Objective:To investigate the clinical manifestations of medically refractory hereditary movement disorders in children and the efficacy of deep brain stimulation (DBS).Methods:A case series study.The clinical and follow-up data of 20 children with medically refractory hereditary movement disorders who underwent DBS treatment at the Neurology and Functional Neurosurgery Departments of Beijing Children′s Hospital, Capital Medical University, from July 2018 to April 2024, were retrospectively analyzed.The severity of movement disorder symptoms and surgical effects were evaluated using the Burke-Fahn-Marsden Dystonia Rating Scale Movement(BFMDRS-M) or the Unified Parkinson′s Disease Rating Scale Ⅲ(UPDRS Ⅲ).Results:There were 12 males and 8 females among the 20 children, with an onset age ranging from 4 months to 12 years and 5 months.Fourteen patients had hereditary dystonia, which is related to KMT2B in 11 patients, TOR1A in 2 patients and SGCE in 1 patient.Two patients had choreoathetosis, which is related to ADCY5-related familial movement disorders.Two patients had early-onset Parkinson′s disease, which is related to ATP6AP2 in 1 patient and VPS13C in 1 patient.Two patients had neurodevelopmental disorders with involuntary movements, which is related to GNAO1 in 1 patient, and the other patient was idiopathic.All the children were given oral Levodopa, Benzhexol, Baclofen, Tiapride Hydrochloride, Clonazepam alone or in combination.Three children showed obvious dyskinesia after Levodopa treatment.The symptoms of movement disorders in all children exhibited little to no improvement.Levetiracetam and Zonisamide had unstable effects in the treatment of myoclonia.DBS surgery was performed on all the patients aged from 3 to 16 years.Electrodes were successfully inserted into bilateral globus pallidus internus in 14 cases and bilateral subthalamic nuclei in 4 cases.The target was unknown in 2 cases.No surgery-related complications were observed.The patients were followed up for 3 months to 6 years, and the last follow-up age of the patients ranged from 5 years and 7 months to 22 years and 1 month.The rate of improvement in BFMDRS-M score was 37%-100% in 16 patients and >70% in 7 patients with hereditary dystonia.The rate of improvement in UPDRS Ⅲ score was 23% in 1 patient with VPS13C-related early-onset Parkinson′s disease. Conclusions:Childhood medically refractory hereditary movement disorders are a case series that exhibits significant phenotypic and genotypic heterogeneity.DBS surgery demonstrates significant efficacy for KMT2B-, TOR1A-, and SGCE-related hereditary movement disorders.

Objective:To investigate the impact of chromate exposure on pulmonary function indices in occupational populations and explore the potential role of alterations in inflammatory indicators in this process.Methods:In July 2024, A cross-sectional analysis was conducted using occupational health examination data of 30875 workers from chromate-related enterprises in Jiangsu Province in 2020 and 2021. Based on the occupational positions and whether there is chromium acid salt exposure in the occupational hazards of the research subjects over the years, they are divided into chromium acid salt exposure group and non-exposure group. For those exposed to chromium acid salts, based on job position descriptions and duration of chromium acid salt exposure, they are further categorized into intermittent exposure group and continuous exposure group; among them, the actual exposure time in the intermittent exposure group is less than half of the working shift time, and the exposure duration is less than the total working life. Pulmonary function test indicators include forced vital capacity (forced vital capacity, FVC) %, first-second forced expiratory volume (forced expiratory volume in one second, FEV 1.0) %, and the ratio of first-second forced expiratory volume to forced vital capacity (FEV 1.0/FVC) %. Peripheral blood samples from the upper limbs of the research subjects were collected on an empty stomach for routine blood tests, selecting neutrophil count, platelet count, and lymphocyte count results, calculating the neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR). Quantitative data that meet normal distribution are expressed using Mean±SD, while variables that do not meet normal distribution are represented by M ( Q1, Q3). Categorical data are expressed using frequency or proportion (%). Linear variable comparisons between groups use t-tests. Generalized linear models were employed to assess the correlation between chromate exposure and pulmonary function indices, while mixed regression models were utilized to explore potential underlying effects. Results:Compared with the non-exposed group, the pulmonary function indices FVC (%), FEV 1.0/FVC (%), and FEV 1.0 (%) in the chromate-exposed group showed a decreasing trend ( P<0.01), indicating statistically significant differences. As the frequency of chromate exposure increased, the prevalence of pulmonary dysfunction rose. The prevalence rates of obstructive, restrictive, and mixed respiratory dysfunction among the chromate-exposed population (including intermittent exposure and continuous exposure groups) were 0.26%, 4.67%, and 0.12%, which were higher than those in the non-exposed group (0.09%, 0.84%, and 0.07%, respectively). All these differences were statistically significant ( P<0.05). After stratification by gender, a negative correlation was observed between chromate exposure and the inflammatory indicator platelet-to-lymphocyte ratio (PLR) ( P<0.05). Potential effect analysis revealed that PLR played a certain mediating role between chromate exposure and the decline in pulmonary function indices, with a mediating proportion of 2.2%. Conclusion:Chromate exposure in occupational populations may lead to a decline in pulmonary ventilatory function and alterations in peripheral blood inflammatory indicators. Inflammatory indicators may be involved in the pulmonary function decline caused by chromate exposure.

