Altered brain structure in preschool-aged children with tetralogy of Fallot.

BACKGROUND: Neurodevelopmental abnormalities are prevalent in children with tetralogy of Fallot. Our aim was to investigate the structural brain alterations of preschool-aged children with tetralogy of Fallot and its correlation with neurodevelopmental outcome. METHODS: T1-weighted structural images were obtained from 25 children with tetralogy of Fallot who had undergone cardiopulmonary bypass surgery and from 24 normal controls. Cortical morphological indices including gray matter volume, cortical thickness, sulcal depth, gyrification, and cortical surface complexity were compared between the two groups. Neurodevelopmental assessments of the children with tetralogy of Fallot were performed with the Wechsler Preschool and Primary Scale of Intelligence. RESULTS: Cortical morphological differences between groups were distributed throughout the right caudal middle frontal gyrus, right fusiform gyrus, right lateral occipital gyrus, right precuneus, and left inferior parietal lobule. Among children with tetralogy of Fallot, altered cortical structures were correlated with the visual spatial index, working memory index, and perioperative variables. CONCLUSION: Our results suggested that abnormal cortical structure in preschool-aged children with tetralogy of Fallot may be the persistent consequence of delayed cortical development in fetuses and cortical morphology can be used as an early potential biomarker to capture regional brain abnormalities that are relevant to neurodevelopmental outcomes. IMPACT: Altered cortical structures in preschool-aged children with ToF were correlated with both neurodevelopmental outcomes and clinical risk factors. Cortical morphology can be used as an effective tool to evaluate neuroanatomical changes and detect underlying neural mechanisms in ToF patients. Abnormal cortical structure may be the continuous consequence of delayed fetal brain development in children with ToF.

Study on the protective effect of RNA-binding motif protein 3 in mild hypothermia oxygen-glucose deprivation/reoxygenation cell model.

Mild hypothermia is proven neuroprotective in clinical practice. While hypothermia leads to the decrease of global protein synthesis rate, it upregulates a small subset of protein including RNA-binding motif protein 3 (RBM3). In this study, we treated mouse neuroblastoma cells (N2a) with mild hypothermia before oxygen-glucose deprivation/reoxygenation (OGD/R) and discovered the decrease of apoptosis rate, down-regulation of apoptosis-associated protein and enhancement of cell viability. Overexpression of RBM3 via plasmid exerted similar effect while silencing RBM3 by siRNAs partially reversed the protective effect exerted by mild hypothermia pretreatment. The protein level of Reticulon 3(RTN3), a downstream gene of RBM3, also increased after mild hypothermia pretreatment. Silencing RTN3 weakened the protective effect of mild hypothermia pretreatment or RBM3 overexpression. Also, the protein level of autophagy gene LC3B increased after OGD/R or RBM3 overexpression while silencing RTN3 decreased this trend. Furthermore, immunofluorescence observed enhanced fluorescence signal of LC3B and RTN3 as well as a large number of overlaps after RBM3 overexpressing. In conclusion, RBM3 plays a cellular protective role by regulating apoptosis and viability via its downstream gene RTN3 in the hypothermia OGD/R cell model and autophagy may participate in it.

Perioperative Brain Injury in Children with Aortic Arch Anomalies: A Retrospective Study of Risk Factors and Outcomes.

Complex pediatric cardiac disease is associated with brain impairment and neurodevelopmental disorders, particularly in patients requiring cardiac surgery for aortic arch anomalies. This study examines the incidence, risk factors, and outcomes of perioperative brain injury in children undergoing aortic arch repair who had aortic arch anomalies. A total of 145 children with aortic arch anomalies in our center undergoing aortic arch repair between January 2014 and December 2022 were enrolled. There were 129 (89.0%) with coarctation of the aorta (COA) and 16 (9.7%) with interrupted aortic arch (IAA). Risk factor analysis of brain injuries was done using perioperative imaging and included symptoms of hemorrhagic stroke, arterial ischemic stroke, white matter injury, cerebral sinus venous thrombosis, and other pathologies. Preoperatively, 50/145 (34.5%) patients had brain injuries. Multivariate analysis showed that an increased risk of hemorrhagic stroke was associated with newborns (odds ratio [OR], 2.09 [95% CI 0.08-3.50]), isolated COA (OR, 3.69 [95% CI 1.23-7.07]), mechanical ventilation (MV) ([OR, 2.56 [95% CI 1.25-4.03]), and sepsis (OR, 1.73 [95% CI 0.46-3.22]). Newborns ([OR, 1.91 [95% Cl 0.58-3.29]) and weight-for-age z score ([OR, -0.45 [95% CI -0.88 to -0.1]) were associated with an increased risk of white matter injury. New postoperative brain injuries were present in 12.9% of the patients (16/124). Deep hypothermic circulatory arrest (DHCA) was associated with new postoperative brain injuries compared with deep hypothermic low-flow (DHLF) plus antegrade cerebral perfusion (ACP) (([OR, 2.67 [95% CI, 0.58-5.75])). Isolated COA was almost associated with new postoperative brain injuries (OR, 1.13 [95% CI, -0.04 to 2.32]). Children diagnosed with isolated COA appeared to have a higher risk of perioperative brain injury, but the underlying mechanisms are still unclear. We focused on the intrinsic mechanism by which changes in hemodynamics caused by COA result in perioperative brain injury. Further research will be needed to optimize the personalized treatment and cerebral perfusion techniques for complex pediatric cardiac surgery.

