Objective To investigate the auditory effects of cochlear implantation in quiet and noisy environ-ments in children with different bilateral intervention modes,as well as the factors influencing these effects.Methods A total of 185 children with bilateral severe to profound sensorineural hearing loss were divided into three groups:bimodal hearing mode group(BIM,n=55),simultaneous bilateral cochlear implantation group(SCI,n=70),and sequential bilateral cochlear implantation group(SBCI,n=60).The Parents' Evaluation of Aural/Oral Performance of Children(PEACH)was used to assess the PEACH scores of the three groups in quiet and noisy environments one year after binaural hearing aid intervention.Additionally,the effects of cochlear implantation age,preoperative residual hearing,hearing aid usage,rehabilitation training mode,family system,and other factors on auditory per-formance in quiet and noisy environments were analyzed.Results The PEACH scores in quiet environments were higher than those in noisy environments for all three groups(all P＜0.05).The SCI group had higher PEACH scores in both quiet and noisy environments compared to the BIM group(P＜0.05).Multifactorial analysis revealed differences in factors influencing auditory performance in quiet and noisy environments among the three groups.First cochlear implantation before 3 years of age,preoperative hearing aid usage,and home-based rehabilitation training mode were common favourable influencing factors for auditory performance in both environments.Preopera-tive residual hearing below 95 dB HL was an favourable influencing factor for auditory performance in quiet environ-ments in the BIM group.The higher the level of parental education,the better auditory performance in both quiet and noisy environments for the SCI and SBCI groups.Implantation interval of 24 months or less and hearing aid usage during the inter-implantation period were favourable influencing factors for auditory performance in both envi-ronments for the SBCI group.Conclusion Children with severe to profound prelingual deafness after simultaneous bilateral CI implantation had better hearing performance than bimodal listening in quiet and noise environments.Ear-ly implantation,preoperative or inter-implantation hearing aid usage are recommended to improve auditory perform-ance in noisy environments,regardless of the bilateral intervention mode.The interval between bilateral cochlear im-plantations should be less than 12 months.

This article reports a rare case of pantothenate kinase-associated neurodegeneration: the patient′s initial symptoms were Parkinson-like symptoms such as tremor and bradykinesia, as well as dysarthria. Brain magnetic resonance imaging showed abnormal signals in the bilateral basal ganglia region similar to the tiger′s eye sign. Two heterozygous mutations including c.803A>G(p.Asp268Gly) and c.1021C>T(p.Arg341 *) were found in the pantothenate kinase 2 gene by whole exome sequencing, and the final diagnosis was neurodegeneration with brain iron accumulation type 1, also known as pantothenate kinase-associated neurodegeneration. By reviewing the clinical characteristics, diagnostic approaches and therapeutic strategies, this article aims to enhance the recognition of this disease, avoid misdiagnosis and missed diagnosis, and provide guidance for clinical diagnosis and treatment.

Objective:To evaluate the effects and underlying mechanisms of metabolites derived from the kidney-reinforcing, blood circulation-activating, and collateral dredging decoction on the proliferation of multiple myeloma (MM) KM3 cells.Methods:MM KM3 cells in the logarithmic growth phase were treated with 3%, 6%, 9%, or 12% metabolites of kidney-reinforcing, blood circulation-activating, and collateral dredging decoction. Cell viability was assessed using the CCK-8 assay. Apoptosis and necrosis were evaluated using flow cytometry and TUNEL staining. Mitochondrial and cellular ultrastructural changes were examined using transmission electron microscopy. mRNA and protein expression levels of dynamin-related protein 1 (Drp1), mitochondrial fission protein 1 (Fis1), mitochondrial fission factor (MFF), PTEN-induced kinase 1 (Pink1), and E3 ubiquitin ligase (Parkin) were determined through quantitative real-time PCR and western blotting. High-performance liquid chromatography-tandem mass spectrometry (HPLC-MS/MS) combined with network pharmacology, was utilized for reverse verification of the pharmacodynamic mechanisms and therapeutic targets underlying the anti-MM activity of this decoction.Results:The metabolites of the kidney-reinforcing, blood circulation-activating, and collateral dredging decoction inhibited KM3 cell proliferation and induced apoptosis in a dose-dependent manner. Transmission electron microscopy revealed increased mitochondrial fission and autophagic structures, with effects intensifying at higher metabolite concentrations. mRNA and protein expression of Drp1, Fis1, MFF, Pink1, and Parkin were significantly upregulated in treatment groups compared to controls ( P<0.05), with the most pronounced effects observed in the 12% metabolite group ( P<0.01). HPLC-MS/MS identified 121 bioactive compounds in BHTF, which shared 474 overlapping targets with MM. Enrichment analysis suggested that BHTF exerts antitumor effects primarily through apigenin, palmatine, and other key components by modulating TNF, NF-κB, and mitophagy pathways. Conclusion:The kidney-reinforcing and blood circulation-activating and collateral dredging decoction suppresses the proliferation of MM KM3 cells, potentially through mechanisms involving the regulation of mitochondrial dynamics and induction of autophagy.

