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.

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.

【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 investigate the change of the error positivity(Pe)of event-related poten-tial(ERP)under Flanker task in adolescents with smoking addiction. Method The signals of ERP during the Flanker task in fifteen adolescents with smoking addiction were compared with that of 15 non-smoking ad-olescents. Results The Pe amplitude in adolescents with smoking addiction((3.575±2.299)μV)was sig-nificantly lower than that of non-smokers((5.865 ± 4.290)μV)(F=5.324,P=0.029),and the reaction time of the Flanker task in smoking addiction adolescent group((440.754± 36.051)ms)was significantly higher than that in the control group((422.850±20.194)ms)(F=0.620,P<0.05).The Pe amplitude in ado-lescents with smoking addiction was positively correlated with smoking index(pack-years)(r=0.599,P=0.018). Conclusion The adolescents with smoking addiction may exit worse ability of error monitoring dur-ing the Flanker task.

Objective To establish a quality standard of yinqiao xiaozhen mixture. Methods Preparation of Forsyth-ia,Arctium lappa L.,and Honeysuckle were identified by TLC method.The concentration of baicalin in yinqiao xiaozhen mixture was determined by HPLC method. Results The qualitative identification method can detect Forsythia,Arctium lappa L.,and Honeysuckle.TLC spots were clear.TLC method has strong specificity.The linear range of baicalin was 0.122 5-1.531 2 μg,r=0.999 9,the average sample recovery rate was 99.42%,RSD was 2.19%,respectively. Conclusion The method is simple,ac-curate and repeatable,which can be used for quality control of Qinqiao xiaozhen mixture.

Objective To investigate the application value of combined detection of antikeratin (AKA) antibody,anti-cyclic citrullinated peptide(anti-CCP) antibody,anti-RA33 antibody,RF and ESR in the diagnosis of rheumatoid arthritis(RA).Methods One hundred cases of RA and 60 cases of suspected RA in our hospital from June 2014 to May 2015 were collected.One hundred and twenty cases of non-RA other autoimmune diseases served as the control group.The indirect immunofluorescence assay (IFA) was used to detect anti-KA antibody,anti-CCP antibody and anti-RA33 antibody were detected by using ELISA.The rate scatter turbid assay was used to detect RF.The Westergren method was used to detect ESR.The diagnostic performance of each diagnostic indicator was evaluated.Results The detection rates of anti-KA antibody,anti-CCP antibody,anti-RA33 antibody,RF and ESR in the RA group were in turn 64%,75%,44%,84% and 51% respectively,the detection rate(sensitivity) of 5-indicator parallel detection were 97%, the specificity of 5-indicator series detection was 89.2%;in the suspected RA group,the detection rates of 5-indicator were in turn 16.7%, 31.7%,13.3%,20%,15% respectively,which in the control group were in turn 0.8%,2.5%,1.7%,0.8% and 5.8% respectively.The detection rates of 5-indicator in the RA group was significantly higher than that in the suspected RA group,showing extremely significant difference (P<0.001),the detection rate of 5-indicator had extremely significant difference between the suspected RA group and control group (P<0.001),the sensitivity of five-indicator parallel detection was 97%,which was significantly higher than that of single indicator detection (P<0.05),the detection specificity of 5-indicator series detection was up to 100%,which was also significantly higher than that of single indicator detection (P<0.05),the missed diagnosis rate of parallel detection was minimal,while the negative predictive value was highest,the misdiagnosis rate of series detection was lowest,the positive predictive value was highest,the Youden index of parallel detection was largest.Conclusion The single detection of anti-CCP antibody and RF have good sensitivity and specificity, but 5-indicator combined detection has higher sensitivity and specificity,which can better reduce the missed diagnosis rate and misdiagnosis rate,can greatly improve the efficiency of diagnosis,and has an important clinical significance for the early diagnosis of RA.

Objective To investigate the role of phosphatidylinositol 3 kinase (PI3K)/protein kinase B (Akt) signaling pathway in Dexamethasone (DEX) inhibiting podocytes apoptosis which was induced by Puromycin (PAN).Methods Mouse glomerular podocytes were cultured in vitro,and were divided into control group,dimethyl sulfoxide (DMSO) group,PAN group,DEX group,and LY294002 (inhibitor of PI3 K) group.The mRNA expression of CD2-associated protein (CD2AP) was measured by using real time fluorescent quantitative polymerase chain reaction,and intracellular distribution was detected by using indirect immunofluorescence staining.Co localization of CD2AP and p85 was detected by using confocal fluorescence microscopy.The expressions of Akt,phosphorylated (p)-Akt,glycogen synthase kinase-3β (GSK3 β) and phosphorylated (p)-GSK3β were evaluated by using Western blot.Results The expressions of CD2AP mRNA in PAN group at each time point (8 h,24 h,48 h) (1.11 ± 0.16,0.78 ±0.09,0.56 ± 0.43) were significantly lower than those in the control group (1.90 ± 0.26,2.09 ± 0.12,2.28 ±0.95),and the differences were statistically significant (all P ＜ 0.05);CD2AP distributed in foot process with uniform filament and discontinuous coarse particle around perinuclear;CD2AP and p85 distributed in cell membrane and cytoplasm evenly in control group,but accumulated in nuclei in the PAN group.The expressions of CD2AP mRNA in DEX group at each time point (8 h,24 h,48 h) (1.53 ± 0.14,2.15 ± 0.27,2.13 ± 0.15) were significantly higher than those in the PAN group,and the differences were also statistically significant (all P ＜ 0.05);the distribution density and range of CD2AP were greater than those in the PAN group,and the accumulation with p85 in nuclei decreased obviously.The expressions of p-Akt and p-GSK3β were inhibited by PAN in a dose-dependent manner (P ＜0.05).The expressions of p-Akt and p-GSK3 β were lowest after PAN stimulated at 15 min and 30 min respectively.However,the expressions of p-Akt and p-GSK3 β increased depending on the concentration of DEX (P ＜ 0.05).In addition,the expressions of p-Akt and p-GSK3 β could be blocked by LY294002 (P ＜ 0.01).Conclusion DEX can protect podocytes and inhibit podocytes apoptosis through stabilizing the expression and distribution of CD2AP.The stale expression of PI3K/Akt signaling pathway is the key factor in DEX protecting podocytes.

