Hypertrophic cardiomyopathy (HCM) in children is one of the most common hereditary cardiomyopathies caused by gene mutations encoding cardiac carcomeric proteins. It is mainly characterized by ventricular hypertrophy and non-enlarged cardiac chambers, with a potential risk of sudden cardiac death. Usually, the assessment is based on the general condition, clinical symptoms, imaging examination results, family history, and genetic testing of the patients, thereby providing a basis for judging the risk of sudden cardiac death, and then identifying the risk factors for sudden cardiac death in hypertrophic cardiomyopathy. Based on the relevant domestic and foreign literature in recent years and the accumulated work experience in the department of cardiology of Beijing Children's Hospital, these risk factors are divided into major and minor risk factors. A comprehensive understanding of these risk factors can guide the clinical early warning of high-risk children with possible sudden death, which is helpful for more accurate assessment of the prognosis of hypertrophic cardiomyopathy in children and the implementation of targeted intervention. This article reviewed the research progress of related risk factors for the prognosis of hypertrophic cardiomyopathy in children.

Cardiogenic syncope in children is common in clinic and is highly regarded because of its high risk of sudden death.The main causes of cardiogenic syncope in children are organic and arrhythmia.In the diagnosis of syncope in children, the clinical characteristics and the primary diagnosis of the etiology of cardiogenic syncope should be emphasized.Holter electrocardiogram and intracardiac electrophysiology are indispensable in arrhythmia syncope.Cardiac ultrasound and cardiac MRI in syncope with structural heart disease is importance.Genetic testing is highly recognized.Different causes of cardiogenic syncope should be treated in different ways.The aim of this review was to improve the ability of the clinician to identify cardiogenic syncope quickly and accurately, so as to improve the treatment of such children.

Objective:To investigate the pathogenic genes, clinical features and treatment as well as follow-up of children with congenital long QT syndrome (LQTS).Methods:The clinical data, genetic test results and follow-up data of 16 congenital LQTS children with syncope as the first manifestation admitted to the Department of Cardiology, Beijing Children′s Hospital Affiliated to Capital Medical University from August 2016 to March 2023 were collected and retrospectively analyzed.Results:Among the 16 LQTS patients, the age of first syncope onset was 1.3-13.3 (7.37±3.41) years, and the interval between first syncope onset and clinical diagnosis was 0-48 (14.8±16.2) months.A total of 13 (81.3%) patients had triggers of syncope, of which nine were exercise-induced and four were emotional induced.Genetic testing was performed in 13 patients with LQTS, of which 12 (92.3%) were found to have pathogenic or suspected pathogenic mutations from KCNQ1, KCNH2, and SCN5A gene.The corrected QT interval of 16 patients was (550.0±50.2) ms, all cases≥460 ms.Schwartz scored 6.0 (5.0, 6.0) points, all cases≥4 points.All patients were initially treated with metoprolol or propranolol, of which 14 patients were followed up to date, three patients had recurrent syncope, and five patients stopped taking the medicines by themselves.One patient with high-dose metoprolol (LQT2) was treated with mexiletine after recurrent episodes.One patient who was intolerant to high-dose propranolol underwent left cardiac sympathectomy and was followed up after surgery without syncope episodes.None of the patients underwent implantable cardioverter defibrillator implantation. Conclusion:Children with LQTS and syncope symptoms have high positive rate of genetic tests.The genetic results could assist typing of patients with LQTS and guide treatment.Routine electrocardiogram screening in children with syncope may diagnose LQTS earlier and reduce misdiagnosis and missed diagnosis.β-blockers are the cornerstone of treatment for patients with LQTS.Strengthening follow-up management and improving patients′ treatment compliance is conducive to further improving the treatment response rate of patients.

