ObjectiveTo introduce the basic design， development plan and objectives of a population-based birth cohort in Shanghai， and further present the main data and baseline characteristics of enrolled participants in the cohort， and to provide key information for reproductive health-related studies. MethodsThe Shanghai population-based birth cohort initiated on January 1， 2005， included newborns born in Shanghai every year and their parents， and collected information on reproductive health， reproductive treatment， birth characteristics， growth and development status， as well as the incidence， treatment and death of diseases by employing data linkage technology and investigations. This formed a birth cohort spanning the entire life cycle. ResultsAs of October 2022， a total of 2 978 538 newborns and their parents were included in the cohort. Among them， 2 905 135 （97.54%） were naturally conceived （NC）， and 73 403 （2.46%） were born through assisted reproductive technologies （ART）. The average age of parents was （32.56±4.12） years old for females and （34.62±5.34） years old for males in the ART group， which was higher than （28.02±4.71） years and （30.07±5.54） years for parents in the NC group. Among parents， females and males aged 30 and above accounted for 77.12% and 85.08%， respectively， which were higher than that of parents （35.28% for females and 49.66% for males） in the NC group. Furthermore， the percentage of parents with a college degree or above in the ART group was 73.23% for females and 73.66% for males， which were higher than those in the NC group （49.98% and 50.91%， respectively）. The multiple births rate in the ART group was 33.81%， which was higher than that in the NC group （1.88%）. The incidence of premature birth and low birth weight in the ART group were 24.47% and 19.08%， respectively， which was higher than that in the NC group （5.47% and 3.73%）. ConclusionThe comprehensive collection of reproductive health-related information in the birth cohort in Shanghai can provide essential resources to determine the influence of genetics， environment， reproductive treatment and other related factors on the health of offspring after birth.

ObjectiveTo establish the Shanghai twin birth cohort （STBC） and analyze the effects of genetic factors， shared environment， and non-shared environment interactions on birth health and growth and development of newborns. MethodsBased on the population-wide birth cohort in Shanghai， a comprehensive survey was conducted on the families with double and multiple babies born after January 1， 2015 to collect information on birth health， growth and development， and the family environment of the babies. ResultsBy December 31， 2021， a total of 7 195 pairs （14 405 cases） of twins were successfully included in the STBC survey. The average birth length of twins was 47.2 cm and average birth weight was 2 465.3 g. Heterozygous twins accounted for 69.05% and preterm babies accounted for 57.07%. The average age of the mothers of twins was 31.82 years， and the average age of the fathers was 33.87 years， with more than 80% of the parents having a college degree or above. 44.50% of the mothers used assisted reproductive technologies， 7.40% had illnesses during pregnancy， and 15.90% were exposed to passive smoking during pregnancy. During the survey period， the average monthly increase in the length of the twin infants was 2.09 cm， and the average monthly weight gain was 0.53 kg. ConclusionThe incidence of adverse outcomes such as maternal cesarean section rate， preterm birth， and low birth weight is higher in the twin birth population. Information on birth health as well as growth and development in childhood and adolescence in the twin birth population is collected based on STBC， which can provide a solid data foundation for studying children’s chronic non-communicable diseases， psychological and behavioral disorders and other complex health problems caused by the combined effects of genetics and the environment.

ObjectiveTo elucidate the principles and methods of the Bayesian probabilistic linkage model， and to demonstrate the effect of applying the model in linking birth and death data. MethodsThrough the Shanghai birth and death registration system， data of 199 025 infants born in 2017 and 1 512 infants who died in 2017 and 2018 were collected. After cleaning the data， the data were divided into monthly blocks and fully linked. The Jaro-Winkler algorithm and Euclidean distance were employed to measure the similarity of fields for matching. A Bayesian probabilistic linkage model was constructed and the linking effect was evaluated using a confusion matrix. ResultsUsing the Bayesian probabilistic linkage model， the birth and death data of infants were effectively linked， revealing that 36.71% of infants who died in Shanghai were born outside the city， and the probability of infant death was 2.6‰. The confusion matrix of the test set showed a recall rate of 0.86， precision of 0.76， and an F-score of 0.81. ConclusionThe practical application of Bayesian probabilistic linkage demonstrates a good model performance， enabling the establishment of birth-death cohorts that more accurately reflect the true levels of infant mortality. Utilizing this technique to integrate data from different departments can effectively improve research efficiency in the field of public health.

