Objective     To summarize the surgical treatment experience in neonates with coarctation of the aorta (CoA) and aortic arch hypoplasia (AAH). Methods     The neonates with CoA and AAH who underwent surgical treatment in the Department of Pediatric Cardiac Surgery of Guangdong Provincial People's Hospital from 2013 to 2020 were retrospectively enrolled. The postoperative complications, long-term survival rate, and freedom from aortic reobstruction were analyzed. Patients undergoing extended end-to-end anastomosis were allocated into an extended end-to-end group, those undergoing extended end-to-side anastomosis into an extended end-to-side group, and those undergoing pulmonary autograft patch aortoplasty into a patch aortoplasty group. Results     Finally 44 patients were  enrolled, including 37 males and 7 females, aged 5.00-30.00 (19.34±7.61) days and weighted 2.00-4.50 (3.30±0.60) kg. There were 19 patients of extended end-to-end anastomosis, 19 patients of extended end-to-side anastomosis, and 6 patients of pulmonary autograft patch aortoplasty. The mean values of the Z scores of the proximal, distal, and isthmus of the aortic arch were –2.91±1.52, –3.40±1.30, and –4.04±1.98, respectively. The mean follow-up time was 45.6±3.7 months. There were 2 early deaths and no late deaths. Aortic reobstruction occurred in 8 patients, and 3 patients underwent reoperation intervention. The 5-year rate of freedom from reobstruction was 78.8%. The Cox multivariable regression analysis showed that the related factors for postoperative reobstruction were the Z score of the preoperative proximal aortic arch (HR=0.152, 95%CI 0.038-0.601, P=0.007) and the postoperative left main bronchus compression (HR=15.261, 95%CI 1.104-210.978, P=0.042). Conclusion     Three surgical procedures for neonates with CoA and AAH are safe and effective, but the aortic reobstruction rate in long term is not low. The smaller Z score of the preoperative proximal aortic arch and the postoperative left main bronchus compression are risk factors for long-term aortic reobstruction.

Objective    To characterize surgical outcomes of supra-cardiac total anomalous pulmonary venous connection (TAPVC), investigate risk factors for postoperative death, and explore informed LASSO machine learning methods to solve "small sample size problem" in research of rare congenital heart diseases. Methods    A retrospective analysis of 241 patients with supra-cardiac TAPVC who underwent surgical repair in Guangdong Provincial People's Hospital from 2009 to 2019 was conducted, including 179 males and 62 females with a median surgical age of 71 (33, 232) d. Detailed clinical data of the postoperative death-related factors were extracted. Univariable Cox proportional hazard models were used to initially screen potential risk factors for postoperative death. Factors with P鈮?.05 were retained. To solve the limitation of small sample size and the "P>n" problem, we proposed a novel LASSO method for conducting multivariable Cox regression analysis that was capable of bringing in findings of related studies to improve analysis power and to reduce false-negative findings. Results 聽 聽Univariable Cox analyses showed several potential clinical risk factors, among which highly significant factors (P<0.001) included: surgical weight鈮?.5 kg (HR=16.00), main pulmonary artery diameter (HR=0.78), prolonged cardiopulmonary bypass time (HR=1.21), aortic block time (HR=1.28), and postoperative ventilator-assisted time (HR=1.13/d). LASSO multivariable analysis revealed that independent risk factors for postoperative death included cardiopulmonary bypass time (aHR=1.308/30 min), age (aHR=0.898), postoperative ventilator-assisted time (aHR=1.023/d), weight鈮?.5 kg (aHR=2.545), right vertical venous return (aHR=1.977), preoperative pulmonary venous obstruction (aHR=1.633) and emergency surgery (aHR=1.383). Conclusion 聽 聽Our proposed informed LASSO method can use previous studies' results to improve the power of analysis and effectively solve the "P>n" and small sample size limitation. Cardiopulmonary bypass time, surgical age, postoperative ventilator-assisted time, weight, right vertical venous return, preoperative pulmonary venous obstruction, and emergency surgery are risk factors for postoperative death of supra-cardiac TAPVC.

Sutureless technique is effective for the treatment of pulmonary vein obstruction after total anomalous pulmonary venous connection (TAPVC) surgery and has been widely used for primary correction of TAPVC. Sutureless repair had more convincing surgical outcomes, including lower mortality and reoperation rate, compared with conventional repair. However, sutureless technique also has some complications, including potential bleeding at the anastomotic site, phrenic nerve injury, air embolism. The safety and effectiveness are still controversial. This article reviewed the research status of sutureless technology in surgical correction of TAPVC in recent years.

