摘要
Tic disorder (TD) is a neurodevelopmental disorder that occurs in childhood, characterized by involuntary movements or vocal tics and occasionally accompanied by various comorbidities.It may seriously affect children′s daily life, learning and social activities.Exploring its etiology and pathological mechanisms and developing drugs require extensive research on animal models.There are currently various methods for constructing TD models, including induced and spontaneous animal models, such as induced animal models with abnormal neurotransmitters and immune functions and spontaneous animal models based on genetic engineering.However, no animal model can fully match the manifestations of TD.This article summarizes the commonly used animal models for TD to provide references for exploring pathological mechanisms and developing drugs.
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Objective To explore the clinical efficacy of laser-assisted sclerectomy and cataract extraction combined with angle separation in the treatment of angle-closure glaucoma.Methods A total of 162 patients with angle closure glaucoma were selected as the research subjects.Eighty-one patients in the experimental group under-went laser-assisted sclerectomy and cataract extraction combined with angle separation,and another 81 patients in the control group underwent cataract extraction combined with angle separation.The therapeutic effects of two groups of patients were observed.Results The postoperative visual acuity,BCVA,angle width,and corneal endothelial cell count of the experimental group were higher than those of the control group,but the intraocular pressure,central anterior chamber depth,and corneal thickness were all lower than those in the control group.The incidence of adverse reactions in the experimental group(6.2% )was lower than that in the control group(13.6% ).During postop-erative follow-up,there was no further increase in intraocular pressure in the two groups,while the height of filtering blebs in the control group decreased significantly when compared with that in the experimental group.One month after surgery,the BCVA and corneal endothelial cell count in the experimental group were higher than those in the control group(P<0.05),and the corneal thickness was lower than that in the control group(P<0.05),but no statistical significance was found at 3 and 6 months after surgery.The astigmatism in the experimental group was better than that in the control group 3 months after surgery,but there was no statistically significant difference at 1 month and 6 months after surgery.Conclusion Laser-assisted sclerectomy and cataract extraction combined with angle separa-tion are effective and safe in the treatment of angle closure glaucoma.
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Abstract@#Currently, the school health system in China is transitioning from basic safeguarding to comprehensive health management and has been elevated to a national strategic level. However, the diversification of students health issues and the imbalance in resource allocation remain major challenges. To address issues such as adolescent myopia, obesity, spinal curvature, mental health, and infectious diseases, there is an urgently need to build a highlevel school health system that encompasses multiple aspects such as resource allocation optimization, professional skill enhancement, and health education improvement. Simultaneously, it is need to deepen the research and implementation of common disease prevention strategies for students. It will play a vital role in promoting the construction of Healthy China, the modernization of education, and the establishment of a highquality disease control system.
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Objective@#To explore the current status and progress of regional school health work to provide policy reference for school health improvement.@*Methods@#Survey data on school health work in Tianjin from 2019, 2021 and 2023 was used. School health staff allocation and expenditure of the health administrative department, CDC and education department, as well as the annual implementation of health education, prevention and control of common diseases and infectious diseases, sports activities and food nutrition in primary and secondary schools were analyzed. Statistical analysis was conducted using KruskalWallis test, Chisquare test, and Fishers exact test.@*Results@#The number of school health staff in the health commissions and education departments from 2019, 2021 and 2023 was relatively stable. Parttime staffs were often employed by health commissions while fulltime staffs were mainly employed by education departments. The number of school health staff at CDCs increased gradually (H=12.65, P<0.01). School health expenditure of administrative departments and schools in 2021 and 2023 increased significantly compared with that in 2019 (H=22.28, 23.75, P<0.05). More than 95% of schools set up clinics or health care rooms, and about 97% of schools had school health technicians or health teachers. More than 90% of schools had health education courses over 4 hours per semester. The rate of mental health education increased by year (86.87%, 89.91%, 96.30%, Z=2.40，P<0.05). Lack of courses regarded safety emergency and risk avoidance, growth and development, and adolescent health education. The provision rate of psychological counseling services (89.00%, 97.25%, 100.00%) and psychological problem prevention and control (56.12%, 71.56%, 81.48%) also increased by year (Z=3.83, 3.96, P<0.01). The implementation rates of prevention and control of poor vision, dental caries, overweight and obesity were all higher than 80%, and the prevention and control rate of abnormal spinal curvature showed an increasing trend (38.78%, 77.06%, 72.22%, Z=4.87, P<0.01). More than 90% of schools met the standard for physical education class hours, and the proportion of schools conducting at least 30 minutes of recess physical activities every day increased year by year (65.00%, 80.73%, 85.98%, Z=3.59, P<0.01). All schools did not have shops.@*Conclusions@#School health work in Tianjin is effective and constantly developing. It is necessary to continue to increase the investment of human resources and expenditure in school health, explore the approaches of cooccurrence and prevention of common diseases, and improve the school sports and nutrition environment.