Objective:To analyze the clinical features and follow-up of children with cardiogenic syncope (CS), and accurately and efficiently guide clinical diagnosis as well as improve the prognosis of children with CS.Methods:Ninety-eight children with CS who were hospitalized in the Department of Cardiology, Beijing Children′s Hospital Affiliated to Capital Medical University from April 1, 2016 to June 31, 2023 were selected as the study objects.According to the etiology type, the children with CS were divided into arrhythmia group, organic cardiovascular disease group and mixed group.The causes of syncope episodes, type of aura, frequency of syncope at first diagnosis, duration of loss of consciousness, concomitant symptoms, past history, family history, physical examination and follow-up were collected and statistically analyzed in each group.Results:A total of 98 children with CS were included, including 59 males and 39 females.The age of first onset was (8.69±3.90) years old.There were 60 cases in arrhythmia group, 18 cases in organic cardiovascular disease group and 20 cases in mixed group.There were no statistically significant differences among three groups of children in whether had inducement, whether had aura, incidence of aura types, duration of loss of consciousness, incidence of urinary and fecal incontinence and associated symptoms of fall injury, incidence of liver macrosis, and recurrence of syncope during follow-up.The children in arrhythmia group were more likely to induce syncope due to intense exercise than those in mixed group ( χ2=9.785, P<0.05). Compared with the organic cardiovascular disease group and the mixed group, the number of syncope attacks in the arrhythmia group was more than five times at the first diagnosis ( P=0.020). Compared with the organic cardiovascular disease group, the children in mixed group and arrhythmia group were more likely to have accompanying symptoms during syncope( P<0.05), and the incidences of convulsion were the higher in both groups.The positive signs of heart in mixed group were more than those in arrhythmia group and organic cardiovascular disease group( P<0.05). Compared with arrhythmia group, facial cyanosis was more common in mixed group and organic cardiovascular disease group ( P<0.05). Of the 87 children with CS who were followed up regularly, 73 (83.9%) did not have recurrent syncope after timely treatment and regular outpatient medication adjustment. Conclusion:Children with CS have special clinical characteristics, such as syncope is easily induced by strenuous exercise or emotional excitement, syncope is often preceded by no aura of seizure, loss of consciousness lasts for a relatively short period of time, the main accompanying symptom of syncope is convulsions, positive cardiac signs can be seen on physical examination, and there can be cardiac disorders in the past history or sudden death in the family history.It is of great significance to improve the diagnosis and prognosis of children with CS by mastering its characteristics and giving timely and appropriate treatment.

Abstract Allergic diseases can occur in all systems of the body, covering the whole life cycle, from children to adults and to old age, can be lifelong onset and even fatal in severe cases. Children account for the largest proportion of the victims of allergic disease, Children s allergies start from scratch, ranging from mild to severe, from less to more, from single to multiple systems and systemic performance, so the prevention and treatment of allergic diseases in children is of great importance, which can not only prevent high risk allergic conditions from developing into allergic diseases, but also further block the process of allergy. At present, there is no consensus on the management system of allergic children in kindergartens and primary schools. The "Consensus on Allergy Management and Prevention in Kindergartens and Primary Schools", which includes the organizational structure, system construction and management of allergic children, provides evidence informed recommendations for the long term comprehensive management of allergic children in kindergartens and primary schools, and provides a basis for the establishment of the prevention system for allergic children.