Objective:This study aimed at analyzing risk factors associated with surgical outcomes of neonatal total anomalous pulmonary venous connection (TAPVC) in our center.Methods:A total of 105 neonates who underwent surgical repair for TAPVC from January 1st, 2009 to January 1st, 2018 were retrospectively analyzed. The anatomical types of TAPVC included supracardiac 42(40%, 42/105), cardiac 21(20%, 21/105), infracardiac 36(34.3%, 36/105), and mixed 6(5.7%, 6/105). The Cox proportional hazards analysis was used to analyze the risk factors related to postoperative pulmonary venous obstruction (PVO) and mortality. Kaplan- Meier analysis was used to analyze the overall survival rates. Results:Twenty-six patients (24.8%, 26/105) were diagnosed with preoperative PVO. The 30-day, 1 year, and 5 years survival rate was 92.4%, 86.7%, and 86.7% respectively. Postoperative PVO occurred in 17 patients (16.2%, 17/105). Preoperative acidosis, low surgical weight, prolonged duration of cardiopulmonary bypass time, increasing postoperative central venous pressure (CVP), and reoperation were risk factors associated with mortality. Preoperative acidosis ( P<0.001), prolonged duration of cardiopulmonary bypass time ( P<0.001), and increasing postoperative CVP ( P=0.005) were independent risk factors for mortality. Mixed TAPVC, preoperative acidosis, low surgical age, prolonged cardiopulmonary bypass time, postoperative pulmonary arterial hypertension were risk factors associated with postoperative PVO. Prolonged cardiopulmonary bypass time ( P=0.029), postoperative pulmonary arterial hypertension ( P<0.001), and mixed TAPVC ( P=0.017) were independent risk factors associated with postoperative PVO. Conclusion:The surgical outcomes of neonatal TAPVC in our center were acceptable, with low mortality rate and incidence of PVO. However, neonates with preoperative acidosis, prolonged duration of cardiopulmonary bypass time, and increased postoperative CVP had a poor prognosis. Patients with mixed TAPVC were at increased risk for postoperative PVO.

Objective    To quest the risk factors of poor prognoses in children with tetralogy of Fallot (TOF) during perioperative period and evaluate its clinical application values. Methods    A retrospective analysis of the clinical data of 119 children who underwent one-stage correction of TOF in Guangdong Provincial People's Hospital from September 2016 to January 2019. The cohort includes 75 males and 44 females, with ages ranging from 3.2-137.1 (13.2±1.4) months and weights ranging from 4.6-21.0 (8.3±0.2) kg. Perioperative poor prognosis was defined as duration of mechanically assisted ventilation >48 h or secondary intubation, vasoactive-inotropic score (VIS) within 48 h >40, postoperative length of stay >14 d, and the occurrence of the major adverse events. Major adverse events were defined as early death, malignant arrhythmia, low cardiac output syndrome, non-fatal cardiac arrest, postoperative reintervention, diaphragm paralysis, and other clinical complications. Univariate and multivariate logistic analyses were used to analyze the correlation between risk factors and poor prognoses. Results    There was 1 perioperative death, and 9 with major adverse events. Variables selected by Least Absolute Shrinkage and Selection Operator (LASSO) included 2 preoperative variables (McGoon index, aortic root diameter index) and 4 intra-operative variables [left-right direction of bicuspid pulmonary valve, total length of right ventricular outflow tract (RVOT) incision index, pulmonary valve with commissurotomy, and minimum temperature in cardiopulmonary bypass (CPB)]. Univariate and multivariate logistic analyses were used to the above factors, respectively. The variables with statistical significance (P≤0.05) were McGoon index, aortic root diameter index, left-right direction of bicuspid pulmonary valve, and minimum temperature in CPB. A nomogram was established based on the above factors, and the results showed that the left-right direction of bicuspid pulmonary valve was more risky than the tricuspid pulmonary valve and the anterior-posterior direction of bicuspid pulmonary valve. The lower the McGoon index, the higher aortic root diameter, and the lower temperature in CPB, the higher risk of poor prognostic events in children with TOF. Conclusion    The left-right direction of the pulmonary bicuspid valve has a higher risk of poor prognosis than the tricuspid pulmonary valve and the anterior-posterior direction of bicuspid pulmonary valve. With the smaller McGoon index and the larger aortic root diameter, the risk of poor prognoses in children with TOF is higher. The temperature in CPB being lower than medium-low temperature obviously relates to the high incidence of poor prognostic events, which can be used as an auxiliary reference standard for decision-making in pediatric TOF surgery in the future.

