Objective:To analyze the clinical characteristics of different types of anomalous origin of the coronary artery.Methods:A case-series study was conducted.Based on the clinical data of children diagnosed with anomalous origin of the coronary artery at Shanghai Children′s Medical Center, Shanghai Jiao Tong University School of Medicine from January 2013 to January 2023, the diseases of different types of anomalous origin of the coronary artery were summarized.Results:A total of 177 children with anomalous origin of the coronary artery were treated.Among them, 122 children developed the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), including 54 males and 68 females, with a median age of 1.2 years; 6 children developed the anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA), including 3 males and 3 females, with a median age of 4.3 years; 9 children developed the anomalous left coronary artery from the right coronary sinus (ALCA), including 6 males and 3 females, with a median age of 9.5 years; 40 children developed the anomalous right coronary artery from the left coronary sinus (ARCA), including 24 males and 16 females, with a median age of 7.7 years.Most children diagnosed with ALCAPA had onset within 1 year of age, with chronic heart failure as the main manifestation, and young children were often accompanied by severe mitral regurgitation.A total of 111 children underwent surgery, and 11 children died.Six children with ARCAPA had no obvious clinical symptoms and were treated by operation according to the principle of double coronary circulation after diagnosis.Nine children with ALCA started with syncope, chest pain or abdominal pain after exercise.Eight of the children underwent surgical treatment, including 1 who received a heart transplant.Of the 40 children with ARCA, 23 children had clinical manifestations, with chest tightness, syncope, and chest pain after exercise as chief complaints; 16 children were tested positive for treadmill exercise before surgery; and a total of 13 children received surgical treatment.Conclusions:Different types of anomalous origin of the coronary artery vary in severity.The clinical manifestations of the anomalous origin of the left coronary artery are generally serious, and most of such patients have the risk of cardiac insufficiency or sudden death.Once diagnosed, surgical treatment should be performed timely.The clinical manifestations of the anomalous origin of the right coronary artery are relatively mild, and only a few may have serious consequences, which are usually treated according to the principle of individualization or double coronary circulation.

Objective:To analyze the coronary angiographic (CAG) characteristics of coronary artery lesion (CAL) in children with Kawasaki disease (KD), and to clarify the necessity of CAG in the diagnosis and treatment of KD combined with CAL in children.Methods:It was a retrospective study to analyze the clinical data, electrocardiogram, echocardiography, time and findings of CAG in children with KD and CAL who underwent CAG in Shanghai Children′s Medical Center of Shanghai Jiao Tong University School of Medicine from January 2013 to August 2022.The distribution, type, severity, and prognosis of CAL were analyzed.Results:A total of 117 children with KD and CAL were included in the analysis.The onset age of KD was from 2 months to 12.8 years old, and the age of performing CAG was from 8 months to 18.1 years old.A total of 234 coronary artery lesions were detected in 117 cases.Among them, CAL in the right coronary artery (RCA), left anterior descending branch (LAD), left main coronary artery and left circumflex artery were detected in 96 branches(41.1%), 78 branches(33.3%), 44 branches(18.8%), and 16 branches(6.8%), respectively.Unilateral coronary artery involvement was detected in 43 cases (36.8%), of which LAD was the dominant; while bilateral involvement was detected in 74 cases (63.2%), among which, LAD and RCA were the most involved arteries.Stratified by the degree of coronary involvement, large coronary aneurysms and severe coronary stenosis were most frequently occurred in the RCA and LAD.In contrast, 10 cases (13.6%), 20 cases (24.3%), 55 cases (45.8%) and 37 cases (67.3%) of intraluminal lesions were found in small, medium and large coronary aneurysms, and stenosis or occlusion, respectively.The incidence of intraluminal lesions tended to be higher in the site of severe lesions.CAG showed stenosis or occlusion in a total of 55 cases, and collateral circulation at varying degrees was found in cases of severe stenosis or occlusion.Conclusions:CAL in children with KD are complex and varied.Although clinical symptoms, routine electrocardiogram and cardiac ultrasound may indicate severe CAL.Their applications are limited by the diagnosis of the type (especially stenosis), degree, and extent of CAL, as well as the detection of extracoronary lesions.CAG is of great significance to identify vascular lesions and guide clinical management of KD combined with CAL in children.

