OBJECTIVE To investigate a dynamic monitoring of drug consumption （DMDC） model based on the seasonal Mann-Kendall trend test， aiming to provide scientific evidence for the efficient and macroscopic monitoring of drug use. METHODS A monitoring list of key outpatient drugs was established based on the top 20% of drugs ranked by sales volume in the outpatient pharmacy in October 2024. A DMDC model based on the Mann-Kendall trend test was constructed using the monthly usage data of key outpatient drugs from November 2021 to October 2024， aiming to eliminate the impact of seasonal fluctuations and analyze the temporal trends in drug consumption. Taking mucolytic expectorants， triazole derivatives for dermatophytosis， and single-agent hydroxymethylglutaryl coenzyme A （HMG-CoA） reductase inhibitors as examples， the monitoring effectiveness of the DMDC model was demonstrated， and its performance was compared with that achieved by the traditional sequential growth rate ranking method. RESULTS A total of 215 drug varieties were included in the monitoring list， and DMDC models were successfully established for all of them. Among these， 119 showed a significant increasing trend （P＜0.05， S′＞0）. The model successfully monitored the monthly consumption of mucolytic expectorants， triazole derivatives for dermatophytosis， and single- agent HMG-CoA reductase inhibitors. The precision and recall rates of the DMDC model for identifying abnormal drug use were 60.7% and 85.0%， respectively， both significantly higher than those of the sequential growth rate ranking method （8.3% and 15.0%， respectively） （χ2＝20.114， P＜0.001； χ2＝19.600， P＜0.001）. CONCLUSIONS DMDC model based on the seasonal Mann-Kendall trend test can effectively identify long-term trends in drug consumption， eliminate seasonal interference， enhance monitoring accuracy and management efficiency， and is suitable for the dynamic monitoring of drug consumption.

ObjectiveTo investigate the differences in clinical features and outcomes between patients with hepatic Wilson's disease （WD） presenting with the syndrome of combined phlegm and stasis and the syndrome of dampness-heat internal accumulation. MethodsA retrospective cohort study was conducted by consecutively recruiting patients with hepatic WD from the Encephalopathy Center of the First Affiliated Hospital of Anhui University of Chinese Medicine between January 2022 and August 2025. According to traditional Chinese medicine （TCM） syndrome differentiation， the patients were assigned into a combined phlegm and stasis group and a dampness-heat internal accumulation group. All the patients received standard treatment. Baseline data， laboratory indicators， complications， Model for End-Stage Liver Disease （MELD） score， Child-Turcotte-Pugh （CTP） score， and Chronic Liver Failure-Sequential Organ Failure Assessment （CLIF-SOFA） score were recorded. The clinical features and outcomes of the two groups of patients were compared by t-test， U-test and multivariate logistic regression. ResultsA total of 141 patients with hepatic WD were included. The combined phlegm and stasis group comprised 68 patients with an average age of （28.22±10.47） years， including 43 males and 25 females. The dampness-heat internal accumulation group comprised 73 patients with an average age of （30.22±8.79） years， including 44 males and 29 females. Univariate analysis showed no statistically significant differences in age or gender between the two groups. The combined phlegm and stasis group had lower platelet （PLT）， alanine aminotransferase （ALT）， aspartate aminotransferase （AST）， creatine （CRE）， total cholesterol （TC）， and triglycerides （TG） levels （P<0.05 or P<0.01） and higher total bilirubin （TBIL） and prothrombin time （PT） （P<0.05） than the dampness-heat internal accumulation group. There were no statistically significant differences in the incidence of hepatic encephalopathy， infection， spontaneous bacterial peritonitis， ascites， or gastrointestinal bleeding between the two groups. The incidence of splenomegaly and the MELD score were higher in the combined phlegm and stasis group （P<0.05）. The CTP and CLIF-SOFA scores were also higher in the combined phlegm and stasis group， while these differences were not statistically significant. Eleven patients in the combined phlegm and stasis group and 9 patients in the dampness-heat internal accumulation group developed liver failure. Multivariate logistic regression analysis showed that PT （OR=1.794， 95%CI 1.249-2.576）， TBIL （OR=1.111， 95%CI 1.026-1.203）， ALT （OR=1.053， 95%CI 1.004-1.105）， and TCM syndrome （OR=5.420， 95%CI 1.384-21.227） were independent risk factors for the development of liver failure in hepatic WD. ConclusionCompared with the hepatic WD patients with the syndrome of dampness-heat internal accumulation， those with the syndrome of combined phlegm and stasis exhibit severe liver function impairment and disease conditions. Furthermore， TCM syndrome serves as an independent predictive factor for the occurrence of liver failure in patients with hepatic WD.