The membrane protein claudin-3 (CLDN3) is critical for the formation and maintenance of tight junction and its high expression has been implicated in dictating malignant progression in various cancers. However, the post-translational modification of CLDN3 and its biological function remains poorly understood. Here, we report that CLDN3 is positively correlated with ovarian cancer progression both and Of interest, CLDN3 undergoes -palmitoylation on three juxtamembrane cysteine residues, which contribute to the accurate plasma membrane localization and protein stability of CLDN3 Moreover, the deprivation of -palmitoylation in CLDN3 significantly abolishes its tumorigenic promotion effect in ovarian cancer cells. By utilizing the co-immunoprecipitation assay, we further identify ZDHHC12 as a CLDN3-targating palmitoyltransferase from 23 ZDHHC family proteins. Furthermore, the knockdown of ZDHHC12 also significantly inhibits CLDN3 accurate membrane localization, protein stability and ovarian cancer cells tumorigenesis Thus, our work reveals -palmitoylation as a novel regulatory mechanism that modulates CLDN3 function, which implies that targeting ZDHHC12-mediated CLDN3 -palmitoylation might be a potential strategy for ovarian cancer therapy.

Corpus callosum is an important constituent of central nervous system.It is located in the bottom of the fissurae longitudinalis cerebri,linking the cross the nerve fibers of left and right hemispheres.Corpus callosum is the largest commissural fibers.There are a variety of lesions in corpus callosum,presenting a variety of clinical symptoms,but most of them are not specific.Common corpus callosum lesions include corpus callosum hemorrhage,corpus callosum infarction,corpus callosum demyelinating,agenesis of the corpus callosum,corpus callosum tumor,corpus callosum injury,clinically mild encephalitis/encephalopathy with a reversible splenial lesion and so on.At present,there are few studies on corpus callosum lesions in children in China.This paper summarizes the current situation and the latest research progress of corpus callosum lesion in children.

Objective: To explore the effect of 18β-sodium glycyrrhetinic acid on thymic stromal lymphopoietin (TSLP) in nasal mucosa of allergic rhinitis (AR) rats. Methods: One hundred Wistar rats,half male and half female,were randomly divided into 5 groups by random number table method: control group, AR model group,budesonide group,18β-sodium glycyrrhetinic acid at dose of 20 mg/kg and 40 mg/kg groups, with 20 rats in each group. AR animal models were established by ovalbumin (OVA) sensitization in the other four experimental groups. After successful modeling, budesonide and 18β-sodium glycyrrhetinic acid were given in each group,and the detection time points were 2 weeks and 4 weeks. The distribution of TSLP in rat nasal mucosa was detected by immunohistochemistry,and the expression of TSLP in rat nasal mucosa was determined by Western blot at the protein level. The expression of TSLP-mRNA in rat nasal mucosa was detected and compared by real-time fluorescence quantitative PCR (RT-PCR) at mRNA level. The concentrations of IL-4 and OVA-sIgE in rat serum were measured and compared by ELISA. One-way analysis of variance and the least significant difference method were used for the comparison among groups, LSD t test was used for the comparison between the two groups,and the difference was statistically significant (P<0.05). Results: Immunohistochemistry confirmed existence of TSLP in rat nasal mucosa, especially in epithelial cells,endothelial cells and epithelial cilia. Western blot and RT-PCR suggested that the expression of TSLP and TSLP-mRNA in nasal mucosa of AR model group was significantly higher than that of control group (2 weeks TSLP: 1.795 9±0.131 4 vs 0.990 5±0.164 2, 4 weeks TSLP: 1.809 7±0.253 4 vs 0.870 3±0.124 4; 2 weeks TSLP-mRNA:4.582 9±0.697 7 vs 1.108 7±0.081 1, 4 weeks TSLP-mRNA:4.814 4±0.662 8 vs 1.001 0±0.155 3; all P<0.05). After 2 weeks and 4 weeks of drug intervention,the expression of TSLP and TSLP-mRNA was inhibited in nasal mucosa of budesonide group,18β-sodium sodium glycyrrhetinic acid at dose of 20 mg/kg and 40 mg/kg group,which was significantly different from that of AR model group (2 weeks TSLP: (0.897 8±0.081 8)/(1.072 1±0.113 6)/(1.396 6±0.133 9) vs 1.795 9±0.131 4; 4 weeks TSLP: (1.191 0±0.161 3)/(1.141 0±0.152 3)/(1.200 5±0.189 6) vs 1.809 7±0.253 4; 2 weeks TSLP-mRNA: (1.175 6±0.100 9)/(1.254 4±0.078 2)/(2.037 2±0.559 2) vs 4.582 9±0.697 7; 4 weeks TSLP-mRNA: (1.158 3±0.104 3)/(1.224 0±0.034 0)/(1.275 2±0.099 6) vs 4.814 4±0.662 8; all P<0.05), and not significantly different from control group. With the inhibition of TSLP, the concentrations of IL-4 and OVA-sIgE in rat serum were also decreased. Conclusion 18β-sodium glycyrrhetinic acid has obvious inhibitory effect on TSLP in nasal mucosa of AR rats, which can control Th2 type immune inflammatory reaction.

