Case Report: A heterozygous mutation of NLRP3 in a Chinese child with NLRP3-AID.

Background: NLR family pyrin domain containing 3 (NLRP3)-associated autoinflammatory disease (NLRP3-AID), formerly known as cryopyrin-associated periodic syndrome, is a group of AIDs comprising neonatal-onset multisystem inflammatory disorder, Muckle-Wells syndrome, and familial cold autoinflammatory syndrome. Mutations in the NLRP3 gene are considered central to its pathogenesis.

Case Report: Here, we present a Chinese infant diagnosed with severe NLRP3-AID who carried a heterozygous variant in the NLRP3 gene.

Decoupled dynamics of absolute and relative lymphocyte counts and age-polarized CD4/CD8 ratio in infants versus older adults.

Background: Significant phenotypic and functional differences in peripheral lymphocyte subsets between infants and the elderly contribute to age-related variations in disease susceptibility and clinical outcomes. However, we are unable to specifically analyze the underlying causes owing to a lack of data on lymphocyte absolute counts and functional markers from two extremes of age.

Methods: A total of 111 infants (≤ 6 months) and 111 older adults (≥ 65 years) were enrolled to assess the percentages and absolute counts of peripheral blood lymphocyte (PBL) subsets.

The YY1-ABCB7 regulatory Axis is associated with malignant progression and ferroptosis sensitivity in lung adenocarcinoma.

Aim: Ferroptosis, a newly identified form of regulated cell death, has emerged as a promising therapeutic target in cancer treatment. The study was to clarify the regulatory effects of Yin Yang 1 (YY1) on ATP Binding Cassette Subfamily B Member 7 (ABCB7) expression and its related functions in lung adenocarcinoma (LUAD), focusing on ferroptosis.

Methods: The bioinformatics analysis of ABCB7 expression in LUAD was initially conducted using public databases and subsequently validated through the examination of LUAD tissue samples.

Hepatic inflammatory myofibroblastic tumor: A case report and literature review.

Rationale: Hepatic inflammatory myofibroblastic tumor (HIMT) is a rare intermediate neoplasm, characterized by localized proliferation and low metastatic potential. Despite its benign nature, HIMT exhibits malignant features including recurrence and multifocal growth. The etiology of HIMT is multifactorial and involves infections, trauma, surgery, and radiotherapy.

Identification of cellular senescence-associated genes for predicting the diagnosis, prognosis and immunotherapy response in lung adenocarcinoma via a 113-combination machine learning framework.

Background: Lung adenocarcinoma (LUAD) is a prevalent malignant tumor of the respiratory system, with high incidence and mortality rates. Cellular senescence (CS) widely affects the tumor microenvironment (TME) and tumor growth, and is related to the invasion and immune escape of tumor cells. This study aims to develop a robust CS-related signature of LUAD.

Elastomer Mechanics of Cross-Linked Linear-Ring Polymer Blends.

Cross-linking a blend of linear and ring polymers creates a new topology-based dual-network elastomer in which the two components differ significantly in their topology. We use molecular simulations and topological analysis to examine key mechanical properties as functions of ring polymer volume fraction ϕ. For ϕ < ϕ, where the rings begin to overlap, the network shear modulus and the maximum stretch ratio λ are weakly dependent on ϕ.

Behavioral outcomes in the bystander and demonstrator male mice following a socially-transferred allodynia paradigm.

The state of mechanical allodynia can be socially transferred from one individual to another during a brief empathetic contact. Our recent research has identified inter-individual differences in behavioral adaptations among bystander (BY) mice after a brief social contact with a demonstrator mouse experiencing complete Freund's Adjuvant (CFA)-induced inflammatory pain. However, the impact of the duration of social contact on the development of socially transferred allodynia is not yet clear.

Long-term safety of fecal microbiota transplantation in Chinese children from 2013 to 2023: a single-center retrospective study.

Background: The gut microbiome plays a vital role in influencing various health conditions. Fecal Microbiota Transplantation (FMT) has emerged as a rapid, safe, and effective method for modifying the microbiome. However, there is a lack of long-term safety data regarding FMT in children.

