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.

The seco-prezizaane-type sesquiterpenes (SPS), as a special class of sesquiterpenes with a highly oxidative five-ring cage structure and seven consecutive chiral centers, are isolated from the genus Illicium, which have a variety of biological activities, including neurotoxicity and neurotrophic effects, etc. This review summarizes the chemical constituents and pharmacological effects of SPS, and discusses the potential trend and scope of future research.

Objective:To explore the combination of high risk screening and family screening for potential patients with Fabry disease in adult hemodialysis population, and to improve the diagnostic efficiency of the disease.Methods:It was a cross-sectional investigation study. High-risk screening for Fabry disease was performed on adult hemodialysis patients with end-stage kidney disease who were admitted to Yongkang First People's Hospital of Zhejiang Province between November 2022 and February 2023. Dry blood paper α-galactosidase A (α-Gal A) detection assay was performed in males, or glycosphingolipids (Lyso-GL-3) detection assay was performed in females. GLA genetic assay was performed for further diagnosis after abnormal screening results. Family screening was carried out on the family members of the confirmed Fabry disease patients, and α-Gal A activity and Lyso-GL-3 of peripheral blood were measured. Additionally, urine routine, blood biochemistry, eye examination, hearing test, cranial magnetic resonance imaging, and electrocardiogram were performed to assess organ damage. Results:Among 244 hemodialysis patients, 139 (56.97%) were males and 105 (43.03%) were females. The age ranged from 25 to 81 years (with median age of 61 years). One female patient with Fabry disease was identified GLA IVS4+919G>A mutation, resulting in a total prevalence of 0.41%. Pedigree screening was conducted on 41 family members of the patient, leading to the confirmation of 12 patients (including the proband), including 3 males and 9 females. Among them, 9 patients were abnormal in enzyme examination, 10 patients were abnormal in substrate, and 11 patients were abnormal in gene sequencing. None of the 12 patients exhibited limb pain, hypohidrosis, angiokeratoma, corneal opacity, and hearing impairment. Eight patients had heart abnormalities. Nine patients had abnormal urine routine (albuminuria or hematuria) and one patient had abnormal renal function. Four patients had abnormal cranial magnetic resonance imaging findings. Conclusions:One GLA IVS4+919G>A mutation family is successfully identified through the combination of high-risk screening and family screening in adult hemodialysis patients, with a total of 12 cases of Fabry disease. The combination of high-risk screening and family screening proves to be effective in detecting potential patients with Fabry disease, and improve the screening efficiency of Fabry disease.

Objective:By analyzing the relationship between the efficiency of traditional Chinese medicine services and the efficiency of comprehensive medical services in county-level traditional Chinese medicine hospitals in Shandong,it explores the path of coordinated development between traditional Chinese medicine and western medicine in traditional Chinese medicine hospitals,providing references for the high-quality development of county-level traditional Chinese medicine hospitals.Methods:The efficiency of traditional Chinese medicine services and comprehensive medical services in county-level traditional Chinese medicine hospitals in Shandong Province from 2019 to 2022 was analyzed by Data Envelopment Analysis(DEA),and the correlation was analyzed by Spearman correlation coefficient.Results:The efficiency of traditional Chinese medicine services in county-level traditional Chinese medicine hospitals in Shandong Province is lower than that of comprehensive medical services,and the scale return coefficient of traditional Chinese medicine category indicators is better than that of comprehensive category indicators.There is a positive correlation between the efficiency of traditional Chinese medicine services and the efficiency of comprehensive medical services.Conclusion:Traditional Chinese medicine hospitals should appropriately expand their investment in traditional Chinese medicine resources.Adhere to the hospital management model of traditional Chinese medicine services as the main focus,and build a coordinated development path between traditional Chinese and western medicine from both the supply and demand sides.

Objective:By analyzing the relationship between the efficiency of traditional Chinese medicine services and the efficiency of comprehensive medical services in county-level traditional Chinese medicine hospitals in Shandong,it explores the path of coordinated development between traditional Chinese medicine and western medicine in traditional Chinese medicine hospitals,providing references for the high-quality development of county-level traditional Chinese medicine hospitals.Methods:The efficiency of traditional Chinese medicine services and comprehensive medical services in county-level traditional Chinese medicine hospitals in Shandong Province from 2019 to 2022 was analyzed by Data Envelopment Analysis(DEA),and the correlation was analyzed by Spearman correlation coefficient.Results:The efficiency of traditional Chinese medicine services in county-level traditional Chinese medicine hospitals in Shandong Province is lower than that of comprehensive medical services,and the scale return coefficient of traditional Chinese medicine category indicators is better than that of comprehensive category indicators.There is a positive correlation between the efficiency of traditional Chinese medicine services and the efficiency of comprehensive medical services.Conclusion:Traditional Chinese medicine hospitals should appropriately expand their investment in traditional Chinese medicine resources.Adhere to the hospital management model of traditional Chinese medicine services as the main focus,and build a coordinated development path between traditional Chinese and western medicine from both the supply and demand sides.