Objective:To explore the clinical characteristics and genetic etiology of a child with Oral-facial-digital syndrome type Ⅰ(OFDSⅠ).Method:A child with OFDSⅠ who received treatment at the Women and Children′s Hospital Affiliated to Ningbo University in March 2023 was selected as the study subject. A retrospective research method was used to collect the clinical data of the child. Peripheral venous blood samples were collected from the child, her parents and sister. Genomic DNA was extracted, and whole exome sequencing (WES) was performed. Candidate variants were validated using Sanger sequencing for familial verification. According to the Standards and Guidelines for the Interpretation of Sequence Variants developed by the American College of Medical Genetics and Genomics (ACMG) (hereinafter referred to as the " ACMG Guidelines" ), the pathogenicity of the candidate variant was rated. This study was approved by the Medical Ethics Committee of Ningbo University Affiliated Women and Children′s Hospital (Ethic No.: EC 2024-063).Results:The child was a prematurely born female with deformities of the oral cavity, fingers, and toes. She was admitted to the Neonatal Department of the Hospital where she was born due to shortness of breath 15 minutes after birth. The WES results indicated that the child has harbored a heterozygous c. 710dup(p.Y238Vfs*2) frameshifting variant of the OFD1 gene. Sanger sequencing confirmed that neither of the child′s parents nor her sister had carried the same variant. According to the ACMG guidelines, the variant was rated as pathogenic (PVS1+ PS4_Moderate+ PM2-Supporting+ PM6_Supporting+ PP4). Conclusion:Children with OFDSⅠ have clinical features such as oral, finger, and toe deformities. The c. 710dup(p.Y238Vfs*2) variant of the OFD1 gene probably underlay the OFDSⅠ in this child. Above result has enriched the mutational spectrum of the OFD1 gene.

OBJECTIVE: To explore the clinical characteristics and genetic etiology of a child with Oral-facial-digital syndrome type Ⅰ(OFDSⅠ). METHODS: A child with OFDSⅠ who received treatment at the Women and Children's Hospital Affiliated to Ningbo University in March 2023 was selected as the study subject. A retrospective research method was used to collect the clinical data of the child. Peripheral venous blood samples were collected from the child, her parents and sister. Genomic DNA was extracted, and whole exome sequencing (WES) was performed. Candidate variants were validated using Sanger sequencing for familial verification. According to the Standards and Guidelines for the Interpretation of Sequence Variants developed by the American College of Medical Genetics and Genomics (ACMG) (hereinafter referred to as the "ACMG Guidelines"), the pathogenicity of the candidate variant was rated. This study was approved by the Medical Ethics Committee of Ningbo University Affiliated Women and Children's Hospital (Ethic No.: EC 2024-063). RESULTS: The child was a prematurely born female with deformities of the oral cavity, fingers, and toes. She was admitted to the Neonatal Department of the Hospital where she was born due to shortness of breath 15 minutes after birth. The WES results indicated that the child has harbored a heterozygous c.710dup (p.Y238Vfs*2) frameshifting variant of the OFD1 gene. Sanger sequencing confirmed that neither of the child's parents nor her sister had carried the same variant. According to the ACMG guidelines, the variant was rated as pathogenic (PVS1+PS4_Moderate+PM2-Supporting+PM6_Supporting+PP4). CONCLUSION Children with OFDSⅠ have clinical features such as oral, finger, and toe deformities. The c.710dup (p.Y238Vfs*2) variant of the OFD1 gene probably underlay the OFDSⅠ in this child. Above result has enriched the mutational spectrum of the OFD1 gene.
Humans ; Female ; Orofaciodigital Syndromes/genetics* ; Exome Sequencing ; Retrospective Studies ; Mutation ; Child ; Proteins