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Objective@#To explore the efficacy and safety of clonidine adhesive patch in Tourette syndrome (TS) patients with comorbid attentiondeficit/hyperactivity disorder (ADHD). @*Methods@#This study was conducted on a sample of children and adolescents with TS who had comorbid ADHD between May 2012 and March 2015. The patients were diagnosed according to Diagnostic and Statistical Manual of Mental Disorders Fourth Edition, and were randomly assigned to four different dose groups: 1.0 mg/week, 1.5 mg/week, 2.0 mg/week and placebo group, and the symptom was evaluated by Swanson, Nolan, and Pelham Rating Scale, Version IV (SNAP-IV) and Yale Global Tic Severity Scale scales every 2 weeks. The primary outcome was tic disorders (TD) effective rate at week 8. @*Results@#One hundred and twenty-seven TS patients with comorbid ADHD in 2.0 mg/week (n=35), 1.5 mg/week (n=27), 1.0 mg/week (n=36) and placebo groups (n=29) were included in this subgroup analysis. The TD effective rate of the 2.0 mg, 1.5 mg, and 1.0 mg groups at week 8 were significantly better than that in placebo group (85.7%, 81.5%, and 86.1% vs. 20.7%, all p<0.0001). All groups demonstrated significant improvements in SNAP-IV total scale scores compared to baseline (p=0.0004), with treatment groups showing only a trend for better performance compared to placebo group at week 8, without statistical differences (22.1±15.41, 21.3±11.96, and 21.2±12.48 vs. 26.0±13.37, p=0.3385). A total of 9 adverse reactions occurred, all recovered spontaneously without additional medication. @*Conclusion@#Clonidine adhesive patch could safely and effectively reduce the tic symptoms of TS patients with comorbid ADHD, and might be potentially helpful in the ADHD symptoms control.
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OBJECTIVE@#To explore the clinical phenotype and genetic features of two children with MEGDEL syndrome due to variants of the SERAC1 gene.@*METHODS@#Two children who had presented at the Fujian Medical University Union Hospital respectively on July 14, 2020 and July 28, 2018 were selected as the study subjects. Clinical features and results of genetic testing were retrospectively analyzed.@*RESULTS@#Both children had featured developmental delay, dystonia and sensorineural deafness, along with increased urine 3-methylglutaric acid levels. Magnetic resonance imaging revealed changes similar to Leigh-like syndrome. Gene sequencing revealed that both children have harbored pathogenic compound heterozygous variants of the SERAC1 gene, including c.1159C>T and c.442C>T in child 1, and c.1168C>T and exons 4~9 deletion in child 2.@*CONCLUSION@#Children with MEGDEL syndrome due to SERAC1 gene variants have variable clinical genotypes. Delineation of its clinical characteristics and typical imaging changes can facilitate early diagnosis and treatment. Discovery of the novel variants has also enriched the spectrum of SERAC1 gene variants.
Subject(s)
Humans , Retrospective Studies , Metabolism, Inborn Errors , Hearing Loss, Sensorineural/genetics , Dystonia , Carboxylic Ester Hydrolases摘要
The clinical data of a child with MORC2 gene mutation related neurodevelopmental disorder treated in Fujian Medical University Union Hospital in July 2020 were analyzed retrospectively.The male (7-year-old)patient was global retardation from infant, with special face, short stature, small head circumference, decreased muscle strength and positive pyramidal tract sign of lower limbs.Brain magnetic resonance imaging was similar to the changes of Leigh syndrome.Genetic testing found de novo mutation in MORC2 gene chr22: 31345763, c.292G>A(p.Gly98Arg). And literature review found that there was only one related report. MORC2 gene mutation related neurodevelopmental disorder is a newly discovered syndrome, and c. 79G>A(p.Glu27Lys) is the most common mutation.This case enriched the clinical phenotype and genotype of neurodevelopmental disorder related to MORC2 gene.
