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: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.

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 analyse the safety and feasibility of laparoscopic techniques in the treatment of elderly patients with acute intestinal obstruction.Methods A prospective two-center single-arm clinical study was conducted.A total of 441 patients with acute intestinal obstruction treated at the department of general surgery of Longyou County People's Hospital and Hospital Affiliated to Qinghai University from October 2017 to October 2021 and met the inclusion criteria were selected as study subjects.Among them,10 patients had chronic obstructive pulmonary disease(COPD)and were given mechanical ventilation,noninvasive ventilation,continuous low-flow oxygen inhalation,nebulization inhalation,and bronchodilator treatment after surgery.Twelve patients had chronic kidney disease(CKD)and were given hemodialysis treatment after surgery.All 441 patients underwent laparoscopic exploration,small intestine decompression,and definitive surgery.After surgery,they received intravenous nutrition,antibiotics,and subcutaneous injection of low-molecular-weight heparin(10 U/kg).The blood gas analysis(for patients with COPD),renal function(for patients with CKD),weight,body mass index(BMI),complications,and prognosis were observed before surgery,after surgery,and at discharge.Results There were 441 elderly patients with intestinal obstruction in the study,including 240 males and 201 females.The mean duration of the disease was(74.2±6.4)hours,and the BMI was(25.4±6.7)kg/m2.A total of 280 patients with adhesions underwent adhesion lysis surgery,while 87 patients with small bowel necrosis and 19 patients with small bowel lipoangiomatosis underwent small bowel resection and anastomosis.Additionally,21 patients with obturator hernia underwent hernia repair surgery,and 34 patients with small bowel persimmon stones underwent small bowel incision and stone removal surgery.For the 10 patients with COPD,there was a significant increase in arterial partial pressure of oxygen[PaO2 mmHg(1 mmHg≈0.133 kPa):80.3±3.3 vs.72.6±2.7,P<0.01],and a significant decrease in arterial partial pressure of carbon dioxide(PaCO2)compared to preoperative levels(mmHg:35.7±3.6 vs.47.6±1.3,P<0.01).Four patients died of type Ⅱ respiratory failure,and 6 patients had a significant decrease in PaO2[(77.4±6.7)mmHg]and a significant increase in PaCO2[(42.1±5.2)mmHg]at discharge compared to postoperative levels(both P<0.01).Among the 12 patients with CKD,both blood urea nitrogen(BUN)and serum creatinine(SCr)increased significantly after surgery compared to preoperative levels[BUN(μmol/L):79.5±8.5 vs.18.8±4.7,SCr(μmol/L):312.7±12.1 vs.138.4±9.7,both P<0.01].Two patients died of renal failure,and 8 patients had a significant decrease in BUN[(9.2±0.7)μmol/L]and SCr[(112.5±3.8)μmol/L]at discharge compared to postoperative levels(both P<0.01).There were no significant differences in BMI and weight between preoperative,postoperative,and discharge times[BMI(kg/m2):25.4±6.7,24.9±3.9 vs.23.9±3.5;weight(kg):74.2±6.8,73.7±3.3 vs.72.8±4.6;all P>0.05].A total of 435 patients recovered from acute intestinal obstruction with no complications such as lower extremity venous thrombosis,intestinal fistula,wound dehiscence,or wound infection.Conclusion The strategy of treating elderly patients with acute intestinal obstruction by laparoscopic exploration,intestinal decompression,definitive surgery,treatment of underlying diseases,and subcutaneous injection of low-molecular-weight heparin is safe and feasible.

Objective To compare the impact of continuous profound neuromuscular blockade versus con-ventional neuromuscular blockade on postoperative shoulder pain in patients undergoing robot-assisted laparoscopic surgery during steep Trendelenburg position.Methods This study was a single-center,randomized,double-blind clinical trial.The inclusion criteria encompassed individuals aged between 18 and 80 years,with an American Society of Anesthesiologists status of Ⅰ or Ⅱ,and a body mass index ranging from 18 kg/m2 to 30 kg/m2.A total of one hundred patients were randomly assigned to either the deep neuromuscular blockade group(D group)or the conventional neuromuscular blockade group(C group),with equal distribution of fifty cases in each group.Rocuronium dosage was titrated to achieve post-tetanic count values of 1～2 and train-of-four stimulation levels of 1～2 during surgery for D and C groups respectively.At the end of surgery,sugammadex was administered for reversal of neuromuscular blockade.The primary endpoint assessed the incidence of postoperative shoulder pain within three days after surgery.Secondary endpoints included Leiden score evaluation during intraoperative period,number of additional neuromus-cular blockers required by the surgeon,recovery time for muscle relaxation postoperatively,nausea and vomiting scores during recovery phase,visual analog scale(VAS)scores in Post-Anesthesia Care Unit(PACU)as well as within three days after surgery,incidence rate for postoperative pulmonary complications,length of hospital stay duration and patient satisfaction score.Results The incidence of postoperative shoulder pain was significantly lower in group D compared to group C(D group 32%vs.C group 56%;P<0.05).However,there were no significant differences in postoperative shoulder pain VAS scores between the two groups(P>0.05).No significant differences were observed between the groups in terms of Leiden score,surgeon's requirement for additional neuromuscular blockers,nausea and vomiting in PACU,and VAS score(P>0.05).Group D exhibited better early postoperative activity pain scores than group C(P<0.05).There were no significant differences in VAS scores between the groups at other time points(P>0.05).Furthermore,there were no significant differences in the incidence of postoperative pulmonary complications,length of stay,and satisfaction scores between the two groups.Conclusion The imple-mentation of continuous deep neuromuscular blockade in patients undergoing robot-assisted laparoscopic surgery with steep Trendelenburg position can effectively mitigate the occurrence of postoperative shoulder pain.