We aimed to analyse the clinical characteristics of children with different types of anomalous aortic origin of the coronary artery (AAOCA) at different ages, and to discuss the factors related to myocardial ischaemia. In this retrospective study, we included 69 children diagnosed with AAOCA using CT coronary angiography; we classified the participants based on the type of AAOCA, age, and high-risk anatomy. The clinical characteristics of the different AAOCA types and age groups were compared, and the correlation between manifestations and high-risk anatomy was analysed. Anomalous origin of the left coronary artery from the right coronary artery sinus, anomalous origin of the right coronary artery from the left coronary artery sinus, and a coronary artery origin without coronary sinuses was found in 10 (14.5%), 57 (82.6%), 2 (2.9%) patients, respectively. There were no significant differences in sex, clinical manifestations, percentage of positive myocardial injury markers, electrocardiogram, transthoracic echocardiography, or proportion of high-risk anatomy among the groups with different AAOCA types. According to age group, the proportion of asymptomatic infants and pre-schoolers was the highest (p < 0.001). Forty-three patients (62.3%) had high-risk anatomy and were more likely to present with severe symptoms and cardiac syncope (p < 0.05). Conclusion : There were no significant differences in the proportions of high-risk anatomy and clinical characteristics among children with different AAOCA types. We found a relation between the severity of AAOCA clinical symptoms and anatomical risk. What is Known: • Clinical symptoms in children with AAOCA are varied and the results of routine cardiological examinations lack specificity. • High-risk anatomical features, exercise, cardiac symptoms, and ALCA are risk factors for the occurrence of SCD in patients with AAOCA. What is New: • Compared the clinical characteristics of different types of AAOCA and ages. • Analysed the correlation between symptoms and high-risk anatomical features.

BACKGROUND Left-sided accessory pathways (APs) can be accessed with either a transaortic (TA) or transseptal approach (TS). For children with Marfan syndrome (MFS) who have aortic disease, the use of TA can aggravate the disease, making TS the best choice for these patients. CASE SUMMARY A 10-year-old girl was hospitalized because of intermittent heart palpitations and chest tightness. She was diagnosed with MFS, supraventricular tachycardia, Wolff-Parkinson-White syndrome, and left-sided AP was detected by cardiac electrophysiological. Catheter ablation was successfully performed via TS under the guidance of the Ensite system. During the follow- up, no recurrence or complications occurred. CONCLUSION The TS for catheter ablation of left-sided APs can be considered in children with MFS. Adequate evaluation and selection of the appropriate puncture site are particularly important.

Infant tachycardia is a critical disease, mainly with supraventricular tachycardia and ventricular tachycardia.The treatment of tachycardia in infant is quite different from that of older children, and there is no relevant guidelines at present.Drug therapy in the acute stage of supraventricular tachycardia and atrial fluttery is mainly intravenous adenosine injection.Digoxin is widely used in neonates.Propranolol is the first choice for prophylactic treatment, and landilolol is in the development stage.Ventricular tachycardia can be spontaneously subsided, the treatment is dominated by intravenous lidocaine.For non-drug therapy, heart cardioerter is the emergency treatment for serious rapid arrhythmia.Radiofrequency ablation is used in infants with more severe conditions and where the onset of tachycardia can not be controlled.Bury cardioverter-defibrillator is effective in preventing infant ion channel disease complicated with malignant ventricular tachycardia induced sudden cardiac death.Subcutaneous implantion of a defibrillator may be superior to intravenous implantation in infants and young children.

