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 investigate the epidemiological characteristics of dengue fever in Shenzhen City in 2024, so as to provide insights into formulation of the preventive and control measures for dengue fever. Methods The epidemiological data of dengue cases reported in Shenzhen City in 2024 were extracted from the China Disease Prevention and Control Information System and field epidemiological survey data of dengue fever in Shenzhen City, and the temporal, regional and population distributions of dengue fever cases, source of acquire dengue virus infections, disease diagnosis and treatment and outbreaks were analyzed. The dengue virus nucleic acid was tested and the serotypes of dengue virus were characterized using real-time quantitative reverse transcription PCR (RT-qPCR) assay, and the dengue virus gene was sequenced using next-generation sequencing (NGS). In addition, the surveillance on the density of Aedes albopictus was performed using Breteau index (BI) and mosquito oviposition index (MOI). Results A total of 1 735 dengue fever cases were reported in Shenzhen City in 2024, including 952 local cases and 783 imported cases. Most imported dengue fever cases acquired infections from eight cities of Foshan, Guangzhou, Zhongshan, Jiangmen, Dongguan, Zhaoqing, Huizhou, and Zhuhai in the Pearl River Delta region (664 cases, 84.8% of total imported cases) into Baoan, Longgang, and Nanshan districts. The epidemic exhibited an early onset and rapid progression, peaking during the period between September and November (1 632 cases, 94.1% of total cases), and dengue fever cases were distributed across 73 subdistricts in 10 districts, with most cases reported in densely populated central and western regions. The dengue fever cases had a male-to-female ratio of 1.9∶1.0, and a median age of 37 (21) years, with a higher median age among local cases than among imported cases [40 (20) years vs. 33(15) years; Z = -10.30, P < 0.05]. Housework, unemployment, workers, and business service were predominant occupations (1 405 cases, 81.0% of total cases), and there was a significant difference in the constituent ratio of occupations between local and imported cases (χ² = 92.30, P < 0.05). Among the 1 735 dengue fever cases, the median duration from onset to definitive diagnosis was 3.3 (2.9) days, and 1 686 cases (97.2%) were identified in healthcare facilities, with a low rate of hospitalization and isolation seen in 1 701 inpatients with available epidemiological data (485 cases, 28.5% of total inpatients). A total of 29 outbreaks of dengue fever occurred in Shenzhen City across 2024, which primarily in construction sites (27 outbreaks, 93.1% of total). Dengue virus type I was the dominant serotype causing dengue fever in Shenzhen City in 2024. Sequencing showed that the genomes of dengue virus from multiple dengue fever cases in Shenzhen City shared a high sequence homology with those from cities neighboring Shenzhen City, and there might be intra-city transmission of dengue virus among multiple construction sites in Shenzhen City. The Aedes albopictus density was significantly higher in Shenzhen City in 2024 than in 2023, peaking from May to September. The annual MOI values ranged from 0.9 to 14.0, and the BI values ranged from 0.6 to 6.0. Conclusions The overall epidemic of dengue fever was severe in Shenzhen City in 2024, which was greatly affected by case importation from neighboring cities, construction sites-centered local transmission, and the effectives of routine mosquito vector control was not satisfactory. Integrated dengue fever control measures should be implemented, focusing on regional joint prevention and control mechanisms, capacity building for mosquito vector control, addressing challenges in epidemic containment at construction sites, and strengthening case detection and management systems.

OBJECTIVE: To compare the effect of acupuncture-moxibustion on idiopathic facial palsy (IFP) at acute phase and recovery phase. METHODS: According to whether received acupuncture-moxibustion at acute phase or not, 198 IFP patients were divided into an early-phase intervention group (118 cases) and a non-early-phase intervention group (80 cases). With the propensity score matching employed, 70 cases were included in each group. On the basis of the conventional treatment of western medicine, acupuncture-moxibustion was supplemented in the two groups. In the early-phase intervention group, acupuncture-moxibustion was delivered at the acute phase (duration of illness≤7 days); in the non-early-phase intervention group, acupuncture-moxibustion was operated at the recovery phase (duration of illness>7 days). At the acute phase, warm needling was performed at Yifeng (TE17), Xiaguan (ST7), Hegu (LI4) and Zusanli (ST36) on the affected side; and at the recovery phase, electroacupuncture was delivered at Cuanzhu (BL2), Sizhukong (TE23) and Yangbai (GB14), etc. on the affected side, with the disperse-dense wave and 2 Hz/100 Hz of frequency. The intervention was operated for 30 min each time, once every two days, three treatments weekly and for 4 weeks. Before treatment, 1 week and 4 weeks of treatment, the House-Brackmann (H-B) facial nerve function grade, the score of Sunnybrook facial nerve function, and the score of facial disability index (FDI) were compared between the two groups. The clinical effect in 1 and 4 weeks of treatment and safety were evaluated. RESULTS: In 1 and 4 weeks of treatment, the H-B grade was improved when compared with that before treatment in each group (P<0.05), and in 4 weeks of treatment, H-B grade in the early-phase intervention group was superior to that of the non-early-phase intervention group (P<0.05). In 1 and 4 weeks of treatment, Sunnybrook scores and the scores of physical function of FDI were elevated in comparison with those before treatment in the two groups (P<0.05); and in 4 weeks of treatment, the elevation of these two indexes in the early-phase intervention group was greater than that of the non-early-phase intervention group (P<0.05). In 4 weeks of treatment, the scores of social function in FDI were reduced when compared with those before treatment in the two groups (P<0.05). In 4 weeks of treatment, the total effective rate (97.1%, 68/70) in the early-phase intervention group was higher than that (87.1%, 61/70) of the non-early-phase intervention group (P<0.05). There was no significant difference in the incidence of adverse events between the two groups (P>0.05). CONCLUSION Acupuncture-moxibustion therapy starting at the acute phase is more beneficial to the functional recovery of the impaired facial nerve than at the recovery phase in the IFP patients.
Humans ; Female ; Male ; Acupuncture Therapy ; Moxibustion ; Adult ; Middle Aged ; Young Adult ; Acupuncture Points ; Treatment Outcome ; Facial Paralysis/therapy* ; Cohort Studies ; Aged ; Bell Palsy/therapy* ; Adolescent