Objective:To investigate the specific method, efficacy and safety of intrathecal baclofen (ITB) in the treatment of moderate and severe spasticity.Methods:The clinical features, response to ITB trial, clinical outcomes and treatment-related complications after baclofen pump implantation surgery were retrospectively analyzed in patients with moderate to severe spasticity who received ITB treatment in Hong Kong University Shenzhen Hospital from August 2022 to August 2023.Results:The study included 22 patients with moderate to severe spasticity, of whom 18 patients (81.8%) were effective in ITB trial. For these patients with effective trial, the lowest mean Modified Ashworth Scale (MAS) score of lower extremity muscle groups after ITB bolus injection was decreased significantly compared with the baseline mean value before injection (1.29±0.81 vs 3.31±1.18 , t=11.364, P<0.001), and the M/H amplitude ratio of soleus H-reflex at 4 hours after ITB bolus injection was significantly higher than the baseline value (9.61±4.66 vs 3.83±2.69, t=7.780, P<0.001). Among the 18 patients with effective ITB trial, 10 patients received baclofen pump implantation surgery with the informed consent of the patients or their guardians. Compared with the 8 patients without surgery, the baseline mean MAS score of lower extremity muscle groups of the 10 patients who received surgery was higher (3.71±1.06 vs 2.83±1.16, F=5.645, P<0.05), the maximum reduction of mean MAS score of lower extremity muscle groups of the 10 patients who received surgery was greater (2.61±0.99 vs 1.30±0.64, F=20.696, P<0.001). In 10 patients who received baclofen pump implantation surgery, the mean MAS score of lower limb muscle groups was significantly decreased in the first and sixth months after surgery compared with the baseline mean value before surgery (1.64±0.91, 1.16±0.89 and 3.71±1.06, F=39.946, P<0.001). Among them, 2 patients were followed up to 12 months after surgery, 2 patients were followed up to 18 months after surgery, and the mean MAS score of their lower limb muscle groups showed continuous improvement, and 1 patient with cerebral palsy combined with lower urinary tract hyperactive syndrome also showed significant improvement of urgent urinary incontinence after surgery. Up to the last follow-up, the daily dose of baclofen in 10 patients receiving baclofen pump implantation was (143.60±96.24) μg, and no treatment-related complications occurred. Conclusions:ITB can effectively relieve spasticity symptoms and other related neurological dysfunction in patients with moderate and severe spasticity, and the efficacy can be maintained for a long time, and treatment-related complications are rare. Preoperative ITB trial is helpful to screen out patients more suitable for baclofen pump implantation. Patients with higher grade of spasticity at baseline and better efficacy of ITB trial are more suitable for baclofen pump implantation. The soleus H reflex can effectively reflect the change of stretch reflex of lower limb muscle groups in patients with spasticity treated by ITB, and can be used to evaluate the treatment effect of ITB.

Objective:To explore the application potential of 18F-Asp-Glu-val-Asp (DEVD)-Cys(StBu)-PPG(CBT)-AmBF 3 ( 18F-1; PPG: propargyl-glycine; CBT: 2-cyanobenzothiazole; AmBF 3: ammoniomethyl-trifluoroborate) PET imaging in early monitoring of triple-negative breast cancer (TNBC) radiotherapy response. Methods:Ten MDA-MB-231 tumor bearing nude mice models were constructed and divided into radiotherapy group ( n=5) and non-radiotherapy group ( n=5) by random sampling method. The radiotherapy group was treated with single irradiation at a dose of 8 Gy. 18F-1 microPET imaging was performed in the radiotherapy and non-radiotherapy groups, and the tumor uptake and muscle uptake in 2 groups at different time points (2.5, 7.5, 12.5, 17.5, 22.5, 27.5, 32.5, 37.5, 42.5, 47.5, 52.5, 57.5 min after injection) were analyzed. The specific uptake of the probe in apoptotic cells was verified by radioautography, HE staining and immunofluorescent staining. Repeated measures analysis of variance and one-way analysis of variance were used to analyze data. Results:18F-1 microPET imaging showed that there was significant difference between tumor uptake and muscle uptake in radiotherapy group ( F=20.27, P=0.011). The uptake of radiotherapy group was the highest at 7.5 min after injection ((4.64±0.35) percentage activity of injection dose per gram of tissue(%ID/g)). There was no significant difference between tumor uptake and muscle uptake in the non-radiotherapy group ( F=1.81, P=0.215). The tumor/muscle (T/M) ratio of radiotherapy group was higher than that of non-radiotherapy group ( F=31.95, P=0.005), with the highest at 47.5 min after injection (2.49±0.46). Radioautography showed that the tumor radioactivity in radiotherapy group was higher than that of muscle in radiotherapy group, and was also higher than tumor and muscle radioactivies in non-radiotherapy group ( F=116.79, P<0.001). HE staining and immunofluorescent staining verified that 18F-1 could specifically detect the activity of caspase-3 activated in tumor cells after radiotherapy. Conclusion:18F-1 can specifically recognize the activated caspase-3 after TNBC radiotherapy, and monitor radiotherapy response at the molecular level by apoptosis PET imaging.

