Objective To investigate the clinical,brain MRI and MT-ATP6 gene variation characteristics of late-onset Leigh syndrome (LS). Methods The clinical data,brain imaging and genetic test results of two patients with late-onset Leigh syndrome in a family were collected in detail,and discussed in combination with the literature. Results The proband,male,9 years old,complained of "bilateral ptosis,unstable walking for half a year,abnormal mental behavior for 4 months".He was admitted to our hospital on April 10,2020.Patient was born normally,his physique and intelligence was worse that of his peers since childhood.Six months ago,the patient had bilateral ptosis and double vision,and he gradually developed unstable walking and difficulty in running.Brain MRI examination showed abnormal signals in bilateral basal ganglia and brain stem.4 months ago,he had intermittent nausea and vomiting,abnormal mental behavior without any inducement.Two months ago,he suddenly fell into a coma and twitched after catching a cold and fever.He was rescued and treated in a local hospital.His vital signs were stable and transferred to our hospital.Physical examination:short stature,thin physique,coma with eyes open.Bilateral ptosis covers more than half of the pupil,bilateral eyeball center fixation,dysphagia,nasal feeding diet.Muscle tension in the limbs was high,tendon reflex was active,and the limbs were flexing,Bilateral pathological signs were positive.Family history:the child's parents are not consanguineous marriage,the father is in good health,the mother is short and has a history of mental and behavioral abnormalities.The proband's brother,14 years old,was less developed than his peers.At the age of 7,he developed ptosis,unstable walking and no history of coma and convulsions.No ragged red fibers were found in skeletal muscle pathological biopsies of the proband and his brother.The mutation of MT-ATP6 gene 9176T>C was found by mitochondrial genome detection. Conclusions The clinical phenotype of late-onset Leigh syndrome is heterogeneous,which may be accompanied by mental and behavioral abnormalities and cognitive impairment.Brain MRI is characterized by abnormal signals in bilateral basal ganglia and midbrain periaqueductal region.Gene detection is an important basis for the diagnosis of Leigh syndrome.

Objective To study the effect of neck restrain on traumatic brain injury during airbag inflation in traffic accidents. Methods Based on the previously validated 3-year-old child head finite element (FE) model, the impact on out-of-position (OOP) child occupant during airbag inflation was simulated by FE method, so as to investigate the effects of neck restraint on intracranial response and injury mechanism in traffic accidents. Results The head kinematics with neck restrain was different from that without neck restrain under the impact of airbag inflation. The neck restraint would obviously decrease the maximum Von Mises stress of pediatric brain. When airbag-head distance was 20 cm or 25 cm, the neck restraint would obviously decrease the maximum intracranial pressure. Conclusions Neck restraint had a relatively large influence on pediatric intracranial response. When the FE method is used to predict pediatric craniocerebral injury, consideration of neck restrain on child brain response is necessary.

ObjectiveTo explore the risk factors related to senile dementia to develop intervention method.MethodsA combination of qualitative and quantitative methods was used to analyze the factors and their degree related to the incidence of dementia for the elderly.ResultsAgeing was the deterministic factor to senile dementia, and vascular factor was common risk for Alzheimer's disease (AD) and vascular dementia (VD).ConclusionActive intervention in early life, concern about women's groups especially in the mental health, and detemination of value range of the risk factors are needed.

Objective:To study the effects of ~(125)I-(α_v)ASODN on the in vitro invasive ability of heptocellular carcino-ma cell line(HepG2) through PEI-RGD-mediated receptor process. Methods: Intergrin α_v-specific antisense oligonucle-otide was labeled with ~(125)I, and PEI-RGD/~(125)I-(α_v)ASODN complex was prepared by combining ~(125)I-(α_v)ASODN with polyethyleneimine derivative PEI-RGD. PEI-RGD/~(125)I-(α_v)ASODN complex was transferred into HepG2 cells through the receptor-mediated process. The effect of PEI-RGD/~(125)I-(α_v)ASODN complex on the invasive ability of HepG2 cells was examined by Boyden chamber invasive assay. Results: (1) The labeling yield and radiochemical purity of ~(125)I-(α_v) ASODN were(73.78±4.09)% and(96.68±1.38)%, respectively, and the labeled compound had a good stability in vitro after 48 h at 37℃; (2) The ability of HepG2 cells to uptake PEI-RGD/~(125)I-(α_v)ASODN reached its peek ([12.77±0.85] % ) when PEI-RGD/~(125)I-(α_v)ASODN was at 4 μl/2 μg ([12.77±0.85] %), and then gredually decreased thereafter. So the dosage of PEI-RGD/~(125)I-(α_v)ASODN for the following experiment was chosen as 2 μl/1 μg; (3) The invasive capacity of HepG2 cells was significantly reduced in PEI-RGD/~(125)I-(α_v)ASODN group compared with those in other experiment and control groups (P <0.01 ). Conclusion: ~(125)I-(α_v)ASODN mediated by PEI-RGD can effectively inhibit the invasive capacity of HepG2 cells.

