Hepatic alveolar echinococcosis (HAE) is a common zoonotic endemic parasitic disease in western China. It lacks of typical clinical manifestations in the early stage, and symptoms become prominent during the end stage, with an alarmingly high mortality rate. Among the treatment of end-stage HAE (es-HAE), orthotopic liver transplantation is almost the only radical treatment due to insufficient remnant liver volume, uncontrollable bleeding and difficulty in vascular reconstruction in vivo. However, the shortage of donor liver and long-term postoperative use of immunosuppressants limit its application. The introduction of ex vivo liver resection and autotransplantation (ELRA) resolves this dilemma and significantly broadens the indications of es-HAE. In addition, multiple centers in China have optimized and modified ELRA to further improve the treatment system of es-HAE. At present, liver transplantation (including ELRA) of es-HAE remains a hot topic for clinicians. In this article, orthotopic liver transplantation, ELRA, auxiliary ELRA and other surgical treatment of es-HAE were reviewed, aiming to further enhance the diagnosis and treatment of es-HAE and improve clinical prognosis of the patients.

Objective To investigate the molecular mechanism of Brusatol(BRU)regulating apoptosis of Hela cells through TLR9-MyD88 signaling pathway.Methods Hela cells preserved in our laboratory were treated with Brucea javanica at different concentrations(0,5.0,10.0,20.0,40.0,80.0 nmol·L-1).Hela cells were divided into Control group(normal cultured Hela cells),BRU-L group(treated with 10.0 nmol·L-1 Brucea javanica)and BRU group-H(treated with 20.0 nmol·L-1 Brucea javanica)Cells were treated with nmol·L-1 of Brucea javanica),BRU+pcDNA-NC group(transfected with pcDNA-NC+20.0 nmol·L-1 of Brucea javanica),BRU-H+pcDNA-TLR9 group(transfected with pcDNA-TLR9+20.0 nmol·L-1 of Brucea javanica).Cell proliferation was detected by CCK-8 and EdU methods.Apoptosis was detected by TUNNEL staining and flow cytometry.The protein expression levels of TLR9,MyD88,Bax,Bcl-2,Cleaved Caspase-3 and Cleaved Caspase-9 were detected by Western blot.Cell apoptosis and mitochondrial membrane potential detection kit(JC-1)were detected by flow cytometry,and the contents of adenosine triphosphate(ATP)and reactive oxygen species(ROS)were detected by ELISA.Results Compared with 0 nmol·L-1 group,the survival rate and IC50 value of 10 nmol·L-1 group were significantly decreased(P<0.05).After stimulation of BRU with different concentrations,the proliferation ability of cells was significantly decreased in a dose-dependent manner(P<0.05).Compared with control group,the 5-ethynyl-2'-deoxyuracil(EDU)positive cell rate,TUNEL positive cell rate,apoptosis rate and Bcl-2 protein of cells in BRU-L,BRU-H and pcDNA-NC groups were significantly decreased.The protein levels of TLR9 and MyD88,Bax,Bax/Bcl-2,Cleaved Caspase-3 and Cleaved Caspase-9 were significantly increased(P<0.05).In control group,BRU-L,BRU-H group/BRU-H+pcDNA-NC,there was a continuous decreasing trend(P<0.05).Compared with the BRU-H+pcDNA-NC group,the EDU positive cell rate,TUNEL positive cell rate,apoptosis rate and Bcl-2 protein in the BRU-H+pcDNA-TLR9 group were significantly increased.The protein levels of TLR9 and MyD88,Bax,Bax/Bcl-2,Cleaved Caspase-3 and Cleaved Caspase-9 were significantly decreased(P<0.05).Compared with Control group,JC-1 level and ATP content in BRU-L,BRU-H and BRU-H+pcDNA-NC groups were significantly decreased,while ROC content and mitotracker positive cell level were significantly increased(P<0.05).Compared with BRU-L group,JC-1 level and ATP content in BRU-H group and BRU-H+pcDNA-NC group were further decreased,while ROC content and mitotracker positive cell level were further increased(P<0.05).Compared with the BRU-H+pcDNA-NC group,the levels of JC-1 and ATP in the BRU-H+pcDNA-TLR9 group were increased,while the levels of ROC,mitotracker staining positive cells were decreased(P<0.05).Conclusion Brucea javanica can produce Hela cell proliferation by regulating TLR9-MyD88 signaling pathway.

