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.

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:Most of the studies on combined orthodontic-orthognathic treatment of skeletal class Ⅲ malocclusions have focused on the improvement of the patient's lateral appearance and recovery in the later stages of the treatment,while there are fewer studies observing the microcosmic nature of the alveolar bone remodeling of the lower anterior teeth. OBJECTIVE:To evaluate the therapeutic effect of lower anterior tooth decompensation and alveolar bone remodeling in patients with skeletal class Ⅲ malocclusion before and after orthodontic-orthognathic treatment based on oral X-ray lateral films and oral cone-beam CT. METHODS:From January 2015 to May 2023,15 patients with skeletal class Ⅲ malocclusion who underwent orthodontic-orthognathic surgery at Qingdao Hospital of Rehabilitation University were enrolled.All patients underwent lateral cephalography and cone beam computed tomography before and after treatment.Cephalometric measurement items related to the angle and line distance,lip/lingual bone cracking length(d-La/d-Li)and bone cracking/bone fenestration of the lower anterior teeth before and after treatment were measured. RESULTS AND CONCLUSION:Lateral X-ray films showed that the amount of alveolar bone remodeling after decompensation of the lower anterior teeth showed significant changes compared to before treatment.The root of the tooth moved significantly towards the center of the alveolar bone,and the specific data was closer to normal data,but there were still some differences compared with normal individuals.Based on the cone-beam CT measurement,the bone cracking/bone fenestration length and width of the alveolar bone were improved in almost all the teeth after orthodontic-orthognathic combined treatment,alveolar bone remodeling in some teeth even reached the level of healthy individuals.Before treatment,most patients often experienced bone fenestration/cracking on the lip/lingual side of the lower incisor due to compensatory tooth growth.However,during the preoperative orthodontic stage,decompensation triggered alveolar bone remodeling and significant changes in tooth angle.Preoperative orthodontic treatment caused the upper anterior teeth to retract and the lower anterior teeth to tilt and control the root,but the amount of decompensation before surgery was often insufficient.In the orthognathic surgery stage,the jaw was removed through the positioning guide plate,the maxilla moved forward,and the mandible retreated.During the postoperative orthodontic process,the effect of fine adjustment was better.Although there is a certain degree of recurrence trend in the position of teeth and jawbones,the postoperative orthodontic treatment is closer to the normal value.

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.

The department of critical care medicine, the clinical base of critical care medicine, is the centralized management unit for the treatment of critical patients. Medicine and nursing are independent disciplines, but both can be fully utilized from different perspectives when dealing with critically ill patients. With the development of critical care medicine understanding and technology, the concept of doctor-nurse integration construction in the department of critical care medicine has emerged. Doctor-nurse integration construction in the department of critical care medicine needs to take severe diseases as the core and academic development as the orientation. Clinical practice should be carried out through medical cooperation, based on critical cognition and unified thinking mode.

ObjectiveTo understand the death status of major chronic diseases among the registered residents in Xuhui District， Shanghai from 2007 to 2021， and to analyze the probability and trend of premature death， so as to provide an evidence for the policy-making of disease control and prevention. MethodsBased on the data from the death registration system of the registered residents in Xuhui District， Shanghai from 2007 to 2021， the mortality rate of major chronic diseases， life expectancy without cause of death， potential years of life lost （PYLL）， average years of life lost （AYLL）， and premature death rate were calculated. Joinpoint 4.8 software was used to calculate the annual percentage change （APC） of premature death rate and its trend. ResultsIn 2021， the mortality rate and standardized mortality rate of four major chronic diseases， namely cardiovascular and cerebrovascular diseases， malignant tumors， diabetes mellitus and chronic lower respiratory diseases among the registered residents in Xuhui District， Shanghai was 767.75/100 000 and 234.69/100 000， respectively， accounting for 85.63% of the total causes of death among the residents in Xuhui District， Shanghai. The PYLL， AYLL and PYLL rate were 10 710.00 person-years， 1.49 years per person， and 11.43‰， respectively. Life expectancy increased by 14.91 years after removing the four major chronic diseases. From 2007 to 2021， the premature death rate of chronic diseases among the registered residents in Xuhui District decreased from 9.38% to 7.91% （APC=-1.09%，P<0.05）. Malignant tumors had the highest rate in premature death， and the premature death rate was higher in males than that in females， with a slower rate of decline than in females. Cardiovascular and cerebrovascular diseases， malignant tumors and chronic lower respiratory diseases showed a significant decreasing trend in premature death rate （APC=-1.30%， -1.20%， -5.42%， P<0.05）， while diabetes mellitus showed a significant increasing trend in premature death rate （APC=3.20%， P<0.05）. There was a significant increasing trend in the rate of premature death from diabetes mellitus in males （APC=5.15%， P<0.05） and a decreasing trend in the rate of premature death from diabetes mellitus in females （APC=-0.76%， P<0.05）. ConclusionCardiovascular and cerebrovascular diseases， malignant tumors， diabetes mellitus and chronic lower respiratory diseases are the main causes of death among the registered residents in Xuhui District. The probability of premature death of the residents in Xuhui District from major chronic diseases is at a low level， indicating that the prevention and control of chronic diseases in Xuhui District has achieved some success， but it is still difficult to realize the goals of the Outline of the "Healthy China 2030" Plan. Therefore， it is necessary to comprehensively carry out the control of risk factors such as tobacco use， harmful alcohol consumption， salt intake， hypertension， hyperglycemia and obesity， especially to strengthen the control of risk factors for male residents， as well as early diagnosis and treatment of malignant tumors， cardiovascular and cerebral vascular diseases and diabetes mellitus， and to implement the disease management mechanism to reduce premature death.