Objective:To summarize characteristics and rules of blood pressure variability (BPV) in children with H-type hypertension, in an attempt to explore some basis for further selection of the time points of treatment.Methods:A total of 117 children diagnosed as essential hypertension in Children′s Hospital Affiliated to Capital Institute of Pediatrics from March 2018 to September 2019 were selected as the research objective.According to the serum level of homocysteine (Hcy), those children were divided into the simple essential hypertension group (74 cases, Hcy≤13.9 μmol/L) and the H-type hypertension group (43 cases, Hcy>13.9 μmol/L). All children received 24 h ambulatory blood pressure monitoring (24 h ABPM), and BPV indexes were recorded and subject to group comparison.Meanwhile, the serum Hcy level was monitored.Besides, the demographic data and such blood biochemical indexes as blood glucose, blood fat and renal function were recorded or measured for an analysis of the correlation between BPV and each index.Results:There were no significant differences in gender, age, body mass index (BMI) and biochemical indexes between the simple essential hypertension group and the H-type hypertension group (all P>0.05). Compared with the simple essential hypertension group, the H-type hypertension group had a significantly higher 24 h systolic blood pressure standard deviation [24 h SSD, (11.21±3.23) mmHg vs.(9.64±2.73) mmHg, 1 mmHg=0.133 kPa, t=-2.806, P<0.05], nighttime systolic blood pressure standard deviation [nSSD, (10.79±3.89) mmHg vs.(9.26±3.23) mmHg, t=-2.292, P<0.05], and nighttime diastolic blood pressure standard deviation [nDSD, (10.23±3.53) mmHg vs.(8.73±2.93) mmHg, t=-2.617, P<0.05]. Pearson correlation analysis showed that the serum Hcy level was significantly positively correlated with 24 h, SSD, nSSD and nDSD ( r=0.194, 0.183, 0.182, all P<0.05). Conclusions:24 h SSD in children with H-type hypertension is significantly higher, with an obvious increase in both nSSD and nDSD, and the serum Hcy level was significantly positively correlated with BPV, which suggested that it was required to effectively control the serum Hcy level, reduce the range of blood pressure fluctuation and select the optimal time points of treatment, thus delaying the progress of hypertension.

Objective:To provide a theoretical basis for diagnosing and treating recurrent Kawasaki disease based on data analysis of clinical symptoms.Methods:Data analysis of children with recurrent Kawasaki disease admitted to Beijing Children′s Hospital of Capital Medical University from January 2016 to December 2019 was conducted, including comparisons of the initial onset and the recurrence on patients′ clinical features, auxiliary examination and treatment.Results:During the four-year scale, 3 041 children with Kawasaki disease were admitted to the department of Cardiology.The recurrence data involved 65 children[male∶female 3.1∶1, average aged(2.42 ± 2.04) years]. First, children′s fever duration was(5.66 ± 2.37) days in recurrence data, significantly shorter than that of their initial onset.The recurrence data also reported a lower incidence of rash and limb changes, together with respiratory and gastrointestinal symptoms.Then, 19 cases out of the 65 children got coronary artery lesion(CAL) at their initial onset.A relief of CAL was reported from 15 children when the disease recurred, along with 11 new-reported CAL cases.Intravenous immunoglobulin(IVIG) refractory Kawasaki disease cases accounted for nine at the initial onset and 12 at the recurrent onset, respectively.Five IVIG refractory recurrent cases reported significant relief after 2 g/kg IVIG treatment.Conclusion:The recurrent Kawasaki disease in children usually occurs among children under three years old, within the first year after the initial onset.Most of the recurrent cases report a shorter fever duration and less typical clinical symptoms than their first onset.In some cases, IVIG treatment showed effects initially but failed to work when the disease recurred.Therefore, glucocorticoid or infliximab should be considered for further treatment.

Objective:To summarize the characteristics of hypertension and target organ damage in hospitalized children, so as to provide scientific basis for comprehensive prevention and management of hypertension children.Methods:The clinical data of 165 hospitalized children diagnosed with hypertension at Beijing Children′s Hospital from January 2017 to December 2019 were analyzed retrospectively.Data including medical history, clinical symptoms, signs, auxiliary examination and treatment strategy were collected and analyzed statistically.Results:Of 165 admitted hypertensive children who met the inclusion criteria, 35(21.2%)were classified as having primary hypertension, and 130(78.8%)were secondary hypertension.The body mass index(BMI)and the rate of family history of children with primary hypertension were higher than those of children with secondary hypertension, the differences were statistically significant( t=2.052, P=0.026; χ2=3.321, P=0.009). Among 165 children with hypertension, 137 had varying degrees of clinical symptoms(83.0%), only nine primary hypertension cases showed symptoms.The original etiologies of secondary hypertension included 78 cases of renal disease(60.0%), 23 cases of cardiogenic disease(17.7%), six cases of endocrine disease(4.6%)and five cases of rheumatic disease(3.8%). Of all admitted patients, the blood pressure of 158 cases(95.8%)decreased to normal with weight control, low-fat and low-salt diet, antihypertensive drugs and treatment of primary disease.Besides, the parents of seven cases gave up any treatment and left hospital. Conclusion:Secondary hypertension is the main type of hypertension in children, among which renal parenchymal disease is the most common etiology.The clinical symptoms also vary by its original etiology.The overall goal of this study is to reduce the risk of target organ damage, and get blood pressure under control relatively early and effectively.

