Objective To evaluate the feasibility and safety of mediastinum examination with flexible endoscope via suprasternal fossa.Methods A total of 20 healthy pigs were enrolled.Mediastinocosopy was performed via a tunnel to thoracix through a 1.5 cm incision on suprastemal fossa.Mediastinum was observed and complications were recorded.Results Mediastinocosopy was successfully achieved in all pigs.No severe bleeding or death occurred during the procedure.Five pigs got fever after the procedure with elevated white blood cell count.Four pigs had pneumothorax and three of them died in 1-6 days.Conclusion Mediastinocosopy with flexible endoscope through suprastemal fossa is feasible to diagnose mediastinal diseases,which can be important experimental evidence for the mini-invasive therapy of mediastimum diseases.

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.

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.

Objectives: . The annual prevalence of chronic rhinosinusitis (CRS) is increasing, and the lack of effective treatments imposes a substantial burden on both patients and society. The formation of nasal polyps in patients with CRS is closely related to tissue remodeling, which is largely driven by the epithelial-mesenchymal transition (EMT). MicroRNA (miRNA) plays a pivotal role in the pathogenesis of numerous diseases through the miRNA-mRNA regulatory network; however, the specific mechanism of the miRNAs involved in the formation of nasal polyps remains unclear. Methods: . The expression of EMT markers and Smad3 were detected using western blots, quantitative real-time polymerase chain reaction, and immunohistochemical and immunofluorescence staining. Differentially expressed genes in nasal polyps and normal tissues were screened through the Gene Expression Omnibus database. To predict the target genes of miR-145-5p, three different miRNA target prediction databases were used. The migratory ability of cells was evaluated using cell migration assay and wound healing assays. Results: . miR-145-5p was associated with the EMT process and was significantly downregulated in nasal polyp tissues. In vitro experiments revealed that the downregulation of miR-145-5p promoted EMT. Conversely, increasing miR-145-5p levels reversed the EMT induced by transforming growth factor-β1. Bioinformatics analysis suggested that miR-145-5p targets Smad3. Subsequent experiments confirmed that miR-145-5p inhibits Smad3 expression. Conclusion . Overall, miR-145-5p is a promising target to inhibit nasal polyp formation, and the findings of this study provide a theoretical basis for nanoparticle-mediated miR-145-5p delivery for the treatment of nasal polyps.

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.

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.

Objective To compare and explore the differences of clinical characteristics between human adenovirus type 7 (HAdV7) and type 55 (HAdV55) infections in adults,and to provide evidences for clinical management.Methods The data of clinical manifestations,laboratory examinations,chest computed tomography and prognosis of 214 cases with HAdV7 and 235 cases with HAdV55 infections from 2012 to 2018 were retrospectively collected and analyzed.The chi-square test was used for the categorical variables,and the rank sum test was used for the continuous variables of non-normal distribution.Results Compared with patients in HAdV55,those in HAdV7 group displayed more diarrhea (12.1％ [26/214] vs 2.6％ [6/235],x2 =15.583),more laryngeal lymphatic follicles (33.2％ [71/214] vs 17.9％ [42/235],x2 =23.566),more tonsil enlargement (56.5％ [121/214] vs 20.0％ [47/235],x2 =63.870) with secretions (33.2％ [71/214] vs 11.5％ [27/235],x2 =30.878),more leukocytosis (24.8％ [53/214] vs 14.0％ [33/235],x2 =8.318),more monocytosis (78.0％ [167/214] vs 52.8％ [124/235],x2 =31.364),more thrombocytosis (7.1％ [15/212] vs 3.8％ [9/235],x2 =5.835),more elevated level of C-reactive protein (80.8％ [97/120] vs 64.3％ [137/213],x2 =10.020),more abnormalities of liver function (alamne aminotransferase [ALT]:13.5％ [28/207] vs 5.6％ [13/232],x2 =8.111) and myocardial enzymes (creatine kinase [CK]:37.6％ [77/205] vs 26.4％ [61/231],x2 =6.246),creatine kinase isoenzymes ([CK-MB]:35.6％ [73/205] vs 11.1％ [24/216],x2 =35.600),and all the differences were statistically significant (all P ＜ 0.05,0.01).Compared with patients in HAdV7 group,those in HAdV55 group displayed longer duration of diseases (7 [5]days vs 6[5] days,Z=-2.632),more sore throat (72.8％ [171/235] vs 62.1％ [133/214],x2 =0.016),more headache (62.1％ [146/235] vs 16.4％ [35/214],x2 =97.527),more pharyngeal congestion (93.6％ [220/235]vs 74.8％ [160/214],x2 =30.602),more leukopenia (14.0％ [33/235] vs 24.8％ [53/214],x2 =8.318),and more elevated level of lactate dehydrogenase (12.6％ [29/230] vs 6.3％ [13/205],x2 =4.881),more pneumonia (71.5％ [168/235] vs31.8％ [68/214],x2 =70.846) (all P＜0.05,0.01).Conclusions The clinical characteristics of HAdV7 and HAdV55 infections in adults are different.The type 55 infection is more likely to develop to pneumonia,while the type 7 infection has wider tissue tropism.