A curcumin-loaded pectin/carboxymethyl cellulose/gelatin film: synthesis, characterization, and application.

Traditional plastic packaging causes environmental pollution and biosafety issues, and developing simple and effective biodegradable active packaging remains a challenge. This study aimed to develop pectin/carboxymethyl cellulose (CMC)/gelatin-based films formulated with curcumin (Cur) to investigate the changes in the films' physicochemical properties and the application in grass carp preservation. The results indicated that Cur was uniformly distributed in the film matrix through hydrogen bonding.

Uncovering the genetic basis for enhanced mushroom flavor in through genome sequencing and metabolic profiling.

is a common timber oak tree species widely distributed in subtropical areas of China. In this study, we presented a chromosome-scale reference genome assembly of . achieved by integrating PacBio Sequel II, DNBseq™, and Hi-C sequencing platforms, and the results indicated the .

Clinical Characteristics, Genotypes, and Treatment Outcomes in 133 Patients With Spinal Muscular Atrophy: A Retrospective Cohort.

Aim: To characterise spinal muscular atrophy (SMA) phenotypes, genetic profiles, and nusinersen efficacy in China.

Methods: In 133 SMA patients (age 6.38 ± 3.

A Flexible Seamless Phase 2/3 Design With Biomarker-Driven Subgroup Enrichment and Sample Size Re-Estimation.

To support the expedited drug development addressing unmet medical needs, the seamless phase 2/3 design that makes the phase switching decision based on early surrogate endpoints is gaining popularity in practice. For also catering to potentially more beneficial patient subgroups based on predictive biomarkers, it is appealing to incorporate the subgroup enrichment feature into the seamless phase 2/3 design. However, the sample size planning for such a complex adaptive design is challenging, as it must strike a balance among shortening development timeline, mitigating development risks, and accounting for uncertainty related to subgroup effects.

Comparison of clinical features and outcomes of Chinese patients with Takotsubo syndrome and acute myocardial infarction-results from the first Chinese Takotsubo syndrome registry.

Background: Takotsubo syndrome (TTS) differs significantly from acute myocardial infarction (AMI) in clinical features and pathological mechanisms, but evidence in Asian populations remains limited. The aim of this study is to compare clinical features and outcomes between patients with TTS and AMI in the first Chinese registry of TTS (ChiTTS Registry, ChiCTR1900026725).

Methods: In this multicenter 6-year retrospective cohort study (February 2016-June 2022), a total of 116 consecutive TTS patients diagnosed according to the international Takotsubo diagnostic criteria and 232 age- and sex-matched AMI patients (1:2 ratio) meeting the 2023 European Society of Cardiology guidelines criteria were enrolled from 10 tertiary medical centers across China.

The Characteristically Slow Proton Transfer Coupled to Platinum Oxidation in Alkaline Polyelectrolyte as Elucidated at the Molecular Level.

The proton transfer in alkaline polyelectrolyte membrane (APEM)/electrode interfaces is significantly coupled to the electrochemical reactions in energy conversion and green synthesis. The OH in APEM/electrode interfaces is characteristically without cations in the surroundings but ambiguous in proton-transfer-coupled electrochemical reactions at the molecular level. Here we employed electrochemical surface-enhanced Raman spectroscopy and high-level quantum-chemical calculations to elucidate the proton transfer in the APEM/Pt interface by using electrochemical Pt oxidation as an indicator.

Administration practices of and adherence to nusinersen in children with spinal muscular atrophy: a multicenter disease registry study in China.

Background: Nusinersen was the first approved disease modifying therapy (DMT) for spinal muscular atrophy (SMA). Intrathecal administration of nusinersen enables drug delivery directly to the central nervous system, where the motor neurons are located. Per the package insert, individuals with SMA receive 4 loading doses of nusinersen followed by maintenance doses every 4 months thereafter.

The comparison of gut microbiota between different types of epilepsy in children.

Objective: To better understand the variations in gut microbiota in children with different types of epilepsy.