Objective:By analyzing the relationship between the efficiency of traditional Chinese medicine services and the efficiency of comprehensive medical services in county-level traditional Chinese medicine hospitals in Shandong,it explores the path of coordinated development between traditional Chinese medicine and western medicine in traditional Chinese medicine hospitals,providing references for the high-quality development of county-level traditional Chinese medicine hospitals.Methods:The efficiency of traditional Chinese medicine services and comprehensive medical services in county-level traditional Chinese medicine hospitals in Shandong Province from 2019 to 2022 was analyzed by Data Envelopment Analysis(DEA),and the correlation was analyzed by Spearman correlation coefficient.Results:The efficiency of traditional Chinese medicine services in county-level traditional Chinese medicine hospitals in Shandong Province is lower than that of comprehensive medical services,and the scale return coefficient of traditional Chinese medicine category indicators is better than that of comprehensive category indicators.There is a positive correlation between the efficiency of traditional Chinese medicine services and the efficiency of comprehensive medical services.Conclusion:Traditional Chinese medicine hospitals should appropriately expand their investment in traditional Chinese medicine resources.Adhere to the hospital management model of traditional Chinese medicine services as the main focus,and build a coordinated development path between traditional Chinese and western medicine from both the supply and demand sides.

Objective:By analyzing the relationship between the efficiency of traditional Chinese medicine services and the efficiency of comprehensive medical services in county-level traditional Chinese medicine hospitals in Shandong,it explores the path of coordinated development between traditional Chinese medicine and western medicine in traditional Chinese medicine hospitals,providing references for the high-quality development of county-level traditional Chinese medicine hospitals.Methods:The efficiency of traditional Chinese medicine services and comprehensive medical services in county-level traditional Chinese medicine hospitals in Shandong Province from 2019 to 2022 was analyzed by Data Envelopment Analysis(DEA),and the correlation was analyzed by Spearman correlation coefficient.Results:The efficiency of traditional Chinese medicine services in county-level traditional Chinese medicine hospitals in Shandong Province is lower than that of comprehensive medical services,and the scale return coefficient of traditional Chinese medicine category indicators is better than that of comprehensive category indicators.There is a positive correlation between the efficiency of traditional Chinese medicine services and the efficiency of comprehensive medical services.Conclusion:Traditional Chinese medicine hospitals should appropriately expand their investment in traditional Chinese medicine resources.Adhere to the hospital management model of traditional Chinese medicine services as the main focus,and build a coordinated development path between traditional Chinese and western medicine from both the supply and demand sides.

Objective:By analyzing the relationship between the efficiency of traditional Chinese medicine services and the efficiency of comprehensive medical services in county-level traditional Chinese medicine hospitals in Shandong,it explores the path of coordinated development between traditional Chinese medicine and western medicine in traditional Chinese medicine hospitals,providing references for the high-quality development of county-level traditional Chinese medicine hospitals.Methods:The efficiency of traditional Chinese medicine services and comprehensive medical services in county-level traditional Chinese medicine hospitals in Shandong Province from 2019 to 2022 was analyzed by Data Envelopment Analysis(DEA),and the correlation was analyzed by Spearman correlation coefficient.Results:The efficiency of traditional Chinese medicine services in county-level traditional Chinese medicine hospitals in Shandong Province is lower than that of comprehensive medical services,and the scale return coefficient of traditional Chinese medicine category indicators is better than that of comprehensive category indicators.There is a positive correlation between the efficiency of traditional Chinese medicine services and the efficiency of comprehensive medical services.Conclusion:Traditional Chinese medicine hospitals should appropriately expand their investment in traditional Chinese medicine resources.Adhere to the hospital management model of traditional Chinese medicine services as the main focus,and build a coordinated development path between traditional Chinese and western medicine from both the supply and demand sides.

Objective:By analyzing the relationship between the efficiency of traditional Chinese medicine services and the efficiency of comprehensive medical services in county-level traditional Chinese medicine hospitals in Shandong,it explores the path of coordinated development between traditional Chinese medicine and western medicine in traditional Chinese medicine hospitals,providing references for the high-quality development of county-level traditional Chinese medicine hospitals.Methods:The efficiency of traditional Chinese medicine services and comprehensive medical services in county-level traditional Chinese medicine hospitals in Shandong Province from 2019 to 2022 was analyzed by Data Envelopment Analysis(DEA),and the correlation was analyzed by Spearman correlation coefficient.Results:The efficiency of traditional Chinese medicine services in county-level traditional Chinese medicine hospitals in Shandong Province is lower than that of comprehensive medical services,and the scale return coefficient of traditional Chinese medicine category indicators is better than that of comprehensive category indicators.There is a positive correlation between the efficiency of traditional Chinese medicine services and the efficiency of comprehensive medical services.Conclusion:Traditional Chinese medicine hospitals should appropriately expand their investment in traditional Chinese medicine resources.Adhere to the hospital management model of traditional Chinese medicine services as the main focus,and build a coordinated development path between traditional Chinese and western medicine from both the supply and demand sides.

Objective To explore the relationship between perceived stress and suicidal ideation as well as the mediating effect of resilience and depression.Methods A sample of 875 college students was recruited for the study to complete a set of self-report measures of Chinese Perceived Stress Scale(CPSS),the Resiliency Scale of University Students,Suicidal Ideation Questionnaire,and Beck Depression Inventory(BDI).Results ①College students'perceived stress and depression had significantly positive correlation with their suicidal ideation,while the students'resilience had a significantly negative correlation with their perceived stress and depression.②Perceived stress did not affect suicidal ideation of the college students directly,but indirectly affected their suicidal ideation through three significant mediating pathways:the separate mediating effects of(a)resilience and(b)depression,and the serial mediating effect of(c)resilience and depression.Conclusion Resilience and depression exert a chain mediating effect between perceived stress and suicidal ideation of college students.