Dorsal raphe GABA-ergic neurons regulate the susceptibility to social transfer of pain in mice.

Some individuals are more susceptible to developing or suffering from pain states than others. However, the brain mechanisms underlying the susceptibility to pain responses are unknown. In this study, we defined pain susceptibility by recapitulating inter-individual differences in pain responses in mice exposed to a paradigm of socially transferred allodynia (STA), and with a combination of chemogenetic, molecular, pharmacological and electrophysiological approaches, we identified GABA-ergic neurons in the dorsal raphe nucleus (DRN) as a cellular target for the development and maintenance of STA susceptibility.

Atomistic Simulations of Mechanical Properties of Lignin.

The mechanical properties of lignin, an aromatic heteropolymer constituting 20-30% plant biomass, are important to the fabrication and processing of lignin-based sustainable polymeric materials. In this study, atomistic simulations are performed to provide microscopic insights into the mechanics of lignin. Representative samples of miscanthus, spruce, and birch lignin are studied.

Sirolimus alleviated intractable diarrhea of IPEX syndrome: a case report and literature review.

Background: Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare single-gene X-linked immunodeficiency disease caused by mutations in the forkhead box protein 3 (FOXP3) gene. The typical clinical manifestations of IPEX mainly include severe atopic dermatitis, insulin-dependent type 1 diabetes mellitus, and intractable diarrhea.

Case Presentation: Here, we report a boy with intractable diarrhea diagnosed with early-onset IPEX syndrome due to the c.

Exploring the Updated Roles of Ferroptosis in Liver Diseases: Mechanisms, Regulators, and Therapeutic Implications.

Ferroptosis, a newly discovered mode of cell death, is a type of iron-dependent regulated cell death characterized by intracellular excessive lipid peroxidation and imbalanced redox. As the liver is susceptible to oxidative damage and the abnormal iron accumulation is a major feature of most liver diseases, studies on ferroptosis in the field of liver diseases are of great interest. Studies show that targeting the key regulators of ferroptosis can effectively alleviate or even reverse the deterioration process of liver diseases.

Measuring Topological Constraint Relaxation in Ring-Linear Polymer Blends.

Polymers are an effective test bed for studying topological constraints in condensed matter due to a wide array of synthetically available chain topologies. When linear and ring polymers are blended together, emergent rheological properties are observed as the blend can be more viscous than either of the individual components. This emergent behavior arises since ring-linear blends can form long-lived topological constraints as the linear polymers thread the ring polymers.

Diblock Rings as Topological Adhesives at Immiscible Polymer Interfaces.

Disparate polymers often do not mix well, and the resulting immiscible interfaces are mechanically weaker than the bulk, which is undesirable for many technological applications. Large-scale molecular simulations are performed to demonstrate the effectiveness of diblock ring polymers as a new type of adhesive for immiscible polymer interfaces. The peak stress σ and the failure strain γ upon shear deformation approach the bulk values with increasing diblock ring length and coverage.

[Oblique Lumbar Interbody Fusion Combined With 4-Screw Fixation for Treating Two-Level Degenerative Lumbar Diseases:A Finite Element Study].

Objective To demonstrate the feasibility of oblique lumbar interbody fusion (OLIF) combined with 4-screw fixation for treating two-level lumbar degenerative diseases.Methods An intact finite element model of L3-S1 (M0) was constructed and validated.Then,we constructed the M1 model by simulating OLIF surgery at L3/4 and L4/5 segments on the M0 model.

Immune signature and phagocytosis of circulating DC subsets in healthy adults during aging.

Dendritic cells (DC) play a pivotal role in the onset and progression of immunosenescence-associated diseases, serving as a link between innate and adaptive immunity. Thus, there is a need to establish reference ranges for DC subset levels in healthy adults and investigate the potential impact of age on DC subset levels and phagocytic activity. Single-platform multi-color flow cytometry was performed to assess the proportions of circulating conventional type 1 DC (cDC1), conventional type 2 DC (cDC2), and plasmacytoid DC (pDC), as well as the percentages of CD80, CD86, CD83, PD-L1, and CD32 in cDC1, cDC2, and pDC.