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Objective:To observe the clinical effect of Perampanel on the add-on therapy in children with drug-resistant epilepsy.Methods:Clinical data of children with drug-resistant epilepsy treated with add-on therapy of Perampanel in the Department of Pediatrics, Fujian Medical University Union Hospital from January to June 2020 were retrospectively analyzed, aiming to assess the therapeutic efficacy of Perampanel on the add-on therapy of drug-resistant epilepsy.The self-control effective rate of Perampanel before and after treatment were counted.Results:A total of 20 cases of 2-12 year-old children with drug-resistant epilepsy were collected, including 14 males and 6 females.Their mean age, age of onset and course of disease were (5.82±2.39) years, (3.41±1.96) years and (2.40±1.48) years, respectively.Among them, 1 case had simple partial seizures, 7 cases had complex partial seizures, 1 case had generalized seizures and 5 cases had epilepsy syndromes, there were 6 cases with undetermined seizure attack.After 3-month add-on therapy of Perampanel, 4 cases of children with drug-resistant epilepsy were seizure-free, 8 cases had the reduced frequency of seizure for 50% or more, 2 cases had the reduced duration of seizure, 1 case had the reduced severity of seizure, while 4 cases did not respond to perampanel and 1 case was aggravated.Based on the criteria of reduced frequency of seizure for more than 50%, the therapeutic efficacy of add-on therapy of Perampanel achieved 60% in children with drug-resistant epilepsy.Conclusions:The third generation of anti-seizure medication Perampanel can effectively reduce the frequency of seizure, especially in the elder children and those with certain epilepsy syndromes.
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Objective:To explore the relationship between serum cortisol and attention deficit hyperactivity disorder (ADHD), and to investigate the application of cortisol in the diagnosis of ADHD, so as to provide clues and theoretical basis for the comprehensive prevention and treatment of ADHD in the future.Methods:Serum cortisol levels were detected in 159 ADHD children [ADHD group, 58 cases of predominately inattentive presentation (ADHD-I), 32 cases of predominately hyperactive/impulsive presentation (ADHD-HI), 69 cases of combined presentation (ADHD-C)], and 58 healthy control children (healthy control group) from July 2018 to June 2019, at Fujian Medical University Union Hospital.The receiver operating characteristic (ROC) was used to evaluate the diagnostic value of serum cortisol levels in ADHD groups.Results:(1) The serum cortisol levels of ADHD-I group[(216.58±70.55) nmol/L], ADHD-HI group[(182.26±51.34) nmol/L]and ADHD-C group[(222.81±75.70) nmol/L]were significantly lower than that of the healthy control group[(344.83±98.17) nmol/L](all P<0.001). The level of cortisol in ADHD-HI group was lower than that in ADHD-I group and ADHD-C group ( P<0.05). (2)According to the ROC analysis of serum cortisol, the area under the ROC curve for the diagnosis of ADHD group was 0.866 (95% CI: 0.814-0.917), the maximum Youden index was 0.583, the corresponding sensitivity was 89.3%, the specificity was 69.0%, and the cut-off was 302.88 nmol/L.When the specificity was 85.0%, 246.13 nmol/L was the diagnostic threshold and its corresponding sensitivity was 71.1%.Under the ROC curve for the diagnosis of ADHD-I group, the area was 0.857 (95% CI: 0.792-0.922), the maximum Youden index was 0.552, the corresponding sensitivity was 69.0%, the specificity was 86.2%, and the cut-off was 243.39 nmol/L.Under the ROC curve for the diagnosis of ADHD-HI group, the area was 0.934 (95% CI: 0.887-0.980), the maximum Youden index was 0.745, the corresponding sensitivity was 96.9%, the specificity was 77.6%, and the cut-off was 261.55 nmol/L.Under the ROC curve for the diagnosis of ADHD-C group, the area was 0.841 (95% CI: 0.774-0.908), the maximum Youden index was 0.559, the corresponding sensitivity was 87.0%, the specificity was 69.0%, and the cut-off was 302.82 nmol/L.In view of parents′ lack of understanding of ADHD behavior, it is suggested that the diagnostic threshold of cortisol level of 246.13 nmol/L should be based on 85.0% specificity principle, combined with behavior verification in clinical practice, so as to improve the diagnostic accuracy. Conclusions:Cortisol levels in ADHD-I, ADHD-HI and ADHD-C groups are lower than that in control group.There is functional impairment of hypothalamic-pituitary-adrenal axis in ADHD children.The level of cortisol has certain accuracy in the diagnosis of simple ADHD and can be used in clinical diagnosis.
