BACKGROUND Tourette syndrome(TS)is recognized as a neurodevelopmental disorder profoundly influenced by familial factors,particularly family functioning.However,the relationship among family functioning,tic severity,a...BACKGROUND Tourette syndrome(TS)is recognized as a neurodevelopmental disorder profoundly influenced by familial factors,particularly family functioning.However,the relationship among family functioning,tic severity,and quality of life in individuals with TS during childhood and adolescence remains unclear.We hypothesized that family functioning plays a role in the association between the severity of TS and quality of life in children.AIM To determine the role of family functioning in the relationship between TS severity and quality of life.METHODS This study enrolled 139 children(male/female=113/26)with TS.We assessed tic severity using the Yale Global Tic Severity Scale,quality of life via the Tourette Syndrome Quality of Life Scale,and family functioning through the Family Assessment Device.Our analysis focused on correlating these measures and exploring the mediating role of family functioning in the relationship between tic severity and quality of life.Additionally,we examined if this mediating effect varied by gender or the presence of comorbidity.RESULTS We found that family communication dysfunction had a significant mediating effect between tic severity and both psychological symptoms(indirect effect:Β=0.0038,95%confidence interval:0.0006-0.0082)as well as physical and activities of daily living impairment(indirect effect:Β=0.0029,95%confidence interval:0.0004-0.0065).For vocal tic severity,this mediation was found to be even more pronounced.Additionally,in male participants and those without attention deficit hyperactivity disorder,the mediating effect of family communication dysfunction was still evident.CONCLUSION Our study highlights the impact of family functioning on the tic severity and the quality of life in children.This relationship is influenced by gender and comorbid conditions like attention deficit hyperactivity disorder.展开更多
Objective:This study designs and implements the health prescription of children’s health education specialized nursing clinic based on the physical and mental characteristics of children with Tourette’s syndrome.Met...Objective:This study designs and implements the health prescription of children’s health education specialized nursing clinic based on the physical and mental characteristics of children with Tourette’s syndrome.Methods:Applying the evidence-based nursing model and nursing intervention theory based on the survey research by searching China Knowledge Network,Wanfang Database and Wipro Chinese Science and Technology Journal Database,the study collected the literature on the health prescription intervention research in children’s Tourette’s disorders health education in children’s health education of specialized nursing outpatient clinic for review and combined with the specialty characteristics to formulate“Children’s Tourette’s Disorder Health Education Prescription.”Results:Tourette’s syndrome in children is a common pediatric neurological disorder,mainly manifested as involuntary,rapid and repeated muscle twitching and vocal tics,which brings serious psychological pressure and life disturbance to the affected children.Conclusion:For children with Tourette’s syndrome,healthcare professionals should give enough care and understanding to the children and their parents,provide timely and effective health education to them,help them improve their bad behavioral habits,establish correct cognitive attitudes,enhance self-confidence,improve patient compliance,reduce the frequency of recurrence,reduce the incidence of complications and promote children’s physical and mental health development.展开更多
Tourette＇s syndrome is treated by behavioral or pharmacological therapy.However,patients with malignant Tourette＇s syndrome also exhibit life-threatening symptoms,which are unresponsive to conservative treatments or...Tourette＇s syndrome is treated by behavioral or pharmacological therapy.However,patients with malignant Tourette＇s syndrome also exhibit life-threatening symptoms,which are unresponsive to conservative treatments or neurosurgical procedures,such as deep brain stimulation.In recent years,mesenchymal stem cells（MSCs）have shown therapeutic potential in many neurological diseases.Therefore,the present study proposed to use MSC transplantation as a novel therapy for Tourette＇s syndrome.Stereotypic behaviors in Tourette＇s syndrome rats decreased significantly at21 days after human MSCs transplantation into the striatum.Immunohistochemistry analyses revealed survival of transplanted human MSCs and differentiation into neurons and astrocytes in the rat brain.Results suggest that intrastriatal transplantation of human MSCs could provide therapeutic potential for Tourette＇s syndrome.展开更多
