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 analyze the distribution characteristics of fluoride and trichloromethane in drinking water in rural schools in Guizhou Province and assess their health risks, so as to provide a scientific basis for ensuring the safety of drinking water in rural schools. Methods: During the dry season (March to May) and wet season (July to September) of 2020 to 2022, 788 rural primary and secondary schools in agricultural counties (districts) in Guizhou Province were selected for investigation by using a direct sampling method. A total of 1 566 drinking water samples were collected from these schools, and the mass concentrations of fluoride and trichloromethane in the water samples were detected. The Mann-Whitney U test was used for intergroup comparison, and a health risk assessment model was employed to evaluate the health risks of students oral intake of fluoride and trichloromethane. Results: From 2020 to 2022, the mass concentrations of fluoride and trichloromethane in the drinking water of rural schools in Guizhou Province all met the standards, and the ranges were no detection to 0.99 mg/L and (no detection to 0.06)×10 -3 mg/L, respectively. The mass concentrations of fluoride in dry and wet seasons were 0.05(0.05,0.10), 0.05(0.05,0.10) mg/L, the mass concentrations of trichloromethane were [0.02(0.02,1.00)]×10 -3 , [0.02(0.02,1.00)]×10 -3 mg/L, the mass concentrations of fluoride in factory water and terminal water were 0.05(0.05,0.05), 0.05(0.05,0.10) mg/L, and the differences were not statistically significant ( Z=-0.04, -0.88, - 0.98 , P >0.05). There was a statistically significant difference in the mass concentration of trichloromethane between factory water and peripheral water [0.02(0.02,0.02)×10 -3 , 0.02(0.02,1.05)×10 -3 mg/L]( Z=-2.16, P < 0.05 ). The non-carcinogenic risk assessment values for students oral exposure to fluoride and trichloromethane were in the range of 0.01(0.01,0.03)-0.03(0.03,0.06) and [0.26( 0.26 ,14.54)]×10 -4 -[0.52(0.52,48.62)]×10 -4 , respectively, all of which were at acceptable levels; the carcinogenic risk assessment values for oral exposure to trichloromethane were in the range of [0.08(0.08, 4.51 )]×10 -7 -[0.16(0.16,15.07)]×10 -7 , indicating a low risk. Conclusions The health risks of students expore to fluoride and trichloromethane in drinking water in rural schools of Guizhou Province are low. It is necessary to strengthen the standardized management of disinfection in some rural drinking water projects and the monitoring of fluoride in water sources to reduce the exposure risk to children.

Nasal drug delivery efficiency is highly dependent on the position in which the drug is deposited in the nasal cavity. However, no reliable method is currently available to assess its impact on delivery performance. In this study, a biomimetic nasal model based on three-dimensional (3D) reconstruction and three-dimensional printing (3DP) technology was developed for visualizing the deposition of drug powders in the nasal cavity. The results showed significant differences in cavity area and volume and powder distribution in the anterior part of the biomimetic nasal model of Chinese males and females. The nasal cavity model was modified with dimethicone and validated to be suitable for the deposition test. The experimental device produced the most satisfactory results with five spray times. Furthermore, particle sizes and spray angles were found to significantly affect the experimental device's performance and alter drug distribution, respectively. Additionally, mometasone furoate (MF) nasal spray (NS) distribution patterns were investigated in a goat nasal cavity model and three male goat noses, confirming the in vitro and in vivo correlation. In conclusion, the developed human nasal structure biomimetic device has the potential to be a valuable tool for assessing nasal drug delivery system deposition and distribution.

Objective: The study aims to investigate the impact of moderate intensity gymnastics on the development of executive function in children aged 5-6, thereby providing a theoretical foundation for exercise interventions targeting executive function. Methods: A total of 63 preschool children, randomly seleted from 3 senior classes in a private kindergarten in Shangqiu, were randomly allocated to the intervention group ( n =31) and control group ( n =32). Children in the intervention group participated in 60 minute gymnastics at a moderate intensity, three times per week, for a total duration of 12 weeks. Concurrently, myzone technology was utilized to monitor exercise intensity throughout the entire intervention period. Children in the control group maintained their regular activities. Inhibitory control (Flanker task), working memory (Empty house task), and cognitive flexibility (Dots task) were assessed before and after the experiment. Results: There was no statistically significant difference in the performance of inhibitory control, working memory, and cognitive flexibility tasks between the two groups of children before intervention ( P >0.05) .The results of covariance analysis revealed significant differences in reaction time [(782.88±24.29，805.13±23.74；719.90±119.99， 833.55± 177.87；1 042.39±72.75，1 091.29±49.42) ms] and accuracy[(73.86±7.26)%，(67.02±8.22)%;(86.36±7.63)%，( 80.50± 9.39 )%;(76.45±9.48)%，(69.59±7.66)%] across inhibitory control, working memory, and cognitive flexibility between the intervention group and the control group ( F =6.84, 4.50，4.87, 6.11, 3.74 , 5.06 , P <0.05). The intervention effect exhibited modest effects( d =0.17-0.74). Conclusions Moderate intensity gymnastics can make modest or moderate effect on improving children s executive function. Brain imaging technology can be incorporated into future research designs to investigate the underlying mechanisms of gymnastics impact on the brain structure and executive function in young children.

