.05), and its inhibitory effect positively correlation with the concentration of PFSP-2-1, and when the concentration of PFSP-2-1 was 1600 µg/mL, it showed the highest inhabitation rate on three hepatocellular carcinoma cells (HepG-2, Hep3b, and SK-Hep-1), for which the survival rates of HepG-2, Hep3b, and SK-Hep-1 were 53.34%, 70.33%, and 71.06%. This study clearly elucidated the structure and antitumor activity of PFSP-2-1, which lays a theoretical foundation for revealing the molecular mechanism of antitumor activity of Perilla seed polysaccharides and provides an important theoretical basis for the development of high-value Perilla.

Neutron spectrometry of D2O-moderated 252Cf with Bonner sphere spectrometer.

For neutron spectrometry of the D2O-moderated 252Cf source with a Bonner sphere spectrometer (BSS), it is difficult to use the large and heavy shadow cone to correct the neutron scattering effect. To overcome this problem, Monte Carlo (MC) simulation method was applied to calculate the neutron scattering ratio and to establish the BSS response functions. The simulated response functions were verified by experimental measurements in reference mono-energetic neutron fields. MC simulation based scattering-correction was validated by measurement of 252Cf neutron field. The measured and simulated values of the neutron scattering ratio were very close with relative errors within ±6%. Finally, the neutron spectrum and the spectrum averaged conversion coefficients of the D2O-moderated 252Cf were measured using BSS after scattering-correction by MC simulation, and the results agreed with the values recommended by ISO 8529-1:2021. It shows that the MC simulation can be a useful substitute to shadow cones method for neutron scattering-correction.

ADA2 deficiency (DADA2) misdiagnosed as systemic onset juvenile idiopathic arthritis in a child carrying a novel compound heterozygous ADA2 mutation: a case report.

Background: The deficiency of adenosine deaminase 2 (DADA2) is caused by an autosomal recessive bi-allelic loss-of-function mutation in the adenosine deaminase 2 (ADA2) gene. DADA2 is a monogenic inherited autoinflammatory disorder characterized by early-onset vasculopathy for which the symptoms range from skin lesions to very severe multiorgan involvement, including life-threatening ischemia and/or hemorrhagic strokes. Owing to the diversity of clinical presentation and the absence of suggestive features, differentiating DADA2 from other inflammatory disorders in the early stages of disease presentation is difficult. Here, we describe the case of a 3-year-old boy who had been misdiagnosed for nearly 2 years before he was definitively diagnosed with DADA2. Case Description: A previously healthy 3-year-old boy was initially diagnosed with systemic onset juvenile idiopathic arthritis (soJIA) owing to recurrent unprovoked fever and elevated acute phase reactants. He developed intractable hypertension during treatment, which his doctor considered an adverse drug reaction. Monogenic inherited autoinflammatory disorders were not suspected until the patient developed intestinal perforation and ensuing recurrent abdominal pain that coincided with fever. Gene sequence analysis revealed a novel compound heterozygous mutation in ADA2. The ADA2 enzyme activity was almost completely lost in the patient. Conclusions: The broad phenotypic spectrum of DADA2 makes early diagnosis challenging. DADA2 should be considered in case of early-onset vasculitis, which is the most common phenotype of DADA2. Early identification and treatment will result in significant improvement of the disease.

Responses of Bacterial Community Structure, Diversity, and Chemical Properties in the Rhizosphere Soil on Fruiting-Body Formation of Suillus luteus.

Mycorrhiza helper bacteria (MHB) play an important role in driving mycorrhizal formation. There are few reports on the relationship between bacteria and fruiting growths. Taking mycorrhizal rhizosphere soil from sporocarps of the S. luteus and non-mycorrhizal rhizosphere soil of the host plant (Larix gmelinii), we measured the bacterial community structure and diversity and chemical properties to clarify the effect of bacteria on fruiting-body formation. The bacterial diversity was significantly higher in mycorrhizal rhizosphere soil (p < 0.05) than that in non-mycorrhizal rhizosphere soil. The relative abundance of Burkholderia, Bradyrhizobium, Pseudomonas, and Rhizobium was significantly higher (p < 0.05) in mycorrhizal rhizosphere soil than in non-mycorrhizal rhizosphere soil. The soil organic matter (SOM), total nitrogen (TN), total phosphorus (TP), total potassium (TK), ammonium nitrogen (AN), available phosphorus (AP), available potassium (AK), and the activity of catalase, urease, and phosphatase in mycorrhizal rhizosphere soil were significantly higher (p < 0.05) than those in non-mycorrhizal rhizosphere soil. A redundancy analysis (RDA) showed that dominant bacteria are closely related to soil enzyme activity and physicochemical properties (p < 0.05). The boletus recruits a large number of bacteria around the plant roots that speed up nutrient transformation and increase the soil nutrient content, providing an important guarantee for mycelium culture and fruiting-body formation. These findings provide ideas for the nutritional supply of boletus sporocarps and lay the theoretical foundation for the efficient artificial cultivation of boletus.