Purpose: This study aimed to evaluate the safety and efficacy of gemcitabine and cisplatin (GP) regimen in combination with immune checkpoint inhibitor sintilimab as neoadjuvant therapy for muscle-invasive bladder cancer (MIBC) patients and the feasibility of the following selective bladder sparing surgery. Materials and Methods: Patients with histopathologically confirmed urothelial carcinoma without distant metastases (T2-4a, N ≤ 1, M0, American Joint Committee of Cancer 8th) and with adequate organ function will be enrolled. The therapeutic regimen was sintilimab 200 mg once on day 8, gemcitabine 1,000 mg/m2 and cisplatin 35 mg/m2 once on days 1 and 8, every 21 days for four cycles. The primary endpoint was pathologic complete response (pCR, pT0N0) rate. The secondary end points were ypT < 2 rate, R0 resection rate, event-free survival, and safety. Results: From May 4, 2020, to May 20, 2023, 55 patients were enrolled. Forty-six patients were evaluated for efficacy. Among the 42 patients who underwent surgery, 16 patients (38.0%) achieved pCR. Thirty-three patients (78.6%) achieved pT < 2. With a median follow-up of 15.7 months, the 1-year event-free survival was 91.3%. Notwithstanding the poor pathological baseline characteristic of a high T3-T4a proportion (39.1%), a promising bladder preservation (including 22 patients transurethral resection of bladder tumor, 5 patients partial cystectomy, and 4 surveillances) rate was achieved (67.4%). The most common grade ≥ 3 treatment-related adverse events was neutropenia (n=15, 27.3%), which was related to chemotherapy. There were no grade 3 immune-related adverse events. Conclusion Neoadjuvant GP plus sintilimab is a promising regimen for MIBC patients, with relatively high pT < 2 rate and triggering the emerging roles for the multi-disciplinary team decision-making for bladder sparing surgery.

Objective:To investigate the role of Fem-1 homolog C(FEM1C)in breast cancer progression and elucidate its underlying regulat-ory mechanism.Methods:The expression of FEM1C in breast cancer tissues and cells were detected with qPCR.The binding of FEM1C to ELAVL1 protein was predicted with an online database and validated by CoIP analysis;and the binding of ELAVL1 protein to OPA1 mRNA was predicted by using the starBase database and validated by RIP analysis.Next,breast cancer cell MDA-MB-231 was transfected with FEM1C shRNA(sh-FEM1C)or overexpression vector(FEM1C)or/and ELAVL1 overexpression vector(ELAVL1)or/and OPA1 overexpression vector(OPA1),or treated with 100 μM Mdivi-1,an DRP1 inhibitor,or MYLS22,an OPA1 inhibitor.Finally,nude mice were injected with sh-FEM1C lentiviral vectors to construct xenograft tumor models,and tumor growth was monitored.Results:The expression of FEM1C was upregu-lated in breast cancer tissues(P<0.01).Silencing FEM1C inhibited the proliferation,induced apoptosis,promoted the expression of auto-phagy protein LC3 Ⅱ/Ⅰ,inhibited p62 protein expression,upregulated the protein level of PINK1 in mitochondrial,promoted the expres-sion of mitochondrial fission proteins DRP1 and MIEF2,and inhibited the expression of fusion proteins OPA1 and MFN1 in MDA-MB-231 cells(P<0.01).Mdivi-1 treatment inhibited DRP1 expression(P<0.01),but had no effect on cell viability(P>0.05);MYLS22 treatment inhibited OPA1 expression and counteracted the effect of FEM1C overexpression on MDA-MB-231 cells(P<0.01).Mechanistic studies revealed that FEM1C binds to ELAVL1 protein and promotes its expression(P<0.01);ELAVL1 protein stabilizes OPA1 mRNA by binding to it and upregu-lates OPA1 protein levels(P<0.01).Overexpression of OPA1 reversed the effect of FEM1C silencing on MDA-MB-231 cells(P<0.01).In vivo results showed that knockdown of FEM1C inhibited tumor growth in vivo(P<0.01).Conclusions:FEM1C promotes the stability of OPA1 mRNA by upregulation of ELAVL1 protein to promote mitochondrial fusion and inhibit autophagy,thereby promoting breast cancer progression.

