Syncope is a common emergency in children and adolescents, which has serious influence on the quality of life.After nearly 30 years′ exploration, great progress has been made in the diagnosis and treatment of children′s syncope in China.We have established the diagnosis procedure of syncope in line with health economics.We have also proposed the concept of individualized precise treatment of syncope in children for the first time.The disease spectrum of syncope in children has been continuously enriched.Relevant progresses have been cited by the guidelines of the United States, Europe and Canada, which has also been unanimously recognized by international experts.

Postural orthostatic tachycardia syndrome (POTS) is a common condition that results in syncope among children and adolescents.It primarily manifests as a range of chronic orthostatic intolerance symptoms, accompanied by an abnormal increase in heart rate upon standing, significantly impacting the learning and quality of life in young individuals.POTS demonstrates diversity and heterogeneity in clinical symptoms, underlying pathophysiological mechanisms, and associated complications.Consequently, relying on experience to treatment often fails to achieve desired clinical outcomes.Delving deeper into potential clinical biomarkers capable of predicting POTS treatment efficacy and implementing individualized treatment approaches are crucial for enhancing patient prognosis and quality of life.This review provided a comprehensive exploration of the latest research findings on biomarkers for predicting POTS clinical outcomes, aiming to offer perspectives and approaches for the clinical treatment of pediatric POTS.

Objective:To investigate the regulatory effects of endogenous nitric oxide (NO) on the activity of superoxide dismutase-1 (SOD1) and apoptosis of human umbilical vein endothelial cells (HUVECs).Methods:HUVECs were taken as the research object.The endothelial NO synthase (eNOS) short hairpin RNA(shRNA) lentivirus was employed to transfect HUVECs to knock down eNOS.HUVECs were divided in 4 groups: the scramble group, the eNOS shRNA group, the eNOS shRNA + sodium nitroprusside(SNP) group and the eNOS shRNA+ SNP+ tris (2-carboxyethyl) phosphine hydrochloride (TCEP) group.The protein expressions of eNOS and SOD1 dimer/monomer in cells were detected by western blot.The activity of SOD was detected by the enzyme-linked immunosorbent assay.The NO content in cells was detected with NO fluorescence probe.The level of superoxide anion in HUVECs was detected with dihydropyridine (DHE). The terminal deoxynucleotidyl transferase (TdT) dUTP nick-end labeling (TUNEL) assay was adopted to detect the apoptosis of HUVECs in situ.Results:Compared with the scramble group, the endogenous NO content (2.690±0.420 vs.15.029±2.193, P<0.01), eNOS protein expression (1.000±0.778 vs.3.141±0.199, P<0.01), SOD1 dimer/monomer ratio (4.6±1.0 vs.7.6±2.0, P<0.05) and SOD activity [(0.432±0.254) Carmen′s unit/10 4 cell vs.(1.000±0.116) Carmen′s unit/10 4 cell, P<0.01] were significantly decreased, while the level of intracellular superoxide anion (11.180±1.560 vs.6.146±1.007, P<0.01) and HUVECs apoptosis [75.0 (55.0, 100.0)% vs.0 (0, 0)%, P<0.01] were significantly increased in the eNOS shRNA group.Compared with the eNOS shRNA group, the content of endogenous NO (16.705±0.116 vs.2.690±0.420, P<0.01), the ratio of SOD1 dimer/monomer (7.3±2.0 vs.4.6±1.0, P<0.05) and the activity of SOD [(0.737±0.060) Carmen′s unit/10 4 cell vs.(0.432±0.254) Carmen′s unit/10 4 cell, P<0.05] were significantly increased, while the level of superoxide anion (6.897±1.648 vs.11.180±1.560, P<0.01) and the HUVECs apoptosis [0 (0, 0)% vs.75.0 (55.0, 100.0)%, P<0.01] were significantly decreased in the eNOS shRNA+ SNP group.Compared with the eNOS shRNA + SNP group, the ratio of SOD1 dimer/monomer (4.4±0.9 vs.7.3±2.0, P<0.05) and the activity of SOD [(0.214±0.084) Carmen′s unit/10 4 cell vs.(0.737±0.060) Carmen′s unit/10 4 cell, P<0.01] were significantly decreased, while the level of superoxide anion (10.917±1.552 vs.6.897±1.640, P<0.01) and the apoptosis level of HUVECs[63.6 (55.0, 90.0)% vs.0 (0, 0)%, P<0.01] were significantly increased in the eNOS shRNA+ SNP+ TCEP group.However, there was no significant difference in the NO content (16.112±0.926 vs.16.705±0.116, P>0.05). Conclusions:Endogenous NO could effectively antagonize the apoptosis of endothelial cells by increasing the cysteine-dependent SOD1 dimer/monomer ratio, enhancing SOD activity and inhibiting the accumulation of reactive oxygen species.