Methods: Thirty-seven children with epilepsy were included in the case group, which was further divided into focal (group A, n = 28) and generalized epilepsy groups (group B, n = 9) based on the origin and extent of the seizures. The focal epilepsy group was subdivided into the benign childhood epilepsy with centrotemporal spikes (BECT) (group C, n = 9) and non-BECT groups (group D, n = 19) based on the appearance of typical centrotemporal spikes or spike-wave complexes on the electroencephalogram (EEG).

Lipidomics Combined with Network Pharmacology to Explore Differences in the Mechanisms of Grey Hair Development Between Type 2 Diabetes Mellitus and Normal Populations (Female).

Type 2 diabetes is usually accompanied by premature grey hair. In this study, we analysed differences in the lipid composition of black and white hair follicles between women with type 2 diabetes and healthy populations, using lipidomic methods. We examined the correlation between the lipid composition of female grey hair follicles and type 2 diabetes mellitus, and we screened for potential grey-hair-delaying ingredients using network pharmacology.

Optimizing Quality Tolerance Limits Monitoring in Clinical Trials Through Machine Learning Methods.

The traditional clinical trial monitoring process, which relies heavily on site visits and manual review of accumulative patient data reported through Electronic Data Capture system, is time-consuming and resource-intensive. The recently emerged risk-based monitoring (RBM) and quality tolerance limit (QTL) framework offers a more efficient alternative solution to traditional SDV (source data verification) based quality assurance. These frameworks aim at proactively identifying systematic issues that impact patient safety and data integrity.

Post-traumatic growth and influencing factors of parents with children with Duchenne muscular dystrophy: a cross-sectional survey study.

Background: The aim of the study was to identify the post-traumatic growth status and influencing factors of parents with children with Duchenne muscular dystrophy (DMD).

Methods: We adopted a cross-section survey study. Between February and December 2022, 181 parents responded to the survey including a participants' characteristics section, post-traumatic growth assessment scale, caregiver burden scale, and social support assessment scale.

Sample Size Adjustment in Sequential Multiple Assignment Randomized Trials.

Clinical trials are often designed based on limited information about effect sizes and precision parameters with risks of underpowered studies. This is more problematic for SMARTs where strategy effects are based on sequences of treatments. Sample size adjustment offers flexibility through re-estimating sample size during the trial to ensure adequate power at the final analysis.

Polyphenol metabolomics reveals the applications and prospects of polyphenol-rich plants in natural dyes.

Polyphenols, as one of the primary compounds produced by plant secondary metabolism, have garnered considerable attention because of their non-toxic, environmentally friendly, and biodegradable properties, as well as their notable medicinal value. This study presents a metabolomic analysis of polyphenols from 11 woody plants, including , , and , investigating a total of 40 polyphenolic metabolites. A differential metabolite dynamics map highlighted the five most differentiated substances among the 11 plants, including vitexin, dihydromyricetin, genistin, resveratrol, and isorhamnetin.

[Genetic analysis of a child with Leukoencephalopathy with ataxia caused by a homozygous variant of CLCN2 gene and a literature review].

Objective: To explore the clinical manifestations and genetic characteristics of a child with Leukoencephalopathy with ataxia (LKPAT) caused by a CLCN2 gene variant.

Methods: A retrospective analysis was conducted on the clinical data of a child admitted to Hunan Children's Hospital in June 2024 due to "intermittent convulsions for 13 days". Peripheral blood samples were collected from the child and his parents for whole exome sequencing, followed by Sanger sequencing validation and pathogenicity analysis of candidate variants.

Multi-omics analysis reveals the regulatory mechanism of branching development in Quercus fabri.

The ability of axillary meristems to form axillary buds and subsequently develop into branches is influenced by phytohormones, environmental conditions, and genetic factors. The main trunk of Quercus fabri is prone to branching, which not only impacts the appearance and density of the wood and significantly reduces the yield rate. This study conducted transcriptomic, proteomic, and metabolomic analyses on three stages of axillary bud development in Q.

Transcriptomic Analysis Reveals the Opposite Regulatory Effects of WRKY and CAMTA Transcription Factors on Total Tannin Production in Fruit.