Objective:By systematically evaluate the implementation of GBZ 2.1, so as to provide technical basis for the future revisions of this standard.Methods:From May to October 2023, Based on the pre survey questionnaire, the semi-structured interview method was used to interviews with experts from CDC, Occupational disease prevention and control hospital institutes, employers, occupational hygiene technical service intermediaries and universities, and the inductive method was used to extract the topics and relevant suggestions.Results:Generally, GBZ 2.1 is scientific, practical, progressiveness and operable. There are still some issues such as OELs overlapping and the correspondence between Chinese and English names. The outstanding problem is the coordination with other standard contents.Conclusion:The technical indicators in GBZ 2.1 could adapt to the needs of current practical work. The coordination between standards needs to be clarified, and the new recommended content needs further promotion and exploration on how to implement it.

Objective:To investigate the clinical manifestations of medically refractory hereditary movement disorders in children and the efficacy of deep brain stimulation (DBS).Methods:A case series study.The clinical and follow-up data of 20 children with medically refractory hereditary movement disorders who underwent DBS treatment at the Neurology and Functional Neurosurgery Departments of Beijing Children′s Hospital, Capital Medical University, from July 2018 to April 2024, were retrospectively analyzed.The severity of movement disorder symptoms and surgical effects were evaluated using the Burke-Fahn-Marsden Dystonia Rating Scale Movement(BFMDRS-M) or the Unified Parkinson′s Disease Rating Scale Ⅲ(UPDRS Ⅲ).Results:There were 12 males and 8 females among the 20 children, with an onset age ranging from 4 months to 12 years and 5 months.Fourteen patients had hereditary dystonia, which is related to KMT2B in 11 patients, TOR1A in 2 patients and SGCE in 1 patient.Two patients had choreoathetosis, which is related to ADCY5-related familial movement disorders.Two patients had early-onset Parkinson′s disease, which is related to ATP6AP2 in 1 patient and VPS13C in 1 patient.Two patients had neurodevelopmental disorders with involuntary movements, which is related to GNAO1 in 1 patient, and the other patient was idiopathic.All the children were given oral Levodopa, Benzhexol, Baclofen, Tiapride Hydrochloride, Clonazepam alone or in combination.Three children showed obvious dyskinesia after Levodopa treatment.The symptoms of movement disorders in all children exhibited little to no improvement.Levetiracetam and Zonisamide had unstable effects in the treatment of myoclonia.DBS surgery was performed on all the patients aged from 3 to 16 years.Electrodes were successfully inserted into bilateral globus pallidus internus in 14 cases and bilateral subthalamic nuclei in 4 cases.The target was unknown in 2 cases.No surgery-related complications were observed.The patients were followed up for 3 months to 6 years, and the last follow-up age of the patients ranged from 5 years and 7 months to 22 years and 1 month.The rate of improvement in BFMDRS-M score was 37%-100% in 16 patients and >70% in 7 patients with hereditary dystonia.The rate of improvement in UPDRS Ⅲ score was 23% in 1 patient with VPS13C-related early-onset Parkinson′s disease. Conclusions:Childhood medically refractory hereditary movement disorders are a case series that exhibits significant phenotypic and genotypic heterogeneity.DBS surgery demonstrates significant efficacy for KMT2B-, TOR1A-, and SGCE-related hereditary movement disorders.

