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Abstract@#There are many problems in emotion ability development of hearing impaired children, such as emotion recognition, emotion expression obstacle, emotion understanding limitation and emotion regulation impropriety, etc., which seriously affect their social adaptation and mental health. Making systematic and individualized intervention programs can effectively promote the development of emotion ability of hearing impaired children. Based on the analysis of the characteristics and influencing factors of the emotion ability development of hearing impaired children, this paper reviews the existing intervention studies, and proposes future research directions in view of the deficiencies of existing studies, in order to provide reference for the research on emotion ability intervention of hearing impaired children.
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Abstract@#Emotion ability has substantial influence on individuals survival and development as well as mental health. Emotion ability development disorder has become a prominent performance of hearing impaired children, which has attracted the attention of researchers at home and abroad. From the four aspects of emotion recognition, emotion understanding, emotion expression and emotion regulation, this paper sorts out and comments on the previous studies, puts forward the intervention measures in the light of the concrete problems existing in the development, and proposes some suggestions for the curtent study limitations, in order to provide fundamental evidance for promotion of emotion ability of hearing impaired children.
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<p><b>OBJECTIVE</b>To investigate the clinical features and surgical strategy for pediatric intractable epilepsy due to posterior quadrantic cortical dysplasia and to assess the surgical outcomes.</p><p><b>METHODS</b>The clinical features and preoperative evaluation results of 14 children with intractable epilepsy due to posterior quadrantic cortical dysplasia were retrospectively analyzed. The localization values of video-electroencephalography and intraoperative monitoring and the indications, advantages and disadvantages of temporoparietooccipital disconnection were evaluated.</p><p><b>RESULTS</b>The 14 children had different seizure types, of which spasm was the most common one. The lesions of cortical dysplasia involved the central cerebral region in 2 cases. After temporoparietooccipital disconnection in 14 patients, 13 cases were seizure-free; only one case still had seizures, but the frequency dropped by more than 50%.</p><p><b>CONCLUSIONS</b>Temporoparietooccipital disconnection is a safe and effective surgical procedure for children with intractable epilepsy due to posterior quadrantic cortical dysplasia.</p>
Subject(s)
Child , Child, Preschool , Female , Humans , Infant , Male , Electroencephalography , Epilepsy , General Surgery , Evoked Potentials, Somatosensory , Malformations of Cortical DevelopmentABSTRACT
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is one of the most common autoimmune encephalitis in children with characterized clinical features. Here we review clinical presentations of typical and atypical anti-NMDAR encephalitis and characteristics of clinical presentations of pediatric anti-NMDAR encephalitis.
Subject(s)
Child , Humans , Anti-N-Methyl-D-Aspartate Receptor Encephalitis , DiagnosisABSTRACT
<p><b>BACKGROUND</b>Wolf-Hirschhorn syndrome (WHS) results from the partial deletion of 4p. This study aimed to identify and fine map the chromosome deletion regions of Chinese children with Wolf-Hirschhorn syndrome among the developmental delay/mental retardation (DD/MR) patients.</p><p><b>METHODS</b>We analyzed the relationship of phenotype and genotype. Inclusion criteria were: moderate to severe DD/MR, no definite perinatal brain injury, and no trauma, toxication, hypoxia, infection of central nervous system; routine karyotyping was normal, no evidence of typical inherited metabolic disorder or specific neurodegenerative disorders from cranial neuro-imaging and blood/urinary metabolic diseases screening; no mutation of FMR1 in male patients, no typical clinical manifestation of Rett syndrome in female patients. Multiplex ligation-dependent probe amplification (MLPA) and Affymetrix genome-wide human SNP array 6.0 assays were applied to accurately define the exact size of subtelomeric aberration region of four WHS patients.