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Objective:To respond to the national requirements on the construction of research integrity, and improve the capacity building of research integrity at hospitals.Methods:Dealing with research integrity problems identified at hospital before 2018, measurements that include " policy and procedures development, standardization of the management of scientific research activities, and optimizing the scientific and technological evaluation system" were adopted to promote the construction of research integrity. Such corrective activities were monitored to summarize experiences and lessons for the continuing improvement of research integrity construction.Results:Both " heteronomy" and " self-discipline" of research integrity have been sublimated simultaneously, at the same time, a tailored research integrity management system with the characteristics of local hospitals has been established.Conclusions:Updating institutional policy and procedures, taking paper management as a priority, as well as research integrity training, these measurements played important roles in promoting the research integrity capacity building at hospitals.
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Objective@#To investigate the clinical features of childhood epilepsy with occipital paroxysms.@*Methods@#The clinical data of 33 children admitted to our hospital from 2015 to 2019 were retrospectively analyzed.@*Results@#The children were divided into three groups: idiopathic group (n=20), structural group (n=10) and genetic group (n=3). Among the structural etiology group, there were 1 case of megagyrus malformation, 1 case of focal cortical dysplasia type Ⅱ (FCDⅡ), 1 case of gestational age infant with enlarged ventricle, and 7 cases of perinatal ischemic-hypoxic encephalopathy (HIE). In the genetic group, there were 1 case of PCDH19 gene mutation, 1 case of cyclin-dependent kinase like 5 (CDKL5) deficiency disorder and 1 case of Wolf-Hirschhorn syndrome. All three groups had ocular motor symptoms and secondary motor symptoms. Compared with the structural group, patients of the idiopathic group had more autonomic symptoms, less visual symptoms, longer ictal duration, less episodes, less abnormal background rhythm, and more epileptic discharges in the frontal lobe or Rolandic area. The genetic group had frequent seizures and the CDKL5 deficiency disorder patient had slowed background and spasms. Compared with the other two groups, patients of the idiopathic group had fewer MRI abnormalities, better seizure control effect, and better prognosis.@*Conclusions@#There are many causes of childhood epilepsy with occipital paroxysms. The idiopathic group accounts for a large proportion. The visual symptoms and autonomic symptoms are different between groups. Background abnormalities are detected more often in the structural group and the genetic group, which is also helpful for differential diagnosis. The treatment options and prognosis of different groups vary greatly. The overall therapeutic effect and prognosis of the idiopathic group are better than those of the structural group and the genetic group.
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Objective To investigate the clinical features of childhood epilepsy with occipital paroxysms.Methods The clinical data of 33 children admitted to our hospital from 2015 to 2019 were retrospectively analyzed.Results The children were divided into three groups:idiopathic group (n =20),structural group (n =10) and genetic group (n =3).Among the structural etiology group,there were 1 case of megagyrus malformation,1 case of focal cortical dysplasia type Ⅱ (FCD Ⅱ),1 case of gestational age infant with enlarged ventricle,and 7 cases of perinatal ischemic-hypoxic encephalopathy (HIE).In the genetic group,there were 1 case of PCDH19 gene mutation,1 case of cyclin-dependent kinase like 5 (CDKL5) deficiency disorder and 1 case of Wolf-Hirschhorn syndrome.All three groups had ocular motor symptoms and secondary motor symptoms.Compared with the structural group,patients of the idiopathic group had more autonomic symptoms,less visual symptoms,longer ictal duration,less episodes,less abnormal background rhythm,and more epileptic discharges in the frontallobe or Rolandic area.The genetic group had frequent seizures and the CDKL5 deficiency disorder patient had slowed background and spasms.Compared with the other two groups,patients of the idiopathic group had fewer MRI abnormalities,better seizure control effect,and better prognosis.Conclusions There are many causes of childhood epilepsy with occipital paroxysms.The idiopathic group accounts for a large proportion.The visual symptoms and autonomic symptoms are different between groups.Background abnormalities are detected more often in the structural group and the genetic group,which is also helpful for differential diagnosis.The treatment options and prognosis of different groups vary greatly.The overall therapeutic effect and prognosis of the idiopathic group are better than those of the structural group and the genetic group.