The Structure and Function of Microbial Community in Rhizospheric Soil of American Ginseng (Panax quinquefolius L.) Changed with Planting Years.

The changes of microbial communities of rhizospheric soil in different ages are speculated to cause soil-borne diseases and replanting problem in American ginseng (Panax quinquefolius L.) cultivation. This study analyzed the physicochemical properties and microbial communities of rhizospheric soil during the planting of American ginseng in the Wendeng area of Weihai, China. The water content and organic matter content of American ginseng rhizospheric soil decreased year by year. A decline in the diversity of bacteria and fungi was observed in the rhizospheric soils planting American ginseng compared with the traditional crop wheat in the control group. During the later planting stage, the abundances of Proteobacteria, Actinobacteria, and Basidiomycota were lower, whereas that of Acidobacteria, Firmicutes, and Mucoromycota were higher. Through the correlation analysis between environmental factors and microbial community, it was found that the content of soil phosphorus was significantly positively correlated with the root rot pathogen Fusarium. The results of functional prediction showed that the decrease of secondary metabolite synthesis of rhizospheric soil bacteria and the increase of plant pathogenic fungi may be the important reasons for the increase of diseases in the later stage of American ginseng planting. This study revealed the evolution of rhizosphere microbial community and function in the process of American ginseng planting, which is valuable for planting management.

X-linked hyper IgM syndrome with severe eosinophilia: a case report and review of the literature.

BACKGROUND: Hyper IgM syndromes (HIGMS) are a group of rare primary immunodeficiency disorders. There are limited reports about HIGMS combined with severe eosinophilia. CASE PRESENTATION: In this report, we described a 2-year-old boy with chronic cough and symptoms of hypoxia. Lung computed tomography (CT) scan showed that diffuse ground-glass changes and eosinophils in peripheral blood increased significantly. Subsequent tests revealed a notable decrease in serum IgG and IgA. The lymphocyte subgroup classification was basically normal. Pneumocystis jirovecii were detected from the bronchoalveolar lavage fluid (BALF) of the patient by metagenomic next-generation sequencing (mNGS). After treatments of caspofungin combined with sulfamethoxazole, intravenous immunoglobulin (IVIG) replacement and anti-inflammatory steroid, the clinical symptoms and pulmonary imaging noticeably improved. The absolute eosinophil count (AEC) also returned to normal range. X-linked hyper IgM syndrome was confirmed by gene test. Two months after the diagnosis, the patient underwent allogeneic stem cell transplantation (HSCT) and has recovered well. CONCLUSIONS: Children with HIGMS are prone to opportunistic infections such as Pneumocystis jirovecii pneumonia (PJP). Diffuse interstitial lung disease and hypoglobulinemia in a young child predict the diagnosis of a primary immunodeficiency (PID). mNGS has obvious advantages for obtaining etiological diagnosis of children with PIDs. Severe eosinophilia is rarely reported in this kind of PIDs. Considering literature review and the corresponding reaction to steroid, we proposed that eosinophilia in HIGMS might be related to infections. Steroid therapy can quickly relieve eosinophilia but is easy to rebound if the reduction is too fast. Once the diagnosis of HIGMS is confirmed, the earlier the HSCT, the better the prognosis.

De novo mutation of CYBB gene in a boy presenting as intra-abdominal infection of Burkholderia contaminans: a case report.

BACKGROUND: Chronic granulomatous disease (CGD) is an inborn error of immunity. It is characterized by recurrent bacterial or fungal infections, including infections by Burkholderia species. This is due to respiratory burst dysfunction of phagocytes. Currently, there is no report on Burkholderia contaminans (B. Contaminans) infection in children with CGD. CASE PRESENTATION: We present a previously healthy, 17-month-old Chinese boy infected with B. Contaminans in the intra-abdominal regions. Immunological screening, including assessment of cellular immunity and humoral immunity did not yield conclusive results. The level of nicotinamide adenine dinucleotide phosphatase (NADPH) activity was decreased and whole-exome sequencing identified a de novo mutation in the CYBB gene. CONCLUSIONS: For specific pathogens such as B. Contaminans, immune assessment should be carried out even if there is no positive medical history or specificity in basic immunity screening.

Chronic immune thrombocytopenia in a child with X-linked agammaglobulinemia-an uncommon phenotype.