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Objective:To investigate the efficacy and safety of ketogenic diet (KD) therapy in the epilepsy of infancy with migrating focal seizures (EIMFS) associated with TBC1D24 gene mutation.Methods:Clinical data of two children with TBC1D24 gene-related EIMFS were collected retrospectively, who were admitted to Department of Pediatrics, Fujian Medical University Union Hospital from 2019 to 2020. Their clinical characteristics and the efficacy and safety of KD therapy were analyzed, and literature review was conducted.Results:Seizures were onset before six months old in the two children with TBC1D24 gene-related EIMFS. Multifocal myoclonic seizures were manifested and happened frequently, lasting for more than 30 minutes sometimes. Developmental retardation was obvious in the two children. A small amount of focal sharp, spike, sharp-slow complex, and spike-slow complex waves were showed in the interictal electroencephalography (EEG). TBC1D24 gene mutations were found in the two children, one with a compound heterozygous mutation (c.1025C>T, p.S342L; c.229_c.240delATCGTGGGCAAG,p.I77_K80del), and the other with a homozygous mutation [c.119G>A,p.R40H(Arg40His)]. Both of those were potentially pathogenic. A variety of anti-epileptic drugs showed poor outcome for the two children. The epilepsy was drug-refractory one. After four to 17 months of KD therapy, the epilepsy in the two children was controlled effectively. There was not obvious adverse reactions. Among six children with TBC1D24 gene-related EIMFS in the literature review, four cases were effective or partially effective for KD therapy, one was discontinued due to insufficient ketogenic ratio, and one was discontinued without effect. There were no obvious adverse reactions in the six children.Conclusions:TBC1D24 gene-related EIMFS is mostly drug-refractory epilepsy. Early KD therapy may help to control seizures.
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Objective To study the effect of ketogenic diet(KD)on vascular endothelial function in children with intractable epilepsy. Methods Clinical informations of 14 children with intractable epilepsy in Fujian Medical University Affiliated Union Hospital from May 2014 to March 2018 were collected. Their blood index values were tested and retested before and after 3 months of KD treatment,including triglycerides( TG),total cholesterol( TC),high－density lipoprotein( HDL),low density lipoprotein( LDL),nitric oxide( NO),endothelin －1( ET －1),and von Willebrand factor(vWF). These data were statistically analyzed by using repeated measurement analysis of variance. Results (1)Changes in blood lipid levels:the levels of TG and TC increased slightly[(1. 08 ± 0. 14)mmol/L, (5. 19 ± 0. 64)mmol/L νs.(0. 97 ± 0. 10)mmol/L,(4. 57 ± 0. 29)mmol/L]and the level of HDL decreased slightly [(1. 19 ± 0. 08)mmol/L νs.(1. 31 ± 0. 08)mmol/L]after 3 months of KD treatment,but the differences were not sta﹣tistically significant in the above indexes(all P＞0. 05). The level of LDL before KD was significantly lower than that after 3 months of KD[(2. 93 ± 0. 25)mmol/L νs.(3. 73 ± 0. 40)mmol/L ],and the difference was statistically signi﹣ficant(P﹦0. 034).(2)Assessment of vascular endothelial function:compared with before KD,all the levels of NO [(60. 84 ± 5. 29)μmol/L νs.(66. 45 ± 5. 39)μmol/L ],ET－1[(1. 24 ± 0. 30)ng/L νs.(2. 13 ± 0. 78)ng/L]and vWF[(150. 53 ± 12. 97)μg/L νs.(137. 57 ± 13. 10)μg/L]had no statistically significant changes after 3 months of KD(all P＞0. 05). Conclusions KD treatment lasting 3 months in children with intractable epilepsy can raise the level of LDL,but can′t change the vascular endothelial function.