Objective:To investigate the impact of chromate exposure on pulmonary function indices in occupational populations and explore the potential role of alterations in inflammatory indicators in this process.Methods:In July 2024, A cross-sectional analysis was conducted using occupational health examination data of 30875 workers from chromate-related enterprises in Jiangsu Province in 2020 and 2021. Based on the occupational positions and whether there is chromium acid salt exposure in the occupational hazards of the research subjects over the years, they are divided into chromium acid salt exposure group and non-exposure group. For those exposed to chromium acid salts, based on job position descriptions and duration of chromium acid salt exposure, they are further categorized into intermittent exposure group and continuous exposure group; among them, the actual exposure time in the intermittent exposure group is less than half of the working shift time, and the exposure duration is less than the total working life. Pulmonary function test indicators include forced vital capacity (forced vital capacity, FVC) %, first-second forced expiratory volume (forced expiratory volume in one second, FEV 1.0) %, and the ratio of first-second forced expiratory volume to forced vital capacity (FEV 1.0/FVC) %. Peripheral blood samples from the upper limbs of the research subjects were collected on an empty stomach for routine blood tests, selecting neutrophil count, platelet count, and lymphocyte count results, calculating the neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR). Quantitative data that meet normal distribution are expressed using Mean±SD, while variables that do not meet normal distribution are represented by M ( Q1, Q3). Categorical data are expressed using frequency or proportion (%). Linear variable comparisons between groups use t-tests. Generalized linear models were employed to assess the correlation between chromate exposure and pulmonary function indices, while mixed regression models were utilized to explore potential underlying effects. Results:Compared with the non-exposed group, the pulmonary function indices FVC (%), FEV 1.0/FVC (%), and FEV 1.0 (%) in the chromate-exposed group showed a decreasing trend ( P<0.01), indicating statistically significant differences. As the frequency of chromate exposure increased, the prevalence of pulmonary dysfunction rose. The prevalence rates of obstructive, restrictive, and mixed respiratory dysfunction among the chromate-exposed population (including intermittent exposure and continuous exposure groups) were 0.26%, 4.67%, and 0.12%, which were higher than those in the non-exposed group (0.09%, 0.84%, and 0.07%, respectively). All these differences were statistically significant ( P<0.05). After stratification by gender, a negative correlation was observed between chromate exposure and the inflammatory indicator platelet-to-lymphocyte ratio (PLR) ( P<0.05). Potential effect analysis revealed that PLR played a certain mediating role between chromate exposure and the decline in pulmonary function indices, with a mediating proportion of 2.2%. Conclusion:Chromate exposure in occupational populations may lead to a decline in pulmonary ventilatory function and alterations in peripheral blood inflammatory indicators. Inflammatory indicators may be involved in the pulmonary function decline caused by chromate exposure.

Objective:By systematically evaluate the implementation of GBZ 2.1, so as to provide technical basis for the future revisions of this standard.Methods:From May to October 2023, Based on the pre survey questionnaire, the semi-structured interview method was used to interviews with experts from CDC, Occupational disease prevention and control hospital institutes, employers, occupational hygiene technical service intermediaries and universities, and the inductive method was used to extract the topics and relevant suggestions.Results:Generally, GBZ 2.1 is scientific, practical, progressiveness and operable. There are still some issues such as OELs overlapping and the correspondence between Chinese and English names. The outstanding problem is the coordination with other standard contents.Conclusion:The technical indicators in GBZ 2.1 could adapt to the needs of current practical work. The coordination between standards needs to be clarified, and the new recommended content needs further promotion and exploration on how to implement it.

Objective:To evaluate the effect of social media based behavioral intervention on promoting joint testing of HIV and syphilis in young men who have sex with men (MSM).Methods:After the recruitment, the participants who met the inclusion criteria were randomly divided (1∶1) into two groups, i.e. social media intervention group and control group. The control group was given routine voluntary counseling and testing (VCT) services. The intervention group was also given VCT services, besides; the comprehensive strategies through social media, including regular health education message and testing information sending, were given to them to strengthen the behavioral intervention. Follow up was conducted for the participants for 12 months after the intervention. The number and the proportion of young MSM receiving HIV and syphilis testing, and the reported proportion of the young MSM with sexually transmitted diseases (STD) symptoms between the intervention group and the control group were compared to evaluate the effect of the intervention.Results:A total of 315 young MSM were recruited (158 in the intervention group and 157 in the control group), in whom 248 young MSM completed the follow up. The follow-up rate was 78.7%. There was no significant difference in baseline characteristics between the intervention group and the control group (all P>0.05). The proportion of young MSM receiving more than one joint testing in the intervention group was slightly higher than that in the control group (53.2% vs. 48.4%, rate difference (RD): 4.8%, 95% CI: -7.5%-17.0%, P=0.448) without significant difference. However, in the young MSM who used condoms in the last anal sex, the proportion of those receiving more than one joint testing in the intervention group was higher than that in the control group (63.8% vs. 46.1%, RD: 17.7%, 95% CI: 1.5%-32.6%, P=0.035). In addition, the reported proportion of young MSM with STD symptoms in the intervention group was significantly lower than that in the control group (6.3% vs. 18.0%,RD: -11.7%, 95% CI: -20.6%- -3.0%, P=0.005). Conclusion:Compared with routine VCT, social media based behavioral intervention might promote joint HIV and syphilis testing in the young MSM who used condom in the study. It could significantly reduce the reporting proportion of STD symptoms, suggesting that this method can promote the AIDS and STD prevention related behaviors in young MSM.