Objective To study the death status of pancreatic cancer among residents in Xuhui district, Shanghai, from 1992 to 2021, and analyze its trends of change, so as to provide evidence for the prevention and treatment of pancreatic cancer. Methods Based on the database of Shanghai death registration system from 1992 to 2021, the crude mortality rate, standardized mortality rate, age-specific mortality rate and other indicators of pancreatic cancer among registered residents in Xuhui district were calculated. The Joinpoint software was used to analyze the trends of average annual percent change (AAPC) of pancreatic cancer mortality rate, and the age-period-cohort model was used to analyze the age effect, period effect and birth cohort effect pairs significant changes in pancreatic cancer mortality. Results In 2021, the mortality rate of pancreatic cancer in Xuhui district, Shanghai, ranked fourth among malignant tumors, and the winning rate and world standard rate of the whole population, males and females were 8.34/100 000 (8.81/100 000, 7.98/100 000) and 7.28/100 000 (7.69/100 000, 6.96/100 000), respectively, with males higher than females. AAPC of crude mortality rate and the standardized (6) mortality rate were higher in males than that in females. The age-specific mortality rate increased with the increase of age, and the highest mortality rate was found in 60-84 years old group. The age-period-cohort model showed that from 1992 to 2021, the annual net shift of pancreatic cancer mortality among the whole population, male and female residents in Xuhui district, Shanghai, was 1.22%, 1.58%, 1.15% (P=0.20, 0.19, 0.45) respectively, and the time trend was not significant. From the perspective of age effect, the risk of death from pancreatic cancer in the whole population and with age deviation in males had an obvious trend with increasing age (P<0.05), while the age effect in females had no obvious trend. From the perspective of period effect, no period deviation was significant in the whole population, males and females (P>0.05). In terms of cohort effects, there were significant differences in the whole population and the male cohort deviations(P<0.05). No significant cohort effect was observed in the female population. Conclusions The mortality rate of pancreatic cancer among registered residents in Xuhui district, Shanghai from 1992 to 2021, was on the rise, especially in the 60-84 years old group and male. The prevention and control of pancreatic cancer needs to develop effective epidemic prevention measures for corresponding populations.

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 investigate the clinical phenotype and genetic characteristics of patients with Fabry disease caused by a GLA variant, IVS4+919G>A.Methods:It was a prospective study. Fabry disease screening was conducted among high-risk population in Ninghai from October 2021 to August 2023. Those children with decreased α-galactosidase enzyme activity<2.40 μmol/(L·h) or elavated Lyso-GL-3 level>1.10 μg/L in dried blood spot (DBS) method underwent GLA genetic testing for diagnosis confirmation. Meanwhile, family screening was carried out. A proband and his family members diagnosed with Fabry disease were research subjects. The clinical and genetic characteristics of patients with Fabry disease caused by the GLA variant (IVS4+919G>A) were analyzed.Results:The female proband aged 9.8 years with pain in both lower limbs as the initial symptom was found to have a heterozygous GLA variant IVS4+919G>A among 102 patients. In family screening, there were 4 family members (proband's father, elder sister, elder male cousin and elder female cousin) with Fabry disease and a family member (proband's fifth aunt) with a GLA variant. Among these 4 diagnosed family members, the elder male cousin of the proband, a boy aged 13.2 years had a heterozygous GLA variant, IVS4+919G>A with intermittent pain in both lower limbs as the initial symptom. The proband′s father had knee joint pain. The proband′s elder sister had decreased vision and his elder female cousin had no obvious symptoms. The proband′s fifth aunt with a GLA variant had decreased vision.Conclusions:High-risk screening in children and family screening are helpful for early diagnosis and treatment of Fabry disease. Neuropathic pain may be a early symptom in children with Fabry disease caused by the GLA variant, IVS4+919G>A.

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.

Over the past decade, mitochondrial dysfunction has been investigated as a key contributor to acute and chronic kidney disease. However, the precise molecular mechanisms linking mitochondrial damage to kidney disease remain elusive. The recent insights into the cyclic guanosine monophosphate-adenosine monophosphate (GMP-AMP) synthetase (cGAS)-stimulator of interferon gene (STING) signaling pathway have revealed its involvement in many renal diseases. One of these findings is that mitochondrial DNA (mtDNA) induces inflammatory responses via the cGAS-STING pathway. Herein, we provide an overview of the mechanisms underlying mtDNA release following mitochondrial damage, focusing specifically on the association between mtDNA release-activated cGAS-STING signaling and the development of kidney diseases. Furthermore, we summarize the latest findings of cGAS-STING signaling pathway in cell, with a particular emphasis on its downstream signaling related to kidney diseases. This review intends to enhance our understanding of the intricate relationship among the cGAS-STING pathway, kidney diseases, and mitochondrial dysfunction.

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.