Objective:To investigate the influencing factors of textbook outcomes in liver surgery (TOLS) after radical resection of gallbladder carcinoma.Methods:The retrospective case-control study was conducted. The clinicopathological data of 530 patients who underwent radical resection of gallbladder carcinoma in 15 medical centers, including the First Affiliated Hospital of Army Medical University et al, from January 2014 to January 2020 were collected. There were 209 males and 321 females, aged (61±10)years. Patients underwent radical resection of gallbladder carcinoma, including cholecystectomy, hepatectomy, invasive bile duct resection, and lymph node dissection. Observation indicators: (1) situations of TOLS; (2) influencing factors of TOLS. Measure-ment data with normal distribution were represented as Mean± SD, and comparison between groups was conducted using the independent sample t test. Measurement data with skewed distribution were represented as M( Q1, Q3), and comparison between groups was conducted using the Mann-Whitney U test. Count data were described as absolute numbers or percentages, and comparison between groups was conducted using the chi-square test. Comparison of ordinal data between groups was conducted using the Mann-Whitney U test. The univariate analysis was conducted using the corresponding statistical methods based on data type, and variables with P<0.10 were included in multivariate analysis. Multivariate analysis was conducted using the Logistic stepwise regression model. Results:(1) Situations of TOLS. All 530 patients underwent radical resection of gallbladder carcinoma, and there were 498 cases achieving R 0 resection, 508 cases without ≥grade 2 intra-operative adverse events, 456 cases without postoperative grade B and grade C biliary leakage, 513 cases without postoperative grade B and grade C liver failure, 395 cases without severe com-plications within postoperative 90 days, 501 cases did not being re-admission caused by severe com-plications within postoperative 90 days. Of the 530 patients, 54.53%(289/530) of patients achieved postoperative TOLS, while 45.47%(241/530) of patients did not achieve postoperative TOLS. (2) Influencing factors of TOLS. Results of multivariate analysis showed that American Society of Anesthesiologists classification >grade Ⅱ, preoperative jaundice, T staging as T3?T4 stage, N staging as N2 stage, liver resection as right hemi-hepatectomy, and neoadjuvant therapy were independent factors influencing TOLS in patients undergoing radical resection of gallbladder carcinoma ( odds ratio=2.65, 1.87, 5.67, 5.65, 2.55, 3.34, 95% confidence interval as 1.22?5.72, 1.18?2.95, 2.51?12.82, 2.83?11.27, 1.41?4.63, 1.88?5.92, P<0.05). Conclusion:American Society of Anesthesiologists classification >grade Ⅱ, preoperative jaundice, T staging as T3?T4 stage, N staging as N2 stage, liver resection as right hemi-hepatectomy, and neoadjuvant therapy are independent factors influencing TOLS in patients undergoing radical resection of gallbladder carcinoma.