Background Atherosclerosis cardiovascular disease (ASCVD) is the main cause of morbidity and mortality in type 2 diabetes mellitus (T2DM). As most diabetic patients with ASCVD are asymptomatic, it is most neglected in clinical practice. For this reason, identifying high-risk ASCVD population with intensified treatment is very important. In recent years, the relationship between diabetic retinopathy (DR) and ASCVD has caused much academic concern, but the results are inconsistent. Moreover, whether all grades of DR increase the risk of ASCVD remains controversial. Most importantly, very few data can be found in China. Objective Our aim is to discuss whether all grades of DR increase the risk of ASCVD after adjustment for the traditional cardiovascular risk factors and to assess the independent contribution of DR to cardiovascular events in patients with T2DM, hoping to provide more evidence for early identification of ASCVD. Research design and methods A total of 425 T2DM patients with complete physical and biochemical data were included in the study. The grade of DR was assessed with two 45 color digital retinal images. Based on the presence of history of ASCVD, 425 T2DM patients were divided into 2 groups: ASCVD group and non-ASCVD group. Results ASCVD patients were older and had a significantly higher fasting plasma glucose (FPG) and glycated haemoglobin (HbA1c) and proportion of history of ASCVD. At the same time, they were more likely to be females, and had lower level of alcohol and calculated glomerular filtration rate (eGFR) than non-ASCVD patients. Their trend to develop DR with ASCVD was significantly higher than patients with non-ASCVD (χ 2 = 5.805, P = 0.016). DR was an independent statistical indicator of the presence of ASCVD [odds ratio (OR) (95% CI): 2.321 (1.152–4.678), P = 0.018]. Furthermore, when DR was divided into non-proliferative retinopathy (NPDR) and proliferative retinopathy (PDR) according to its severity, only PDR was significantly associated with incident ASCVD [OR (95% CI): 8.333 (1.813–38.304), P = 0.006]. After adjusting for traditional ASCVD risk factors, such an association still existed [OR (95% CI): 7.466 (1.355–41.137), P = 0.021]. Conclusion DR associates strongly with ASCVD in the Chinese population with T2DM. With the increasing severity of DR, the risk of ASCVD also increases. After adjustment for traditional risk factors, PDR is still an independent risk marker for ASCVD.

Objective:To analyze the clinical characteristics of Kawasaki disease(KD)with complications of acute abdominal disease and to improve the diagnosis, treatment and prognosis of patients.Methods:A total of 2 931 cases with KD hospitalized from January 2016 to December 2019 in our department were retrospectively analyzed, including 14 children with acute abdominal disease(acute abdomen group). And 62 patients with KD but without acute abdominal disease were randomly selected as the control group.The clinical characteristics, laboratory examination and treatment between two groups were compared and analyzed.Results:There were 8 males and 6 females.The average age of patients was(4.46±0.74) years.Compared with control group, there was no significant difference in age, sex and proportion of coronary artery injury in acute abdomen group( P>0.05). Acute abdominal disease group were more likely to have gastrointestinal symptoms, intravenous immunoglobulin(IVIG) resistance and longer fever duration( P<0.05). C-reactive protein(CRP), alanine aminotransferase(ALT), aspartate aminotransferase(AST), γ-glutamyltranspeptidase(GGT) and total bilirubin(TBIL) in acute abdominal disease group were significantly higher( P<0.05). There was no significant difference in white blood cell, erythroayte sedimentation rate, hemoglobin, platelet, Na + , albumin between two groups( P>0.05). All of the 14 patients used IVIG, 3 of them were treated with hormone(methylprednisolone)and 1 patient accepted infliximab.All patients were discharged from hospital.After follow-up for 6 months to 3 years, all patients had no sequelae of digestive system. Conclusion:KD can be complicated with acute abdominal disease, some of which as the first symptom.Gastrointestinal manifestations such as abdominal pain are common.For children with obvious gastrointestinal symptoms, IVIG resistance, and long duration of fever, attention should be paid to the possibility of acute abdominal disease.For children with significantly elevated CRP, ALT, AST, GGT, and TBIL should to be alert to the possibility of acute abdominal disease.KD complicated with acute abdominal disease generally has good prognosis.It is important to treat KD in the first place.