Objective:To observe the changes of growth parameters and glucose and lipid metabolism indexes in GHD children treated with rhGH for 2 years, and analyze the influence of sex and age on these indexes.Methods:Clinical data of children with 80 cases GHD admitted to the Endocrine and Nutrition Clinic of the Beijing Children's Hospital affiliated to the Capital Medical University from July 2016 to December 2022 were analyzed retrospectively. All patients were treated with rhGH. The growth parameters, growth factors, glucose metabolism and lipid metabolism indexes were collected and calculated before treatment and at 3, 6, 12, 18 and 24 months after treatment, the influence of sex and age on these indexes and the correlations between these indicators and height growth rate were analyzed. Independent-sample t-test was used to compare two groups with normal distribution, one-way ANOVA was used to compare multiple groups, and repeated measures ANOVA was used to compare the mean of each time point within groups. The nonparametric rank sum test was used for the comparison of non-normal distribution measurement data. Pearson correlation analysis was used to analyze the correlation between HGV and each index.Results:A total of 80 children were enrolled, 39 boys and 41 girls. Grouped by age, there were 20 in the 3.00-5.99 age group, 41 in the 6.00-9.99 age group, and 19 in the ≥10.00 age group. All patients after 24 months of treatment had a higher height ((135.13±13.17) cm), HtSDS (-0.73 (-1.04, -0.41)), body weight (29.25 (23.13, 35.00) kg), weight standard deviation score (WtSDS) (-0.44 (-1.03, 0.03)), and body mass index (BMI) (15.99 (14.90,16.92) kg/m 2) compared to before treatment ((115.44±12.87) cm, -2.11 (-2.57, -2.03), 20.00 (16.00,25.00) kg, -1.48 (-2.12, -0.89) and 15.30 (14.45, 16.21) kg/m 2) all increased, and the differences were statistically significant (all P<0.05). The increase in HtSDS in the group aged 3.00-5.99 (1.74±0.29) was higher than that in the group aged 6.00-9.99 (1.57±0.33) and ≥10.00 (1.39±0.45), and the difference was statistically significant ( F=4.84, P=0.010). All patients showed an increase in insulin-like growth factor 1 (IGF-1) (329.50 (268.00, 417.25) μg/L) and insulin-like growth factor binding globulin 3 (IGFBP-3) (6.27 (5.50,6.95) mg/L) after 24 months of treatment compared to before treatment (131.50 (96.48,177.25) μg/L, 4.07 (3.60,4.88) mg/L), with statistical significance (all P<0.05). After treatment for 3 months, 6 months, 12 months, 18 months, and 24 months, children aged ≥ 10.00 years old with IGF-1 (353.00 (221.00, 493.00), (414.84±147.91), 441.00 (287.00, 578.00), (421.68±138.30), 376.00 (290.00, 581.00) μg/L) were higher than