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.

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 evaluated the clinical optimization of treatment strategies for the disease and risk factors for prognosis.Methods:The records of 34 patients with scimitar syndrome evaluated at our medical center between Jan 2013 and Dec 2018 were reviewed, including clinical data, treatment outcome and follow-up.Results:Scimitar syndrome was identified in 34 patients, 16 males and 18 females, aged (21.16±33.19) months. The mean length of follow-up after diagnosis was (50.30±26.99) months. Compared with adult form patients, patients presenting less than 1 year of age had a higher incidence of pulmonary hypertension and pulmonary veins stenosis. 8 patients didn’t require surgical treatment after interventional coil embolization of aortopulmonary collaterals or other interventional maneuvers in associated congenital heart diseases. 15 patients had scimitar vein surgery, 4 patients had postoperative pulmonary vein obstruction that occurred with similar frequency after baffle or reimplantation procedures( P=0.569), and tended to be more common in patients had preoperative obstruction of scimitar vein( P=0.009). Overall, 7(20.5%) of 34 died. High risk factors for death included age at diagnosis( P=0.000), obvious pulmonary hypertension( P=0.007) and pulmonary vein stenosis( P=0.014). Conclusion:Patients with pulmonary artery systolic pressure near normal and absence of congenital heart disease excluding atrial septal defect do not require surgery. Postoperative pulmonary vein obstruction is related with scimitar vein stenosis before surgery regardless of redirection technique. Age at diagnosis, obvious pulmonary hypertension and pulmonary vein stenosis are risk factors for death in patients with scimitar syndrome.

Objective:This study intends to explore the clinical outcomes of liposuction combined with a vacuum-assisted biopsy device in the treatment of Simon grade Ⅰ or Ⅱ gynecomastia (GYN) caused by glandular proliferation and excrescent fat deposition.Methods:The clinical data of Simon grade Ⅰ or Ⅱ GYN caused by glandular proliferation and excrescent fat deposition treated by liposuction and vacuum-assisted biopsy device in the Department of Plastic and Reconstructive Surgery of the Ninth People’s Hospital Affiliated to Medical College of Shanghai Jiao Tong University from June 2016 to June 2019 were analyzed retrospectively. Surgical procedures: A 5-mm skin incision was made at the lower outer margin of the breast, through which the excrescent fat deposition was removed by liposuction. In addition, a vacuum-assisted biopsy device was applied to remove the residual glandular tissue. The operation time, liposuction volume, removed glandular tissue weight, blood loss, drainage volume, drainage duration, patient satisfaction, and complications were observed and recorded. The patients were followed up from June to December 2020. Patients were asked to grade the cosmetic outcome between 0 and 5 (0 was extremely dissatisfied, 5 was extremely satisfied). The data were sorted out and analyzed by SPSS 19.0, and the data were expressed as Mean±SD or M ( P25, P75). Results:A total of 27 patients with 51 breasts were enrolled in this study. Unilateral operation time, liposuction volume, removed glandular tissue weight, blood loss, drainage volume, drainage duration were (45.3±11.1) min, (206.0±66.7) ml, (28.9±9.5) g, (10.0±4.3) ml, (78.8±33.9) ml and (2.3±0.4) d respectively. Bruising occurred in 9 breasts. No other complications were observed. The postoperative follow-up time was 21.0 (17.0, 28.0) months, and the score of patient satisfaction was 4.98±0.14.Conclusions:Liposuction combined with vacuum-assisted biopsy device can be used as a feasible and minimally invasive approach to treat the Simon grade Ⅰ or Ⅱ GYN caused by glandular proliferation and excrescent fat deposition, which is safe and reliable with few complications, excellent cosmetic results, and high patient satisfaction. However, it has some shortcomings, such as hemostasis under the indirect vision.

Objective:This study intends to explore the clinical outcomes of liposuction combined with a vacuum-assisted biopsy device in the treatment of Simon grade Ⅰ or Ⅱ gynecomastia (GYN) caused by glandular proliferation and excrescent fat deposition.Methods:The clinical data of Simon grade Ⅰ or Ⅱ GYN caused by glandular proliferation and excrescent fat deposition treated by liposuction and vacuum-assisted biopsy device in the Department of Plastic and Reconstructive Surgery of the Ninth People’s Hospital Affiliated to Medical College of Shanghai Jiao Tong University from June 2016 to June 2019 were analyzed retrospectively. Surgical procedures: A 5-mm skin incision was made at the lower outer margin of the breast, through which the excrescent fat deposition was removed by liposuction. In addition, a vacuum-assisted biopsy device was applied to remove the residual glandular tissue. The operation time, liposuction volume, removed glandular tissue weight, blood loss, drainage volume, drainage duration, patient satisfaction, and complications were observed and recorded. The patients were followed up from June to December 2020. Patients were asked to grade the cosmetic outcome between 0 and 5 (0 was extremely dissatisfied, 5 was extremely satisfied). The data were sorted out and analyzed by SPSS 19.0, and the data were expressed as Mean±SD or M ( P25, P75). Results:A total of 27 patients with 51 breasts were enrolled in this study. Unilateral operation time, liposuction volume, removed glandular tissue weight, blood loss, drainage volume, drainage duration were (45.3±11.1) min, (206.0±66.7) ml, (28.9±9.5) g, (10.0±4.3) ml, (78.8±33.9) ml and (2.3±0.4) d respectively. Bruising occurred in 9 breasts. No other complications were observed. The postoperative follow-up time was 21.0 (17.0, 28.0) months, and the score of patient satisfaction was 4.98±0.14.Conclusions:Liposuction combined with vacuum-assisted biopsy device can be used as a feasible and minimally invasive approach to treat the Simon grade Ⅰ or Ⅱ GYN caused by glandular proliferation and excrescent fat deposition, which is safe and reliable with few complications, excellent cosmetic results, and high patient satisfaction. However, it has some shortcomings, such as hemostasis under the indirect vision.

Objective:To establish a novel decision tree-based algorithm in complete Kawasaki disease(cKD)and evaluate its diagnostic value in incomplete Kawasaki disease(iKD)and pediatric infectious disease(IF)with common clinical characteristics, which facilitates early and accurate diagnosis of iKD.Methods:Based on inclusion criteria of KD and IF, clinical and laboratory data of patients with cKD, iKD and IF from Shanghai Children′s Medical Center between December 2018 and December 2019 were collected.The training data set included cKD and random half number of IF patients, and validation data was constituted with iKD and the rest of IF patients.The decision tree algorithm analysis was performed in training data set to generate a clinical diagnostic panel for cKD.Finally, the decision tree-based algorithm was verified and evaluated among the iKD patients.Results:A single statistical analysis was performed on 26 examination indexes of constructing decision tree-based algorithm.It was found that 16 examination indexes were obviously different between cKD and IF patients, and 17 examination indexes were significantly different between iKD and IF patients.According to date set of cKD and IF patients, the decision tree-based algorithm was established.The erythrocyte sedimentation rate>35mm/h, N-terminal atrial brain natriuretic peptide precursor≥315 pg/ml, CD3 -/CD19 + %≥21%, and the amount of neutrophil≥8.5×10 9/L were constructed as key elements.The algorithm had a sensitivity of 0.947 and a specificity of 0.963, and correctly classified subjects with iKD who were difficult to be distinguished from patients with IF. Conclusion:A decision tree-based algorithm based on the examination indexes of cKD is one of the effective methods to identify iKD and IF, which provides strong support for the early clinical diagnosis of iKD.

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.