背景:临床决策和应急处理能力是麻醉住院医师培养过程中的难点,也是保证临床安全的基础.目的:本研究在骨科临床手术麻醉教学中引入模拟病例运行教学方式,以提高住院医师临床决策和应急处理能力,并提高教学效果.方法:以麻醉科第二年规范化培训住院医师为培训对象,开展骨科手术麻醉应急处理的模拟培训课程.通过2个罕见、后果严重的骨科手术病例——恶性高热和术中肺栓塞进行模拟教学演练,并在演练结束后进行引导性反馈.在培训前后通过主观及客观问卷评估教学效果.结果:培训课程后,学员的客观评估评分显著提高(P<0.05),同时学员对培训课程满意度较传统模式教学高.结论:模拟病例教学方式弥补了传统教学的缺点,具有较好的教学效果,可以应用于骨科手术麻醉的临床教学中.

BACKGROUND:Peripheral artery disease (PAD) is a manifestation of atherosclerotic cardiovascular disease (ASCVD) and significantly increases the risk of myocardial infarction and stroke. As most patients with PAD are asymptomatic, this condition is largely neglected in clinical practice. Type 2 diabetes (T2DM) patients have higher prevalence of PAD. Therefore, early detection and intervention of diabetic PAD are very important. Metabolic syndrome (MetS) is a group of interrelated metabolic risk factors, a predictor of poor prognosis in elderly patients with ASCVD. Recently, many of the metabolic risk factors as well as the overall concept of MetS itself have sparked a great deal of debate regarding their precise roles in PAD. OBJECTIVE:To evaluate the relationship between metabolic syndrome (MetS) and peripheral arterial disease (PAD) in elderly patients with type 2 diabetes (T2DM). METHODS:Two hundred and eighty-one elderly T2DM patients admitted to Beijing Tongren Hospital from October 2016 to December 2017 were divided into PAD group (n=136) and non-PAD (NPAD) group (n=145). Their medical records, physical examination parameters and laboratory testing parameters were later recorded and analyzed by multivariate logistic regression analysis. RESULTS:No significant difference was detected in general clinical data and laboratory testing parameters between the two groups (P>0.05). The incidence of MetS was significantly higher in PAD group than in NPAD group (88.2% vs 70.3%, P=0.001). Multivariate logistic regression analysis showed that the risk of PAD was 1.762-fold higher in MetS patients after adjustment for age, sex, history of smoking and alcohol consumption, WC, SBP, serum TC and HbA1c level, eGFR and duration of DM (95% CI: 1.205-6.330, P=0.016). CONCLUSION:MetS closely correlates with PAD in elderly T2DM patients, though no significant difference has been detected in their metabolic indicators.

Department of Geriatrics, Beijing Tongren Hospital, Capital Medical University, Beijing, People’s Republic of China Background: Peripheral artery disease (PAD) is a manifestation of atherosclerotic cardiovascular disease (ASCVD) and significantly increases the risk of myocardial infarction and stroke. As most patients with PAD are asymptomatic, this condition is largely neglected in clinical practice. Type 2 diabetes (T2DM) patients have higher prevalence of PAD. Therefore, early detection and intervention of diabetic PAD are very important. Metabolic syndrome (MetS) is a group of interrelated metabolic risk factors, a predictor of poor prognosis in elderly patients with ASCVD. Recently, many of the metabolic risk factors as well as the overall concept of MetS itself have sparked a great deal of debate regarding their precise roles in PAD. Objective: To evaluate the relationship between metabolic syndrome (MetS) and peripheral arterial disease (PAD) in elderly patients with type 2 diabetes (T2DM). Methods: Two hundred and eighty-one elderly T2DM patients admitted to Beijing Tongren Hospital from October 2016 to December 2017 were divided into PAD group (n=136) and non-PAD (NPAD) group (n=145). Their medical records, physical examination parameters and laboratory testing parameters were later recorded and analyzed by multivariate logistic regression analysis. Results: No significant difference was detected in general clinical data and laboratory testing parameters between the two groups (P>0.05). The incidence of MetS was significantly higher in PAD group than in NPAD group (88.2% vs 70.3%, P=0.001). Multivariate logistic regression analysis showed that the risk of PAD was 1.762-fold higher in MetS patients after adjustment for age, sex, history of smoking and alcohol consumption, WC, SBP, serum TC and HbA1c level, eGFR and duration of DM (95% CI: 1.205–6.330, P=0.016). Conclusion: MetS closely correlates with PAD in elderly T2DM patients, though no significant difference has been detected in their metabolic indicators.