Objective:To explore the significance of plasma homocysteine (Hcy) as a new biomarker for the differential diagnosis of postural tachycardia syndrome (POTS) and suspected myocarditis in children.Methods:A total of 24 children diagnosed with POTS and 21 children diagnosed with suspected myocarditis treated in the Pediatrics Department of the Peking University First Hospital from July to December 2016 were included in the study.Plasma Hcy levels were measured in each subject and compared between children with POTS and suspected myocarditis.The receiver operating characteristic (ROC) curves were depicted for assessing the diagnostic potential of Hcy in distinguishing POTS from suspected myocarditis.Results:Plasma Hcy level in the POTS group was significantly higher than that in the suspected myocarditis group [(14.25±8.09) μmol/L vs.(8.99±3.19) μmol/L], which was also significantly higher than that of the mean levels in Beijing children [(8.82±5.58) μmol/L] (all P<0.05). When the cut-off was 9.36 μmol/L, the area under the ROC curve was 0.76, and the sensitivity and specificity for distinguishing POTS from suspected myocarditis were 71% and 68%, respectively. Conclusions:Plasma Hcy levels are helpful in the differential diagnosis of POTS and suspected myocarditis in children.

Objective:To understand the current status of nurses' awareness and needs for the Employee Assistance Program (EAP), as to provide basis for the hospital to formulate EAP.Methods:Using the purpose sampling method, 352 ICU nurses from the Nanjing Drum Tower Hospital Affiliated of Nanjing University Medical School were selected as the research subjects from December 2019 to January 2020. Finally, 350 valid questionnaires were obtained. Use the EAP demand questionnaire to investigate the ICU nurses' awareness and demand for EAP.Results:Among the 350 nurses in the intensive unit, 214 cases (61.14%) had never understood EAP, only 8 (2.29%) had understood and experienced the EAP, and 332 cases (94.86%) believed that the hospital′s implementation of EAP was a benefit provided to nurses. The top two service requirements were: "Relief of work pressure" and "mental health consultation", and hope to receive mixed-mode EAP services; in ICU and CCU, nurses hope EAP can help resolve work-family conflicts; in EICU, they need to provide Specific job skills training services.Conclusions:ICU nurses have insufficient cognition of EAP and urgently need to obtain EAP implementation knowledge; at the same time, it is necessary to formulate an implementation plan that meets ICU nurses′ needs for EAP, that is, to relieve work pressure and carry out psychological counseling to ensure that EAP is launch successfully in ICU nurses.

Objective:To investigate the relationship between orthostatic intolerance (OI) and body mass index (BMI), blood lipid and serum protein levels in children and adolescents.Methods:A total of 122 children and adolescents aged from 6 to 17 years old, who were diagnosed with OI at the Department of Pediatric Cardiology, the Second Hospital of Lanzhou University from April 2018 to April 2019, were selected as the subjects.While, 56 children and adolescents in the health management center were selected as the healthy control group during the same period.Subjects were divided into syncope group and non-syncope group according to whether there was syncope in clinical history.The height and body mass of all children were measured, and venous blood were taken to detect blood lipids and serum protein in the morning.Date analysis were conducted with SPSS 22.0 software.Results:(1) The level of triglyceride in the OI group was lower than that in the healthy control group[(0.98±0.45) mmol/L vs. (1.28±1.04) mmol/L], and there was statistically significant( t=2.025, P<0.05); the BMI were respectively (17.56±3.23) kg/m 2 and (16.46±2.58) kg/m 2 in syncope group and non-syncope group, whose result indicated that the BMI in syncope group was higher than that in non-syncope group( t=2.085, P<0.05). (2) The results of binary Logistic regression analysis showed that the triglyceride level was an independent risk factor for OI( OR=0.504, 95% CI: 0.272-0.931, P<0.05). (3) The receiver operating characteristic curve evaluated the predictive value of triacylgly-cerol to OI.Results showed the sensitivity and specificity of OI were respectively 72.1% and 48.2%when the triacylglycerol was 1.09 mmol/L. Conclusions:Low triglyceride level and high BMI may be susceptible factors to OI in children and adolescents.Therefore, the diet of children with OI should be highly valued by clinicians and parents.

