Objective To investigate the predictive value of serum Nesfatin-1 and 25-hydroxyvitamin D3[25(OH)D3]levels for the short-term prognosis of status epilepticus(SE)in children.Methods A total of 104 children with SE admitted to the hospital from March 2020 to March 2023 were enrolled in the study,and the clinical data of the children were collected.According to the Glasgow outcome Scale(GOS)score at dis-charge,the children were divided into a good prognosis group(equal to 5 points)and a poor prognosis group(＜5 points).Univariate analysis and multivariate Logistic regression were used to analyze whether serum Nesfatin-1 and 25(OH)D3 levels were risk factors for poor short-term prognosis in children with SE.The re-ceiver operating characteristic(ROC)curve was drawn to analyze the predictive value of serum Nesfatin-1 and 25(OH)D3 levels for the short-term poor prognosis in children with SE.Results At discharge,85 children[81.73％(85/104)]with a GOS score of 5 were included in the good prognosis group,and 19 children[8.27％(19/104)]with a GOS score of＜5 were included in the poor prognosis group.There was no significant differ-ence in gender,age,previous history of epilepsy,and seizure types between the two groups(P＞0.05).There were significant differences in the duration of SE,the time from medication to seizure cessation,electroenceph-alogram(EEG)results,head CT results,and serum Nesfatin-1 and 25(OH)D3 levels between the two groups(P＜0.05).Multivariate Logistic regression analysis showed that SE duration＞60 min,abnormal head CT results,serum Nesfatin-1 and 25(OH)D3 levels were independent risk factors for the short-term poor progno-sis of children with SE(OR=1.945,2.343,1.731,1.505;P＜0.05).The area under the ROC curve of serum Nesfatin-1 and 25(OH)D3 levels combined to predict poor short-term prognosis of children with SE was 0.840(95％CI:0.732-0.949),which was better than that of serum Nesfatin-1 and 25(OH)D3 levels alone[0.607(95％CI:0.453-0.761),0.742(95％CI:0.604-0.880)],respectively.Conclusion Serum Nesfatin-1 and 25(OH)D3 levels are risk factors for poor prognosis in children with SE,and the combination of them has high predictive value for poor prognosis in children with SE.

ObjectiveTo clone coumarate-3-hydroxylase gene （C3H） from Angelica sinensis， and analyze the correlation between its bioinformatics， expression patterns and content of ferulic acid， and to explore the functions of ASC3H. MethodReal-time polymerase chain reaction （Real-time PCR） was used to clone the full-length cDNA of ASC3H based on the transcriptome dataset of A. sinensis， and the bioinformatics analysis of the gene sequence was carried out. Real-time PCR and high performance liquid chromatography （HPLC） were used to determine relative expression of ASC3H and content of ferulic acid in different root tissues of A. sinensis （periderm， cortex and stele）. ResultThe open reading frame （ORF） of ASC3H （GenBank accession number： MN2550298） was 1 530 bp， encoding 509 amino acids， with a theoretical molecular weight of 57.86 kDa and an isoelectric point of 8.36. It was a hydrophilic protein that was located in the chloroplast with multiple phosphorylation sites and a transmembrane region， and contained a conserved domain CGYDWPKGYGPIINVW_P450 （383-399 aa） in cytochrome P450. Multiple amino acid sequence alignment analysis showed that ASC3H had high similarity with C3H from other plants， especially Ammi majus in Umbelliferae. The Real-time PCR revealed that ASC3H had different expressions in periderm， cortex and stele tissues of A. sinensis roots. It was found from HPLC that the cortex tissues had the highest content of ferulic acid， and the stele tissues had the lowest. ConclusionASC3H was successfully cloned from A. sinensis， and its sequence characteristics were understood more clearly， suggesting that ASC3H might be involved in the ferulic acid biosynthesis pathway of A. sinensis. This paper provided a basis for further studying the functions of the gene and exploring the biosynthesis and regulation mechanism of ferulic acid in A. sinensis， while laying the foundation for the genetic improvement of A. sinensis．