From June 16 to 30, 2023, men who have sex with men (MSM) who had visited Voluntary Counseling Testing (VCT) clinics in the Luohu, Futian and Nanshan districts of Shenzhen were included in this study to analyze their awareness of Mpox and the influencing factors. The mean age of the 262 MSM was (34.78±8.94) years, with the majority being unmarried (75.2%) and 79.0% confirmed to be infected with HIV. The awareness rates for five primary indicators, current status of Mpox, pathogen and source of infection, mode of transmission, population susceptibility, clinical manifestations and treatment were 68.4%, 84.7%, 60.3%, 87.8%, and 52.5%, respectively. The awareness rates for five secondary indicators, earliest transmission location (44.7%), main mode of transmission (54.2%), role of masks (46.9%), drug accessibility (46.6%), and self-limiting nature (38.2%) were all below 60%. The MSM population in Shenzhen perceived their likelihood of being infected (2.76±1.32) and discriminated against (3.87±1.26) as relatively low. The logistic analysis showed that the high school or vocational school education ( OR:3.094, 95 %CI:1.180-9.299), college or above education ( OR:5.360, 95% CI:2.159-15.501), and higher scores on questions affecting learning or work ( OR:2.196, 95% CI:1.409-3.599) were promoting factors for Mpox awareness, while higher scores on questions concerning the possibility of Mpox mortality ( OR:0.591, 95% CI:0.432-0.791) was the hindering factor for Mpox awareness.

Objective To construct a perioperative exercise program for older frail patients with colorectal cancer,and to verify its effect.Methods Based on Goal-directed Theory and literature review,expert consultation was carried out to establish the perioperative exercise program for older frail patients with colorectal cancer.Then,from July to December 2022,the perioperative exercise program was established for older frail patients with colorectal cancer hospitalized in the General Surgery Department of a tertiary A hospital in Suzhou by convenience sampling method for preliminary application.It was divided in to the intervention group and the control group by the ward.The intervention group was applied with the constructed perioperative exercise program on the basis of routine perioperative nursing.The safety,feasibility and intervention effect of the program were evaluated.Results 15 experts were included for 2 rounds of expert consultations.The authority coefficient was 0.880;the coefficient of variation was＜0.250;the Kendall concordance coefficient was 0.167 and 0.224,respectively.The final program contained 4 stages:preoperative exercise,postoperative bed rest,sitting and standing exercise.In the end,54 specific interventions were constructed.During the preliminary application,all the patients completed the program.Generalized estimation equation showed that,on the interaction effect,there was no statistical difference(P=0.752);there were significant differences on the intervention effect and the time effect(P＜0.05).At discharge,there were significant differences in timed up and go test and Barthel Index between the 2 groups(P＜0.05).Only 2 patients experienced mild pain in the intervention group,and no other adverse events occurred.Conclusion The perioperative exercise program for older frail patients with colorectal cancer,based on Goal-directed Theory,is scientific,reliable and safe.It has been preliminarily verified in improving postoperative physical function,activities of daily living and early postoperative recovery of elderly frail patients with colorectal cancer.

From June 16 to 30, 2023, men who have sex with men (MSM) who had visited Voluntary Counseling Testing (VCT) clinics in the Luohu, Futian and Nanshan districts of Shenzhen were included in this study to analyze their awareness of Mpox and the influencing factors. The mean age of the 262 MSM was (34.78±8.94) years, with the majority being unmarried (75.2%) and 79.0% confirmed to be infected with HIV. The awareness rates for five primary indicators, current status of Mpox, pathogen and source of infection, mode of transmission, population susceptibility, clinical manifestations and treatment were 68.4%, 84.7%, 60.3%, 87.8%, and 52.5%, respectively. The awareness rates for five secondary indicators, earliest transmission location (44.7%), main mode of transmission (54.2%), role of masks (46.9%), drug accessibility (46.6%), and self-limiting nature (38.2%) were all below 60%. The MSM population in Shenzhen perceived their likelihood of being infected (2.76±1.32) and discriminated against (3.87±1.26) as relatively low. The logistic analysis showed that the high school or vocational school education ( OR:3.094, 95 %CI:1.180-9.299), college or above education ( OR:5.360, 95% CI:2.159-15.501), and higher scores on questions affecting learning or work ( OR:2.196, 95% CI:1.409-3.599) were promoting factors for Mpox awareness, while higher scores on questions concerning the possibility of Mpox mortality ( OR:0.591, 95% CI:0.432-0.791) was the hindering factor for Mpox awareness.