BACKGROUND:The treatment of distal tibial fractures with soft tissue injury has always been challenging,and the new retrograde tibial nailing is a new choice.Up to now,there were few reports on the biomechanical properties between the new retrograde tibial nailing,anterograde intramedullary nailing and supercutaneous locking plate. OBJECTIVE:To explore the biomechanical stability of new retrograde tibial nailing,antegrade intramedullary nailing and supercutaneous locking plate in the treatment of distal tibial fractures with soft tissue injury using finite element analysis so as to offer a scientific foundation for clinical application. METHODS:The finite element model of transverse distal tibia fracture was established by relevant software utilizing the CT data of the tibia from a 42-year-old healthy male.Retrograde tibial nailing,antegrade intramedullary nailing and supercutaneous locking plate finite element models were assembled under the principle of fracture fixation.Finally,meshing,applying loads,and data processing were accomplished with the ANSYS 2019 software.Moreover,the stress distribution and displacement of the tibia and internal fixation of each model were compared. RESULTS AND CONCLUSION:(1)The displacement of fracture end in the three groups increased with the increase of load.In all mode loads,the average displacement of the fracture end was the least in the retrograde tibial nailing group,followed by the supercutaneous locking plate group,and the highest in the antegrade intramedullary nailing group.At 800 N vertical load,the displacement difference of the fracture end was statistically significant(P<0.05).There was no statistical significance in other load modes.(2)Under different loads,the tibial stress in the three groups was the highest in the middle of the tibia,and gradually decreased to the proximal and distal ends.The stress distribution of the tibial shaft was the highest in the retrograde tibial nailing group,followed by the supercutaneous locking plate group,and the least in the antegrade intramedullary nailing group.(3)Under different loads,the stress of the tibial stress raiser in the three groups was significantly higher in the supercutaneous locking plate group than in the other two groups,with statistical significance(P<0.05).(4)Under different loads,the stress of the fixators in the three groups was the largest in the supercutaneous locking plate group,followed by the retrograde tibial nailing group,and the minimum in the antegrade intramedullary nailing group.There were significant differences in the stress of fixator stress raiser among the three groups under different loading modes(P<0.05).(5)It is indicated that all three fixation methods have the good anti-rotation ability and axial stability.Retrograde tibial nail shows better biomechanical stability.