Tannins are prevalent compounds found in plant fruits, contributing to the bitter taste often associated with these fruits and nuts, thereby influencing their overall taste quality. Numerous studies have been conducted to investigate the regulatory factors involved in tannin synthesis. Among these factors, transcription factors exhibit the most significant capacity to regulate tannin production as they can modulate the expression of several key enzyme genes within the tannin synthesis pathway.

Mesenchymal Stem Cell-Derived Exosomes: Emerging as a Promising Cell-Free Therapeutic Strategy for Autoimmune Hepatitis.

Autoimmune hepatitis (AIH) is an immune-mediated liver disease that currently faces limited treatment options. In its advanced stages, AIH can progress to liver fibrosis and cirrhosis. Recent research has increasingly focused on cell-free therapies, particularly the use of mesenchymal stem cell (MSC)-derived exosomes (Exos), which have shown promise in treating autoimmune diseases, including AIH.

Safety and efficacy of viltolarsen in ambulatory and nonambulatory males with Duchenne muscular dystrophy.

Duchenne muscular dystrophy (DMD) is an X-linked recessive disease characterized by mutations in the dystrophin gene, causing motor and pulmonary function decline. Viltolarsen is indicated for patients with dystrophin gene mutations amenable to exon 53 skipping. Here, we report safety, motor function, and the first pulmonary function results from the open-label, phase II Galactic53 trial of viltolarsen (NCT04956289).

A Novel m.1636A > G Variant in Mitochondrial TV Gene Might Cause New Phenotype of Mitochondrial Disease in a 2-Year Old Chinese Boy.

Pathogenic variants of mitochondrial DNA (mtDNA) are associated with a large number of heterogeneous diseases involving multiple systems with which patients may present with a wide range of clinical phenotypes. Clinical data of the proband and his family members were gathered in a retrospective study. Whole-exome sequencing and full-length sequencing of the mitochondrial genome that was performed on peripheral blood, urine, and oral mucosa cells were applied for genetic analysis.

Nusinersen effectiveness and safety in pediatric patients with 5q-spinal muscular atrophy: a multi-center disease registry in China.

Objective: To evaluate the effectiveness and safety of nusinersen for the treatment of 5q-spinal muscular atrophy (SMA) among Chinese pediatric patients.

Methods: Using a longitudinal, multi-center registry, both prospective and retrospective data were collected from pediatric patients with 5q-SMA receiving nusinersen treatment across 18 centers in China. All patients fulfilling the eligibility criteria were included consecutively.

A novel mutation of DNA2 regulates neuronal cell membrane potential and epileptogenesis.

Mesial temporal lobe epilepsy (MTLE) is one of the most intractable epilepsies. Previously, we reported that mitochondrial DNA deletions were associated with epileptogenesis. While the underlying mechanism of mitochondrial DNA deletions during epileptogenesis remain unknown.

Clinical and genetic analyses of APMR4 syndrome caused by novel biallelic variants.

Alopecia intellectual disability syndromes 4 (APMR4) caused by Lanosterol synthase () gene variants is a very rare autosomal recessive neuroectodermal syndrome. It is characterized by congenital alopecia and variable degrees of intellectual disability (ID), frequently associated with developmental delay (DD) and epilepsy. Currently, only three studies regarding -related APMR4 have been reported, the pathogenesis of APMR4 is poorly understood.

Clinical analysis and literature review of two paediatric cases of anti-IgLON5 antibody-related encephalitis.

Introduction: Anti-IgLON5 antibody-related encephalitis is a rare autoimmune disorder of the central nervous system, predominantly occurring in middle-aged elderly individuals, with paediatric cases being exceptionally rare. This study aims to enhance the understanding of paediatric anti-IgLON5 antibody-related encephalitis by summarising its clinical and therapeutic characteristics.

Method: A retrospective analysis was conducted on two paediatric patients diagnosed with anti-IgLON5 antibody-related encephalitis at Hunan Children's Hospital from August 2022 to November 2023.