Objective:To conduct a survey on major surgeon′s mental trauma caused by iatrogenic biliary injury (IBI) during laparoscopic cholecystectomy (LC), and explore its influencing factors.Methods:The retrospective cross-sectional survey was conducted. Surgeons who have registered in Chinese College of Surgeons of Chinese Medical Doctor Association and Chinese Surgical Society of Chinese Medical Association were recruited to participate as respondents between December 1, 2018 and January 1, 2019. The survey was conducted by the questionnaires of influencing factors for surgeon′s mental trauma caused by IBI during LC. The questionnaires were distributed to participants via WeChat on the Wenjuanxin platform. Observation indicators: (1) results of questionnaire survey; (2) analysis of influencing factors for severe mental trauma of major surgeons caused by IBI during LC. Measurement data with normal distribution were expressed as Mean± SD, and count data were expressed as absolute numbers or percentages. The chi-square test was used for univariate analysis. Factors with P<0.10 in the univariate analysis were included in multivariate analysis, and Logistic regression model was used for multivariate analysis. Results:(1) Results of questionnaire survey. A total of 606 questionnaires were retrieved. ① Basic information of major surgeons: of the 606 major surgeons, there were 596 males and 10 females, aged (41±7)years, with a range from 18 to 62 years. Of the 606 major surgeons, 59.24%(359/606) came from non-teaching hospitals, and 64.03%(388/606) encounted the most impressive case of IBI during LC when they were in the primary or intermediate professional title. For 76.24%(462/606) of the major surgeons, the first case of IBI during LC was the most impressive case, and 69.80%(423/606) believed that careful operation during LC could avoid IBI. ② Patient information: of the patients with the most impressive IBI during LC in each major surgeon′s memory, there were 400 females and 206 males. The proportion of patients younger than 35 years old, in 35-65 years old and older than 65 years old was 9.57%(58/606), 65.51%(397/606), and 24.92%(151/606), respectively. ③ IBI related information: 57.43%(348/606) of the major surgeons indicated that they could receive help from senior surgeons in time for the occurrence of IBI during LC, and 78.88%(478/606) of the major surgeons invited senior surgeons to participate in the initial repair. For the most impressive case of IBI during LC, 66.83%(405/606) of the primary repair surgeries were performed during LC, 11.06%(67/606) were performed within postoperative 3 days and 22.11%(134/606) were performed after 3 days. The main repair methods included local repair, bile duct to end anastomosis, and bilioenteric anastomosis, accounting for 24.92%(151/606), 30.20%(183/606), 33.17%(201/606), respectively. The proportion of patients requiring partial hepatectomy, with perioperative death, and requiring multiple repair was 2.48%(15/606), 2.15%(13/606), and 9.24%(56/606), respectively. ④ Subsequent processing on major surgeons: after the occurrence of IBI during LC, 64.85%(393/606) of the major surgeons gained the understanding of patients and their families, 35.15%(213/606) of the major surgeons were involved in medical disputes, 15.68%(95/606) of the major surgeons received administrative punishment from the hospital. About the compensation, 14.36%(87/606) of the major surgeons had to pay for the compensation by themselves, and only 6.77%(41/606) of the major surgeons had medical liability insurance. There were 9.24%(56/606) of the major surgeons invloved in violent conflicts during the medical disputes. ⑤ Psychology-related information of major surgeons: of the 606 major surgeons, 544 had mental trauma including 279 with severe mental trauma. After the occurrence of IBI during LC, 82.01%(497/606) of the major surgeons experienced anxiety and/or depression for more than one month; 63.37%(384/606) of the major surgeons expected to avoid LC or showed tension during LC; 44.72%(271/606) of the major surgeons had a physiological response when recalling the case; 36.14%(219/606) of the major surgeons initiated the idea of not being a surgeon; 6.44%(39/606) of the major surgeons asked psychologists for help; and 5.61%(34/606) of the major surgeons had taken psychiatric drugs such as antianxiety and anti-depression drugs for more than one month. (2) Analysis of influencing factors for severe mental trauma of major surgeons caused by IBI during LC. Results of univariate analysis showed that the hospital type of the major surgeons, participation of senior surgeons in the repair, surgical method of the primary repair, IBI requiring repeated repair, medical disputes, administrative punishment from the hospital, compensation paid by the major surgeon, having medical liability insurance, violent conflicts in medical disputes were related factors for severe mental trauma of the major surgeons caused by IBI during LC ( χ2=7.688, 3.932, 19.764, 13.837, 61.488, 24.904, 30.976, 5.344, 26.285, P<0.05) . Results of multivariate analysis showed that the surgical method of the primary repair, IBI requiring repeated repair, medical disputes, administrative punishment from the hospital, compensation paid by the major surgeon, violent conflicts in medical disputes were independent risk factors for severe mental trauma caused by IBI during LC of the major surgeons ( odds ratios=1.203, 2.198, 2.922, 1.830, 2.405, 2.171, 95% confidence interval: 1.033-1.402, 1.143-4.226, 1.944-4.391, 1.083-3.093, 1.076-5.375, 1.002-4.702, P<0.05). Having medical liability insurance was an independent protective factor for severe mental trauma of the major surgeons caused by IBI during LC ( odds ratios=0.336, 95% confidence interval: 0.126-0.896, P<0.05). Conclusions:Most surgeons in China are troubled by IBI during LC, and nearly half of them suffer from severe mental trauma. Surgical method of the primary repair, IBI requiring repeated repair, medical disputes, administartive punishment from the hospital, compensation paid by the major surgeon, and violent conflicts in medical disputes are independent risk factors for severe mental trauma of major surgeons caused by IBI during LC. Having medical liability insurance is an independent protective factor for severe mental trauma of major surgeons caused by IBI during LC.