Objective To observe the changes of electroencephalogram (EEG) signal during the resting state in adolescents with smoking addiction.Methods Spectral analysis during the resting state was used to observe and analyze the EEG signals between adolescents with smoking addiction (n=21) and nosmoking control group (n=21) by using Brain Vision Analyzer software.Results Compared with the nosmoking control group,adolescents with smoking addiction showed increased alpha band signals in 3 brain regions (including left-anterior,right-anterior and midline-anterior regions,P＜0.05),and decreased delta band signals in 3 brain regions (including right-posterior,midline-anterior and midline-posterior regions,P＜0.05).Moreover,the signals in these brain regions were positively correlated with pack-years (alpha band:left-anterior(r=0.648,P=0.003),right-anterior (r=0.629,P=0.004),midline-anterior (r=0.456,P=0.043);delta band:right-posterior (r=0.602,P =0.005),midline-anterior (r =0.533,P=0.019),midline-posterior (r=0.692,P=0.001))in adolescents with smoking addiction.Conclusion The changes of EEG signals during the resting state in adolescents with smoking addiction may be associated with cumulative effects of smoking.

Objective To investigate the clinical effect of large dosage of Methylprednisolone on epilespy combined with electrical status epilepticus during sleep(ESES) in children.Methods Forty-six epielpsy patients with ESES were treated with additive large dosage of Methylprednisolone.The seizures and video electroencephalogram discharges were observed before and after using Methylprednisolone.The Methylprednisolone effect in eliminating the methylprednisolone of ESES and controlling of clinical seizures and improving cognitive function were analyzed.Results Two cases were lost and 44 cases were included,male 30,female 14,the age at onset was (5.37 ±2.52) (2-9) years old.The duration of follow-up was (4.12 ± 2.00)(1-9) years old.The age at diagnosis of ESES was (8.17 ± 2.09) (4.0-12.5) years old.The efficacy of Methylprednisolone on seizures was 72.7％ (32/44 cases),while the efficacy of Methyl prednisolone on electroencephalograph (EEG) was 59.1％ (26/44 cases).For patients who were resistant to Levetiracetam or Clonazepam,Methylprednisolone was still effective.Intelligence quotient had no significant changes before and after treatment(P ＞ 0.05).The earlier onset age,the worse effect of Methylprednisolone.The efficacy of methylprednisolone for atypical benign epilepsy with cento-temporal spike(BECT) was higher than other syndromes.Conclusions Large dosage of Methyl prednisolone therapy for children with ESES,especially for those resistant to traditional or new antiepileptic drug for ESES,was effective and safe.The onset age and syndrome classification may have a certain value for prognosis and prediction of the effect of Methylprednisolone treatment.

Objective: To compare the efifcacy of direct and pre-dilated atherectomy (RA) for treating the patients with calciifed coronary lesions. Methods: A total of 137 coronary artery disease (CAD) patients received RA treatment in our hospital from 2010-04 to 2014-09 were retrospectively studied. The ischemic related lesions were all deifned as calciifed coronary lesions. The patients were divided into 2 groups: Direct RA group,n=81 and Pre-dilated RA group, the patients received balloon dilatation followed by RA,n=56. The procedural features, complications, in-hospital and 1 year occurrence rates of major cardiaccerebral vascular events (MACCE) were compared between 2 groups. Results: Compared with Pre-dilated RA group, Direct RA group had the less pre-stent balloon application,P=0.000 and the higher maximum post-dilatationpressure,P=0.004; lower rate of in-operative complication (14.8% vs 32.1%),P=0.016; higher rate of acute lumen gain (128.52±75.77) % vs (77.12±27.01) %,P=0.004; lower MACCE occurrencerate(7.3% vs 23.6%) at 1 year period,P=0.006.Cox regression analysis presented that the following indexes were related to MACCE occurrence within 1 year of RA treatment: balloon dilatation before RA (HR=8.166, 95% CI 1.872-35.614,P=0.005), left main disease (HR=13.649, 95% CI 2.983-62.440,P=0.001), minimum post-operative lumen area (HR=0.583, 95% CI 0.378-0.879,P=0.010), post-dilatation (HR=0.066, 95% CI 0.013-0.332,P=0.001) and EF>40% (HR=0.019, 95% CI 0.002-0.158, P=0.000). Conclusion: Direct RA had the lower MACCE occurrence rate at 1 year period, this might be related to less operative complication and the optimal lumen gain.