Pediatric neurogenic bladder (PNB) is defined as dysfunction of the detrusor and/or urethral sphincter due to myelodysplasia or spinal cord injury, manifesting as impaired urination or defecation, which seriously affects the patients’ quality of life. The main indication for sacral neuromodulation (SNM) is overactive bladder syndrome, which presents as refractory urinary frequency and urgency, urgency incontinence and non-obstructive urinary retention. Age more than 16 years are also recommended for this technique. Previous studies have revealed that SNM could significantly improve the outcome of refractory bladder bowel dysfunction. This paper reviews the advances of the application of SNM in the treatment of pediatric neurogenic bladder so as to provide reference for pediatricians.

Objective:To explore the clinical characteristics, therapeutic efficacy and prognosis of congenital coronary artery fistula (CAF) in children.Methods:Clinical data of 71 pediatric patients diagnosed with congenital CAF at Department of Cardiology and Department of Cardiac Surgery, Shanghai Children′s Medical Center, Shanghai Jiao Tong University School of Medicine from January 2013 to June 2019 were retrospectively analyzed.The median age was 2.2 years (0.1-14.0 years), and the median body weight was 18.3 kg (3.2-55.8 kg), including 37 males (52.1%) and 34 females (47.9%). They were divided into the transcatheter closure group (30 cases) and surgical repair group (41 cases). The therapeutic effect and follow-up data of the 2 groups were compared by the Fisher′ s exact test. Results:Among the 71 congenital CAF children, 70 had heart murmurs, 2 had chest tightness after activity, and 5 were prone to recurrent respiratory tract infection.Transcatheter closure and surgical repair were successfully performed in 22/30 (73.3%) and 41/41 (100.0%) cases with a statistically significant difference ( P=0.001). However, in the surgical repair group, 1 (2.4%) case died after operation and 2(4.9%) needed further transcatheter closure due to large residual shunt during the follow-up period.At the last follow-up, there were 2 cases with minimal or small residual shunt in both groups ( P=0.567). There were 2/71(2.8%) cases suffering from postoperative thrombosis. Conclusions:Cardiac murmur is the main sign of congenital CAF in children, and some of them may have frequent respiratory tract infection due to increased lung blood caused by a large amount of shunt.A few children have chest tightness and chest pain due to myocardial ischemia caused by coronary steal.Both percutaneous closure and surgical repair are safe and effective with few complications.

Objective:To investigate the effects of hydrogen rich-saline (HRS) on intestinal mucosal barrier in rat with intestinal ischemia/reperfusion injury (IIRI).Methods:Twenty-four healthy male Sprague-Dawley rats, aged 8 weeks, were randomly divided into 3 groups (8 in each group) by random number table method: sham group, model group and HRS group.Rats in HRS group were intraperitoneally injected with HRS (10 mL/kg) at 30 min of ischemia, and the same amount of normal saline was intraperitoneally injected in model group.After 45 min of ischemia and 6 h of reperfusion, rats were sacrificed.Serum and ileum were collected for further detection.Tumor necrosis factor alpha (TNF-α), interleukin (IL)- 1β and IL-17A expression levels in serum were detected by conducting enzyme-linked immunosorbent assay (ELISA). The localization expressions of tight junction protein Occludin was detected by immunohistochemical staining (IHC), while the localization expression of tight junction protein zonula occluden-1 (ZO-1) were detected by immunofluorescence staining (IF). The protein expression of Occludin, ZO-1, and Lysozyme were detected by performing Western blot.The mRNA expression of Lysozyme and α-defensin were detected by real-time PCR (qPCR).Results:ELISA results proved that the levels of serum TNF-α and IL-1β in HRS group rats were significantly lower than those in model group [(62.02±29.97) ng/L vs.(113.40±44.58) ng/L, (21.68±0.35) ng/L vs.(28.29±3.49) ng/L], while the level of IL-17A increased [(28.18±5.28) ng/L vs. (15.10±3.60) ng/L] (all P<0.05). IHC staining: compared with model group, the expression of Occludin in HRS group was uniform and continuous, and the staining was darker.IF results: compared with model group, the fluorescence signal intensity of ZO-1 in HRS group rats significantly increased, and the distribution was clear and continuous.Wes-tern blot results: compared with model group, the expression levels of Occludin and ZO-1 proteins in HRS group rats remarkably increased (0.79±0.06 vs. 0.54±0.04, 0.91±0.11 vs. 0.51±0.13), while Lysozyme protein decreased (1.50±0.40 vs. 2.99±0.80) (all P<0.05). qPCR results revealed that the expression level of Lysozyme mRNA in HRS group rats was lower than that in model group (1.64±0.33 vs. 2.20±0.40), while α-defensin mRNA obviously increased (0.82±0.19 vs. 0.47±0.13) (all P<0.01). Conclusions:HRS protects intestinal mucosal barrier by inhibiting the expression of tight junctions and the secretion of antimicrobial peptides in rat suffering from IIRI.