The prevalence of Class III malocclusion varies among different countries and regions. The populations from Southeast Asian countries (Chinese and Malaysian) showed the highest prevalence rate of 15.8%, which can seriously affect oral function, facial appearance, and mental health. As anterior crossbite tends to worsen with growth, early orthodontic treatment can harness growth potential to normalize maxillofacial development or reduce skeletal malformation severity, thereby reducing the difficulty and shortening the treatment cycle of later-stage treatment. This is beneficial for the physical and mental growth of children. Therefore, early orthodontic treatment for Class III malocclusion is particularly important. Determining the optimal timing for early orthodontic treatment requires a comprehensive assessment of clinical manifestations, dental age, and skeletal age, and can lead to better results with less effort. Currently, standardized treatment guidelines for early orthodontic treatment of Class III malocclusion are lacking. This review provides a comprehensive summary of the etiology, clinical manifestations, classification, and early orthodontic techniques for Class III malocclusion, along with systematic discussions on selecting early treatment plans. The purpose of this expert consensus is to standardize clinical practices and improve the treatment outcomes of Class III malocclusion through early orthodontic treatment.
Humans ; Malocclusion, Angle Class III/classification* ; Orthodontics, Corrective/methods* ; Consensus ; Child

Objective To investigate the multi-target mechanisms of quercetin in treating endometriosis(EMT)through integrative network pharmacology analysis.Methods Active targets of quercetin were collected from the TCMSP database,while EMT-related differentially expressed genes(DEGs)were identified through the Gene Expression Omnibus(GEO)dataset.A comparative analysis was conducted to pinpoint potential therapeutic targets of quercetin for EMT treatment.Functional enrichment analyses were employed to investigate the biological functions associated with these targets,and a protein-protein interaction(PPI)network was conducted to identify core targets.Molecular docking and dynamics simulations were performed to validate the binding characteristics between quercetin and the core targets.The top 2 target protein pairs,HSP90AB1 and AR,exhibiting the lowest binding energy,were selected for subsequent cellular experimental validation.Human EMT-immortalized ectopic endometrial epithelial cell line 12Z(n=6,independent replicates)was subjected,and CCK-8 assay was used to determine ehe effects of quercetin on cell viability and proliferation,and the half-maximal inhibitory concentration(IC50)was calculated at 48 h after treatment.Then the 12Z cells were treated with quercetin at a concentration gradient of 0,30,60 and 90 μmol/L,the migration and invasion abilities were assessed with cell scratch and cell invasion assays.Western blotting was conducted to detect the changes in the expression of HSP90AB1 and AR proteins after different doses of treatment.Results There were 49 potential EMT-related therapeutic targets and 10 core targets identified.Functional enrichment analyses revealed that these targets were significant enriched in inflammation-related signaling pathways,including AGE-RAGE,ErbB and TNF;immune-related pathways,such as Th17 cell differentiation,T/B cell receptor signaling;angiogenesis-related pathways like VEGF;and hormonal regulatory pathways involving estrogen and GnRH.Molecular docking demonstrated that quercetin exhibited favorable binding activity(binding energy<-5 kcal/mol)with all core target proteins,with particularly strong binding energies(<-7 kcal/mol)observed for AR,EGFR,FOS,ERBB2,and HSP90AB1.Molecular dynamics simulations revealed that quercetin forms sustained hydrogen bond interactions with AR and HSP90AB1,facilitating the formation of stable complexes.CCK-8 assay,cell scratch assay,and transwell invasion assay indicated that quercetin inhibited the proliferative activity,and migrative and invasive abilities of 12Z cells in a concentration-dependent manner,with more pronounced inhibitory effects observed at 60 and 90 μmol/L quercetin(P<0.001);Western blotting revealed that treatment of 12Z cells with varying quercetin concentrations for 48 h up-regulated the expression of HSP90AB1 and AR,with the most significant increase observed at 90 μmol/L quercetin(HSP90AB1,P<0.05;AR,P<0.001).The restored expression levels of HSP90AB1 and AR showed positive correlations with the proliferative activity,migrative and invasive abilities of ectopic endometrial cells.Conclusion Quercetin effectively addresses endometriosis through multiple molecular targets and signaling pathways,and stabilization of the HSP90AB1/AR complex and subsequent protein upregulation represents a key therapeutic mechanism.

