Objective:To analyze the efficacy of enzyme replacement therapy and anti-drug antibody production in children with Fabry disease.Methods:The clinical data of 7 children with Fabry disease treated with enzyme replacement therapy for more than 1 year at Children′s Hospital of Zhejiang University School of Medicine from July 2021 to June 2024 were retrospectively analyzed. The basic information and the changes of related clinical indicators before and after treatment were collected. Paired sample t test was used to compare renal function, left heart mass index, pain score and other related indexes before and after treatment. The anti-drug antibodies were detected by enzyme-linked immunosorbent assay. Results:A total of 6 boys and 1 girl were included. The age of diagnosis was (12.2±1.8) years. After 1 year of enzyme replacement therapy, the abnormal substrate globotriaosylsphingosine and brief pain inventory scores of all children were significantly lower than those before treatment ((16±11) vs. (63±42) μg/L, 22±19 vs. 45±29, t=3.88, 3.43, both P<0.05). There were no significant differences in glomerular filtration rate, urinary microalbumin to creatinine and left heart mass index before and after treatment ((124±35) vs. (136±26) ml/(min·1.73 m 2), (9.3±8.3) vs. (3.8±2.5) mg/g, (38±9) vs. (33±6) g/m 2.7, t=1.33, 1.74, 1.19, all P>0.05). Patients 4, 5 and 6 developed anti-drug antibodies at 1 month, 4 months and 1 month after medication, respectively. Patient 4 had persistently high anti-drug antibody titers (absorbance 3.65-3.73) accompanied by urticaria, elevated globotriaosylsphingosine and worsening clinical symptoms. Conclusions:The enzyme replacement therapy can effectively improve the clinical symptoms and reduce the level of globotriaosylsphingosine in children with Fabry disease. The anti-drug antibody is common in patients after long-term enzyme replacement therapy and may diminish the efficacy, which needs dynamic monitoring.

Objective: To investigate the epidemic situation of notifiable infectious diseases in Zhejiang Province in 2024, so as to summarize the epidemic characteristics. Methods: Data of notifiable infectious diseases cases in Zhejiang Province from January 1 to December 31, 2024 were collected from the Infectious Disease Surveillance System of Chinese Disease Prevention and Control Information System. The epidemiological characteristics were analyzed according to the classification and transmission routes using the descriptive epidemiological method. Results: A total of 32 types of notifiable infectious diseases with 1 858 695 cases and 392 deaths were reported in Zhejiang Province in 2024, with a reported incidence of 2 804.73/105 and a reported mortality of 0.591 5/100 000. A total of 238 infectious disease public health emergencies were reported, of which 218 (91.60%) occurred in schools and kindergartens. There were 22 types of class A and B notifiable infectious diseases reported, with incidence of 470.62/100 000 and mortality of 0.591 5/100 000. Totally 10 types of class C notifiable infectious diseases, with a reported incidence of 2 334.11/105, and no deaths were reported. Classified by transmission route, respiratory infectious diseases had the highest reported incidence of 2 423.87/100 000, among which influenza exhibited the highest reported incidence of 2 024.22/100 000. The reported incidence of intestinal infectious diseases was 312.94/105, among which the incidence of other infectious diarrhea and hand-foot-mouth disease (HFMD) were high, with reported incidences of 169.52/100 000 and 136.18/100 000, respectively. Blood-borne and sexually transmitted infectious diseases accounted for the largest number of reported deaths, among which AIDS had the highest mortality of 0.424 0/100 000. Natural and insect-borne infectious diseases exhibited a low reported incidence of 1.37/105. The reported incidence of dengue fever was 0.40/100 000, and 95.08% of the cases were imported. Conclusions The reported incidence of respiratory and intestinal infectious diseases and the reported mortality of AIDS were high in Zhejiang Province in 2024. It is recommended to strengthen the prevention and control of infectious diseases such as influenza, other infectious diarrhea, and HMFD in schools and kindergartens.