Objective: To explore the clinical manifestations, diagnosis, treatment and prognosis of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) . Methods: A retrospective study identified 91 patients diagnosed with ALCAPA at Shanghai Children′s Medical Center from March 2010 to August 2017. According to the left ventricular ejection fraction (LVEF) at the time of consultation, patients were divided into the cardiac insufficiency group (n=54) and the normal cardiac function group (n=37). Clinical features (age of onset, clinical performance, etc) and auxiliary examinations (electrocardiogram, echocardiography, etc) between the two groups were compared using a t-test and a Chi-square test. Prognostic factors were analyzed by an ordered logistic regression and a Pearson correlation coefficient. Results: (1) The age of diagnosis of patients in the cardiac insufficiency group who were usually misdiagnosed as cardiomyopathy was (10.0±2.6) months (20/54) , whereas the age of diagnosis of patients in the normal cardiac function group who were usually misdiagnosed as valvular diseases was (40.0±7.8) months (4/37). According to the pathophysiological mechanism, forty of the 54 (74%) patients in the cardiac insufficiency group were infantile type, and 78% patients (29/37) in the normal cardiac function group were adult type. (2) Preoperative electrocardiogram showed the deep Q wave in lead I occurred more frequently in the cardiac insufficiency group than in the normal cardiac function group (28/54 vs. 11/37, χ²=4.388, P=0.036). (3) Twenty patients died in the cardiac insufficiency group including 12 patients who died from postoperative cardiac pump failure and 8 children who did not undergo surgery due to poor prognosis and died from other reasons. There was no death in the normal cardiac function group. (4) Preoperative LVEF was the unique risk factor affecting prognosis (F=16.872, P=0.005). The preoperative LVEF was significantly lower than the postoperative LVEF ((37±11)% vs. (45±14)%, t=3.614, P=0.001) in the cardiac insufficiency group. During the follow-up period, 6 patients in the cardiac insufficiency group still presented with postoperative cardiac dysfunction, and the patients in the normal cardiac function group still had normal cardiac function. Conclusions Preoperative LVEF was the unique risk factor affecting prognosis of ALCAPA. Patients with infantile type ALCAPA and preoperative cardiac insufficiency should receive long-term follow-up treatment.