BACKGROUND:The treatment of distal tibial comminuted fractures with soft tissue injury has always been challenging.The new retrograde tibial nailing and supercutaneous locking plate are important treatment methods,but their strain and stress shielding at the fracture end during different periods of fracture healing and different load conditions have not been reported. OBJECTIVE:To explore the biomechanical stability of retrograde tibial nailing and supercutaneous locking plate in different periods of fracture healing by finite element analysis to offer a scientific foundation for clinical application and rehabilitation exercise. METHODS:The finite element model of distal tibial comminuted fracture was established by utilizing the CT data of the tibia from a 40-year-old healthy male.Retrograde tibial nailing,supercutaneous locking plate,and callus models were assembled in accordance with the principle of fracture fixation.The finite element analysis was performed using ANSYS software to compare the displacement of the fracture end,the stress shielding of the tibia,the stress of the callus,and the stress distribution of the tibia and the fixation device during different periods of fracture healing. RESULTS AND CONCLUSION:(1)The relative displacement of the tibial fracture decreased gradually with the healing of the fracture,and the displacement decreased significantly after 3 months.At 0 and 1 months after operation,the vertical displacement and total displacement of the supercutaneous locking plate group were higher than those in the retrograde intramedullary nail group.The Z-axis displacement(horizontal medial and lateral displacement)of the two fixation methods was more obvious than the X-axis and Y-axis,and the Z-axis displacement of supercutaneous locking plate group was the most obvious.The maximum Z-axis displacement of the two fixation methods was located on the outside of the tibia,and the minimum displacement was located on the inside of the tibia.(2)The stress shielding rate at different periods of fracture healing gradually decreased with time.The stress shielding rate of the retrograde intramedullary nail was higher than that of the supercutaneous locking plate at different stages of fracture healing.After 3 months,the stress shielding rate of the supercutaneous locking plate was reduced to about 4%,and the stress shielding rate of the retrograde intramedullary nail was reduced to about 40%.(3)The stress of the stress concentration site of the callus in the two fixation methods increased with the increase of the load,and the stress of the callus in the supercutaneous locking plate group was always greater than that in the retrograde intramedullary nail group.The maximum stress distribution of the callus was approximately equally distributed among the two modes of fixation,both in the lateral portion of the tibia.(4)As the fracture healed,the maximum stress of the tibia in the two groups decreased gradually,and the stress in the supercutaneous locking plate group was always greater than that in the retrograde intramedullary nail group.The average stress of the maximum stress area of the tibia in the supercutaneous locking plate group under 1 500 N load was 285 MPa,while that in the retrograde intramedullary nail group was 26 MPa.(5)As the fracture healed,the stress of the fixation device in the two groups decreased gradually,and the stress in the supercutaneous locking plate group was significantly higher than that in the retrograde intramedullary nail group.After 3 months,the stress of the two fixation devices decreased significantly.(6)It is indicated that in the early stage of fracture healing,the strain on the fracture end in the retrograde intramedullary nail group is small,and the maximum stress of the tibia is moderate,allowing early loaded.The fractured ends in the supercutaneous locking plate group had too large strain and too large maximum stress of the tibia,which needed to be partially loaded under protection and could not be fully loaded.In the middle and late stages of fracture healing,the tibial retrograde intramedullary nail and the supercutaneous locking plate could be completely loaded,and the stress shielding rate of the supercutaneous locking plate was significantly lower than the tibial retrograde intramedullary nail.

OBJECTIVE To investigate the relationship between the course of antibiotics during perioperative period and postoperative infection in pregnant women with human immunodeficiency virus/acquired immune deficiency syndrome(HIV/AIDS) undergoing cesarean section, and to guide the rational use of antibiotics in clinical practice. METHODS A total of 53 cesarean section women in the hospital from January 2014 to September 2022 were chosen as research objects. According to the course of antibiotics, the puerperae were divided into two groups. The treatment course of antibiotics in the study group(n=31) was >24 h and ≤48 h, and the control group(n=22) was ≤24 h. The postoperative wound healing, inflammatory indexes, postoperative infection rate, and postoperative hospital stay were observed. RESULTS There was no significant difference in postoperative body temperature, white blood cell count, neutrophil ratio, C-reactive protein, postoperative hospital stay, wound healing grade and postoperative infection rate between the study group and the control group. CONCLUSION Antibiotics for HIV/AIDS patients during perioperative period of cesarean section for <24 h may play a better role in preventing postoperative infection.

Aural vertigo frequently encountered in the otolaryngology department of traditional Chinese medicine （TCM） mainly involves peripheral vestibular diseases of Western medicine， such as Meniere's disease， benign paroxysmal positional vertigo， vestibular neuritis， and vestibular migraine， being a hot research topic in both TCM and Western medicine. Western medical therapies alone have unsatisfactory effects on recurrent aural vertigo， aural vertigo affecting the quality of life， aural vertigo not relieved after surgery， aural vertigo with complex causes， and children's aural vertigo. The literature records and clinical practice have proven that TCM demonstrates unique advantages in the treatment of aural vertigo. The China Association of Chinese medicine sponsored the "17th youth salon on the diseases responding specifically to TCM： Aural vertigo" and invited vertigo experts of TCM and Western medicine to discuss the difficulties and advantages of TCM diagnosis and treatment of aural vertigo. The experts deeply discussed the achievements and contributions of TCM and Western medicine in the diagnosis and treatment of aural vertigo， the control and mitigation of the symptoms， and the solutions to disease recurrence. The discussion clarified the positioning and advantages of TCM treatment and provided guidance for clinical and basic research on aural vertigo.