After multidisciplinary treatment including radiotherapy, the median survival of patients with glioblastoma multiforme (GBM) remains approximately 1 year. The heterogeneity of the genome and proteome of glioblastoma stem cells (GSC) is the fundamental factor affecting the prognosis. Proteomics-based sensitization of key radioresistance proteins is expected to improve the prognosis of GBM patients. In this article, literature review was conducted from PubMed and other databases in the previous 10 years to systematically discuss the research progress on various commonly used protein quantitative techniques, tools for data processing analysis and the application in radioresistance and radiosensitization of GSCs.

Objective: To learn the trend of death from injury in Fengcheng residents from 2009 to 2017 and predict from 2018 to 2021,in order to provide the basis for injury prevention and control. Methods: All cause of death monitoring data and demographic data of Fengcheng from 2009 to 2017 were collected,injury mortality,standardized mortality and annual change percentage（APC）were calculated,and GM（1,1）was established to predict the injury mortality from 2018 to 2021. Results: From 2009 to 2017,the injury death rate in Fengcheng was 52.68 per 100 000 and the standardized death rate was 46.50 per 100 000. The APC of the total standardized mortality was -5.10%,showing a decreasing trend year by year（P<0.05）. The injury mortality and standardized mortality in males were higher than that in female（both P <0.05）. The highest injury mortality lay in the group aged 65 years and above,which was 108.13 per 100 000. The top five causes of injury death were traffic accident,suiside,poisoning,fall and drowning,their mortality rate were 27.03 per 100 000,7.84 per 100 000,5.62 per 100 000,5.08 per 100 000 and 2.36 per 100 000,respectively. The mortality of traffic accident in males and suicide in females showed a decreasing trend（P<0.05）,while the mortality of fall showed an increasing trend（all P<0.05）. The model of GM（1,1）predicted that the injury mortality from 2018 to 2021 would be 48.00 per 100 000,44.15 per 100 000,40.61 per 100 000 and 37.35 per 100 000,which showed a decreasing trend year by year. Conclusion The injury death rates in Fengcheng dropp year by year from 2009 to 2017 and will probably keep dropping from 2018 to 2021. Men and people aged 65 years or over are at high risk of death from injury,traffic accident and fall are the main causes.

Objective: To evaluate the precision of image registration between MRI simulation (MRIsim) and CT simulation compared to diagnostic MRI(MRIdiag) and to provide information for further application of MRIsim. Methods: A total of 24 patients who underwent both MRIsim and MRIdiag were enrolled, including 8 patients with gliomas, 8 with nasopharyngeal carcinomas and 8 with prostate cancers. MRIsim and MRIdiag images of each patient were fused with CT. The OARs were delineated on three modalities of images and targets were delineated on fusion image of MRIsim with CT (F_CTMsim) and fusion image of MRIdiag with CT (F_CTMdiag) respectively. The concordance index (CI), Dice′s similarity coefficient (DSC) between the OARs and image similarity index (S) based on images from MRIsim, MRIdiag and CT were evaluated. IMRT plans were designed based targets on F_CTMsim and OARs on CT images, and differences in dosimetry of targets and OARs were evaluated subsequently. Results: Volumes of most OAR from three modalities of images showed no statistically significant difference(P>0.05). All the CI and DSC between the OARs derived from MRIsim and CT were higher than those corresponding values from MRIdiag, and a statistically significant difference was achieved in 50% of these OARs (t=2.58-5.47, P<0.05). The S values of MRIsim and MRIdiag compared with CT were 0.89 and 0.83 respectively (t=5.77, P<0.05). MRIsim improved the S value by 10% (2%-56%) compared with MRIdiag. No further differences in dosimetry were found on all OARs and all targets(P>0.05). Conclusions The precision of image registration can be significantly improved by introducing MRIsim into radiotherapy planning design compared with MRIdiag. However, no significant differences in dosimetry were found on targets produced by rigid registration and manual adjustment method .