Objective:To summarize the clinical and genetic characteristics of catecholaminergic polymorphic ventricular tachycardia (CPVT) in children caused by CASQ2 gene variants. Methods:The clinical data of 8 children (4 males and females, respectively) with CPVT caused by CASQ2 gene variants admitted to Beijing Children′s Hospital, Capital Medical University from January 2017 to November 2018 were retrospectively analyzed.The targeted next generation sequencing was employed to identify CASQ2 variants and Sanger sequencing was conducted to conform the candidate variants and determine the parental origin. Results:As for 8 children in this study, the average age of onset was 6.4 years, the mean age at diagnosis was 9.4 years, and the average interval from onset to diagnosis was 3 years.Only 2 cases had clearly diagnosis at onset, other 6 cases had a delay to diagnosis and 3 cases of them were diagnosed at other hospitals as having epilepsy and did not respond to anti-epileptic therapy.During physical activity and/or emotional stress, 8 cases presented with recurrent syncope and were able to regain consciousness after a few minutes.They had no a history of sudden cardiac death or family history.There was no abnormality on resting electrocardiogram during the paroxysmal interval in 6 cases and mild sinus bradycardia in 2 cases.Typical bidirectional ventricular tachycardia (VT) and/or polymorphic VT were detected in 8/8 cases and 5/5 cases, respectively, based on Holter electrocardiography and cardiac stress test.The CASQ2 gene variant was found in all children, with 6 cases carrying compound heterozygous variants and 2 cases carrying homozygous variants.A total of 9 different CASQ2 variants were detected in 8 cases, of which 5 had not been previously reported.According to the family-line verification, all of them had a familial variant, with no novel variants.All 8 cases were treated orally with β-blockers, with asymptomatically recurrent episodes, with a mean follow-up of 1.5 years, during which implantable cardioverter defibrillation was performed in 1 case owing to severe sinus bradycardia.There was no death case among them. Conclusions:CPVT with CASQ2 variants is characterized by early onset before preschool age, recurrent syncope after exercise or emotional stress and bidirectional/polymorphic VT.Early diagnosis of CPVT remains challenging due to delayed diagnosis or misdiagnosis.Treatment with β-blockers can achieve favorable effectiveness and safety.Five novel variants in this study would further expand the database of CASQ2 genes.

恶性室性心律失常包括器质性和遗传性两大类,可导致猝死严重危害人类健康.这些心律失常发生多与心脏交感神经激活有关.心脏交感神经切除术(CSD)通过切除下半部分星状神经节和前四对胸交感神经节可降低心脏交感神经活性,是恶性室性心律失常有效、安全的治疗手段.对于长QT综合征和儿茶酚胺敏感性多形性室性心动过速(CPVT),单纯左侧CSD可能就已足够;对于器质性心脏病合并室性心律失常,双侧CSD可能具有更好疗效.

Objective:To analyze the changes of myocardial damage markers during ventricular tachycardia (SVT) onset and efficacy of various therapeutic approaches.Methods:The clinical data of 256 children with SVT admitted to Beijing Children′s Hospital Affiliated to Capital Medical University from October 2018 to October 2019 were retrospectively analyzed.According to the age of onset, children were divided into groups of 0~3 years old, >3-6 years old, and >6 years old.According to the different heart rates at the time of attack, patients were divided into the groups of <180 times/min, 180~260 times /min, and >260 times /min.According to the treatment methods, children were divided into physical therapy group, drug therapy group and radiofrequency ablation group.Frequency of SVT attacks, heart rate at the onset, serum creatine kinase isoenzyme (CK-MB), troponin (cTn) I, and brain natiuretic peptide(BNP) were collected within 2 h after the onset stop.The recovery rate of each treatment group was counted.Results:Among the 256 cases, there were 135 males and 121 females, which aged from 1 month to 18 years, mean age was (10.2±2.2) years and SVT attacked from 1 to 8 times.The faster the heart rate during SVT attack, the higher CK-MB, cTnI and BNP ( P<0.05). The recovery rate was 27.6% in the mechanical stimulation group, 98.0% in the drug treatment group and 100% in the radiofrequency ablation group, respectively.Successful recovery rate was 47.2%(34 cases)with ATP, 71.7%(109 cases)with Propafenone, 67.3%(64 cases)with Cediland, 59.6%(31 cases)with Verapamil, 61.5%(24 cases)with Sotalol and 54.7%(29 cases)with Amiodarone.Eithty-two cases were successfully converted by two drugs(32.7%) and 38 cases were successfully converted by three drugs(15.1%). Conclusion:When SVT attacks, most children have myocardial damage.The faster the heart rate, the greater the damage in the heart muscle; The treatment of SVT in children should still be the first choice of physical therapy in older children, and if it is not effective, drug therapy should be selected.Radiofrequency ablation is the most effective method to cure tachyarrhythmia.