Objective:To explore the genetic etiologies of newborn deaths.Methods:A total of 98 newborns who were recruited to the Neonatal Genome Project of the Children′s Hospital of Fudan University and died in the hospital from January 2018 to August 2020 were enrolled in this study. The genetic information and the interventions based on the genetic findings were retrospectively analyzed. T-test, Mann-Whitney U test, Chi square test and Fisher′s exact probability test were used to compare the demographic features and clinical characteristics between the patients with or without a genetic finding. Results:Among 98 newborns (55 males and 43 females), there were 63 preterm and 35 term infants, with a gestational age of (33±5) weeks, a birth weight of (2 107±975) g and the age at death of 12 (2,34) days. Sixteen (16%)patients were identified with genetic variants, including 11 with single nucleotide variants, 4 with copy number variants and 1 with both single nucleotide variant and copy number variant. The detected single nucleotide variants were spanning 12 genes, among which 3 were multiple disorders-related, 2 metabolic disorder-related, 2 hematological disorder-related, 2 respiratory disorder-related, 2 cardiovascular disorder-related and 1 skeletal disorder-related. The patients with a positive genetic finding had significant differences in the birth weight ((2 605±940) vs. (2 009±957) g, t=2.283, P=0.025), the gestational age ((36±5) vs. (33±5) weeks, t=2.131, P=0.036), the age at death ((37 (5, 69) vs. 11 (2, 29) days, Ζ=-2.245, P=0.025) and the history of asphyxia at birth (1/16 vs. 46% (38/82), P=0.002)when compared to those without a genetic finding. In addition, the genetic etiology rates of patients who were born term or with a birth weight ≥ 2 000 g were significantly higher than those who were born preterm (29% (10/35) vs. 10% (6/63), P=0.022) or with a birth weight<2 000 g (25% (13/51) vs. 7% (3/46), χ 2=5.016, P=0.025), respectively. Six cases were medically actionable based on the genetic findings and the treatments included special diet, applying specific medicine, hematopoietic stem cell transplantation and lung transplantation. Conclusions:Genetic etiologies are not rare in newborn deaths and mainly associated with metabolic disorder, multi-system disorders, hematological disorder, respiratory disorder, cardiovascular disorder and skeletal disorder. Some findings are medically actionable, based on which the specific treatments could be scheduled timely. A genetic etiology should be investigated in newborn deaths especially in those who are term birth or with a birth weight ≥2 000 g or without a history of asphyxia at birth.

Objective:To explore the clinical characteristics, plasma levels of hydrogen sulfide(H 2S) and the relationship between the genotype and phenotype of cardiovascular involvement in children with methylmalonic acidemia and homocystinemia. Methods:The clinical and laboratory data of 66 outpatients diagnosed with methylmalonic acidemia combined with homocystinemia in Department of Pediatrics, Peking University First Hospital from January 2014 to July 2014 were collected and analyzed respectively, and the patients were divided into 2 groups: cardiovascular involvement group (10 cases) and non-cardiovascular involvement group(56 cases). The differences in the clinical characteristics, plasma levels of H 2S and genotypes were compared between 2 groups. Results:(1) There were 45 cases of early-onset children under 1 year old, including 4 cases of cardiovascular system involvement and 41 cases of non-cardiovascular system involvement.Twenty-one cases had onset above 1 year old, including 6 cases of cardiovascular system involvement and 15 cases of non-cardiovascular system involvement. There were 44 male children, including 8 cases with cardiovascular system involvement and 36 cases without cardiovascular system involvement; 22 cases female children, including 2 cases with cardiovascular system involvement and 20 cases without cardiovascular system involvement. There was no significant difference in onset age and gender distribution between the 2 groups ( χ2=2.910, 0.368, all P>0.05). (2)In the 10 cases with cardiovascular involvement, there were 3 cases with hypertension, 2 cases with hypertension combined with pulmonary hypertension, 2 cases with mild myocardial hypertrophy, 1 case with atrial septal defect combined with pulmonary hypertension, 1 case with pulmonary hypertension, 1 case with myocardial noncompaction.Compared with the non-cardiovascular involvement group, the proportion of kidney involvement was increased and that of nervous system was decreased in cardiovascular system involvement group( χ2=20.34, 5.79, all P<0.05), the proportion of hematological system involvement between the 2 groups had no significant differences ( χ2=1.28, P>0.05). The plasma levels of hydrogen sulfide of children with cardiovascular involvement was significantly lower than that of non-cardiovascular involvement[(33.8±3.6) μmol/L vs.(39.3±5.2) μmol/L, t=-3.22, P<0.01]. (3) MMACHC gene mutation (cblC type) was identified in all 46 patients.It was found that the most common type of gene mutation was c. 80A>G in cardiovascular involvement group, while c. 609G>A was the most common type of gene mutation in non-cardiovascular involvement group. Conclusions:The clinical manifestations of children with methylmalonic acidemia and homocystinemia involving cardiovascular system are multiple and prone to multiple system involvement, especially renal involvement.A decrease in plasma hydrogen sulfide levels may be involved in the involvement of its cardiovascular system.The MMACHC gene c. 80A>G mutation is the most common genetic mutation site in children with cardiovascular involvement with methylmalonic acidemia and homocystinemia.