Anti-seizure medications (ASMs) are the main therapy for epilepsy.There are many kinds of ASMs with complex mechanism of action, so it is difficult for pharmacists to examine prescriptions.This paper put forward some suggestions on the indications, dosage forms/routes of administration, appropriateness of usage and dosage, combined medication and drug interaction, long-term prescription review, individual differences in pathophysiology of children, and drug selection when complicated with common epilepsy, for the reference of doctors and pharmacists.

The number of investigator initiated research (IIR) is increasing. But the recognition and management of IIR in China is still in its infancy, and there is a lack of specific and operable guidance for the implementation process. Based on our practical experiences, previous literature reports, and current policy regulations, the authors took prospective IIR as an example to summarize the implementation process of IIR into 14 steps, which are as the following: study initiation, ethical review, study registration, study filing, case report form design, database establishment, standard operating procedure making, investigator training, informed consent, data collection, data entry, data verification, data locking and data archiving.

15q11-q13 Duplication syndrome is a rare genetic disease of the nervous system, characterized by developmental retardation, intellectual impairments, hypotonia, autism, epilepsy and so on. This article reports a 33-year-old male patient, with the clinical manifestation of early-onset intractable epilepsy and mental retardation. The high-throughout whole exome sequencing showed a 10.53 Mb repeat sequence in the 15q11.2-q13.3 region, further confirming the diagnosis of 15q11-q13 duplication syndrome. The literature reports of the pathogenic mechanism, classification, typical clinical manifestation, seizure features,accessory examination and therapy of the 15q11-q13 duplication syndrome are summarized and reviewed, so as to enhance the understanding of the disease, as well as to improve the diagnosis and treatment level of the clinicians.

Objective:To analyze the risk factors of bronchopulmonary dysplasia(BPD)in very preterm infants(VPI), and to provide scientific basis for the prevention and treatment of BPD in VPI.Methods:A prospective multicenter study was designed to collect the clinical data of VPI in department of neonatology of 28 hospitals in 7 regions from September 2019 to December 2020.According to the continuous oxygen dependence at 28 days after birth, VPI were divided into non BPD group and BPD group, and the risk factors of BPD in VPI were analyzed.Results:A total of 2 514 cases of VPI including 1 364 cases without BPD and 1 150 cases with BPD were enrolled.The incidence of BPD was 45.7%.The smaller the gestational age and weight, the higher the incidence of BPD( P<0.001). Compared with non BPD group, the average birth age, weight and cesarean section rate in BPD group were lower, and the incidence of male infants, small for gestational age and 5-minute apgar score≤7 were higher( P<0.01). In BPD group, the incidences of neonatal respiratory distress syndrome(NRDS), hemodynamically significant patent ductus arteriosus, retinopathy of prematurity, feeding intolerance, extrauterine growth restriction, grade Ⅲ~Ⅳ intracranial hemorrhage, anemia, early-onset and late-onset sepsis, nosocomial infection, parenteral nutrition-associated cholestasis were higher( P<0.05), the use of pulmonary surfactant(PS), postnatal hormone exposure, anemia and blood transfusion were also higher, and the time of invasive and non-invasive mechanical ventilation, oxygen use and total hospital stay were longer( P<0.001). The time of starting enteral nutrition, cumulative fasting days, days of reaching total enteral nutrition, days of continuous parenteral nutrition, days of reaching 110 kcal/(kg·d) total calorie, days of reaching 110 kcal/(kg·d) oral calorie were longer and the breastfeeding rate was lower in BPD group than those in non BPD group( P<0.001). The cumulative doses of amino acid and fat emulsion during the first week of hospitalization were higher in BPD group( P<0.001). Multivariate Logistic regression analysis showed that NRDS, invasive mechanical ventilation, age of reaching total enteral nutrition, anemia and blood transfusion were the independent risk factors for BPD in VPI, and older gestational age was the protective factor for BPD. Conclusion:Strengthening perinatal management, avoiding premature delivery and severe NRDS, shortening the time of invasive mechanical ventilation, paying attention to enteral nutrition management, reaching whole intestinal feeding as soon as possible, and strictly mastering the indications of blood transfusion are very important to reduce the incidence of BPD in VPI.