Objective: To describe the clinical characteristics, treatment and outcomes of Staphylococcus aureus infective endocarditis (SAIE) in children. Methods: One hundred and fifty-one children with infective endocarditis (IE) and treated in Shanghai Children′s Medical Center of Shanghai Jiaotong University School of Medicine were retrospectively analyzed, from January 2007 to December 2017.There were 88 boys and 63 girls, the age of IE onset ranged from 1 month to 17 years [(6.27±4.80) years]. There were 23 cases (15.2%) with infection of staphyloco-ccus aureus, 128 cases with non-Staphylococcus aureus infective endocarditis (NSAIE). The clinical characteristics, treatment and prognosis of patients between the SAIE group and the NSAIE group were analyzed and compared. Results: Among the SAIE group, there were 15 boys and 8 girls, the age of IE onset ranged from 3 months to 16 years [(6.2±5.6) years]; 10 cases of them had no underlying heart disease.There was no significant difference in gender, age and previous cardiac surgery between the SAIE group and the NSAIE group (all P>0.05). The number of patients without underlying heart disease in the SAIE group was more than that in the NSAIE group, and the difference was statistically significant[43.5%(10/23 cases) vs.20.3%(26/128 cases), χ²=5.762, P=0.016]. Compared with the NSAIE group, the SAIE group was associated with significantly higher levels of C-reactive protein [60.0(128.0) mg/L vs.25.0(58.0) mg/L, Z=-2.033, P=0.042], higher erythrocyte sedimentation rate [(59.3±43.2) mm/h vs.(39.4±31.5) mm/h, t=-2.283, P=0.024], and lower levels of serum albumin [30.1(12.7) g/L vs.34.3(8.4) g/L, Z=-2.282, P=0.022]. The proportion of severe sepsis in the SAIE group was also significantly higher than that in the NSAIE group [21.7%(5/23 cases) vs.7.0%(9/128 cases), Z=5.014, P=0.045]. Twelve cases (52.2%) of the SAIE group had systemic embolic events, including cerebral events (6 cases), pulmonary (2 cases), limbs (1 case), splenic (1 case), limbs and mesenteric (1 case), limbs and cerebral events (1 case). Eighteen cases in the SAIE group were treated with antibiotics combined with surgery and 1 case died, while 5 cases were treated with antibiotics alone, 4 cases died and 1 case abandoned.The SAIE patients had a significantly higher systemic embolic rate [52.2%(12/23 cases) vs.20.3%(26/128 cases), χ²=7.978, P=0.016], neurological events rate [30.4%(7/23 cases) vs.10.9%(14/128 cases), χ²=4.669, P=0.031] and in-hospital mortality [21.7%(5/23 cases) vs.6.3%(8/128 cases), χ²=4.139, P=0.042] than those in the NSAIE group. Conclusions SAIE was most common in those without heart disease.Compared with NSAIE, SAIE is characterized by a higher prevalence of severe sepsis, major neurological events, and inpatient mortality.Timely surgery is recommended in these cases, when possible, before the occurrence of complications.

Objective To explore the clinical manifestations, diagnosis, treatment and prognosis of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Methods A retrospective study identified 91 patients diagnosed with ALCAPA at Shanghai Children′s Medical Center from March 2010 to August 2017. According to the left ventricular ejection fraction (LVEF) at the time of consultation, patients were divided into the cardiac insufficiency group (n=54) and the normal cardiac function group (n=37). Clinical features (age of onset, clinical performance, etc) and auxiliary examinations (electrocardiogram, echocardiography, etc) between the two groups were compared using a t‐test and a Chi‐square test. Prognostic factors were analyzed by an ordered logistic regression and a Pearson correlation coefficient. Results (1) The age of diagnosis of patients in the cardiac insufficiency group who were usually misdiagnosed as cardiomyopathy was (10.0±2.6) months (20/54), whereas the age of diagnosis of patients in the normal cardiac function group who were usually misdiagnosed as valvular diseases was (40.0 ± 7.8) months (4/37). According to the pathophysiological mechanism, forty of the 54 (74%) patients in the cardiac insufficiency group were infantile type, and 78% patients (29/37) in the normal cardiac function group were adult type. (2) Preoperative electrocardiogram showed the deep Q wave in lead I occurred more frequently in the cardiac insufficiency group than in the normal cardiac function group (28/54 vs. 11/37, χ2=4.388, P=0.036). (3) Twenty patients died in the cardiac insufficiency group including 12 patients who died from postoperative cardiac pump failure and 8 children who did not undergo surgery due to poor prognosis and died from other reasons. There was no death in the normal cardiac function group. (4) Preoperative LVEF was the unique risk factor affecting prognosis (F=16.872, P=0.005). The preoperative LVEF was significantly lower than the postoperative LVEF ((37±11)% vs. (45±14)%, t=3.614, P=0.001) in the cardiac insufficiency group. During the follow‐up period, 6 patients in the cardiac insufficiency group still presented with postoperative cardiac dysfunction, and the patients in the normal cardiac function group still had normal cardiac function. Conclusions Preoperative LVEF was the unique risk factor affecting prognosis of ALCAPA. Patients with infantile type ALCAPA and preoperative cardiac insufficiency should receive long‐term follow‐up treatment.

