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 explore the independent influencing factors of kinesiophobia in pa-tients after total knee arthroplasty and analyze the effect of rehabilitation training under the informa-tion-motivation-behavioral skills(IMB)model.Methods A total of 385 patients undergoing primary unilateral total knee arthroplasty were selected,and the independent influencing factors of kinesiopho-bia in these patients after total knee arthroplasty were analyzed.Eighty-nine patients with kinesiopho-bia were divided into control group(n=44)and observation group(n=45)according to random number table method.The control group received routine rehabilitation intervention,while the obser-vation group received rehabilitation intervention under the IMB model.The application effect of reha-bilitation intervention under the IMB model in patients with kinesiophobia after total knee arthroplasty was analyzed.Results Multivariate Logistic regression analysis showed that the analgesic effect,ad-verse reactions to anesthetics,preoperative anxiety level,preoperative depression level,postoperative self-efficacy,and postoperative family care level were all independent influencing factors of kinesio-phobia in patients after total knee arthroplasty(P＜0.05).Compared with before intervention,at 1 month and 3 months after intervention,the Tampa Scale of Kinesiophobia(TSK)scores and Numeri-cal Rating Scale(NRS)scores of both groups decreased,while the Knee Society Score(KSS)and rehabilitation exercise compliance scores increased(P＜0.05).Compared with the control group,at 1 month and 3 months after intervention,the TSK scores and NRS scores of the observation group were significantly lower,while the KSS and rehabilitation exercise compliance scores were signifi-cantly higher(P＜0.05).Conclusion There are multiple influencing factors for kinesiophobia in patients after total knee arthroplasty.Clinically,the implementation of rehabilitation training pro-gram under the IMB model is conducive to changing postoperative rehabilitation behaviors,impro-ving kinesiophobia and pain levels after total knee arthroplasty,and enhancing knee function and re-habilitation training compliance.

Objective To explore the model of decreased ovarian reserve(DOR)induced by experimental autoimmune thyroiditis(EAT)in mice and investigate the protective mechanism of Jiexu Zichong Granules(ZCWS).Methods Ninety-six Kunming female mice were randomly divided into the J control group,BIW control group,J model group,and BIW model group.The mice were immunized with antigen(0.1 mg,once or twice per week)combined with high iodine water(0.64 g/L)for 7-19 weeks to validate the model.Subsequently,30 mice were randomly divided into the ZCWS group,model group,and control group.The DOR model was established by immunization with the antigen(0.2 mg per week)combined with high iodine water feeding for 13 weeks.The ZCWS group received oral administration of traditional Chinese medicine suspension(0.64 g/mL),while the remaining groups received an equivalent volume of physiological saline for 7 weeks.Thyroid and ovarian tissue morphology and related indicators were detected using HE staining,ELISA,IHC,and Western blot.Results Antigen immunization for 13 weeks(0.1-0.2 mg per week)combined with high iodine water feeding stably constructed the EAT DOR model.ZCWS reduced thyroid lymphocyte infiltration,follicular structure destruction,and serum TPOAb and TGAb levels(P<0.01).It inhibited MDA activity(P<0.01),increased GSH-Px and SOD activities(P<0.05),increased primordial,primary,and secondary follicles(P<0.05),and reduced atretic follicles(P<0.01).ZCWS upregulated AMH and downregulated FSH(P<0.01),regulated the expression of Bcl-2,Bax,and Caspase-3 proteins in ovarian tissue,downregulated Keap1 protein(P<0.01),and upregulated Nrf2(P<0.01),HO-1,and LONP1 proteins(P<0.05).It upregulated Bcl-2 protein(P<0.05),downregulated Bax(P<0.01),and Caspase-3(P<0.05)proteins,downregulated Keap1 protein(P<0.01),and upregulated Nrf2(P<0.01),HO-1,and LONP1 proteins(P<0.05)in ovarian tissue.Conclusion ZCWS improves ovarian reserve function in EAT mice by activating the Nrf2 pathway,inhibiting oxidative stress,and reducing follicular atresia.

Public hospitals have formed a relatively perfect working foundation in the introduction and training of young talents,but the evaluation system of young talents is not perfect.Based on the requirements of high-quality development,grasp the principle of party management of talents,combine the talents development situation in Jiading District Maternal and Child Health Care Hospital,takes the special training of young talents in administration as the starting point,comprehensively uses the literature method,interview method and Delphi method to establish the index database,uses the exploratory factor analysis meth-od to calculate the index weight,and constructs the evaluation model of young talents in hospital administrative management,so as to help hospitals better screen and evaluate talents and give full play to the value and role of talents as the first resource.

Objective To analyze the international research hotspots and development trends of social work intervention in chronic disease management,providing reference for domestic research on social work intervention in chronic disease manage-ment.Methods The research literature was retrieve on the core collection of Web of Science,articles published from the estab-lishment of the database from 2000 to 2024.CiteSpace were used to present the keyword co-occurrence,keyword clustering,au-thor collaboration network,and institutional collaboration network of social work intervention in chronic disease management.Results The post-documentation situation showed a fluctuating upward trend as a whole.The keywords that appeared frequently were"social support""management""quality of life""chronic disease"and"Care".There was multi-agency and multi-author collaboration.Conclusion Internationally,social work intervention in chronic disease management focuses on social support and quality of life.The research cooperation system in related fields is diversified,and social workers play an active role in the inter-disciplinary teams for chronic disease management.