目的:总结川崎病(KD)伴休克及心肾综合征5型(CRS-5)患儿的临床特点。方法:回顾性分析首都医科大学附属北京儿童医院2016年6月至2020年2月收治的4例KD伴休克及CRS-5患儿的临床表现、实验室检查、影像学特点、治疗及预后情况。结果:4例患儿中男3例，女1例，发病年龄2～12岁。3例患儿在KD经典症状的基础上于发病第5天出现休克表现，1例患儿以休克起病。所有患儿均同时伴有精神萎靡、呼吸心率增快、心音低钝、奔马律及尿少。4例患儿外周血白细胞计数、中性粒细胞比例、C反应蛋白、降钙素原、肾功能及N末端脑利钠肽前体均显著升高，并伴有低白蛋白血症及蛋白尿，3例患儿肌钙蛋白I升高。超声心动图提示4例患儿左心室射血分数值降低，波动在41%～55%，仅1例出现冠状动脉扩张。2例患儿泌尿系超声提示双肾增大，回声增强。4例患儿予液体复苏、应用血管活性药物、静脉用丙种球蛋白冲击、糖皮质激素抗炎、强心及营养脏器等支持治疗后，均好转出院。随访期间，所有患儿均未遗留脏器功能损害。结论:儿童KD急性期可出现休克及CRS-5表现，常伴强烈的炎症反应，心肾损伤标记物异常升高，及时正确治疗，预后良好。

目的 总结风湿性心脏炎的临床特点及用心脏彩超评价治疗前后心脏结构及功能情况.方法 回顾性分析2007年10月到2019年4月首都医科大学附属北京儿童医院收治的136例风湿性心脏炎患儿的临床特点,用心脏彩超评价治疗前与治疗后6个月心脏结构变化,分析抗风湿治疗的效果.结果 风湿性心脏炎患儿临床症状部分不典型.心脏彩超表现以瓣膜返流最为常见(86.0％),其次为心脏增大(34.6％),极少存在心包积液(0.7％).治疗前后二尖瓣、主动脉瓣、三尖瓣瓣膜返流程度差异有统计学意义(P＜0.05).左心室增大患儿治疗前左心室舒张末期前后径差异有统计学意义(P＜0.05).主动脉瓣狭窄及主动脉瓣脱垂者治疗前后均无明显改变.结论 风湿性心脏炎患儿经积极早期抗风湿及对症治疗后,瓣膜返流可较前减少,扩大的心脏可较前缩小,大部分射血分数减低患儿的射血分数升高,心包积液消失,但瓣膜狭窄及脱垂多无明显改善.

Objective: To investigate the clinical features and improve the diagnosis and treatment of anomalous origin of the left coronary artery from the right coronary sinus with an interarterial course (ALCA-R-IAC) between the ascending aorta and main pulmonary artery in children. Methods: A retrospective analysis of the clinical manifestation, laboratory test, radiological feature, treatment and prognosis were conducted in four female children presented with ALCA-R-IAC in Beijing Children's Hospital from November 2015 to June 2018. Results: The four girls with onset age of 7.5-14.7 years were diagnosed with ALCA-R-IAC by CT coronary angiography (CTCA). Four children presented with exercise-induced syncope and clinical manifestations of acute myocardial infarction including 3 patients with acute left heart failure, 1 cardiogenic shock and 1 cardiac arrest. Nervous system involvement was found in one patient. Troponin I increased significantly to 20.65-50.00 μg/L in the four patients. Electrocardiogram (ECG) developed signs of left main coronary artery involvement. Echocardiography revealed reduced left ventricular ejection fraction (LVEF) of 25%-45% in three children and suspected anomalous origin of the left coronary artery in one child. CTCA showed an anomalous left coronary artery originating from the right coronary sinus, which had an interarterial course between the aorta and pulmonary artery leading to a slim left main coronary trunk. Two children underwent unroofing procedure and the other two children in whom physical activities were restricted received conservative managements. During a regular follow-up period of 12-43 months, all the children survived without recurrent symptoms and had good prognosis. Conclusions: ALCA-R-IAC can present as exercise-related syncope and acute myocardial infarction, even sudden death in children and adolescents. CTCA is helpful to clarify the early diagnosis of ALCA-R-IAC. Surgical intervention is the main treatment for ALCA-R-IAC and strenuous physical activities should be avoided.

