Objective To assess the effect of lung protective ventilation on outcome of children with acute respiratory distress syndrome(ARDS).Methods Between January 1999 and December 2007,43 children with ARDS were enrolled from PICU of Shanghai Children's Medical Center and assigned to the protective-ventilation group(group A) or the conventional-ventilation group(group B).The patients in group A (from January 2004 to December 2007)received lower tidal volume(6～7 ml/kg) and high levels of positive end-expiratory pressure(PEEP),and optimal oxygenation was achieved by adjusting FiO2 and PEEP.The patients in group B(from January 1999 to December 2003) received relatively higher tidal volume(8～12 mL/kg) with lower PEEP(2～6 cm H2O),and optimal oxygenation was achieved by adjusting FiO2.Tidal volume,PEEP,arterial blood gas,mortality and the number of ventilator-free days were compared between the two groups.Results Since protective ventilation was adopted after 2004,tidal volume was significantly lower in group A[(7.09±1.66)ml/kg]as compared with that in group B[(9.82±2.31) ml/kg](P=0.001).PEEP was significantly higher in group A[(7.15±2.08) cm H2O]as compared with that of group B[(5.40 + 1.84) cm H2O](P=0.021).The mortality was 30.3% in group A and 60.0% in group B.The number of ventilator-free days were(10.88±8.84) d in group A and(8.40±10.86) d in group B.Although mortality was lower and number of ventilator-free days was greater in group A,no significant differences were found between the two groups(P＞0.05).Conclusion Lung protective ventilation may improve the outcome for pediatric patients with ARDS,however,larger trials are required before a definite conclusion can be reached.

Objective Severe sepsis, especially complicated multiple organ dysfunction syndrome (MODS) ,remains a serious problem in pediatric intensive care unit (PICU). This study aims to explore the clinical characteristics of sepsis and the cause of death for septic patients. Methods A cohort of severe sepsis admitted to PICU between Jan 2008 and Dec 2008 was enrolled in the study. Forty six variables were included in the univariate analysis as potential risk factors for mortality in severe sepsis, followed by logistic regression analysis. Results Forty five children were enrolled with a mortality of 32. 1%. The risk factors of mortality were assessed using the PRISM Ⅲ score ( OR 1. 502;95% CI 1. 131 ～ 1.995) and maximum platelet count during hospitalization ( OR 0. 991 ;95% CI 0.982 ～ 1.000). The mortality of severe sepsis complicated with 1,2,3,4 or more organ dysfunction was 10.0%, 11.1%, 44. 4% and 68. 8 %, respectively ( P ＜ 0. 001 ). The organ dysfunction most frequently appeared in the cardiovascular system (75.6%) and respiratory system (66.7%). In severely septic children with MODS, respiratory failure (OR 23. 179; 95% CI 2.095 ～256. 522) and renal failure( OR 9. 637 ;95% CI 1. 698 ～ 54. 703) were the main risk factors of death. Conclusion The risk factors of death in severe sepsis were PRISM Ⅲ score and maximum platelet count during hospitalization. Severe sepsis combined with MODS had a poor prognosis, with a positive correlation between the number of dysfunctional organs and mortality rate. Respiratory and renal failure rate was associated with death in severe sepsis with MODS.

Objective To determine the changes of the host's inflammatory response throughout the course of sepsis secondary to pneumonia by evaluating the serum levels of interleukin (IL)-6 and IL-10.Methods Sixty-two patients who were diagnosed with pneumonia and hospitalized in PICU from Sep 2008 to Mar 2009 were enrolled in this study.They were divided into 3 groups:pneumonia group (n =31 ),sepsis group (n =20) and severe sepsis group (n =11 ).The serum levels of IL-6 and Il-10 were measured by enzyme-linked immunosorbent assay at the day 1 and day 4 after admitted to PICU.Results The pediatric risk of score mortality Ⅲ scores and fatality rate were significantly higher in severe sepsis group compared to the other two groups (P ＜ 0.05).The levels of IL-6 were (55.68 ± 61.41 ) pg/ml,(57.46 ± 96.56) pg/ml,and ( 114.86 ± 206.37) pg/ml in pneumonia group,sepsis group and severe sepsis group at day 1 of PICU,with no difference among 3 groups.However,the levels of IL-10 were ( 59.50 ± 57.97 ) pg/ml,( 41.27 ±28.37) pg/ml,and(20.05 ±9.14) pg/ml at day 1 in 3 groups,which showed a significant difference(P ＜0.05).The ratio of IL-6 to IL-10 were 1.51 ±2.42,1.48 ±2.50,and 14.47 ±26.97 at day 1 in 3 groups.There was a significant differrence among 3 groups ( P ＜ 0.05 ).No difference was found among 3 groups at day 4.Conclusion IL-10 may be an determinant factor of the severity and prognosis of the sepsis,and measurement of IL-10 or the ratio of IL-6 to IL-10 can be used to evaluate the severity and prognosis of sepsis.