OBJECTIVE:To study the gene mutation status of epidermal growth factor receptor(EGFR)in non-small cell lung cancer(NSCLC)patients and its relationship with clinical indexes,and to provide reference for individualized administration of EGFR-TKI in NSCLC patients. METHODS:Totally of 274 NSCLC patients from the northern of Jiangsu area were selected from our hospital during Jan. 2015-Dec. 2017. Mutation status of EGFR gene in lung tissue was determined by amplification refractory mutation system (ARMS)-TaqMan PCR assay. The relationship of EGFR gene mutation with clinical indexes as gender,age, smoking status, staging, tumor differentiation and pathological type were analyzed retrospectively. Compared with related literatures,the regional differences of EGFR gene mutation were analyzed. RESULTS:Among 274 NSCLC patients,112 patients suffered from EGFR gene mutation with total mutation rate of 40.88%. There were 50,57,3,2 cases of exon 19,exon 21,exon 20 and exon 19+21 mutation,and the types of EGFR gene mutation were delE746-A750,L858R and insH773-V774H,etc. The mutation rates of EGFR gene exon 19,exon 21 in non-smoking,early,well-differentiated and adenocarcinoma patients were 52.50%,47.24%,46.36% and 45.00%,which were significantly higher than smoking (28.57%),advanced (27.03%), poor-differentiated(31.71%)and squamous cell carcinoma(27.66%)patients,with statistical significance(P＜0.05). There was no statistical significance in mutation rates of EGFR gene exon 19 and exon 21 between male and female,≥65 year-old and ＜65 year-old patients (P＞0.05). EGFR mutation rate of NSCLC subjects from the northern of Jiangsu area was significantly higher than Shanghai area(P＜0.05);there was no statistical significance compared with Yunnan area(P＞0.05)but mutation types were different. CONCLUSIONS:There is the highest EGFR gene mutation rate in its exon 21,lesser in exon 19,rare in exon 20 and exon 19+21 among NSCLC patients from the Northern of Jiangsu area. There are obvious regional differences. The mutation rate of EGFR gene mutation exon 19 and exon 21 are associated with smoking status,staging,tumor differentiation and pathological type of NSCLC patients. The non-smoking, early stage, well-differentiated and adenocarcinoma patients are more likely to benefit from EGFR-TKI targeted therapy.

Objective To explore the behavioral characteristics of response to name in 16-30 months old infants with autism spectrum disorders (ASD),in order to provide a theoretical basis for the early identification and early diagnosis.Methods Two professionals,according to the scoring criteria and using video analysis methods,evaluated the response score (RS),reaction time (RT),duration time (DT) and the rate (RR) of response to their names among ASD infants (ASD group,61 cases),who were diagnosed at Child Developmental and Behavioral Center,the Third Affiliated Hospital of Sun Yat-Sen University from April to December 2017.Then they were compared with infants with developmental delays (DD group,32 cases) and neuro-typical (NT group,33 cases) infants.Finally,researchers predicted the diagnosis for ASD infants according to the behavioral indicators,which had significant differences compared with other groups.Results Compared with DD group [RS 2 (1) score,RT 1.32 (4.65) s,DT 2.69(1.84) s] and NT group [RS 2 (1) score,RT 1.37 (4.37) s,DT 2.90 (2.23) s],RS was significantly lower [1 (1)score],RT was significantly longer [5.87 (4.64) s],and DT was significantly shorter [0.77 (1.88) s] in ASD group,and the differences were statistically significant (H =-4.91,-5.94;5.36,5.41;-4.47,-5.78;all P ＜0.05);while the differences between DD group and NT group were not significant(all P ＞0.05).The RR was significantly lower in ASD group [0.25 (0.50)] and DD group [0.50 (0.25)],compared with NT group [0.75 (0.50)],and the differences were statistically significant (H =-6.39,-4.45,all P ＜ 0.01);while the differences between DDgroup and ASD group were not significant(P ＞0.05).When detecting ASD from ASD and NT infants,the area under the receiver operating characteristic curve (AUC) was 0.889 (P ＜0.01);when detecting ASD from ASD and DD infants,AUC was 0.924 (P ＜ 0.01);when detecting ASD from all infants,AUC was 0.868 (P ＜ 0.01),according to all indicators of response to name.Conclusions There are significant differences between ASD infants and DD and NT infants in response to name domain.Behavioral characteristics in the procedure of response to name can predict ASDwell.Response to name as an early social behavioral indicator,being tested at 2 years old,is still of importance for the early identification and early diagnosis of ASD.