Objective:To evaluate the feasibility, safety and efficacy of indocyanine green (ICG) fluorescence imaging navigation combined with modified pancreaticogastrostomy in laparoscopic duodenum-preserving pancreatic head resection.Methods:The clinical data of 14 patients undergoing laparoscopic duodenum-preserving pancreatic head resection with indocyanine green fluorescence navigation technique combined with modified pancreaticogastrostomy from January 2019 to January 2022 in the Department of Hepatobiliary and Pancreatic Surgery, the First Hospital of Hebei Medical University were retrospectively analyzed, including 4 males and 10 females, aged (40.6±7.1) years. The operation time, intraoperative blood loss, postoperative complications and follow-up were recorded.Results:All 14 operations were successfully completed, and there was no intraoperative ICG allergic reaction. The intraoperative ICG staining facilitated the identification of common bile duct. The operation time was (325.71±23.00) min, and the time of modified pancreaticogastrostomy was (18.32±1.52) min. Intraoperative blood loss was 200 (150, 300) ml. There were no case of intraoperative blood transfusion. Postoperative complications include three cases of grade A pancreatic fistula, one of biliary fistula, and one of biliary stricture. All patients were followed up for one to 18 months, and the median follow-up time was 10 months. One patient had intermittent fever after operation. Magnetic resonance cholangiopancreatography showed biliary stricture in one patient one month after operation, which was managed by endoscopic biliary stent implantation.Conclusion:Indocyanine green fluorescence navigation is safe and feasible in laparoscopic duodenum-preserving pancreatic head resection. Combined with modified pancreaticogastrostomy, it helps skip the jejunal anastomosis and improve the efficiency of surgery.

Objective To explore the candidate genes and potential molecular mechanisms of anti-neutrophil cyto-plasmic antibodies(ANCA)-associated vasculitis by bioinformatics and experimental validation,and to provide a scientific theoretical basis for the treatment of potential inflammatory targets for ANCA-associated vasculitis.Meth-ods The GSE108109 chip data was retrieved from the Gene Expression Omnibus(GEO)database,and the differ-ential genes were processed,analyzed and screened using the R language related program package.Kyoto encyclo-pedia of genes and genomes(KEGG)and gene ontology(GO)enrichment analysis was carried out using DAVID online network cable,and the interaction network of the protein encoded by the selected genes of inflammatory syn-drome was constructed through STRING website.Further endogenous competitive RNA(ceRNA)regulatory net-work was predicted and constructed through miRWalk and DIANA-LncBase databases,and key genes were screened from the network to draw ROC curve.The renal biopsy samples of patients with ANCA-associated vasculi-tis confirmed by our hospital were collected as the experimental group,and the renal biopsy samples of IgA ne-phropathy and micro-adaptive nephropathy were collected as the control group.Immunohistochemical staining was performed on the collected renal biopsy samples,and the average optical density was calculated by semi-quantita-tive analysis of immunohistochemical staining to further verify the expression of the key genes screened by the bioin-formatics analysis.Pearson linear correlation analysis was performed between the average optical density results and the clinical inflammatory data of patients.Results 846 differential genes were screened,of which 444 genes were significantly up-regulated and 402 genes were significantly down-regulated.Through KEGG and GO analysis,im-portant differentially expressed genes related to inflammation regulation were obtained.Among them,CSF1R and TNFRSF1B,two differentially expressed genes never reported in ANCA-associated vasculitis,attracted our atten-tion.At the same time,we constructed multiple ceRNA regulatory axes including KCNQ1OT1-hsa-miR-125a-5p-TNFRSF1B.There were 15 samples of ANCA-associated vasculitis,6 samples of IgA nephropathy,and 3 samples of micropathological kidney.Immunohistochemical results of renal biopsy specimens showed that the expression of CSF1R and TNFRSF1B in ANCA-associated vasculitis kidney tissue was higher than that in the control group.Pearson correlation analysis of clinical data of patients in ANCA group showed that the expression of CSF1R was positively correlated with the content of neutrophil count(r=0.587),and the expression of TNFRSF1B was posi-tively correlated with the content of serum C-reactive protein(r=0.646).Conclusion Key genes related to in-flammatory regulation such as CSF1R and TNFRSF1B were investigated by bioinformatics methods,and a rigorous ceRNA regulatory network was constructed.The expression of CSF1R and TNFRSF1B in ANCA vasculitis was high-er than that in the control group through immunohistochemistry.The results provides a scientific theoretical basis for the molecular mechanism of inflammation,and laid a good foundation for new therapeutic targets of ANCA-related vasculitis for inflammation.