Objective:To analyze the clinical characteristics of late-onset epileptic spasm (LOS), thus providing basis for its early identification and treatment.Methods:Clinical data[electroencephalogram(EEG), imaging, treatment and prognosis]of LOS patient hospitalized in the Department of Pediatrics, the Second Affiliated Hospital of Xi′an Jiaotong University from January 2017 to June 2019 were retrospectively analyzed.Results:The age of onset of spasm in 35 children with LOS ranged from 18 months to 11 years old, with a median of 42 months.There were 21 cases of symptomatic LOS (60.0%) and 14 cases of cryptogenic LOS (40.0%). Epileptic spastic seizures, generalized seizures, partial seizures and myoclonic seizures as the first onset were reported in 13 cases (37.1%), 11 cases (31.4%), 10 cases (28.6%), and 1 case (2.9%), respectively.There were 15 cases (43.9%) of flexion type, 11 cases (30.4%) of extension type and 9 cases (25.7%) of mixed type.Spastic seizures can be presented as genera-lized or focal seizures.Among the 35 cases of LOS, 12 cases (34.3%) had normal EEG background, 18 cases (51.4%) had slow EEG background, and 5 cases (14.3%) had high EEG irregularity.Three cases were in accor-dance with Lennox-Gastaut syndrome and the other 32 cases were not in accordance with the defined epileptic syndrome.Eighteen cases were treated with antiepileptic drugs, and sodium channel blockers were added in 9 cases; 17 cases were treated with glucocorticoid.Eight cases did not have seizures at the last follow-up.There were 17 children with seizure reduction ≥ 50%, 3 cases with seizure reduction < 50%, and 7 cases with no reduction of seizure.Conclusions:LOS is mostly symptomatic and often associated with other types of seizures.Most of cases do not have high irregularity in the EEG, and the focal discharges are mainly in the temporal region or frontotemporal region, with refractory epilepsy mainly.The onset age, etiology and EEG characteristics of LOS differ from those of West syndrome, which should be detected and treated as soon as possible to improve the prognosis in pediatric patients.

【Objective】 To analyze the clinical features and gene mutation characteristics of four children with pyridoxine-dependent epilepsy (PDE) in order to provide evidence for early diagnosis of this rare disease. 【Methods】 The clinical data of four cases of PDE were collected from January 2016 to June 2019 in The Second Affiliated Hospital of Xi’an Jiaotong University. We collected data of the laboratory examination, electroencephalogram, and magnetic resonance imaging (MRI). Peripheral venous blood was collected from children and parents, genomic DNA was extracted from white blood cells, and primers were designed to amplify the aldehyde dehydrogenase 7 family member A1(ALDH7A1) on the long arm of chromosome 5 using PCR; exon and the junction of exon and intron were also amplified to determine whether there is a gene mutation. 【Results】 All the 4 cases had a full-term birth and no history of birth asphyxia. ① Clinical features: onset time from 8 days after birth to 6 months after birth. Type of seizure: 1 case with myoclonus onset, then converted to spasm; 1 case for generalized seizures; 2 cases for partial seizures, then converted to generalized seizures; 3 cases were prone to epileptic seizures; 1 case was significantly worse after infection; MRI: 3 cases showed no abnormalities, 1 case showed non-specific abnormalities; EEG: One case was fragmentary hypsarrhythmia, 3 cases of multifocal epileptiform discharge; Treatment: a small dose of vitamin B6 could control the seizure, 1 of them was controlled at a tiny dose, and 3 cases were controlled by a small dose. ② Genetic analysis results: There were 4 cases of ALDH7A1 gene mutation, of which 3 cases were known gene mutations and 1 case was new mutation. 【Conclusion】 PDE has an early onset, often in the neonatal or small infancy, is prone to epilepticus and has an increased severity after infection. There is no specificity in seizure type, EEG or MRI. The analysis of ALDH7A1 gene and vitamin B6 load test can help to confirm the diagnosis, small dose of Vitamin B6 can control the seizures so as to provide reference for the dose of vitamin B6. However, the number of cases is small, and a large sample size is still needed for verification.