ObjectiveTo identify mutations ofGATA4 andGATA6 genes in children with isolated congenital atrial septal defect (ASD).Methods From November 2012 to November 2013, 101 patients with ASD (99 unrelated patients and one twin) who were submitted to catheter-based intervention and 100 ethnicity-matched children without congenital heart disease, blood disorders and chromosomal abnormalities were enrolled. The blood was collected. The coding regions and lfanking regions of theGATA4 andGATA6 genes were ampliifed by polymerase chain reaction and sequenced using the dideoxvnucleotide chain termination technique, and then compared with the normal sequence in the Genbank.Results Two novel heterozygous missense GATA6mutations, c. G145A and c. G151A, were identiifed in 2 unrelated ASD patients, which were not present in the controls. These two mutations predicted the conversion of glycine into serine at amino acid residue 49 (G49S) and glutamate into lysine at amino acid residue 52 (K52E). A heterozygous missenseGATA6 mutation c.43 G>C, which caused a conversion from glycine to arginine, was found in 9 ASD patients and 7 controls. A single nucleotide polymorphism c.99G>T, which did not cause amino acid conversion inGATA4 gene, was found.ConclusionsGATA6 gene is an important transcription factor in heart development. The mutation ofGATA6 gene may cause the change of its transcriptional activity, and lead to ASD.

GATA6 transcription factor belongs to the GATA family and contains 2 conserved zinc ifnger DNA binding domains. GATA6 not only presents in embryonic tissues but also found in heart, lung and pancreas and is essential for the maintenance of their function.The present review focuses on the critical roles of GATA6 in heart development and atrial septal defect to provide theoretical basis for diagnosis and treatment of atrial septal defect.

The clinical data of 2 children with early graft liver dysfunction (EAD) admitted to the Pediatric Intensive Care Unit, Shanghai Children′s Medical Center, Shanghai Jiaotong University School of Medicine were retrospectively analyzed to discussed the therapeutic significance of non-biological artificial liver technology, such as intermittent plasma exchange (PE) combined with continuous veno-venous hemodiafiltration (CVVHDF) in children with EAD.Case 1 was suffering from biliary atresia, and case 2 was suffering from Niemann-Pick disease.Graft liver dysfunction and multiple organ dysfunction occurred in 2 children after liver transplantation.PE and CVVHDF were initiated early in the first two days after liver transplantation.After one-week therapy with intermittent PE plus CVVHDF, acute multiple organ dysfunction were reversed with liver function remarkably improved in the 2 cases.Therefore non-biological artificial liver technique can be tried after liver transplantation in children.This technique contributes to the recovery of liver function and can improve the secondary multi-organ insufficiency.

Objective:To explore the role and safety of endomyocardial biopsy (EMB) in the diagnosis of pediatric myocardial disease.Methods:Demographic, clinical and histopathological data of all children receiving EMB in Shanghai Children′s Medical Center, Shanghai Jiaotong University School of Medicine between January 2016 and August 2020 were collected.The pathological results and the procedure-related complications were retrospectively analyzed.Results:A total of 22 patients with the mean age of (10.2±3.1) years underwent EMB.Among them, 13 cases (59.1%) underwent right ventricular EMB, 5 cases (22.7%) underwent left ventricular EMB, and 4 cases (18.2%) underwent biventricular EMB.Among the 12 patients with clinically suspected myocarditis or unexplained heart failure, 4 cases were diagnosed with lymphocytic myocarditis, 2 cases were dilated cardiomyopathy, and 1 case was inflammatory cardiomyopathy.Seven patients presented ventricular diastolic dysfunction, including 6 cases of restrictive cardiomyopathy and 1 of constrictive pericarditis.Three patients exhibited hypertrophic cardiomyopathy with pre-excitation syndrome, involving 2 cases were diagnosed with glycogen storage cardiomyopathy.EMB was successfully performed in all patients.No patient died, and procedure-related complications were not reported.Conclusions:EMB assists the diagnosis and treatment of pediatric patients with selected myocardial disease, which is relatively safe with less complications if performed by experienced interventionalists in qualified pediatric cardiovascular medical centers.