AIM:To investigate whether the axial length(AL)/total corneal refractive power(TCRP)ratio is a sensitive and simple factor that can be used for the early diagnosis of Marfan’s syndrome(MFS)in children.METHODS:The rel...AIM:To investigate whether the axial length(AL)/total corneal refractive power(TCRP)ratio is a sensitive and simple factor that can be used for the early diagnosis of Marfan’s syndrome(MFS)in children.METHODS:The relationship between the AL/TCRP ratio and the diagnosis of MFS for 192 eyes in 97 children were evaluate.The biological characteristics,including age,sex,AL,and TCRP,were collected from medical records.Receiver operating characteristic(ROC)curve analysis was performed to investigate whether the AL/TCRP ratio effectively distinguishes MFS from other subjects.The Youden index was used to re-divide the whole population into two groups according to an AL/TCRP ratio of 0.59.RESULTS:Of 96 subjects(mean age 7.46±3.28 y)evaluated,56(110 eyes)had a definite diagnosis of MFS in childhood based on the revised Ghent criteria,41(82 eyes)with diagnosis of congenital ectopia lentis(EL)were included as a control group.AL was negatively correlated with TCRP,with a linear regression coefficient of-0.36(R2=0.08).A significant correlation was found between age and the AL/TCRP ratio(P=0.023).ROC curve analysis showed that the AL/TCRP ratio distinguished MFS from the other patients at a threshold of 0.59.MFS patients were present in 24/58(41.38%)patients with an AL/TCRP ratio of≤0.59 and in 34/39(87.18%)patients with an AL/TCRP ratio of>0.59.CONCLUSION:An AL/TCRP ratio of>0.59 is significantly associated with the risk of MFS.The AL/TCRP ratio should be measured as a promising marker for the prognosis of children MFS.Changes in the AL/TCRP ratio should be monitored over time.展开更多
Objective:To explore the structural and functional characteristics of microbiota in oropharynx of subhealthy children with gastrointestinal heat retention syndrome(GHRS)differentiated by traditional Chinese medicine,a...Objective:To explore the structural and functional characteristics of microbiota in oropharynx of subhealthy children with gastrointestinal heat retention syndrome(GHRS)differentiated by traditional Chinese medicine,and screen the biometric operational taxonomic units(OTUs)to assist the clinical diagnosis.Methods:We recruited children according to the“GHRS diagnostic scale”,collected their oropharyngeal swabs,and sequenced the 16 SrDNA V4 region.We described the bacterial structure with alpha-indexes,beta-distances,and relative abundances;moreover,we screened the differential genera/OTUs with Wilcoxon rank-sum test,Metagenome Seq analysis,and linear discriminant analysis effect size(LEf Se)analysis,in which biometric OTUs were selected to construct the receiver operating characteristic curve to verify the diagnostic value.The bacterial function was predicted with Kyoto Encyclopedia of Genes and Genomes pathways according to 16S rDNA gene by using Phylogenetic Investigation of Communities by Reconstruction of Unobserved States.Results:The study population was composed of 10 children with GHRS and 10 healthy control children.GHRS children were more likely to overeat(gluttony,P=.033).Alpha-indexes,such as Sobs,abundancebased coverage estimator,Bootstrap,and Qstat,were significantly higher in the GHRS group,while betadistances did not exhibit any significant intergroup differences.There were 9 differently distributed nonpredominant genera between the groups in Wilcoxon rank-sum test,as well as 13 non-predominant genera in Metagenome Seq analysis and 3 non-predominant OTUs in LEfSe analysis.OTU44 and OTU196 were used to construct the receiver operating characteristic curve,and the area under curve was 0.92.Predicted functions showed that pathways related to oxidative phosphorylation and carbon metabolism were enriched in healthy control samples,while the pathway related to renin secretion was remarkably enriched in GHRS samples.Conclusion:Unique oropharyngeal microbial structure and function were identified in GHRS children.OTU44 and OTU196 were specific OTUs,which could be used as biomarkers of GHRS to assist clinical diagnosis.展开更多