Verification of genetic differences and immune cell infiltration subtypes in the neuroblastoma tumour microenvironment during immunotherapy.

BACKGROUND: Improved understanding of the tumour microenvironment (TME) has enabled remarkable advancements in research on cancer progression in the past few years. It is crucial to understand the nature and function of the TME because precise treatment strategies, including immunotherapy, for managing specific cancers have received widespread attention. The immune infiltrative profiles of neuroblastoma (NB) have not yet been completely illustrated. The purpose of this research was to analyse tumour immune cell infiltration (ICI) in the microenvironment of NB. METHODS: We applied the CIBERSORT and ESTIMATE algorithms to evaluate the ICI status of 438 NB samples. Three ICI models were selected, and ICI scores were acquired. Subgroups with high ICI scores determined based on the presence of immune activation signalling pathways had better overall survival. RESULTS: Genes involved in the immunosuppressive heparan sulphate glycosaminoglycan biosynthesis signalling pathway were markedly enriched in the low ICI score subgroup. It was inferred that patients with high ICI NB subtypes were more likely to respond to immunotherapy and have a better prognosis than those of patients with low ICI NB subtypes. CONCLUSION: Notably, our ICI data not only provide a new clinical and theoretical basis for mining NB prognostic markers related to the microenvironment but also offer new ideas for the development of NB precision immunotherapy methods.

Exposure to perchlorate, nitrate and thiocyanate was associated with the prevalence of cardiovascular diseases.

AIMS: To determine the association between urinary levels of perchlorate, nitrate and thiocyanate, and the prevalence of cardiovascular diseases (CVD) among general population. METHODS: A total of 16, 570 participants were enrolled from the National Health and Nutrition Examination Surveys (NHANES). Urinary levels of perchlorate, nitrate and thiocyanate were measured using ion chromatography coupled with electrospray tandem mass spectrometry. Multivariable linear regressions and logistic regressions were performed to explore the associations of exposure to perchlorate, nitrate and thiocyanate, and the prevalence of total and specific CVD, including chronic heart failure (CHF), coronary heart disease (CHD), angina, heart failure and stroke. Restricted cubic splines were used to explore the nonlinearity. RESULTS: Participants with CVD had a lower urinary level of nitrate and thiocyanate (all P < 0.001). A null association between urinary perchlorate and total CVD or specific CVD was observed. Comparing with the lowest quartile, the highest quartile of urinary nitrate was independently associated with a decreased presence of total CVD (odds ratio [OR] 0.66, 95% confidence interval [CI] [0.53, 0.82]), CHF (OR 0.48, 95% CI [0.33, 0.71]), and stroke (OR 0.63, 95%CI [0.45, 0.88]). In addition, per one-fold increasement of urinary nitrate decreased a 0.15-fold prevalence of total CVD, 0.29-fold prevalence of CHF, and 0.16-fold prevalence of stroke. However, for urinary thiocyanate, we found that the 2nd and 3rd quartile were associated with total CVD, the 2nd quartile associated with heart attack, and the 2nd, 3rd and 4th quartile associated with stroke. What's more, restricted cubic splines confirmed that the relation between urinary nitrate and CVD was linear (P for nonlinearity = 0.242) and the inverse relation between urinary thiocyanate and CVD was nonlinear (P for nonlinearity < 0.001). CONCLUSION: In the general population, low levels of nitrate were linearly while thiocyanate were nonlinearly associated with an increased presence of cardiovascular diseases.

The effect of selective cerebral perfusion on cerebral versus somatic tissue oxygenation during aortic coarctation repair in neonates and infants.