Purpose: This study aimed to investigate reproductive concerns among breast cancer patients of reproductive age, analyze the influencing factors, explore the relationship between coping styles, fear of progression (FOP), and reproductive concerns, and identify the multiple effects of coping styles on the relationship between FOP and reproductive concerns among Chinese breast cancer patients. Methods: A cross-sectional, descriptive study was conducted among breast cancer patients in four tertiary grade A hospitals in Fujian, China, from January 2022 to September 2022. A total of 210 patients were recruited to complete paper-based questionnaires, which included the general data questionnaires, the Reproductive Concerns After Cancer Scale (RCACS), the Fear of Progression Questionnaire-Short Form (FOP-Q-SF), and the Medical Coping Modes Questionnaire (MCMQ). Structural equation models were utilized to evaluate the multiple effects of coping styles on FOP and reproductive concerns. Results: Reproductive concerns in breast cancer patients had a mean score of 53.02 (SD, 10.69), out of a total score of 90, and coping styles for cancer (confrontation, avoidance) were closely associated with FOP and reproductive concerns. FOP showed a significant positive correlation with reproductive concerns (r = .52, p < .01). At the same time, confrontation was significantly negatively correlated with both FOP (r = −.28, p < .01) and reproductive concerns (r = −.39, p < .01). Avoidance was positively correlated to both FOP (r = .25, p < .01) and reproductive concerns (r = .34, p < .01). The impact of FOP on reproductive concerns is partially mediated by confrontation and avoidance, with effect sizes of .07 and .04, respectively. These mediating factors account for 22.0% of the total effect. Conclusions The FOP directly impacted reproductive concerns, while coping styles could partially mediate the association between FOP and reproductive concerns. This study illustrates the role of confrontation and avoidance in alleviating reproductive concerns, suggesting that it is necessary to focus on the changes in reproductive concerns among reproductive-age breast cancer patients. Healthcare professionals can improve disease awareness and reduce patients' FOP, thereby promoting positive psychological and coping behaviors and ultimately alleviating reproductive concerns.

The main clinical phenotypes, imaging features and genetic test results of a child with Joubert syndrome treated in Shenzhen Children′s Hospital in July 2020 were analyzed retrospectively, and the literature on Joubert syndrome was summarized.The main manifestations of the protester during infancy were respiratory abnormalities and developmental retardation.The brain magnetic resonance imaging (MRI) showed a " molar sign" , which was consistent with the diagnosis of Joubert syndrome.Genetic testing suggested that the protestor carried complex heterozygous variations of KIAA0586 gene.Two variants were not reported previously, one of which was synonymous mutation.The child is the first case of Joubert syndrome caused by KIAA0586 gene in China.Joubert syndrome is a rare congenital brain development malformation characterized by high clinical heterogeneity and MRI molar signs.It may involve multiple systems.Early identification and intervention can improve outcomes.

Objective To investigate the clinical features and prognosis of antimyelin oligodendrocyte glycoprotein-IgG （MOG-IgG） associated disorders （MOGAD）.Methods Eight cases met the diagnostic criteria of MOGAD were reviewed，and the demographics，clinical manifestations，laboratory and imaging results，treatment and prognosis were analyzed. The Expanded Disability Status Scale （EDSS） and the modified Rankin Scale （mRS） were used to dynamically evaluate the neurological disability. Results The ratio of male to female in this 8-case series was 1∶1，the median onset age was 34 years old，and the median disease duration was 42 months. The most common initial symptom is optic neuritis （ON，50%），followed by meningitis and/or encephalitis （25%）. Recurrence occurs in 50% of cases，and the recurrence rate of patients with high serum MOG IgG titer （≥1∶32） is 60%. The white blood cells in the cerebrospinal fluid in cases with meningitis/encephalitis or longitudinally extensive myelitis is often>100×10-6/L，which is higher than other phenotypes. There are 6 cases （75%） of patients with different MRI abnormalities，including hypertensity on T-2/Flair image with enhancement at affected optic nerve and orbital tissue，asymptomatic diencephalon/thalamus lesions on T2 and contrasted image，and MRI findings mimic typical multiple sclerosis may also be present. Cases with myelitis can show short-segment eccentric lesions or long-segment central lesions on MRI. Cases with meningitis/encephalitis showed unilateral cortical high T-2/Flair lesions on cranial MRI with various enhancements. After medication and rehabilitation，the peak EDSS of this case series was 5±1.069，and 2.19±1.689 points at the last visit；mRS was 3.25±1.165 points at the peak and 1.25±1.035 points at the last visit. The difference was statistically significant.Conclusions MOGAD is a rare type of demyelinating disease in central nervous with various manifestations，which is highly treatable. Fully understanding of the clinical characteristics，making a early diagnosis through comprehensive tests，and initiating immunization and rehabilitation treatment in time，will contribute to better prognosis for patients.