目的探索阿立哌唑治疗Tourette综合征共患注意缺陷多动障碍患儿的疗效及安全性。方法选择Tourette综合征共患注意缺陷多动障碍患儿44例,随机分为阿立哌唑组和氟哌啶醇组,分别给予阿立哌唑和氟哌啶醇治疗12周,治疗前后采用耶鲁综合抽动...目的探索阿立哌唑治疗Tourette综合征共患注意缺陷多动障碍患儿的疗效及安全性。方法选择Tourette综合征共患注意缺陷多动障碍患儿44例,随机分为阿立哌唑组和氟哌啶醇组,分别给予阿立哌唑和氟哌啶醇治疗12周,治疗前后采用耶鲁综合抽动严重程度量表(Yale global tic severity scale,YGTSS)和Conners(父母)症状问卷提供的多动指数标准评估患儿抽动症状及多动指数改善情况,并记录治疗过程中发生的药物副反应。结果重复测量方差分析示,对于YGTSS评分,分组主效应无统计学意义(P>0.05),时间主效应、分组与时间的交互效应有统计学意义(P<0.05);对于多动指数,分组主效应、时间主效应、分组与时间的交互效应均有统计学意义(P<0.05)。治疗12周时,两组YGTSS评分无统计学差异(P>0.05),阿立哌唑组多动指数低于氟哌啶醇组(P<0.01)。阿立哌唑组出现副反应者(3/22)比氟哌啶醇组(6/22)少(P<0.05)。结论阿立哌唑治疗Tourette综合征共患注意缺陷多动障碍患儿的抽动症状与氟哌啶醇相当,且可一定程度减轻其多动、注意力缺陷等症状,副作用较氟哌啶醇少。展开更多
Attention deficit hyperactivity disorder(ADHD) is the most common neurodevelopmental disorder in children and adolescents, with prevalence ranging between 5% and 12% in the developed countries. Tic disorders(TD) are c...Attention deficit hyperactivity disorder(ADHD) is the most common neurodevelopmental disorder in children and adolescents, with prevalence ranging between 5% and 12% in the developed countries. Tic disorders(TD) are common co-morbidities in paediatric ADHD patients with or without pharmacotherapy treatment. There has been conflicting evidence of the role of psychostimulants in either precipitating or exacerbating TDs in ADHD patients. We carried out a literature review relating to the management of TDs in children and adolescents with ADHD through a comprehensive search of MEDLINE, EMBASE, CINAHL and Cochrane databases. No quantitative synthesis(meta-analysis) was deemed appropriate. Metaanalysis of controlled trials does not support an association between new onset or worsening of tics and normal doses of psychostimulant use. Supratherapeutic doses of dextroamphetamine have been shown to exacerbate TD. Most tics are mild or moderate and respond to psychoeducation and behavioural management. Level A evidence support the use of alpha adrenergic agonists, including Clonidine and Guanfacine, reuptake noradrenenaline inhibitors(Atomoxetine) and stimulants(Methylphenidate and Dexamphetamines) for the treatment of Tics and comorbid ADHD. Priority should be given to the management of co-morbid Tourette's syndrome(TS) or severely disabling tics in children and adolescents with ADHD. Severe TDs may require antipsychotic treatment. Antipsychotics, especially Aripiprazole, are safe and effective treatment for TS or severe Tics, but they only moderately control the co-occurring ADHD symptomatology. Short vignettes of different common clinical scenarios are presented to help clinicians determine the most appropriate treatment to consider in each patient presenting with ADHD and co-morbid TDs.展开更多
AIM: To review pediatric cases of orofacial granulomatosis (OFG), report disease characteristics, and explore the association between OFG and Crohn’s disease.
基金Supported by the National Natural Science Foundation of China,No.82171538the Beijing High Level Public Health Technology Talent Construction Project,No.Discipline Leader-01-07.
文摘BACKGROUND Tourette syndrome(TS)is recognized as a neurodevelopmental disorder profoundly influenced by familial factors,particularly family functioning.However,the relationship among family functioning,tic severity,and quality of life in individuals with TS during childhood and adolescence remains unclear.We hypothesized that family functioning plays a role in the association between the severity of TS and quality of life in children.AIM To determine the role of family functioning in the relationship between TS severity and quality of life.METHODS This study enrolled 139 children(male/female=113/26)with TS.We assessed tic severity using the Yale Global Tic Severity Scale,quality of life via the Tourette Syndrome Quality of Life Scale,and family functioning through the Family Assessment Device.Our analysis focused on correlating these measures and exploring the mediating role of family functioning in the relationship between tic severity and quality of life.Additionally,we examined if this mediating effect varied by gender or the presence of comorbidity.RESULTS We found that family communication dysfunction had a significant mediating effect between tic severity and both psychological symptoms(indirect effect:Β=0.0038,95%confidence interval:0.0006-0.0082)as well as physical and activities of daily living impairment(indirect effect:Β=0.0029,95%confidence interval:0.0004-0.0065).For vocal tic severity,this mediation was found to be even more pronounced.Additionally,in male participants and those without attention deficit hyperactivity disorder,the mediating effect of family communication dysfunction was still evident.CONCLUSION Our study highlights the impact of family functioning on the tic severity and the quality of life in children.This relationship is influenced by gender and comorbid conditions like attention deficit hyperactivity disorder.