Objective:To summarize the changes of serum cytokines in 301 children with Kawasaki disease(KD) and analyze their clinical significance.Methods:Three hundred and one pediatic patients with KD admitted to Beijing Children′s Hospital, Capital Medical University between June 2018 and July 2019 were enrolled and divided into the immunoglobulin sensitive group and immunoglobulin non-response group according to the therapeutic effect.The levels of interferon gamma (IFN-γ), tumor necrosis factor-alpha (TNF-α), interleukin-10 (IL-10), interleukin-6 (IL-6), interleukin-4 (IL-4), and interleukin-2 (IL-2) were compared between the 2 groups.Results:(1)Fifty (16.6%) children had no-response to immunoglobulin.Before treatment, the level of serum cytokines in the immunoglobulin non-response group and the immunoglobulin sensitive group were as follows: IFN-γ[11.32(10.88) ng/L vs. 4.52(5.09) ng/L], TNF-α[7.43(11.25) ng/L vs. 4.42(6.04) ng/L], IL-10[14.44(20.3) ng/L vs. 8.32(7.99) ng/L], IL-6[100.28(126.39) ng/L vs. 41.34(49.32) ng/L], IL-4[1.06(1.79) ng/L vs. 0.98(1.42) ng/L], IL-2[0.48(0.90) ng/L vs. 0.40(0.62) ng/L]; there were significant differences in IFN - γ, TNF - α, IL-10 and IL-6 between the 2 groups (all P<0.05); there was no significant difference of IL-4 and IL-2 between the 2 groups (all P>0.05). (2)Before treatment and after the temperature was normal, the levels of cyto-kines in the immunoglobulin non-response group were as follows: IFN-γ[11.32(10.88) ng/L vs. 1.26(2.10) ng/L], TNF-α[7.43(11.25) ng/L vs. 1.52(3.46) ng/L], IL-10[14.44(20.3) ng/L vs. 4.63(7.00) ng/L], IL-6[100.28(126.39) ng/L vs. 22.85(39.95) ng/L], IL-4[1.06(1.79) ng/L vs. 0(0.25) ng/L], IL-2[0.48(0.90) ng/L vs. 0(0.02) ng/L]. Before treatment and after the temperature was normal, the levels of cytokines in the immunoglobulin sensitive group were as follows: IFN-γ[4.52(5.09) ng/L vs. 0.79(1.53) ng/L], TNF-α[4.42(6.04) ng/L vs. 1.60(3.4) ng/L], IL-10[8.32(7.99) ng/L vs. 3.59(3.74) ng/L], IL-6[41.34(49.32) ng/L vs. 11.90(18.84) ng/L], IL-4[0.98(1.42) ng/L vs. 0(0.88) ng/L], IL-2[0.40(0.62) ng/L vs. 0(0.14) ng/L]. The levels of cytokines decreased significantly after treatment in between 2 groups (all P<0.05). (3) Logistic regression analysis showed that IFN-γ, IL-6, fever duration and albumin were independent risk factors for no-response to immunoglobulin. Conclusions:Children with abnormally high serum IFN-γ and IL-6 levels before treatment are more likely to resist immunoglobulin.Therefore, the change of the condition in such children should be closely monitored.If there is no response to immunoglobulin, immunoglobulin should be used again as soon as possible or second-line drugs should be applied to improve the prognosis.

伊伐布雷定是一种选择性窦房结If电流抑制药,具有特异性降低心率的作用.美国食品药品监督管理局于2019年批准其用于治疗6个月及以上儿童患者因扩张型心肌病导致的稳定型心力衰竭,为儿童心力衰竭患者提供了一种新的治疗选择.本文对伊伐布雷定治疗儿童心力衰竭的作用机制、药代动力学及临床应用的现状与研究进展进行综述.