Objective:To explore the cognition and intention of patients with lower limb deep vein thrombosis (DVT) to participate in patient-reported outcomes (PROs), so as to provide references for the promotion, application, and practice of PROs in lower limb DVT patients in China.Methods:This study adopted the phenomenological qualitative research method. From February to June 2023, 13 lower limb DVT patients admitted to Peking Union Medical College Hospital were selected for semi-structured in-depth interviews. Colaizzi 7-step analysis method was used for analyzing and summarizing data.Results:Thirteen patients with lower limb DVT highly recognized their own value in participating in PROs, namely health benefits, prevention of DVT complications, and reduction of disease uncertainty. Lower limb DVT patients expected to receive support from healthcare professionals, families, and portable and simplified reporting formats to promote their participation in PROs. From the patient 's own perspective, they perceived that insufficient attention to DVT caused by lack of awareness of the hazards of DVT and limited understanding of PROs made it difficult for them to participate in PROs. Conclusions:Lower limb DVT patients have a high intention to participate in PROs. The application and promotion of PROs in lower limb DVT patients in China require joint support from various sectors of society, among which strengthening effective health education for patients and their families and providing multi-channel reporting forms are the key to promoting of PROs.

Objective:To construct a tool to evaluate the knowledge, attitude, and practice of nurses in the prevention of thrombosis related to peripherally inserted central catheter (PICC) .Methods:From December 2022 to April 2023, based on the theory of knowledge, attitude, and practice, a preliminary draft of the PICC-related Thrombosis Prevention Knowledge, Attitude, and Practice Questionnaire for Nurses was formed through systematic literature review, two rounds of Delphi expert consultations, and pre-survey. In May 2023, convenience sampling was used to select 573 nurses from Chinese Academy of Medical Sciences & Peking Union Medical College Hospital as the research subject for a survey to conduct item analysis, validity testing, and reliability testing on the questionnaire.Results:The final version of the PICC-related Thrombosis Prevention Knowledge, Attitude, and Practice Questionnaire for Nurses included 20 knowledge items, 10 attitude items, and 10 practice items. In the content validity of the questionnaire, the average content validity index was 0.984, the overall consensus content validity index was 0.850, and the item level content validity index was 0.857 to 1.000. Exploratory factor analysis extracted three common factors with eigenvalues>1.000, with a cumulative variance contribution rate of 64.540% and factor loadings of 0.450 to 0.908 for each item. The total Cronbach's α coefficient of the questionnaire was 0.895, the half reliability coefficient was 0.947, and the retest reliability coefficient was 0.966.Conclusions:The PICC-related Thrombosis Prevention Knowledge, Attitude, and Practice Questionnaire for Nurses has good reliability and validity, and can be used to evaluate PICC-related thrombosis prevention knowledge, attitude, and practice among nurses.

Quality-sensitive indicators in nursing, as standards for evaluating nursing quality management, can quantitatively determine nursing outcomes. This article provides a comprehensive review of the framework and content of quality-sensitive indicators for venous thromboembolism (VTE) prevention nursing care. The indicators are discussed from three dimensions: structure, process, and outcome. The aim is to provide a reference for the future development of unified and standardized quality-sensitive indicators for VTE prevention nursing care, in order to guide clinical nurses in standardized preventive practices.

Cancer-associated thrombosis (CAT) is a common complication and cause of death in cancer patients. Accurately and efficiently identifying high-risk groups for CAT using risk assessment models and implementing targeted early prevention is key. Although numerous CAT risk assessment models currently exist, their predictive capabilities vary across different populations. This article provides a comprehensive review of CAT risk assessment models and their application status, aiming to offer a reference for clinical nursing staff to understand and choose appropriate risk assessment tools.

Objective:To develop the Cancer-associated Thrombosis (CAT) Prevention Knowledge, Attitude and Practice Questionnaire for Nurses, and conduct reliability and validity tests.Methods:This study was a questionnaire development study. Guided by the theory of knowledge, attitude, and practice, an initial CAT Prevention Knowledge, Attitude, and Practice Questionnaire for Nurses was formed through literature review, group discussion, two rounds of expert consultation, and pre-survey. From April to May 2023, convenience sampling was used to select 700 in-service nurses from Chinese Academy of Medical Sciences & Peking Union Medical College as the research subject, and the reliability and validity of the questionnaire were tested.Results:A total of 700 questionnaires were distributed, and 593 valid questionnaires were collected, with a valid response rate of 84.71% (593/700). The final version of the CAT Prevention Knowledge, Attitude, and Practice Questionnaire for Nurses included three dimensions of knowledge, attitude, and practice, with a total of 39 items. The scale-level content validity index ( S- CVI) was 1.000, with an average S- CVI of 0.971 and an item-level CVI (I- CVI) of 0.860 to 1.000. Exploratory factor analysis extracted a total of seven common factors, with a cumulative variance contribution rate of 77.376%. The total Cronbach's α coefficient of the questionnaire was 0.937, and the Cronbach's α coefficients for each dimension were 0.923, 0.987, and 0.979. The half reliability coefficient was 0.984. After two weeks, the reliability coefficients for each dimension of retesting were 0.959, 1.000, and 0.994. Conclusions:The CAT Prevention Knowledge, Attitude, and Practice Questionnaire for Nurses developed in this study has good reliability and validity, and can be used as an evaluation tool for the cognition of clinical nurses in CAT prevention.