Objective: To clarify whether the surgical treatment for hilar cholangiocarcinoma combined with artery reconstruction is optimistic to the patients with hilar cholangiocarcinoma with hepatic artery invasion. Methods: There were 384 patients who received treatment in the First Affiliated Hospital to Army Medical University from January 2008 to January 2016 analyzed retrospectively. There were 27 patients underwent palliative operation, 245 patients underwent radical operation, radical resection account for 63.8%. Patients were divided into four groups according to different operation method: routine radical resection group(n=174), portal vein reconstruction group (n=47), hepatic artery reconstruction group (n=24), palliative group(n=27). General information of patients who underwent radical operation treatment was analyzed by chi-square test and analysis of variance. The period of operation time, blood loss, the length of hospital stay and hospitalization expenses of the radical operation patients were analyzed by one-way ANOVA. Comparison among groups was analyzed by LSD-t test. Results: The follow-up ended up in June first, 2016. Each of patients followed for 6 to 60 months, the median follow-up period was 24 months. 1-, 3-, and 5-year survival rates were 81.3%, 44.9% and 13.5% of routine radical operation group, and were 83.0%, 44.7% and 15.1% of portal vein reconstruction group, and were 70.8%, 27.7% and 6.9% of hepatic artery reconstruction group, respectively. And 1-, 3-, and 5-year survival rates of hepatic artery reconstruction group was lower than routine radical group and portal vein reconstruction group significantly (P<0.05). However, the rate of postoperative complications of the hepatic artery reconstruction group and the routine radical operation group and the portal vein reconstruction group were 62.5%(15/24), 55.3%(96/174) and 51.5%(24/47), respectively. There was no significant difference among them (P>0.05). The data shows that the ratio of lymphatic metastasis in hepatic artery reconstruction group (70.8%) is much higher than them in routine radical operation group (20.1%) and portal vein reconstruction group (19.1%) significantly (P<0.05). The presented data also indicate that hepatic artery resection prolongs survival time comparing with patients undergoing palliative therapy for hilar cholangiocarcinoma. Cox regression analysis indicate that hepatic artery resection and reconstruction is a protective factor compare with palliative therapy (RR=0.38, 95%CI: 0.22-0.67). The significant reason for shorter survival time is a positive correlation between hepatic artery invasion and lymph node metastasis. Conclusion Hepatic artery resection and reconstruction has beneficial impact on oncologic long-term outcome in patients with advanced stage hilar cholangiocarcinoma.

Aim To investigate the promoting apoptosis effect of artesunate( ART) on human colon cancer Lovo cells and its mechanisms. Methods MTT assay was performed to determine the anti-proliferative effect of artesunate. Flow cytometry assay and electron micros-copy( EM) were used to evaluate the apoptotic effect of artesunate. Luciferase reporter assay was introduced to measure the activation of Wnt/β-catenin pathway. Western blot was used to detect the pathway-related protein levels of β-catenin, GSK-3β,c-Myc and apop-tosis-related protein level of casepase-3 . Results Compared with the control group, the inhibitory rate of cell proliferation at 72 h and 320 μmol·L-1 ART was (78. 99 ± 1. 95 )% ( F =898. 301, P =0. 000 ); the cell apoptotic rate at 24 h and 160 μmol · L-1 ART was(19. 00 ± 0. 05)% and morphological signs of cell apoptosis were found by EM;the transcriptional activi-ty of TCF4/LEF at 24 h and 160 μmol·L-1 ART was (0. 36 ± 0. 30)%(F =470. 954,P <0. 01); the ex-pressions of caspase-3 and GSK-3β were significantly increased, whileβ-catenin and c-Myc were significant-ly decreased when treated with different concentrations of ART for 48 h ( P <0. 01 ) . Conclusion ART may significantly inhibit proliferation and promote apoptosis of Lovo cells probably by inactivating Wnt/β-catenin pathway.

Aim To investigate the effect of artesunate on the invasion of human colon cancer Lovo cells and the possible mechanisms. Methods After Lovo cells were treated with different doses of artesunate(20,80, 160 μmol·L - 1 ), the soft agar colony formation test was adopted to observe the anchorage-independent pro-liferation of Lovo cells. Transwell assay was used to determine the effect of artesunate on the invasion abili-ty of Lovo cells. And the protein expressions of HMGB1 and MMP-2 were investigated by western blot. Results Artesunate could significantly inhibit both proliferation and invasion ability of Lovo cells in a dose-dependent manner(P < 0. 01). The experimental group treated with artesunate significantly down-regula-ted the protein expressions of HMGB1 and MMP-2 compared with control group(P < 0. 05). Conclusion Artesunate could inhibit the invasion of human colon cancer Lovo cells by down-regulating HMGB1 and MMP-2 expressions.