This paper introduced the project of "the Study on the Evaluation Index System of Rehabilitation Service for All Disabled in Shanghai" in brief, including research background, objectives, researching objects and sampling. The project aimed at constructing the evaluation index system of rehabilitation service for all disabled in Shanghai. According to the research purpose, the persons with disability in 19 regions (or counties) and 2 towns were investigated with 12 consultation conference and 3 Delphi's surveys to find out the rehabilitation demand and service. The Evaluation Index System of Rehabilitation Service for All Disabled in Shanghai that finally comes into being includes 8 first-degree index, 32 second-degree index and 100 third-degree index.

Objective To investigate the status of rehabilitation demands and services of persons with disability in rural area in Shanghai.MethodsXinhe town of Chongmin county was selected as the sample by stratified-cluster random sampling method, and the rehabilitation demands and services of 1128 persons with disability were surveyed.ResultsThe realization rate of rehabilitation demands in Shanghai suburb was 81.3%. The realization rate of different rehabilitation demands was different. Only 4.0% persons with disability had obtained assistive devices.ConclusionIn order to improve the realization rate of rehabilitation demand, more rehabilitation relief, balanceable development of rehabilitation service and assistive devices service should be enhanced.

Objective To create the target value of third-degree index and the weight coefficient of evaluation index system of rehabilitation service for all disabled in Shanghai. Methods Compositive quantitative and qualitative research methods, such as document analysis, Delphi method, questionnaire survey, specialist consultation were carried in the study. Results The enthusiasm coefficient of specialist in three times were 100%, 100%, 92.3% respectively, the degree of specialist authority was 0.84, the coordination coefficient of specialist for the first-and second-index were 0.79 and 0.72 respectively. The weight of the more important first-degree index namely community rehabilitation, outlay support, organization network and policy environment were 0.1836, 0.1781, 0.1360 and 0.1274.Conclusion The enthusiasm, authority and coordination of specialist of this evaluation index system are high. The index weight can offer evaluation basement.

Objective To create the evaluation index system of rehabilitation service for all disabled in Shanghai.MethodsCompositive quantitative and qualitative research methods,such as literature review,focus-group discussion,questionnaire survey,specialist consultation were carried in the study.ResultsThe Evaluation Index System of Rehabilitation Service for All Disabled in Shanghai finally comes into being includes 8 first-degree index,32 second-degree indexes and 100 third-degree index.ConclusionThe evaluation index system would be provided with directive significance to the goal of 'rehabilitation service for all disabled persons'.

Objective To investigate and assess the current situations on information management of rehabilitation service in Shanghai. Methods The information management in 6 districts in Shanghai were assessed in field, the fulfilling of the first- and second-degree index and the standard-reaching rate of the third-degree index were calculated separately according to the first-degree index- information management of the Evaluation Index System of Rehabilitation Service for All Disabled in Shanghai. Results The average fulfilling degree of the first-degree index was 73.1%. There were only 2 districts reached the standard of Registration of Rehabilitation. The average fulfilling degrees of the Development and Dynamic Maintenance of Rehabilitation Service Information Database were 50.0% and 66.7%, respectively. The standard-reaching rate of the Disabled Children First-diagnose Report System, Effectual Providing Referral Rehabilitation Service Across-the-board, Suspicious Disabled Persons Report System in Community were 16.7%, 16.7% and 50%, respectively. Conclusion The development and supervision of rehabilitation service information should be reinforced and the referral rehabilitation service should be improved.

Objective To investigate and assess the current situations of propagandizing and inculcating rehabilitation information in Shanghai. Methods The propagandizing and inculcating rehabilitation information in 6 districts in Shanghai were assessed in field, the fulfilling of the first- and second-degree index and the standard-reaching rate of the third-degree index were calculated separately according to the first-degree index- propagandizing and inculcating rehabilitation information of the Evaluation Index System of Rehabilitation Service for All Disabled in Shanghai.Results The average fulfilling degree of the first-degree index was 82.3%. There was 1 district reached the standard of Propagandizing Rehabilitation Information by Broadcast, Television, Newspaper, Magazine and Internet; the average fulfilling degrees of the Community Rehabilitation Function for the Disabled and the Community School Propagandizes and Inculcates the Rehabilitation Information for the Disabled are 66.7% and 33.3%, respectively. Conclusion The support from the government could make propagandizing and inculcating rehabilitation information carry out more successfully; utilizing the media adequately is important; propagandizing and inculcating rehabilitation information must be strengthened in community.