Objective To explore the clinical manifestation, treatment and prognosis of anomalous left coronary artery from the pulmonary artery (ALCAPA). Method The clinical data of patients with ALCAPA from January 2011 to September 2016 were retrospectively analyzed. All the patients were divided into infant group (<12 months) and child group (≥12 months). Results In the 62 patients (38 infants and 24 children) with ALCAPA, the median ΔLVDD (actually measured LVDD – the normal upper limit of LVDD in this age group) of infant group and child group was 13.6 (8.4～17.5) mm and 8.5 (3.3～13.7) mm respectively. The mean LVEF of the infant group was 40.6±2.4 ％, lower than that of the children group (59.0±2.9) ％, and the difference was statistically significant (P<0.01). Sixty children were treated with surgery. The mean cardiopulmonary bypass duration of infant group (137.1±8.4 min) was longer than that of the children group (105.9±6.6 min), and the difference was statistically significant (P=0.010). The median mechanical ventilation time after operation in infant group (6 d, 3～7 d), was longer than that in the children group (2 d, 1～4 d), and the difference was statistically significant (P<0.01). The median follow-up time of 60 patients was 38 months. Six patients died within 1 month after surgery (5 cases in the infant group and 1 in the children group), and there were no deaths more than 1 month after surgery. In 22 cases, the flow rate of pulmonary valve increased rapidly within 1 week after operation (>1.8 m/s). Conclusion ALCAPA is easily misdiagnosed as dilated cardiomyopathy. The clinical manifestations of infants were more severe than those of children. The mortality was high within 1 month after surgery, and the long-term prognosis was better.

Objective To evaluate the diagnostic value of invasive cardiac catheterization for restrictive cardiomyopathy (RCM) and constrictive pericarditis (CP).Methods Twenty-seven children with CP or RCM hospitalized in Department of Cardiology,Shanghai Children's Medical Center,School of Medicine,Shanghai Jiaotong University from February 2002 to December 2015,were selected,including 10 patients who had been surgically documented CP and 17 patients with RCM who underwent cardiac catheterization.Intracardiac pressure waveforms were recorded and all the measurement indexes of pressure of all the patients were analyzed.The changes of cardiac pressure under deep breathing in 9 patients with local anesthesia was recorded.Results There were significant differences in pulmonary artery pressure,difference between left ventricular end-diastolic pressure (LVEDP) and right ventricular end-diastolic pressure (RVEDP),and the ratio of RVEDP/right ventricular systolic pressure between group RCM and group CP [(50.2 ± 12.0) mmHg(1 mmHg =0.133 kPa) vs.(38.1 ±6.8) mmHg,(7.8±5.5) mmHgvs.(1.8 ±4.7) mmHg,0.27 ±0.10vs.0.45 ± 0.20,respectively;t =2.912,2.787,2.418,all P ＜ 0.05].However,there was overlapping for these criteria,and the predictive sensitivity of any of the criteria was less than 66.7％.In patients with CP,reciprocal changes in the filling between right ventricle and left ventricle occurred during respiration.In patients with RCM,the right ventricle and left ventricle pressures moved concordantly with respiration.The systolic area index was greater in group CP than that in group RCM (1.20 ± 0.03 vs.0.70 ± 0.14),and the difference was significant (t =6.152,P ＜ 0.01).The systolic area index had a sensitivity of 100.0％ and a predictive accuracy of 100.0％ for the identification of patients with surgically proven CP.Conclusions Measurements in catheterization,especially the ratio of right ventricular to left ventricular systolic area during inspiration and expiration is a reliable catheterization criterion for differentiating CP from RCM.