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OBJECTIVE@#To delineate the clinical and genetic features of two pedigrees affected with aromatic L-amino acid decarboxylase (AADC) deficiency.@*METHODS@#The clinical features, family history and results of genetic testing of 2 patients with AADC deficiency were retrospectively analyzed.@*RESULTS@#Both patients featured hypotension, developmental delay and oculogyric crisis during infancy. Genetic testing confirmed that they have respectively carried c.714+ 4 (IVS6) A to T/c.175(exon2)G TO A compound heterozygous variants and c.714+ 4(IVS6)A to T homozygous variant.@*CONCLUSION@#The clinical manifestation of children with AADC deficiency may include hypotonia, developmental delay and paroxysmal oculogyric crisis. The combination of 3-O-methyldopa testing and variant analysis is not only very useful for early diagnosis, but also important for the evaluation of treatment effect and prognosis of the disease. Discovery of the novel variants has enriched the variant spectrum of AADC deficiency.
Subject(s)
Humans , Infant , Amino Acid Metabolism, Inborn Errors , Genetics , Aromatic-L-Amino-Acid Decarboxylases , Genetics , DNA Mutational Analysis , Genetic Testing , Pedigree , Retrospective Studies摘要
Objective@#To study the effect of ketogenic diet (KD) on vascular endothelial function in children with intractable epilepsy.@*Methods@#Clinical informations of 14 children with intractable epilepsy in Fujian Medical University Affiliated Union Hospital from May 2014 to March 2018 were collected.Their blood index values were tested and retested before and after 3 months of KD treatment, including triglycerides (TG), total cholesterol (TC), high-density lipoprotein (HDL), low density lipoprotein (LDL), nitric oxide (NO), endothelin-1 (ET-1), and von Willebrand factor (vWF). These data were statistically analyzed by using repeated measurement analysis of variance.@*Results@#(1)Changes in blood lipid levels: the levels of TG and TC increased slightly [(1.08±0.14) mmol/L, (5.19±0.64) mmol/L vs.(0.97±0.10) mmol/L, (4.57±0.29) mmol/L]and the level of HDL decreased slightly [(1.19±0.08) mmol/L vs.(1.31±0.08) mmol/L]after 3 months of KD treatment, but the differences were not statistically significant in the above indexes (all P>0.05). The level of LDL before KD was significantly lower than that after 3 months of KD [(2.93±0.25) mmol/L vs.(3.73±0.40) mmol/L ], and the difference was statistically signi-ficant(P=0.034). (2)Assessment of vascular endothelial function: compared with before KD, all the levels of NO [(60.84±5.29) μmol/L vs.(66.45±5.39) μmol/L ], ET-1 [(1.24±0.30) ng/L vs.(2.13±0.78)ng/L] and vWF [(150.53±12.97) μg/L vs.(137.57±13.10) μg/L]had no statistically significant changes after 3 months of KD(all P>0.05).@*Conclusions@#KD treatment lasting 3 months in children with intractable epilepsy can raise the level of LDL, but can′t change the vascular endothelial function.
摘要
Objective@#To delineate the clinical and genetic features of two pedigrees affected with aromatic L-amino acid decarboxylase (AADC) deficiency.@*Methods@#The clinical features, family history and results of genetic testing of 2 patients with AADC deficiency were retrospectively analyzed.@*Results@#Both patients featured hypotension, developmental delay and oculogyric crisis during infancy.Genetic testing confirmed that they have respectively carried c. 714+ 4 (IVS6) A>T/c.175(exon2)G>A compound heterozygous variants and c. 714+ 4(IVS6)A>T homozygous variant.@*Conclusion@#The clinical manifestation of children with AADC deficiency may include hypotonia, developmental delay and paroxysmal oculogyric crisis. The combination of 3-O-methyldopa testing and variant analysis is not only very useful for early diagnosis, but also important for the evaluation of treatment effect and prognosis of the disease. Discovery of the novel variants has enriched the variant spectrum of AADC deficiency.