OBJECTIVE To investigate the role of the complement C3/C3aR signaling pathway in the prefrontal cortex and colon neuroglia cell interactions during meth-amphetamine(METH)addiction,to observe the effects of TLR4 inhibitors as well as complement C3 elimination on METH reward and relapse behavior,and to explore the neuroinflammatory mechanisms of complement C3 acti-vation in METH addiction.METHODS ①A 14 d and 28 d rat METH addiction model was established to observe the effects of TLR4 antagonist ibudilast 3 mg·kg-1 and 10 mg·kg-1 on self-administration,reward motivation,relapse,and natural reward behavior in METH-trained 14 d rats and the effects of 0.02 mg·kg-1 complement C3 antago-nist on self-administration behavior in METH-trained 28 d rats.② Differences in the expression of TLR4,NF-κB,GRP94,C3,cathepsin L,CD68,and GFAP in the pre-frontal cortex of each group were examined using West-ern blotting.③ In addition,the expression of ATF6 in the prefrontal cortex of each group and the effects on neuro-nal and microglia/macrophage INOS,CD206 GRP94,and complement C3/C3aR.RESULTS ① Endoplasmic reticulum stress occurred in neurons and microglia after METH exposure depending on GRP94 and unfolded pro-tein responses to the ATF6 pathway.In addition,it acti-vates the TLR4-NF-κB pathway.② Microglia with high complement C3/C3aR expression in the prefrontal cortex were recruited to synaptic pruning and phagocytic responses around neurons with high GRP94,comple-ment C3/C3aR expression and these effects were blocked by complement C3 antagonists.③ In the rec-tum,GRP94 functions as a molecular chaperone for com-plement C3 and cathepsin L.Crosstalk occurs between enteric neurons high in GRP94,complement C3,and macrophages high in C3aR,located in the submucosa,lamina propria,and muscular,respectively,and all of these effects are blocked by complement C3 antago-nists.④ Treatment with the TLR4 antagonist ibudilast inhibits self-administration,reward motivation,and cue-or METH-priming in METH-trained 14 d rats,but fails to affect natural reward behavior.Ibudilast treatment attenu-ates the TLR4-NF-κB inflammatory pathway and comple-ments C3/C3aR pathway in the prefrontal cortex.CON-CLUSION Activation of the complement C3/C3aR signal-ing pathway by TLR4-NF-κB inflammatory signaling in the prefrontal cortex mediates the METH addiction pro-cess,providing an experimental basis for the clinical treatment of METH addiction,and targeting TLR4/NF-κB inflammatory signaling and complement C3/C3aR may be a new way to intervene in METH addiction.

OBJECTIVE To analyze the clinical manifestations and characteristics of adverse drug reactions （ADR） induced by dronedarone，and to provide reference for clinically safe drug use. METHODS Retrieved from PubMed database， Wanfang database，CNKI and VIP （up to August 31st， 2022），ADR cases of dronedarone were analyzed retrospectively in respect of patient’s age，gender，nationality，usage and dosage of dronedarone，and occurrence time，clinical manifestations，treatment measures and outcome of ADR，etc. RESULTS A total of 26 case reports were included，with a total of 27 patients. The age of the patients was 41-86 years old，with an average age of 68.8 years. The proportion of patients aged 60-79 was the largest （20 cases，74.1%）. There was no significant difference in the number of males （14 cases） and females （13 cases）. The patients came from 6 countries， of which the United States was the largest （16 cases，59.3%）. The dosage of 14 patients was 400 mg bid；one patient was 200 mg bid；the dosage for 12 patients was not specified. The most ADR cases （16 cases，59.3%） occurred within 1 month，of which 11 cases（40.7%） occurred within 1 week，and there were no ADR reports with medication more than 12 months. Organs/systems involved in ADRs were mainly liver and biliary diseases （7 case times，23.3%），skin and subcutaneous tissue diseases （6 case times， 20.0%），respiratory tract，thoracic and mediastinal diseases （5 case times，16.7%）. In addition，ADR also occurred in heart diseases， kidney and urinary system diseases，vascular diseases，medical examinations and eye diseases. Among 27 patients，there were 3 cases of death，the ADR were bronchiolitis obliterans with organizing pneumonia，toxic epidermal necrolysis and hepatic failure， respectively. One patient underwent liver transplantation. CONCLUSIONS Dronedarone can cause multiple organ system ADR. Before use，it is necessary to improve the examination including ECG，liver function，lung function，etc. and strengthen drug use monitoring within one month after the start of use，especially the ADR of hepatobiliary，skin and respiratory system. The occurrence of severe ADR has no obvious relationship with the duration of medication； even if it is taken safely for a long time，it still needs continuous pharmaceutical monitoring and follow-up to ensure the clinical medication safety of patients.

Objective:To elucidate the clinical and genetic characteristics of PLA2G6-related parkinsonism. Methods:The clinical, imaging and genetic data of 6 patients with PLA2G6-related parkinsonism admitted to Peking Union Medical College Hospital from January 2015 to December 2022 were retrospectively collected and analyzed. The prognosis was followed up through phone call. Results:There were 3 male and 3 female patients, and the age of disease onset was (24.3±5.4) years. Phenotypically, 5 of them had dystonia-parkinsonism (DP) with obvious atrophy of cerebellum and 1 presented as early-onset Parkinson′s disease (EOPD) with no brain structural abnormality. Only 1 patient presented with abnormal brain iron deposition. All of the patients were partially responsive to levodopa. Three cases underwent levodopa challenge test with the objective levodopa responsiveness varied from 10.3% and 10.6% in 2 DP patients, to 77.0% in 1 EOPD patient. Levodopa-induced dyskinesias were present in 4 of them, and all appeared within the first year since the initiation of dopaminergic treatment. Two patients underwent bilateral deep brain stimulation (DBS) of subthalamic nucleus and globus pallidus internus respectively, albeit revealed poor outcome. Genetically, 8 PLA2G6 variants were identified. Two of them were found to be novel (c.1973A>G and exon2 heterozygous deletion), and the most frequent variant was the c.991G>T mutation which was detected in 4 patients. Conclusions:The phenotype of PLA2G6-related parkinsonism is complex. Cerebellar atrophy is a frequent magnetic resonance imaging feature. Levodopa responsiveness tends to depend on the clinical phenotype, and EOPD is better than DP. DBS might not be promising in DP patients with obvious cerebral atrophy. The c.991G>T mutation is the most frequent mutation, suggesting a common founder effect.