To analyze the differences in cerebral blood flow (CBF) characteristics among children with different subtypes of attention deficit and hyperactivity disorder (ADHD) and their relationship with executive function using arterial spin labeling (ASL) technology.

[Objective]To evaluate the efficacy of Yiqi Yangyin Quyu Prescription in the treatment of primary Sj?gren's syndrome(pSS),and study the epigenetic markers related to the efficacy of traditional Chinese medicine through whole genome bisulfate sequencing(WGBS)technology.[Methods]Fifty patients with pSS were included,including 30 in Chinese medicine group and 20 in western medicine group.Clinical information before treatment and 12 weeks after treatment was evaluated,and the therapeutic effect was analyzed.Three effective patients were selected from Chinese medicine group and their whole blood samples before and after Chinese medicine treatment were subjected to WGBS.Through methylation site analysis,gene ontology(GO)of differentially methylated genes,Kyoto Encyclopedia of Genes and Genomes(KEGG),and molecular signature database(MSigDB)enrichment analysis,efficacy-related differentially methylated genes and pathways were identified.[Results]Compared with pre-Chinese medicine treatment group,the group treated with Chinese medicine after 12 weeks showed a significant decrease in Sj?gren's syndrome disease activity index(SSDAI),erythrocyte sedimentation rate(ESR),and immunoglobulin G(IgG)level(P<0.05).Compared with pre-western medicine treatment group,the SSDAI and ESR levels in western medicine treatment group after 12 weeks decreased significantly(P<0.05).There was no statistically significant difference in SSDAI,ESR,and other indicators between the group treated with Chinese medicine after 12 weeks and the group treated with western medicine after 12 weeks(P>0.05).WGBS identified a total of 29 differentially methylated regions(DMRs),mainly concentrated at the sites of repetitive elements and protein coding genes,covering a total of 68 genes,including three RNA categories:mRNA,long noncoding RNA(lncRNA),and circRNA.Enrichment analysis suggested that the therapeutic effect was closely related to pathways such as antigen processing and presentation,interferon related pathways,and helper T cell(Th)1 and Th2 cell differentiation.[Conclusion]Yiqi Yangyin Quyu Prescription can regulate DNA methylation,thereby inhibiting the immune response in patients with pSS and exerting therapeutic effects.

Objective To explore the correlation between serum levels of miR-193a-3p,activated transcription factor 5(ATF5),clinicopathological characteristics and chemotherapy efficacy in patients with triple negative breast cancer(TNBC).Methods A total of 120 patients with TNBC admitted to our hospital were collected as the research group.In the same period,120 cases with benign breast disease in our hospital were selected as the control group.Serum levels of miR-193a-3p and ATF5 were detected,and the relationship between them and clinicopathological characteristics were detected in two groups.According to the therapeutic effect,TNBC patients were divided into the treatment ineffective group(n=50)and the treatment effective group(n=70).The expression levels of miR-193a-3p and ATF5 were compared between the two groups,and factors affecting the chemotherapy efficacy of TNBC patients were analyzed.Results Compared with before chemotherapy,the serum miR-193a-3p level increased and ATF5 level decreased in TNBC patients after chemotherapy(P＜0.05).Compared with the control group,the serum miR-193a-3p level of TNBC patients decreased in the research group before chemotherapy,and ATF5 level increased(P＜0.05).The expression level of miR-193a-3p was lower and the expression level of ATF5 was higher in patients with tumor diameter≥3 cm,lymph node metastasis,low histological grade,clinical stage Ⅲ and Ki-67＞30%(P＜0.05).In TNBC patients,compared with the treatment effective group,patients in the treatment ineffective group showed a decreased serum miR-193a-3p level and an increased ATF5 level(P＜0.05).Lower level of miR-193a-3p,higher level of ATF5,lymph node metastasis,tumor diameter≥3 cm,low histological grade,and TNM stage Ⅲ were risk factors affecting the efficacy of chemotherapy in TNBC patients(P＜0.05).Conclusion Low level of miR-193a-3p and high level of ATF5 in the serum of TNBC patients are risk factors for chemotherapy efficacy.