Objective:To explore the association between socioeconomic status (SES) and overweight/obesity in Yi people in Sichuan province.Methods:A cross-sectional study was conducted in Liangshan Yi Autonomous Prefecture in 2015. Stratified cluster sampling method was used to enroll Yi farmers and rural-to-urban Yi migrants aged 20-80 years. SES was measured by education level, personal annual income, and compound SES index. Unconditional logistic regression models were used to determine the association between SES and overweight/obesity (BMI≥24.0 kg/m 2). Results:1 894 Yi farmers and 1 162 rural-to urban migrants were included in the analysis. After adjustment for age, smoking, drinking and physical activity, compared with illiteracy, OR for farmer males with higher education level (primary or junior school, senior high school or higher) were 1.71 (95 %CI: 1.13-2.58) and 4.15 (95 %CI: 2.10-8.22). Compared with lower income group (<5 000 CNY/y), the higher income group had increased risk ( OR=1.66, 95 %CI: 1.12-2.44). For farmer males with medium and high SES level, the risk of overweight/obesity were 1.65 (95 %CI: 1.02-2.67) and 3.26 (95 %CI: 1.97-5.42) compared with low level of SES. For farmer females, the risk increased with the higher income, with OR as 1.49 (95 %CI: 1.10-2.02). Compared with low SES level, farmer females with medium level of SES was associated with 1.47 (95 %CI: 1.11-1.95) times higher risk of overweight/obesity. In Yi migrants, the association between SES and overweight/obesity was not found. Conclusion:Socioeconomic status was positively associated with overweight/obesity only in Yi farmers.

Objective To study the effect of sex on reperfusion time delay in ST-segment elevation myocardial infarction (STEMI) patients after diagnosis.Methods A total of 1647 STEMI patients admitted to our hospital were included in this study.Their clinical data and treatment time points were recorded.The reperfusion time delay after diagnosis refers the time from ECG-based diagnosis to PCI.The reperfusion time delay after diagnosis was divided into 0-3 h,＞3-6 h,＞6-12 h,＞12-24 h and ＞24 h.The effect of sex and other risk factors on reperfusion time delay after diagnosis was assessed according to the established logistic regression model.Results The age of female STEMI patients was older than that of male STEMI patients (65±10 years vs 60±11 years,P＜0.05).The rate of past CABG and PCI was significantly higher and the reperfusion time delay was significantly longer in female STEMI patients than in male STEMI patients (4.3 ％ vs 1.5％,20.7％ vs 17.4％,P＜0.05;404±34 min vs 280±14 min,P＜0.01).Multivariate logistic regression analysis showed that female and visiting form were related with the reperfusion time delay for ＞3-6 h and ＞12-24 h (95％CI:1.052-264.306,P=0.046;95％CI:1.089-2.751,P=0.013).Conclusion Female is related with reperfusion time delay,visiting form and call for emergency treatment in STEMI patients.