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X-linked adrenoleukodystrophy is the most common peroxisomal disorder,which belongs to single peroxidase enzyme deficiency disease.It is caused by mutations in the ABCDI gene and alterations in peroxisomal beta-oxidation of long chain fatty acid in plasma and tissues.It manifests a wide range of phenotypes in male and it has been frequently discussed,in which progressive myelopathy is the most common.For X-linked recessive inheritance,female heterozygotes are always thought to be nonpathogenic.There have been only limited studies specifically focused on the phenotype of female heterozygotes,while,these patients also need further study.This article discusses the clinical manifestations,diagnosis and treatment of female heterozygotes with X-linked adrenoleukodystrophy,to enhance people's understanding of clinical diagnosis and treatment,and provide the basis for accurate prognosis assess-ment and diagnosis.
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Objective To observe 100 video-electroencephalogram (EEG)-cases of intellectual disability (ID) children,to analyze their EEG features of different degrees of ID,to explore the relationship between EEG changes and ID.Methods We performed a retrospective analysis of long-range video-EEGs of 100 children with an unexplained reason of ID from July 2016 to May 2018 in the department of pediatrics of Xiangya Hospital of Central South University,sumnarized their EEG manifestations,and compared their differences among different degrees of ID.We carried out x2 test according to the degree of ID,and discussed the relationship between the degree of ID and EEG.Results Among 100 cases of ID children with unexplained pathogenic factor,67 cases (67％) of them were males,33 cases (33 ％) of females,38 cases (38％)of mild ID,27 cases (27％) of moderate ID and 35 cases (35％) of severe ID.The EEG background of ID patients showed fast waves (11％),slow waves (15％),generalized discharges (14％) and focal discharges (36％),etc.Focal sharp and spike-wave were the most common in children with moderate ID,while background changes and multifocal sharp spike wave were more common in severe ID children.Conclusions The EEG background changes in children with severe ID have significant differences than mild and moderate degree ones.The specific features of EEG in mild and moderate degree ones is focal discharges,while multiple discharges in severe ID patients.Changes in the EEG may be related to the extent of intellectual disability.
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Objective To analyze the clinical characteristics and treatment of children with tuberous sclerosis (TSC) complicated with epilepsy,so as to improve the level of diagnosis and treatment and improve the prognosis of children with TSC.Methods The clinical data of TSC children complicated with epilepsy diagnosed and followed up in Xiangya Hospital of Central South University were collected and analyzed retrospectively.Results Of the 51 children,49 (96.1％) had their first visit because of epileptic seizures.Their electroencephalogram (EEG) showed epileptiform discharges during the epileptic period.The therapeutic effect of vigabatrin on TSC patients with spastic seizures was significantly different from that of antiepileptic drugs alone or in combination.Conclusions Epileptic seizure is the most common reason for first visit and seizure control will affect the prognosis of children to a large extent.Vigabatrin had remarkable effect on TSC patients with spasm seizure,and rapamycin has broad prospects in the treatment of children with TSC.
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Infantile neuroaxonal dystrophy (INAD) is a rare autosome-recessive disease characterized by progressive motor and cognitive regression.The PLA2G6 gene is its causative gene,which encodes calcium-independent phospholipase A2 enzyme (iPLA2-VIA).The diagnosis of INAD is difficult because of its clinical heterogeneity,and the rate of misdiagnosis is high.The purpose of this study is to describe the clinical characteristics,molecular genetics,treatment and prognosis of INAD to improve the acknowledgement of INAD in medical workers and to help make an early diagnosis of INAD.