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Objective To investigate the antimicrobial resistance profile of the Streptococcus pneumoniae strains isolated from respiratory tract of children in Zhongshan Boai Hospital, Guangdong Province for better management of such infections. Methods The sputum samples were collected from respiratory tract of children in pediatric outpatient and inpatient from May 2013 to August 2017. S. pneumoniae strains were isolated and identified and their susceptibility to antimicrobial agentswas determined. Results The prevalence of S. pneumoniae was 10.6％ (2 963/28 006) in the sputum samples. S. pneumoniae was mainly isolated from children under 6 years old, and relatively higher in winter and spring. About 43.0％ of the S. pneumoniae isolates was associated with mixed infection, especially Moraxella catarrhalis and Haemophilus influenzae. About 6.0％ of the S. pneumoniae isolates were non-susceptible to penicillin, 59.3％ non-susceptible to ceftriaxone, and more than 95％ non-susceptible to erythromycin, clindamycin or tetracycline. However, more than 95％ of the isolates were susceptible to chloramphenicol or ofloxacin. No S. pneumoniae isolate was found resistant to rifampin, linezolid or vancomycin. Conclusions The respiratory tract infection caused by S. pneumoniae of children is increasing year by year, which is associated with age, season, and higher rate of mixed infection. These data showed that penicillin non-susceptible S. pneumoniae is highly prevalent in Zhongshan. More than 95％ of the S. pneumoniae isolates from children are resistant to erythromycin, clindamycin or tetracycline. S. pneumoniae isolates should be closely monitored for the emergence of multidrug resistant strains. Appropriate control measures must be taken according to the results of susceptibility testing.
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Objective To compare the efficacy and safety of transabdominal preperitoneal prosthetic and lichtenstein operation for treating recurrent inguinal hernia.Methods In this study,116 patients with recurrent inguinal hernia treated in First People's Hospital of Xianyang from June 2012 to June 2016 were divided into transabdominal preperitoneal prosthetic group (n =58) and Lichtenstein group (n =58) according to the operation method.The operation time,hospitalization time,postoperative exhaust time and postoperative temperature and cost were compared between two groups.Postoperative complications including wound infection,urinary retention,visceral injury,and foreign body sensation in groin area were recorded.Results Lichtenstein group had significantly higher operative time,hospital stay,postoperative exhaust time and postoperative temperature than those in transabdominal preperitoneal prosthetic group [(55.4 ± 9.6) min and (41.5 ± 5.2) min;(4.15 ± 1.08) d and(2.66±0.43) d;(0.82 ±0.40) d and (0.45±0.21) d;(38.1 ±0.9) ℃ and (37.2±0.6) ℃ respectively],but the cost of lichtenstein group [(3 110 ± 614) yuan] was lower than that of transabdominal preperitoneal prosthetic group [(4 586 ± 925) yuan],with the statistically significant differenc (P ＜ 0.05).Conclusions Transabdominal preperitoneal prosthetic has better clinical outcome and safety for treating recurrent inguinal hernia,compared to litchtenstein operation.Therefore,transabdominal preperitoneal prosthetic surgery is a clinical treatment worthy listening.
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Objective To explore the change of endogenous glucocorticoids (GC) secretion after intracranial hemorrhage (ICH) of neonatal rats and the impact of Dexamethasone (DEX).Methods Ten-day-old Sprague-Dawley rat pups of both sexes were randomized into 11 groups:normal control group(CON group),sham operated group (SHAM group),ICH group(each of which was further subgrouped into 12 h group,24 h group and 72 h group according to execution time after the modeled operation),glucocorticoids receptor (GR) agonist intervene group (DEX group) and GR antagonist intervene group(RU486 group).The intracranial autologous blood injection model of ICH was employed.Neurological functional deficits was measured by neurological deficit score (NDS),the levels of cerebral homogenate GC were tested by the emission immunology method,and the pathologic change and the expression of GR in hippocampus CA1 were examined by using Nissl staining and immunofluorescence separately.Results (1) Seventy-two-hour after the modeled operation,NDS of rats in the ICH group reached (7.48 ± 2.19) scores.After intervened by DEX,NDS of rats in DEX group decreased to (3.15 ± 1.93) scores,significantly lower than in ICH group,the difference was significant (P ＜ 0.05).The necrotic neurons were found around the hematoma of rats in ICH group,while in DEX group,less necrotic neurons were found.(2)In ICH group,the GC level in cerebral homogenate climbed up to a peak of (1.359 1 ±0.308 5) μg/L at 12 h,and slowly went down.By the end of 72 h,the GC level was (0.951 0 ±0.036 1) μg/L,which was higher than those of the CON group[(0.621 3 ±0.039 3) μg/L],the difference was significant (P ＜ 0.05),while in the DEX group,the level of GC in cerebral homogenate showed no difference with statistics from CON group.(3)The mean integrated optical density (IOD) of GR in hippocampal CA1 of rats in the ICH group (1.282 4 ± 0.035 6) were much more smaller than those in the CON group (1.012 5 ± 0.027 3,P ＜ 0.05),which meant the down-regulated expression of GR.(4) No difference was found in the NDS,pathological change,GC level and GR expression between RU486 group and ICH group.DEX didn't effect the expression of GR.Conclusions ICH in neonatal rat disturbs the modulation of hypothalamus-pituitary-adrenal axis,with an increase in the GC level and less GR expression.Early application of exogenous GC helps protect the neurons.