文摘Objective:This study designs and implements the health prescription of children’s health education specialized nursing clinic based on the physical and mental characteristics of children with Tourette’s syndrome.Methods:Applying the evidence-based nursing model and nursing intervention theory based on the survey research by searching China Knowledge Network,Wanfang Database and Wipro Chinese Science and Technology Journal Database,the study collected the literature on the health prescription intervention research in children’s Tourette’s disorders health education in children’s health education of specialized nursing outpatient clinic for review and combined with the specialty characteristics to formulate“Children’s Tourette’s Disorder Health Education Prescription.”Results:Tourette’s syndrome in children is a common pediatric neurological disorder,mainly manifested as involuntary,rapid and repeated muscle twitching and vocal tics,which brings serious psychological pressure and life disturbance to the affected children.Conclusion:For children with Tourette’s syndrome,healthcare professionals should give enough care and understanding to the children and their parents,provide timely and effective health education to them,help them improve their bad behavioral habits,establish correct cognitive attitudes,enhance self-confidence,improve patient compliance,reduce the frequency of recurrence,reduce the incidence of complications and promote children’s physical and mental health development.
文摘Tourette＇s syndrome is treated by behavioral or pharmacological therapy.However,patients with malignant Tourette＇s syndrome also exhibit life-threatening symptoms,which are unresponsive to conservative treatments or neurosurgical procedures,such as deep brain stimulation.In recent years,mesenchymal stem cells（MSCs）have shown therapeutic potential in many neurological diseases.Therefore,the present study proposed to use MSC transplantation as a novel therapy for Tourette＇s syndrome.Stereotypic behaviors in Tourette＇s syndrome rats decreased significantly at21 days after human MSCs transplantation into the striatum.Immunohistochemistry analyses revealed survival of transplanted human MSCs and differentiation into neurons and astrocytes in the rat brain.Results suggest that intrastriatal transplantation of human MSCs could provide therapeutic potential for Tourette＇s syndrome.
基金Supported by the National Natural Science Foundation of China(No.81770908)the Shanghai Science and Technology Commission(Scientific Innovation Project,No.20Y11911000)。
文摘AIM:To investigate whether the axial length(AL)/total corneal refractive power(TCRP)ratio is a sensitive and simple factor that can be used for the early diagnosis of Marfan’s syndrome(MFS)in children.METHODS:The relationship between the AL/TCRP ratio and the diagnosis of MFS for 192 eyes in 97 children were evaluate.The biological characteristics,including age,sex,AL,and TCRP,were collected from medical records.Receiver operating characteristic(ROC)curve analysis was performed to investigate whether the AL/TCRP ratio effectively distinguishes MFS from other subjects.The Youden index was used to re-divide the whole population into two groups according to an AL/TCRP ratio of 0.59.RESULTS:Of 96 subjects(mean age 7.46±3.28 y)evaluated,56(110 eyes)had a definite diagnosis of MFS in childhood based on the revised Ghent criteria,41(82 eyes)with diagnosis of congenital ectopia lentis(EL)were included as a control group.AL was negatively correlated with TCRP,with a linear regression coefficient of-0.36(R2=0.08).A significant correlation was found between age and the AL/TCRP ratio(P=0.023).ROC curve analysis showed that the AL/TCRP ratio distinguished MFS from the other patients at a threshold of 0.59.MFS patients were present in 24/58(41.38%)patients with an AL/TCRP ratio of≤0.59 and in 34/39(87.18%)patients with an AL/TCRP ratio of>0.59.CONCLUSION:An AL/TCRP ratio of>0.59 is significantly associated with the risk of MFS.The AL/TCRP ratio should be measured as a promising marker for the prognosis of children MFS.Changes in the AL/TCRP ratio should be monitored over time.