Autoimmune disorders are common in patients with primary immunodeficiency diseases (PIDs). However, the prevalence of autoimmunity is low in patients with X-linked agammaglobulinemia (XLA), mostly due to the absence of antibodies. Chronic or persistent immune thrombocytopenia (ITP), which is usually considered an antibody-mediated disease, is uncommon in patients with XLA. In this study, we detailly described a surprising autoimmune phenomenon, chronic ITP, in a small boy diagnosed with XLA. This is an interesting phenotype found in XLA, and it is helpful to understand the immune pathogenesis of autoimmunity in patients with XLA.

Spatially Confined Synthesis of SnSe Spheres Encapsulated in N, Se Dual-Doped Carbon Networks toward Fast and Durable Sodium Storage.

On account of the high theoretical capacity and preferable electrochemical reversibility, tin selenides have emerged as potential anode materials in the field of sodium ion batteries (SIBs). Unfortunately, the large volume changes, low electrical conductivity, and shuttling effect of polyselenides have impeded their real application. In this work, we present a spatially confined reaction approach for controllable fabrication of SnSe spheres, which are embedded in polydopamine (PDA)-derived N, Se dual-doped carbon networks (SnSe@NSC) through a one-step carbonization and selenization method. The NSC shell can not only buffer the volume changes during the cycling but also ensure strong coupling interaction between the SnSe core and carbon shell through Sn-C bonds, leading to excellent conductivity and structural integrity of the composite. Meanwhile, DFT theory calculations confirm that N, Se codoping in the carbon shell can endow the composite with enhanced adsorption energy and accelerated transfer ability of Na+. Consequently, the SnSe@NSC anode exhibits a high discharge capacity of 302.6 mA h g-1 over 500 cycles at 1 A g-1 and a competitive rate capability of 285.3 mA h g-1 at 10 A g-1. Additionally, a sodium ion full battery is assembled by coupling the SnSe@NSC anode with the cathode of Na3V2(PO4)3 and verified with good cycling durability (190 mA h g-1 at 1 A g-1 over 500 cycles) and high energy density (204.3 W h kg-1). Our scalable and facile design of heterostructured SnSe@NSC provides a new avenue to develop novel advanced anode materials for SIBs.

Activated PI3Kδ syndrome 1 mimicking systemic lupus erythematosus and secondary Sjögren's syndrome-like phenotype without recurrent infections: A case report.

Activated phosphoinositide 3-kinase-δ syndrome 1 (APDS1) is a combined immunodeficiency caused by a heterozygous gain-of-function mutation in PIK3CD, encoding the p110δ catalytic subunit of phosphoinositide 3-kinase δ (PI3Kδ). APDS1 is characterized by recurrent sinopulmonary infections, leading to airway damage, chronic herpes viremia, lymphoproliferation, and autoimmune and inflammatory diseases. Several cases of systemic lupus erythematosus (SLE) have been reported in APDS1; however, Sjögren's syndrome (SS) or an SS-like phenotype is rarely described in patients with APDS1. In this study, we report a 4-year-old girl with APDS1 who did not experience recurrent sinopulmonary infections and chronic viremia but presented with cytopenia, proteinuria, hypocomplementemia, and positive antinuclear antibodies that met the classification criteria for SLE. Additionally, the patient also mimicked a secondary SS-like phenotype based on recurrent parotitis and labial salivary gland biopsy. The patient achieved remission after treatment with sirolimus and immunosuppressive therapy. This case report enriches the clinical phenotype of APDS1 and provides a reference for the diagnosis and therapy of patients with APDS1.

The effects of plant-soil feedback on invasion resistance are soil context dependent.

There is growing interest in understanding the role that plant-soil feedbacks (PSFs) may play in invasion resistance. However, recent studies have shown that there is great uncertainty in explaining community patterns by PSF studies regarding invasions. This uncertainty may be partly because soils used for PSF studies are usually collected from open areas rather than natural communities, thus ignoring the effects of community contexts that may specifically influence the soil feedbacks of community residents to invaders. We performed a two-phase pot experiment to study the soil feedback initiated by ten co-occurring native and exotic species to a forest invader, Phytolacca americana, and the experiments were performed in forest soil and open area soil. The context-dependent mechanisms were further explored by studying different components of PSF. The results showed that natives and exotics had positive and negative effects on P. americana in the open area soil, respectively, but both had negative effects in the forest soil. Nutrient limitation was more important for the PSF in open area soil, whereas biotic factors were likely the primary mechanisms explaining the PSF in forest soil. Additionally, the litter-mediated allelopathy of dominant Quercus acutissima caused the strongest inhibition of the invader. These results suggest that native species can effectively resist invasion by producing negative PSF depending on the community context. Evidence that exotic species promote invasion through positive PSFs was not obtained. This study provided preliminary insights into the possibility of bridging PSF studies and community patterns.