Objective: To investigate the self-monitoring blood pressure behavior and its influencing factors among residents in Jiangsu Province, so as to provide the basis for strengthening proactive blood pressure monitoring among residents. Methods: Permanent residents aged 35-75 years in six counties (cities, districts), Jiangsu Province, were selected using the stratified cluster sampling method in 2023. Data on basic information, disease history, and self-monitoring blood pressure behavior were collected, height and weight were measured to calculate the body mass index (BMI); and blood glucose and lipid levels were measured. Self-monitoring blood pressure behavior was defined as having measured blood pressure at least once in the past three months. Factors affecting self-monitoring blood pressure behavior were identified using a multivariable logistic regression model. Results: A total of 12 475 residents were surveyed, including 5 748 males and 6 727 females, with a male-to-female ratio of 1∶1.17. There were 3 855 residents aged 45-<55 years (30.90%) and 5 511 residents who had self-monitoring blood pressure behaviors (44.18%). Multivariable logistic regression analysis showed that the residents who were males (OR=1.167, 95%CI: 1.081-1.261), lived in rural areas (OR=1.430, 95%CI: 1.321-1.547), aged 45-75 years (45-<55 years, OR=1.384, 95%CI: 1.241-1.543; 55-<65 years, OR=1.397, 95%CI: 1.243-1.570; 65-75 years, OR=1.196, 95%CI: 1.049-1.363), had an annual household income ≥30 000 yuan (30 000-<60 000 yuan, OR=1.190, 95%CI: 1.072-1.321; 60 000-<110 000 yuan, OR=1.330, 95%CI: 1.191-1.485; ≥110 000 yuan, OR=1.746, 95%CI: 1.536-1.984), were overweight (OR=1.170, 95%CI: 1.070-1.280) or obese (OR=1.248, 95%CI: 1.120-1.391), were unaware (OR=1.221, 95%CI: 1.103-1.353) or aware (OR=3.937, 95%CI: 3.575-4.335) of having hypertension, were aware of having diabetes (OR=1.538, 95%CI: 1.354-1.749), and aware of having dyslipidemia (OR=1.265, 95%CI: 1.106-1.447) were more likely to have self-monitoring blood pressure behaviors. Conclusions Among the residents aged 35-75 years in Jiangsu Province, 44.18% had self-monitoring blood pressure behavior. Gender, place of residence, age, annual household income, BMI, hypertension, diabetes, and dyslipidemia were identified as influencing factors for self-monitoring blood pressure behavior.

This consensus was developed by the Orthodontic Society of the Chinese Stomatological Association to provide a systematic, scientific, and practical guideline for informed consent in orthodontic care. Orthodontic treatment is typically lengthy, highly individualized, and involves multiple factors such as growth and development, occlusal function, and facial esthetics. Rapid technological advances and diverse risk profiles make the traditional reliance on orthodontist experience or institutional templates insufficient to ensure patients′ full understanding and autonomous decision-making. To address this, the expert panel conducted extensive reviews of domestic and international guidelines, analyzed representative dispute cases, and performed multicenter patient-clinician surveys. Using a multi-round Delphi method, the group established a standardized informed consent framework covering the initial consultation, treatment, and retention phases. The consensus emphasizes that informed consent is not only a fundamental legal and ethical requirement but also a key step in building trust, improving patient compliance, and enhancing treatment satisfaction. Orthodontists should clearly and comprehensively explain treatment plans, potential risks, uncertainties, and associated costs, while respecting the autonomy of patients or guardians, and maintain continuous communication and dynamic evaluation throughout the treatment process. The release of this consensus provides unified and authoritative guidance for clinical orthodontics, helping to standardize informed consent, enhance its transparency, safeguard patient rights, reduce medical risks, and promote high-quality, sustainable development of orthodontic practice.

Objective The application effect of bedside ultrasound combined with joint movement protractor on the accurate monitoring and management of joint range of motion in extremely low birth weight infants was evaluated to reduce the incidence of secondary ectopic PICC in extremely low birth weight infants.Methods Convenience sampling was used to select 128 extremely low birth weight infants admitted to the neonatal intensive care unit of a tertiary hospital in Zhengzhou,Henan Province from February to September 2024.Among them,infants enrolled between June and September 2024 were assigned to an experimental group,while those enrolled between February and May 2024 were assigned to a control group,with 64 cases in each group.Whereas the experimental group,based on the control group,combined bedside ultrasound with a joint goniometer for precise monitoring and management of limb movement range in children with upper limb catheterization;the control group received routine prevention and management of PICC secondary displacement.After intervention,the incidence of PICC secondary displacement,the rate of PICC secondary displacement removal,the incidence of complications related to PICC secondary displacement,and the average length of stay of the infants were compared between the 2 groups.Results Ultimately,60 cases were included in each of the 2 groups.The incidence of secondary ectopic PICC in the post-intervention experimental group was 8.33％,lower of 23.33％in the control group;the extubation rate of secondary ectopic PICC was 3.33％,lower of 16.67％in the control group;the incidence of complications related to secondary ectopic PICC was 13.33％,lower of 51.67％in the control group;the average hospitalization day was 50(48,55)d,shorter of 61(55,65)d in the control group.The difference between the 2 groups was statistically significant(P＜0.05).Conclusion The combination of bedside ultrasound and joint movement protractor was used to accurately monitor and manage the range of motion of limbs in children with upper limb catheterization,which effectively guaranteed the safe position of the catheter tip and reduced the incidence of secondary ectopic complications related to PICC.It can be further promoted and applied in clinical practice.