Establishment of bone-targeted nano-platform and the study of its combination with 2-deoxy-d-glucose enhanced photodynamic therapy to inhibit bone metastasis.

At present, simple anti-tumor drugs are ineffective at targeting bone tissue and are not purposed to treat patients with bone metastasis. In this study, zoledronic acid (ZOL) demonstrated excellent bone-targeting properties as a bone-targeting ligand. The metal-organic framework (MOF) known as ZIF-90 was modified with ZOL to construct a bone-targeting-based drug delivery system.

Single-cell sequencing reveals the reproductive variations between primiparous and multiparous Hu ewes.

Background: In the modern sheep production systems, the reproductive performance of ewes determines the economic profitability of farming. Revealing the genetic mechanisms underlying differences in the litter size is important for the selection and breeding of highly prolific ewes. Hu sheep, a high-quality Chinese sheep breed, is known for its high fecundity and is often used as a model to study prolificacy traits.

Identification of MARK2, CCDC71, GATA2, and KLRC3 as candidate diagnostic genes and potential therapeutic targets for repeated implantation failure with antiphospholipid syndrome by integrated bioinformatics analysis and machine learning.

Background: Antiphospholipid syndrome (APS) is a group of clinical syndromes of thrombosis or adverse pregnancy outcomes caused by antiphospholipid antibodies, which increase the incidence of fertilization failure in patients with infertility. However, the common mechanism of repeated implantation failure (RIF) with APS is unclear. This study aimed to search for potential diagnostic genes and potential therapeutic targets for RIF with APS.

Sericin suppresses high glucose-induced EMT in mouse podocytes via miR-30a-5p and its target Snai1.

The effect of sericin in high glucose (HG)-induced podocyte injury and the mechanisms involving Snai1 and miR-30a-5p were investigated. Bioinformatics and dual-luciferase reporter assay evaluated the relationship of Snai1 with miR-31a-5p. Podocyte injury mouse induced by HG were randomly divided into control (5.

Cellular senescence in liver diseases: From mechanisms to therapies.

Cellular senescence is an irreversible state of cell cycle arrest, characterized by a gradual decline in cell proliferation, differentiation, and biological functions. Cellular senescence is double-edged for that it can provoke organ repair and regeneration in physiological conditions but contribute to organ and tissue dysfunction and prime multiple chronic diseases in pathological conditions. The liver has a strong regenerative capacity, where cellular senescence and regeneration are closely involved.

Dynamics of Associative Polymers with High Density of Reversible Bonds.

An associative polymer carries many stickers that can form reversible associations. For more than 30 years, the understanding has been that reversible associations change the shape of linear viscoelastic spectra by adding a rubbery plateau in the intermediate frequency range, at which associations have not yet relaxed and thus effectively act as crosslinks. Here, we design and synthesize new classes of unentangled associative polymers carrying unprecedentedly high fractions of stickers, up to eight per Kuhn segment, that can form strong pairwise hydrogen bonding of ∼20k_{B}T without microphase separation.

Altered gut microbiota and microbial metabolism in children with hepatic glycogen storage disease: a case-control study.

Background: Accumulating evidence has demonstrated that gut microbiota dysbiosis correlated with altered metabolism are implicated in liver metabolic diseases. However, data on pediatric hepatic glycogen storage disease (GSD) are limited. Here, we aimed to investigate the features of the gut microbiota and metabolites in hepatic GSD children from China.

A Single-Center Study Using IVUS to Guide Rotational Atherectomy for Chronic Renal Disease's Calcified Coronary Artery.

Objective: To assess the effectiveness and safety of an IVUS-guided rotational atherectomy (RA) percutaneous coronary intervention (PCI) in chronic renal patients with complex coronary calcification who are at risk for contrast-related acute kidney injury (AKI).

Methods: From October 2018 to October 2021, 48 patients with chronic renal disease who were receiving PCI with RA at the General Hospital of NingXia Medical University were informed for data collection for this research. They were randomly assigned to the IVUS-guided RA group and the Standard RA group, which did not use IVUS.