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DNA methylation is an important epigenetic modification,functioning under the regulation of DNA methyltransferase(DNMT),interacting with other epigenetic mechanisms and participating in the regulation of gene expression and differentiation process of cells.Many DNA methylation related changes are found in epilepsy and the emerging role of DNA methylation in epileptogenesis is becoming even more obvious.Here,we discuss the research progress of DNA methylation in the pathogenesis of epilepsy,and the perspectives of epigenetic medicine as new therapeutic strategy in epilepsies.
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Objective To explore the nursing measures of teenagers with arthroscopic reduction of avulsed fracture of tibialintercondylar eminence. Methods Eighteen teenagers with avulsed fracture of tibialintercondylar eminence hospitalized from January 2013 to September 2014 were included in the study. Before operation, psychological care and operative preparation were delicately done. After operation, a plan for function training was made, the preventive measures for related complications were taken and discharge instruction was performed. Results All patients got recovered after function training 3 months since operation. The average score by Lysholm evaluation was (97.6 ± 2.3). Among them, one was Lachman test I positive, with mildly limited extension of the knee join. Conclusion The following nursing strategies inclucling psychological nursing before operation can help to reduce the incidence of complications.
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Objective: To study the apoptosis of T lymphocyte subsets of peripheral blood in systemic lupus erythematosus(SLE)and the correlated pathogenesis.Methods: The percentages of T lymphocyte subsets,the positive expression rates of Fas/FasL on T lymphocyte subsets,and the apoptosis of T lymphocyte subsets were determined by three-colorflow cytometry.The level of serum IL-10 was analyzed by ABCELISA.IL-10 antibody and FasL antibody were added respectively into the 48 hours' culture of 10 SLE patients' PBMCs in vitro,whose serum IL-10 significantly increased, and then, the changes of T lymphocyte subsets of SLE PBMCs were determined by three-colodlow cytometry.Results: 1)In SLE, mainly in hyperactive SLE, the apoptesis of CD4~+ T lymphocytes increased significantly(P＜0.05),and the ratio of CD4~+/CD8~+ decreased compared with normal controls,and the abnormal apoptesis of CD4~+ T lymphocytes had a positive correlation with the increased expression rates of Fas/FasL on CD4~+ and CD8~+ T lymphocytes(P＜0.05).2)The serum IL-10 level increased significantly in SLE (P＜0.01), expecially in hyperactive SLE, and serum IL-10 had a positive correlation with the level of serum anti-dsDNA antibodies, CD4~+/CD8 + ratio, and the positive expression rate of FasL on CD4 * T lymphocytes(P＜0.05).3)According to a tracking experiment on two different groups of SLE patients,we found that:with the stabilization of the illness,the level of serum IL-10 decreased significantly, the positive expression rate of Fas/FasL on T lymphocytes,particularly on CD4~+ T lymphocytes, reduced evidently,and the apoptosis of CD4~+ T lymphocytes decreased(P＜0.05).Conclusion: The abnormal activities of T lymphocytes, particularly of CD4~+ T lymphocytes from patients with SLE,which lead to AICD,cause the percentage of CD4~+ T lymphocytes and the ratio of CD4~+/CD8~+ to decrease,disorder immune response, and accelerate the process of SLE.As a key factor in modulating the positive expression rate of Fas/FasL on CD4~+ T lymphocytes,IL-10 plays an important role in causing SLE.
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Objective:To study the apoptosis of T lymphocyte subsets of peripheral blood in systemic lupus erythematosus(SLE) and the correlated pathogenesis.Methods:The percentages of T lymphocyte subsets,the positive expression rates of Fas/FasL on T lymphocyte subsets,and the apoptosis of T lymphocyte subsets were determined by three-colorflow cytometry.The level of serum IL-10 was analyzed by ABC-ELISA.IL-10 antibody and FasL antibody were added respectively into the 48 hours' culture of 10 SLE patients' PBMCs in vitro,whose serum IL-10 significantly increased,and then,the changes of T lymphocyte subsets of SLE PBMCs were determined by three-colorflow cytometry.Results:1) In SLE,mainly in hyperactive SLE,the apoptosis of CD4+ T lymphocytes increased significantly(P