these in 3.00-5.99 years old group (181.00 (151.25, 237.75), (216.30±68.48), 239.50 (216.75, 325.00), (284.30±89.12), 293.00 (245.25, 343.75)) μg/L and 6.00-9.99 age group (253.00 (193.50, 345.50), (294.59±90.37), 284.00 (217.50, 377.50), (325.76±90.04), 345.00 (265.00, 431.00) μg/L, the difference was statistically significant (all P<0.05). At 3 months, 6 months, 12 months, and 18 months of treatment, IGFBP-3 levels were observed in children aged ≥ 10.00 years old (6.15 (5.52, 6.46), (6.56±1.26), (6.78±1.33), (6.78±1.38) mg/L) higher than 3.00-5.99 years old group (4.69 (4.43,5.11), (5.18±0.63), (5.61±0.84), (6.08±1.00) mg/L) and 6.00-9.99 age group (5.51 (4.76, 6.35), (5.61±0.81), (5.72±0.78), (6.03±0.80) mg/L, the difference was statistically significant (all P<0.05). All children with HbA1C (5.40 (5.20, 5.58)%), fasting blood glucose (5.06 (4.76, 5.24) mmol/L), triglycerides (0.67 (0.53, 1.02) mmol/L), TyG index (2.24±0.48), and triglyceride/HDL-C ratio (1.05 (0.73, 1.50)) after 24 months of treatment compared to before treatment (5.10 (5.00, 5.28)%, 4.78 (4.51, 5.09) mmol/L, 0.57 (0.47, 0.72) mmol/L, (1.92±0.36), 0.86 (0.65, 1.08). The level of cholesterol increased, and the total cholesterol (3.74 (3.39, 4.31) mmol/L) decreased compared to before treatment (3.95(3.64, 4.54) mmol/L), with statistical significance (all P<0.05). Female patients had higher levels of triglycerides (0.79 (0.59, 1.09) mmol/L) and TyG index (2.31±0.49) than male patients (0.66 (0.53,0.89) mmol/L, (2.16±0.46)) after 18 months of treatment. The triglyceride/HDL-C at 12 months (1.10(0.67, 1.93)), 18 months (1.16(0.83, 1.68)), and 24 months (1.26 (0.79, 1.81)) of treatment ratio was also higher than male patients (0.76 (0.61, 1.09), 0.90 (0.72, 1.08), 0.98 (0.66, 1.30)). Female HDL-C levels at 18 months (1.52 (1.29,1.75) mmol/L) and 24 months (1.45(1.29,1.76) mmol/L) of treatment were significantly lower in males (1.72 (1.45, 1.84), 1.59 (1.43, 1.92) mmol/L) with statistical significance (all P<0.05). HGV was positively correlated with IGF-1 at 12 months ( r=0.243, P=0.030) , 18 months ( r=0.277, P=0.013) and 24 months ( r=0.289, P=0.009), and it was positively correlated with IGFBP-3 at 18 months ( r=0.242, P=0.030) and 24 months ( r=0.236, P=0.035), but it was negatively correlated with HDL-C at 18 months ( r=-0.331, P=0.003) and 24 months ( r=-0.281, P=0.012). Conclusions:RhGH can obviously improve HtSDS and WtSDS in GHD children. Growth factors, glucose metabolism and lipid metabolism should be monitored during the treatment. Especially for female patients (≥10.00 years old), we should closely monitor the indexes of glucose and lipid metabolism in order to avoid metabolic diseases.