基金supported by the National Natural Science Foundation of China(81373769)the Scientific Research and Postgraduate Training Co-construction Project of Beijing Municipal Education Commission(1000062520115)the Province Natural Science Foundation of Beijing(7172131)。
文摘Objective:To explore the structural and functional characteristics of microbiota in oropharynx of subhealthy children with gastrointestinal heat retention syndrome(GHRS)differentiated by traditional Chinese medicine,and screen the biometric operational taxonomic units(OTUs)to assist the clinical diagnosis.Methods:We recruited children according to the“GHRS diagnostic scale”,collected their oropharyngeal swabs,and sequenced the 16 SrDNA V4 region.We described the bacterial structure with alpha-indexes,beta-distances,and relative abundances;moreover,we screened the differential genera/OTUs with Wilcoxon rank-sum test,Metagenome Seq analysis,and linear discriminant analysis effect size(LEf Se)analysis,in which biometric OTUs were selected to construct the receiver operating characteristic curve to verify the diagnostic value.The bacterial function was predicted with Kyoto Encyclopedia of Genes and Genomes pathways according to 16S rDNA gene by using Phylogenetic Investigation of Communities by Reconstruction of Unobserved States.Results:The study population was composed of 10 children with GHRS and 10 healthy control children.GHRS children were more likely to overeat(gluttony,P=.033).Alpha-indexes,such as Sobs,abundancebased coverage estimator,Bootstrap,and Qstat,were significantly higher in the GHRS group,while betadistances did not exhibit any significant intergroup differences.There were 9 differently distributed nonpredominant genera between the groups in Wilcoxon rank-sum test,as well as 13 non-predominant genera in Metagenome Seq analysis and 3 non-predominant OTUs in LEfSe analysis.OTU44 and OTU196 were used to construct the receiver operating characteristic curve,and the area under curve was 0.92.Predicted functions showed that pathways related to oxidative phosphorylation and carbon metabolism were enriched in healthy control samples,while the pathway related to renin secretion was remarkably enriched in GHRS samples.Conclusion:Unique oropharyngeal microbial structure and function were identified in GHRS children.OTU44 and OTU196 were specific OTUs,which could be used as biomarkers of GHRS to assist clinical diagnosis.
文摘目的探索阿立哌唑治疗Tourette综合征共患注意缺陷多动障碍患儿的疗效及安全性。方法选择Tourette综合征共患注意缺陷多动障碍患儿44例,随机分为阿立哌唑组和氟哌啶醇组,分别给予阿立哌唑和氟哌啶醇治疗12周,治疗前后采用耶鲁综合抽动严重程度量表(Yale global tic severity scale,YGTSS)和Conners(父母)症状问卷提供的多动指数标准评估患儿抽动症状及多动指数改善情况,并记录治疗过程中发生的药物副反应。结果重复测量方差分析示,对于YGTSS评分,分组主效应无统计学意义(P>0.05),时间主效应、分组与时间的交互效应有统计学意义(P<0.05);对于多动指数,分组主效应、时间主效应、分组与时间的交互效应均有统计学意义(P<0.05)。治疗12周时,两组YGTSS评分无统计学差异(P>0.05),阿立哌唑组多动指数低于氟哌啶醇组(P<0.01)。阿立哌唑组出现副反应者(3/22)比氟哌啶醇组(6/22)少(P<0.05)。结论阿立哌唑治疗Tourette综合征共患注意缺陷多动障碍患儿的抽动症状与氟哌啶醇相当,且可一定程度减轻其多动、注意力缺陷等症状,副作用较氟哌啶醇少。
文摘Attention deficit hyperactivity disorder(ADHD) is the most common neurodevelopmental disorder in children and adolescents, with prevalence ranging between 5% and 12% in the developed countries. Tic disorders(TD) are common co-morbidities in paediatric ADHD patients with or without pharmacotherapy treatment. There has been conflicting evidence of the role of psychostimulants in either precipitating or exacerbating TDs in ADHD patients. We carried out a literature review relating to the management of TDs in children and adolescents with ADHD through a comprehensive search of MEDLINE, EMBASE, CINAHL and Cochrane databases. No quantitative synthesis(meta-analysis) was deemed appropriate. Metaanalysis of controlled trials does not support an association between new onset or worsening of tics and normal doses of psychostimulant use. Supratherapeutic doses of dextroamphetamine have been shown to exacerbate TD. Most tics are mild or moderate and respond to psychoeducation and behavioural management. Level A evidence support the use of alpha adrenergic agonists, including Clonidine and Guanfacine, reuptake noradrenenaline inhibitors(Atomoxetine) and stimulants(Methylphenidate and Dexamphetamines) for the treatment of Tics and comorbid ADHD. Priority should be given to the management of co-morbid Tourette's syndrome(TS) or severely disabling tics in children and adolescents with ADHD. Severe TDs may require antipsychotic treatment. Antipsychotics, especially Aripiprazole, are safe and effective treatment for TS or severe Tics, but they only moderately control the co-occurring ADHD symptomatology. Short vignettes of different common clinical scenarios are presented to help clinicians determine the most appropriate treatment to consider in each patient presenting with ADHD and co-morbid TDs.
文摘AIM: To review pediatric cases of orofacial granulomatosis (OFG), report disease characteristics, and explore the association between OFG and Crohn’s disease.