Objective:To explore the predictive value of preoperative frailty assessment using the Geriatric 8 (G8) screening tool for postoperative complications in elderly patients with radical prostatectomy.Methods:In this prospective cohort study, 145 elderly patients who underwent radical prostatectomy in the Urology Department of the Affiliated Central Hospital of Jiangnan University between September 2023 and March 2024 were selected using convenience sampling method. Preoperatively, frailty was assessed using the G8 screening tool. Postoperatively, complications within 30 days were evaluated using the Clavien-Dindo classification. The predictive value of the G8 frailty score for postoperative complications was analyzed using the receiver operating characteristic (ROC) curve.Results:The preoperative frailty incidence among the 145 elderly patients with radical prostatectomy was 43.45% (63/145). The postoperative complication rate was 63.49% (40/63) in the frail patients and 13.41% (11/82) in the non-frail patients, with a statistically significant difference (χ 2=39.184, P<0.01). After adjusting for age, smoking history, polypharmacy, albumin, tumor stage, International Prostate Symptom Score, and activities of daily living, the risk of postoperative complications in the frail patients was 6.011 times higher than in the non-frail patients [ OR=6.011, 95% CI (2.177, 16.594) ]. The area under the ROC curve for G8 frailty score in predicting postoperative complications was 0.798 [95% CI (0.723, 0.873) ] . Conclusions:Elderly patients undergoing radical prostatectomy with frailty have a higher incidence of postoperative complications. G8 screening tool has good efficacy in predicting the risk of postoperative complications in elderly prostate cancer patients.

Public hospitals have formed a relatively perfect working foundation in the introduction and training of young talents,but the evaluation system of young talents is not perfect.Based on the requirements of high-quality development,grasp the principle of party management of talents,combine the talents development situation in Jiading District Maternal and Child Health Care Hospital,takes the special training of young talents in administration as the starting point,comprehensively uses the literature method,interview method and Delphi method to establish the index database,uses the exploratory factor analysis meth-od to calculate the index weight,and constructs the evaluation model of young talents in hospital administrative management,so as to help hospitals better screen and evaluate talents and give full play to the value and role of talents as the first resource.

Objective To analyze the international research hotspots and development trends of social work intervention in chronic disease management,providing reference for domestic research on social work intervention in chronic disease manage-ment.Methods The research literature was retrieve on the core collection of Web of Science,articles published from the estab-lishment of the database from 2000 to 2024.CiteSpace were used to present the keyword co-occurrence,keyword clustering,au-thor collaboration network,and institutional collaboration network of social work intervention in chronic disease management.Results The post-documentation situation showed a fluctuating upward trend as a whole.The keywords that appeared frequently were"social support""management""quality of life""chronic disease"and"Care".There was multi-agency and multi-author collaboration.Conclusion Internationally,social work intervention in chronic disease management focuses on social support and quality of life.The research cooperation system in related fields is diversified,and social workers play an active role in the inter-disciplinary teams for chronic disease management.

Objective To explore the model of decreased ovarian reserve(DOR)induced by experimental autoimmune thyroiditis(EAT)in mice and investigate the protective mechanism of Jiexu Zichong Granules(ZCWS).Methods Ninety-six Kunming female mice were randomly divided into the J control group,BIW control group,J model group,and BIW model group.The mice were immunized with antigen(0.1 mg,once or twice per week)combined with high iodine water(0.64 g/L)for 7-19 weeks to validate the model.Subsequently,30 mice were randomly divided into the ZCWS group,model group,and control group.The DOR model was established by immunization with the antigen(0.2 mg per week)combined with high iodine water feeding for 13 weeks.The ZCWS group received oral administration of traditional Chinese medicine suspension(0.64 g/mL),while the remaining groups received an equivalent volume of physiological saline for 7 weeks.Thyroid and ovarian tissue morphology and related indicators were detected using HE staining,ELISA,IHC,and Western blot.Results Antigen immunization for 13 weeks(0.1-0.2 mg per week)combined with high iodine water feeding stably constructed the EAT DOR model.ZCWS reduced thyroid lymphocyte infiltration,follicular structure destruction,and serum TPOAb and TGAb levels(P<0.01).It inhibited MDA activity(P<0.01),increased GSH-Px and SOD activities(P<0.05),increased primordial,primary,and secondary follicles(P<0.05),and reduced atretic follicles(P<0.01).ZCWS upregulated AMH and downregulated FSH(P<0.01),regulated the expression of Bcl-2,Bax,and Caspase-3 proteins in ovarian tissue,downregulated Keap1 protein(P<0.01),and upregulated Nrf2(P<0.01),HO-1,and LONP1 proteins(P<0.05).It upregulated Bcl-2 protein(P<0.05),downregulated Bax(P<0.01),and Caspase-3(P<0.05)proteins,downregulated Keap1 protein(P<0.01),and upregulated Nrf2(P<0.01),HO-1,and LONP1 proteins(P<0.05)in ovarian tissue.Conclusion ZCWS improves ovarian reserve function in EAT mice by activating the Nrf2 pathway,inhibiting oxidative stress,and reducing follicular atresia.