</p><p><b>RESULTS</b>All four WHS patients presented with severe DD, hypotonia and microcephaly, failure to thrive, 3/4 patients with typical facial features and seizures, 2/4 patients with congenital heart defects and cleft lip/palate, 1/4 patients with other malformations. The length of the deletions ranged from 3.3 Mb to 9.8 Mb. Two of four patients had "classic" WHS, 1/4 patients had "mild"-to-"classic" WHS, and 1/4 patients had "mild" WHS.</p><p><b>CONCLUSIONS</b>WHS patients in China appear to be consistent with those previously reported. The prevalence of signs and symptoms, distribution of cases between "mild" and "classic" WHS, and the correlation between length of deletion and severity of disease of these patients were all similar to those of the patients from other populations.</p>
Subject(s)
Adolescent , Child , Female , Humans , Male , Genotype , Mutation , Phenotype , Polymorphism, Single Nucleotide , Genetics , Wolf-Hirschhorn Syndrome , Genetics , PathologyABSTRACT
FMDV 2A peptide was introduced as a linker between GP5 and M protein of porcine reproduction and respiratory syndrome virus (PRRSV) to allow automatic self-cleavage the polyproteins. This strategy simultaneously displayed the neutralizing action of GP5 protein and cell-mediated immunity of M protein. We put them into the expression cassette of adenovirus vector. The results of RT-PCR, IFA and Western blotting showed that GP5 and M protein were not only expressed correctly, but also self-cleavaged and assemble heterodimers formation. To detect the advantages of rAd-GP5-2A-M, we also constructed some other recombinant adenoviruses (rAd-GP5, rAd-M and rAd-GP5-M) as control. After inoculated subcutaneously into BALB/c mice, the four recombinant adenoviruses can induce PRRSV-specific antibodies and cell-mediated immune response, but the level of humoral and cell-mediated immune response against PRRSV induced by rAd-GP5-2A-M is the strongest among the four recombinant adenoviruses. All of these suggested that it is possible to develop one multi-gene engineering vaccine utilizing FMDV 2A peptide, and also provided a novel strategy for developing other viral disease vaccine.
Subject(s)
Animals , Female , Mice , Adenoviridae , Genetics , Metabolism , Immunization , Mice, Inbred BALB C , Recombinant Fusion Proteins , Genetics , Allergy and Immunology , Metabolism , Swine , Vaccines, Synthetic , Allergy and Immunology , Viral Envelope Proteins , Genetics , Allergy and Immunology , Metabolism , Viral Matrix Proteins , Genetics , Allergy and Immunology , Metabolism , Viral Vaccines , Allergy and ImmunologyABSTRACT
The apoptosis of RK13 cells induced by RHDV was investigated with DAPI staining, DNA ladder, Caspase 3 activity and flow cytometry, etc. The results showed that nuclear staining of infected cells with DAPI showed gradually morphological changes of the nuclei. As shown in the paper, a canonic oligonucleosome-sized DNA ladder was observed in cells harvested at 24h, 48h and 72h post-infection, confirming that DNA fragmentation was induced by RHDV infection. The results of flow cytometry showed that about 63% of cells were in apoptosis at 48h post-infection. Besides, we also demonstrated that the activation of Caspase 3 occurred during the infection process. In conclusion, our results showed that apoptosis in RHD might be determinant in the development of the pathogenesis of RHD.
Subject(s)
Animals , Rabbits , Apoptosis , Caliciviridae Infections , Genetics , Virology , Caspase 3 , Metabolism , Cell Line , Cell Nucleus , Genetics , Virology , DNA Fragmentation , Hemorrhagic Disease Virus, Rabbit , PhysiologyABSTRACT
To provide an efficient and safe technology platform for studying the replication and pathogenesis mechanisms of RHDV, the interaction between the RHDV and its host cells, a replicon system of RHDV, was constructed based on the infectious cDNA clone of RHDV, in which VP60 gene encoding the capsid protein was deleted, but all the necessary protease coding regions and non-coding regions were retained. Results from RT-PCR, IFA and qRT-PCR confirmed that the replicon RNA could efficiently replicate in RK-13 cells. Besides, the results also suggested that the capsid protein which is the structural protein of RHDV is necessary for maintaining the viral infectivity.