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 analyze the progression of children with severe coronary artery lesions due to Kawasaki disease by coronary artery angiography,and evaluate the diagnostic value of echocardiography in these children.Methods·A retrospective analysis was performed to enroll children with Kawasaki disease whose coronary artery lesions were graded Ⅳ or above from Shanghai Children's Medical Center,Shanghai Jiao Tong University School of Medicine,from January 2013 to January 2023.The subjects were required to have received at least 2 times of coronary angiogram,and their clinical and imaging data were collected to analyze the progression of the lesions.Echocardiography results were compared with the results of the coronary angiogram.Results·A total of 21 children were included,including 15 males and 6 females,with a median age at onset of 3 years and 6 months,a median age at initial coronary angiography of 7 years and 11 months,a median interval of 4 years and 5 months between the time of onset and initial angiography,a median age at angiographic review of 9 years and 2 months,and a median interval of 1 year and 3 months between the time of initial angiography and review.Coronary stenosis or occlusion was detected in 13 children in the initial angiography,of whom 6 underwent coronary artery bypass grafting(CABG)and had their angiography reviews 1 year later.The review results showed that the bridging vessels were unobstructed and no obvious stenosis was observed.Fifteen children had progression of the lesions detected by echocardiography in the subsequent follow-up and had their angiogram reviews,of whom 8 had significant progression of the coronary lesions.Intracoronary balloon dilatation was performed in 1 case,and CABG was performed in another case.Sixteen lesions of coronary stenosis or occlusion were detected in the initial angiography in 21 children,while only 1 lesion of coronary stenosis was detected by echocardiography during the same period of time.Twenty-eight medium-to large-sized coronary aneurysms were detected in the initial angiography in the 21 children,and the diameters of the 28 aneurysms measured by echocardiography and coronary angiogram were subjected to the Bland-Altman analysis.The Bland-Altman analysis showed that the difference in maximum diameter between 2 methods was(1.63±2.33)mm,with 95％CI of-2.95-6.21 mm.Conclusion·Coronary artery lesions due to Kawasaki disease may be progressive;in the children with severe lesions,coronary artery stenosis or occlusion may be missed or misdiagnosed and some errors may exist in the measurement of diameters of aneurysms by echocardiography.Regular review of coronary angiography is needed.

Objective: To investigate the incidence and prognosis of tricuspid regurgitation after transcatheter closure of perimembranous ventricular septal defects in pediatric patients as well as the risk factors of regurgitation occurrence or aggravation. Methods: Clinical data of 1 108 patients who underwent percutaneous closure in Shanghai Children′s Medical Center, School of Medicine, Shanghai Jiaotong University from January 2011 to January 2017 was analyzed retrospectively, and the prognosis and risk factors of postoperative tricuspid regurgitation and aggravation were also analyzed. Results: Mild tricuspid regurgitation occurred in 24 cases after surgery with the incidence of 2.1%.Among 5 cases with mild or above preoperative tricuspid regurgitation, the regurgitation was alleviated after surgery in 4 cases.No severe tricuspid regurgitation requiring surgical intervention occurred in any patient during follow-up.Univariate analysis showed that intervention time (P<0.05) and residual shunt (P<0.05) were risk factors for mild or above tricuspid regurgitation after intervention.Binary regression analysis indicated that the size of the occluder (mm) (OR=1.48, 95%CI: 1.13-1.90) and residual shunt (OR=6.53, 95%CI: 1.69-25.30) were risk factors for tricuspid regurgitation after intervention (all P<0.05). Conclusions There is a certain incidence of tricuspid regurgitation after transcatheter closure of perimembranous ventricular septal defects, but most tricuspid regurgitation do not need surgical intervention.The intervention time, size of occluder and residual shunt are risk factors of intraoperatively or postoperatively tricuspid regurgitation.