A 71-year-old male presented with esophageal cancer and severe aortic valve regurgitation. Treatment strategies for such patients are controversial. Considering the risks of cardiopulmonary bypass and potential esophageal cancer metastasis, we successfully performed transcatheter aortic valve implantation and minimally invasive three-incision thoracolaparoscopy combined with radical resection of esophageal cancer (McKeown) simultaneously in the elderly patient who did not require neoadjuvant treatment. This dual minimally invasive procedure took 6 hours and the patient recovered smoothly without any surgical complications.

Objective To compare the differences in clinical features and treatment choices between periph-eral spondyloarthritis(pSpA)and axial spondyloarthritis(axSpA),and better understand the clinical charac-teristics and medication needs of pSpA.Methods Our study is a retrospective cohort study.The patients who first visited the First Medical Center of Chinese PLA General Hospital between January 2016 and December 2022 and were diagnosed with axSpA or pSpA according to the classification criteria established by the Assess-ment of SpondyloArthritis International Society were selected as the study subjects.Demographic data,clinical characteristics,laboratory tests,and treatment information of these patients were obtained through the electronic medical records management system and the intelligent management system for spondyloarthritis.The research compared the distribution of swollen and tender joints between pSpA and axSpA patients,as well as that between pSpA1(excluding patients with psoriatic arthritis)and axSpA patients.Additionally,we analyzed differences in clinical features and treatment options among these groups.Results A total of 1639 pa-tients were included in the study,of which 184 had pSpA(including 97 with psoriatic arthritis),and 1455 had axSpA.Compared to axSpA patients,pSpA patients had fewer male patients(62.5％vs.79.7％,P＜0.001),later onset age(33.8 years vs.22.0 years,P＜0.001),shorter diagnostic delays(6.0 months vs.14.2 months,P=0.004),more associated peripheral arthritis(71.7％vs.9.3％,P＜0.001)and dac-tylitis(6.5％vs.0.3％,P＜0.001),more cases of psoriasis(52.7％vs.1.1％,P＜0.001)and a more common family history of psoriasis(11.4％vs.3.4％,P＜0.001).pSpA patients had higher levels of in-flammatory markers but a lower positive rate of human leukocyte antigen(HLA)-B27(43.5％vs.87.4％,P＜0.001).A positive HLA-B27 was associated with an earlier onset age,fewer cases of psoriasis,and a fami-ly history of ankylosing spondylitis.pSpA patients had a higher proportion of using conventional synthetic dis-ease-modifying antirheumatic drugs(csDMARDs),biologic disease-modifying antirheumatic drugs(bDMARDs),and oral glucocorticoids,and they also more frequently used a combination of bDMARDs and csDMARDs(19.0％vs.12.2％,P=0.009)or multiple csDMARDs(65.8％vs.12.5％,P＜0.001).Compared to axSpA patients,pSpA1 patients(excluding psoriatic arthritis)did not show significant differences in the prevalence of psoriasis,uveitis,family history of psoriasis,or the use of bDMARDs,but the subgroup analysis of other variables was consistent with the results of pSpA patients.Conclusions pSpA patients tend to have a later onset of disease,a lower proportion of male and HLA-B27 positivity,more associ-ated peripheral arthritis,dactylitis,psoriasis,and a more common family history of psoriasis.The disease bur-den in terms of treatment for pSpA is not lower than that for axSpA.Due to the presence of more peripheral symptoms,psoriasis,and higher levels of inflammation,they also require more medication.

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

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.

The “Technical Guideline for Epidemic Prevention and Control Disinfection in Large-Scale Events”(hereinafter referred to as “the Guideline”), organized and compiled by the National Disease Control and Prevention Administration, was officially released in April 2024. This guideline aims to ensure the effective implementation of large-scale group activities, mitigate the impact of infectious disease outbreaks on such events, and maintain hygiene and safety standards at event venues. During the compilation process, data were systematically collected in alignment with epidemic prevention requirements and disinfection principles, incorporating research findings from domestic and international disinfection practices. Information was gathered through field investigations, expert consultations in epidemiology and disinfection, and roundtable discussions with representatives from organizations responsible for disinfection operations at large-scale events, thereby ensuring the scientific rigor and practical applicability of the content. The Guideline provides comprehensive technical disinfection guidance for relevant authorities and event organizers, addressing critical aspects such as disinfection protocols, operational principles, emergency response strategies, and technical specifications. By standardizing hygiene assurance measures for large-scale events, including considerations of participant demographics, venue characteristics, and event scale, the guideline establishes a framework to proactively minimize the risk of infectious disease transmission.