摘要
Objective To explore the change of endogenous glucocorticoids (GC) secretion after intracranial hemorrhage (ICH) of neonatal rats and the impact of Dexamethasone (DEX).Methods Ten-day-old Sprague-Dawley rat pups of both sexes were randomized into 11 groups:normal control group(CON group),sham operated group (SHAM group),ICH group(each of which was further subgrouped into 12 h group,24 h group and 72 h group according to execution time after the modeled operation),glucocorticoids receptor (GR) agonist intervene group (DEX group) and GR antagonist intervene group(RU486 group).The intracranial autologous blood injection model of ICH was employed.Neurological functional deficits was measured by neurological deficit score (NDS),the levels of cerebral homogenate GC were tested by the emission immunology method,and the pathologic change and the expression of GR in hippocampus CA1 were examined by using Nissl staining and immunofluorescence separately.Results (1) Seventy-two-hour after the modeled operation,NDS of rats in the ICH group reached (7.48 ± 2.19) scores.After intervened by DEX,NDS of rats in DEX group decreased to (3.15 ± 1.93) scores,significantly lower than in ICH group,the difference was significant (P ＜ 0.05).The necrotic neurons were found around the hematoma of rats in ICH group,while in DEX group,less necrotic neurons were found.(2)In ICH group,the GC level in cerebral homogenate climbed up to a peak of (1.359 1 ±0.308 5) μg/L at 12 h,and slowly went down.By the end of 72 h,the GC level was (0.951 0 ±0.036 1) μg/L,which was higher than those of the CON group[(0.621 3 ±0.039 3) μg/L],the difference was significant (P ＜ 0.05),while in the DEX group,the level of GC in cerebral homogenate showed no difference with statistics from CON group.(3)The mean integrated optical density (IOD) of GR in hippocampal CA1 of rats in the ICH group (1.282 4 ± 0.035 6) were much more smaller than those in the CON group (1.012 5 ± 0.027 3,P ＜ 0.05),which meant the down-regulated expression of GR.(4) No difference was found in the NDS,pathological change,GC level and GR expression between RU486 group and ICH group.DEX didn't effect the expression of GR.Conclusions ICH in neonatal rat disturbs the modulation of hypothalamus-pituitary-adrenal axis,with an increase in the GC level and less GR expression.Early application of exogenous GC helps protect the neurons.
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Autism spectrum disorder(ASD)is a severe neurodevelopmental disorder of children that leads to disability which lacks of effective treatment.The specific pathogenesis of ASD remains unclear,which may involve in multiple factors.In recent years,many studies have shown that children with ASD have gastroin-testinal(GI)abnormalities and some special diet therapies can effectively ameliorate the symptoms of ASD. These diets include gluten free diet,sensitive or harmful food removing therapy and ketogenic diet.The patients with ASD have GI inflammation or allergy,energy metabolism disorder,oxidative stress injury,neurotransmitter disturbance and dysbiosis of intestinal flora.Diet therapy may improve ASD symptoms by correcting disorders a-bove.This article reviews the application and the related mechanism of diet therapy in ASD children and the effect of diet therapy on the growth and the development of children.
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Objective To explore the clinical and the genetic features of infantile neuroaxonal dystrophy (INAD).Methods The clinical and laboratory data,neuroimaging examination and genetic testing results of one child with INAD were retrospectively analyzed.Results A 2 years old boy presented motor and verbal dexterity regression and hypotonia.Laboratory findings revealed decreased total iron-binding capacity in serum with increased glutamic oxaloacetic transaminase (AST) and lactic dehydrogenase (LDH).Myoelectrography showed neurogenic impairments of the arms and legs,and the color doppler ultrasound of the heart,video-EEG and brain MRI results were normal.A homozygous mutation of c.1077G＞A was found in PLA2G6 gene of the infant.The infant's parents were heterozygous mutation carriers at this locus.Conclusions PLA2G6 gene mutations cause INAD.