The comprehensive changes in soil properties are continuous cropping obstacles associated with American ginseng (Panax quinquefolius) cultivation.

This study aims to verify the time-variant feature of American ginseng (AG) continuous cropping obstacles and to explore the factors impeding continuous cropping. We verified the feature with a plant-soil feedback pot experiment and then investigated the factors by comparing the properties of control soils that had not been previously used for growing ginseng (CS) with those of soils with a 10-year-crop-rotation cycle following the growth of AG (RS). It's found that the survival rate of AG in RS was lower than that in CS. The RS had lower pH, available potassium content, and urease activity. Additionally, p-coumaric, p-hydroxybenzoic, vanillic, caffeic, and cinnamic acid levels were lower in RS than in CS, but salicylic acid levels showed the opposite pattern. RS had higher Rhodanobacter and lower Acidothermus, Sphingomonas relative abundances in bacterial community. It's also found that many bacteria were substantially correlated with phenolic acids and soil physiochemical properties. Results indicate that even after 10-year crop rotation, the negative effects of prior continuous cropping of AG has not been eliminated. The growth of AG can be affected negatively with deterioration of soil physicochemical properties and with lower levels of phenolic acids which promote pathogen reproduction. Probiotics reduction also weighs. Moreover, biotic factors are interrelated with abiotic ones. Therefore, it can be inferred that the comprehensive change of soil properties is the main obstacle for continuous cropping.

Differences in Microbial Community and Metabolites in Litter Layer of Plantation and Original Korean Pine Forests in North Temperate Zone.

In order to explore the relationship between microbial diversity and metabolites in the litter layer of northern temperate forests, the microbial community structure and metabolite species in the litter layer of an original Korean pine forest and Korean pine plantation of northern temperate climate were determined on the basis of high-throughput sequencing and metabonomic techniques. The results showed that there were 698 bacterial genera and 363 fungal genera in the litter samples in the original Korean pine forest. Linear discriminant effect size (LEfSe) analysis showed that there were 35 indicator bacterial species and 19 indicator fungal species. In the litter samples of the Korean pine plantation, there were 622 bacterial genera and 343 fungal genera. Additionally, LEfSe analysis showed that there were 18 indicator bacterial species and 5 indicator fungal species. The litter of the two forest types contained 285 kinds of organic compounds, among which 16 different metabolites were screened, including 6 kinds of organic acids, 5 kinds of amino acids, 2 kinds of sugars, 2 kinds of sugar alcohols, and 1 kind of lipid. Latescibacteria, Rokubacteria, and Olpidiomycota are unique to the original Korean pine forest. They can catalyze the degradation rate of litter and decompose cellulose and chitin, respectively. Subgroup 6 was abundant in the lower litter layer. Subgroup 6 can grow with carbon compounds as substrate. It was clear that the microbial diversity of the litter layer in the original Korean pine forest was higher than that of the Korean pine plantation. Moreover, whether original forest or plantation forest, the lower-litter layer microbial diversity was higher than that in the middle-litter layer. CCA showed that the main metabolites were related to Chitinophagaceae_uncultured were saccharopine. The main metabolites associated with Mortierella and Polyscytalum were myo-inositol. At the same time, analysis of the difference between the litter layer of the original Korean pine forest and the Korean pine plantation also provides a theoretical basis for their participation in the element cycles of forest ecosystems.

Metabolite analysis of wheat dough fermentation incorporated with buckwheat.

Dough fermentation represents an important developmental stage in the manufacturing process. In this study, volatile and nonvolatile metabolite analysis were carried out to investigate time-dependent metabolic changes in the course of wheat dough fermentation incorporated with buckwheat based on gas chromatography-mass spectrometry (GC/MS). A total of 70 nonvolatile metabolites were identified, covering a broad spectrum of polar (e.g., amino acids, sugars, sugar alcohols, and acids) and nonpolar (e.g., fatty acid methyl esters, free fatty acids, and sterols) low molecular weight dough constituents. Meanwhile, sixty-four volatile metabolites comprising aldehydes, ketones, alcohols, organic acids, aromatic compounds, and furans were identified using solid-phase micro-extraction combined with GC-MS. Some differences may exist in the volatile composition between fermented and unfermented dough. Statistical assessment of the nonvolatile data via principal component analysis demonstrated that the metabolic changes during the mixed dough fermentation are reflected by time-dependent shifts of polar nonvolatile metabolites. And some potential nutritional markers, such as amino acids and sugars, could be developed to optimize and control the industrial dough fermentation incorporated with buckwheat.