Animals ; Coronary Vessels ; Diabetes Mellitus ; Muscle, Smooth, Vascular ; Myocytes, Smooth Muscle ; Rats

Objective To analyze the epidemiological characteristics and seasonal distribution of hand, foot and mouth disease (HFMD) in a suburban town in Shanghai from 2012 to 2018, and to provide a scientific basis for formulating effective prevention and control strategies. Methods The incidence data of a suburban town in Shanghai from 2012 to 2018 was collected through the Chinese disease prevention and control information system and analyzed by descriptive epidemiological methods. Results From 2012 to 2018, a total of 538 cases of hand, foot and mouth disease were reported in the suburban town in Shanghai, with an average annual incidence of 186.27/100,000. The difference in incidence between each year was statistically significant (χ²=107.95, P<0.05). The age of onset was mainly children under 5 years old. Peak incidence was from May to September. The main occupation was scattered children. HFMD outbreaks mainly occurred in nurseries and schools, accounting for 60.53% of the total number of outbreaks. Conclusion From 2012 to 2018, the incidence of HFMD in a suburban town in Shanghai had a certain seasonality. It is recommended to implement preventive measures against HFMD for key populations and key places before the high incidence period to reduce the occurrence of severe cases and epidemic situations.

PTPN11 is the most common mutation gene of RAS disease, which is located in the upstream of RAS/MAPK pathway and participates in signal transduction. Because the molecular mechanism of RAS's disease involves the same pathway, it may present a certain commonality in clinic, but the different genotypes with PTPN11 mutation may also express different phenotypes. Therefore, it is not easy to identify and diagnose this disease early in clinic. The present article aims to analyze the correlation between the clinical phenotype and genotype of 4 patients with RAS disease.

Objective:To compare the surgery indicators of early lung cancer patients between video-assisted thoracoscopic surgery and thoracotomy in order to provide suggestion for surgery scheme.Methods: 126 early lung cancer patients, from March 2014 to January 2016 stayed in our hospital, were chosen as observation object. They were divided in control group (63 cases, thoracotomy) and observation group (63 cases, video-assisted thoracoscopic surgery), and to compare series of surgery indicators, IL-6 and TNF-α between the two groups.Results: The operating time and blood loss of observation group were lower, and the dissected lymph node number of observation group was higher than control group, respectively, and both of the differences were statistical significant (t=6.568,t=3.096, P<0.05;t=3.659,P<0.05); the hospital stay, catheter-retaining time and pain score of observation group were lower than control group, and the differences also were statistical significant (t=15.850,t=12.379,t=10.272;P<0.05); the differences of IL-6 and TNF-α between two groups were no statistical significant before treatment, and both of them reduced after treatment; both of the IL-6 and TNF-αof observation group were lower than control group (t=13.073, t=1.629;P<0.05); on the other hand, the total rate of complications of observation group, such as pneumonia, pulmonary atelectasis and arrhythmia, were higher than control group (x2=0.208, x2=2.800,x2=0.341;P<0.05). Conclusion: Early lung cancer patients are more suitable for video assisted thoracoscopic surgery, and its effect is better and is more conducive to the rehabilitation of patients after surgery.

Objective To study the early diagnostic predictors and key follow-up parameters for girls with rapidly progressive central precocious puberty (RP-CPP). Methods A total of 260 girls with CPP participated in a prospective, nonrandomized, multi-center, nested case control study. After follow-up six months without any therapy, 114 girls were divided into RP-CPP (n=70) and slowly progressive CPP (SP-CPP) (n=44) groups. Results The basal serum LH and insulin-like growth factor Ⅰstandard deviation score (IGF-ⅠSDS) were the important risk factors of RP-CPP (OR 4.04, 1.578), especially the former. The receiver operating characteristic (ROC) curve revealed that the areas under the ROC curve of basal LH and IGF-ⅠSDS were 0.83 and 0.807, respectively. The levels of basal LH and IGF-ⅠSDS were at 0.52 mIU/ml and 0.35 respectively for the accuracy diagnosis of RP-CPP with the maximum Youden indexs. After follow-up for six months, the change levels of height, breast stages, bone age/chronological age ratio, serum LH, uterine and ovarian volume in RP-CPP group were significantly higher than those in SP-CPP group (all P<0.05). Conclusions The level of basal serum LH and IGF-ⅠSDS may be used as the risk predictors for early diagnosis for girls with RP-CPP. The change levels of basal LH, progress rates of gonad and sex character, height, and impaired growth potential seem to be the key follow-up parameters for CPP progress.