Objective To explore the differences of clinical and electrophysiological characteristics of different subgroups of generalized myasthenia gravis（MG），and analyze the correlation between electrophysiological results and disease severity.Methods A retrospective analysis of 101 generalized MG patients who accepted diagnosis and treatment in the department of neurology of Shenzhen Hospital of Hongkong University from July 2016 to July 2020 was performed. According to their clinical feature，these patients were divided into early-onset generalized MG group with Acetylcholine receptor（AchR） antibodies（52 cases），late-onset generalized MG group with AchR antibodies（23 cases），thymoma-associated MG group（22 cases） and MuSK associated MG group（4 cases）. We compared the demographic characteristic，Quantitative Myasthenia Gravis score（QMGs），MG Foundation America（MGFA） Clinical Classification，repetitive nerve stimulation（RNS） results of different subgroups and analyze the correlation between RNS results and QMGs.Results In different subgroups of generalized MG without positive MuSK antibodies，the rate of female patients of early-onset MG with AchR antibodies was highest（71.2% vs 65.2% vs 36.4%，P<0.05），the rate of patients with respiratory/bulbar muscles dominantly impaired of thymoma-associated MG was highest（28.8% vs 43.5% vs 63.6%，P<0.05），the rate of patients with RNS positive of whole muscles detected of early-onset MG with AchR antibodies was highest（44.2% vs 17.4% vs 22.7%，P<0.05），the positive correlation between QMGs and highest or whole RNS decrement was demonstrated（P<0.05）；In MuSK associated MG，the rate of patients with respiratory/bulbar muscles dominantly impaired or moderately and severely impaired were relatively higher（both 75%），the rate of patients with RNS positive of whole muscles detected were relatively lower（0%）.Conclusion The population distribution，gender proportion，dominantly impaired muscles and severity and distribution of impaired muscles of different subgroups of generalized MG were significantly different，which would be valuable in the classification diagnosis of generalized MG；The high correlation between RNS results and disease severity existed，which demonstrated the significant values of RNS in severity evaluation of generalized MG.

Objective:To describe the characteristics and the survival status of pneumoconiosis cases reported in Guangzhou City from 1958 to 2018, and to investigate the epidemiological trends of pneumoconiosis. This study is aiming to provide basic data for formulating the guidelines and policies for control of pneumoconiosis, and for evaluating the control effects of pneumoconiosis.Methods:From July 2019 to January 2020, based on the data collected in the pneumoconiosis case cards and database as well as the retrospective follow-up investigate of pneumoconiosis cases, we conducted a descriptive analysis for all the new cases of pneumoconiosis reported from 1958 to 2018 in Guangzhou City. The statistical indices included the number of new pneumoconiosis cases in each decade, types of pneumoconiosis, regional and industrial distributions, survival status, and the change tendency of the diagnosis age and the dust exposure time of pneumoconiosis cases.Results:From 1958 to 2018, a total of 1194 new cases of pneumoconiosis were reported in Guangzhou City, including 1147 males (96.1%) and 47 females (3.9%) . Silicosis (60.1%, 718/1194) and welder's pneumoconiosis (21.5%, 257/1194) were the main types of pneumoconiosis. The top three districts for reporting new cases were Huangpu District (29.0%, 346/1194) , Nansha District (12.6%, 151/1194) and Baiyun District (11.1%, 132/1194) , respectively. The top three industries for reporting new cases were civil engineering construction industry (25.0%, 298/1194) , railway ship aerospace and other transportation equipment manufacturing industry (16.1%, 192/1194) and non-metal mining industry (15.7%, 187/1194) . The diagnosis age for new cases was 47.8 (23.6-79.1) years old, and the dust exposure time was 12.3 (0.4-49.1) years. Both of these two statistical indicators rose first and fell later from 1958 to 2018. As of December 31, 2019, a total of 963 new cases of pneumoconiosis were followed up, of which 467 (48.5%) survived, mainly silicosis (41.3%, 193/467) and welder's pneumoconiosis (43.3%, 203/467) . 496 cases (51.5%) died, and the age of death was 69.9 (32.4-96.9) years old.Conclusion:According to the epidemiological characteristics of pneumoconiosis in Guangzhou, we should focus on key districts and industries in the prevention and control of pneumoconiosis. Besides, the staffs in the occupational disease diagnosis institutions should try the best to collect the complete dust exposure data of new cases of pneumoconiosis. Moreover, the health administrative departments should pay more attention to the management of pneumoconiosis death case reports.