BACKGROUND: Suboptimal tissue perfusion and oxygenation may be the root cause of certain perioperative complications in neonates and infants having complicated aortic coarctation repair. Practical, effective, and real-time monitoring of organ perfusion and/or tissue oxygenation may provide early warning of end-organ mal-perfusion. METHODS: Neonates/infants who were scheduled for aortic coarctation repair with cardiopulmonary bypass (CPB) and selective cerebral perfusion (SCP) from January 2015 to February 2017 in Children's Hospital of Nanjing Medical University participated in this prospective observational study. Cerebral and somatic tissue oxygen saturation (SctO2 and SstO2) were monitored on the forehead and at the thoracolumbar paraspinal region, respectively. SctO2 and SstO2 were recorded at different time points (baseline, skin incision, CPB start, SCP start, SCP end, aortic opening, CPB end, and surgery end). SctO2 and SstO2 were correlated with mean arterial pressure (MAP) and partial pressure of arterial blood carbon dioxide (PaCO2). RESULTS: Data of 21 patients were analyzed (age=75±67 days, body weight=4.4±1.0 kg). SstO2 was significantly lower than SctO2 before aortic opening and significantly higher than SctO2 after aortic opening. SstO2 correlated with leg MAP when the measurements during SCP were (r=0.67, p<0.0001) and were not included (r=0.46, p<0.0001); in contrast, SctO2 correlated with arm MAP only when the measurements during SCP were excluded (r=0.14, p=0.08 vs. r=0.66, p<0.0001). SCP also confounded SctO2/SstO2's correlation with PaCO2; when the measurements during SCP were excluded, SctO2 positively correlated with PaCO2 (r=0.65, p<0.0001), while SstO2 negatively correlated with PaCO2 (r=-0.53, p<0.0001). CONCLUSIONS: SctO2 and SstO2 have distinct patterns of changes before and after aortic opening during neonate/infant aortic coarctation repair. SctO2/SstO2's correlations with MAP and PaCO2 are confounded by SCP. The outcome impact of combined SctO2/SstO2 monitoring remains to be studied.

Preoperative thyroid hormone levels predict ICU mortality after cardiopulmonary bypass in congenital heart disease patients younger than 3 months old.

BACKGROUND: We aimed to study the effectiveness of preoperative thyroid hormone levels in predicting intensive care unit (ICU) mortality after cardiopulmonary bypass (CPB) in infants with congenital heart disease (CHD). METHODS: We retrospectively reviewed and analyzed data from 133 patients younger than 3 months old who underwent cardiac surgery with CPB from June 2017 to November 2019. ICU mortality prediction was assessed by multivariate binary logistic regression analysis and area under the curve (AUC) analysis. RESULTS: Non-survivors were younger (17.46 ± 17.10 days vs. 38.63 ± 26.87 days, P = 0.006), with a higher proportion of neonates (9/13 vs. 41/120, P = 0.017) and a higher proportion of individuals with a Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1) score ≥ 4 (8/13 vs. 31/120, P = 0.020). No significant difference was found in CPB and aortic cross-clamping (ACC) time. The levels of free triiodothyronine (FT3) (3.91 ± 0.99 pmol/L vs. 5.11 ± 1.55 pmol/L, P = 0.007) and total triiodothyronine (TT3) (1.55 ± 0.35 nmol/L vs. 1.90 ± 0.57 nmol/L, P = 0.032) were higher in survivors than in non-survivors. In the ICU mortality prediction assessment, FT3 was an independent mortality predictor and showed a high AUC (0.856 ± 0.040). CONCLUSIONS: The preoperative FT3 level was a powerful and independent predictor of ICU mortality after CPB in infants with CHD younger than 3 months old.

Endoscopic forceps removal for complicated magnetic beads impaction.

A 2-year-old girl was transferred to our center after she was witnessed swallowing a magnetic toy two days previously. An abdominal x-ray at a community hospital showed multiple foreign bodies in the gastric region and close observation was advised. However, radiography the next day showed the magnets' persistence in situ.

Sex differences in the link between blood cobalt concentrations and insulin resistance in adults without diabetes.

BACKGROUND: Little is known about the effects of environmental cobalt exposure on insulin resistance (IR) in the general adult population. We investigated the association between cobalt concentration and IR. METHODS: A total of 1281 subjects aged more than 20 years with complete blood cobalt data were identified from the National Health and Nutrition Examination Survey (NHANES) 2015-2016 cycle. Blood cobalt levels were analyzed for their association with IR among all populations and subgroups by sex. Regression coefficients and 95% confidence intervals (CIs) of blood cobalt concentrations in association with fasting glucose, insulin and homeostatic model assessment of insulin resistance (HOMA-IR) were estimated using multivariate linear regression after adjusting for age, sex, ethnicity, alcohol consumption, body mass index, education level, and household income. A multivariate generalized linear regression analysis was further carried out to explore the association between cobalt exposure and IR. RESULTS: A negative association between blood cobalt concentration (coefficient = - 0.125, 95% CI - 0.234, - 0.015; P = 0.026) and HOMA-IR in female adults in the age- and sex-adjusted model was observed. However, no associations with HOMA-IR, fasting glucose, or insulin were found in the overall population. In the generalized linear models, participants with the lowest cobalt levels had a 2.74% (95% CI 0.04%, 5.50%) increase in HOMA-IR (P for trend = 0.031) compared with subjects with the highest cobalt levels. Restricted cubic spline regression suggested that a non-linear relationship may exist between blood cobalt and HOMA-IR. CONCLUSIONS: These results provide epidemiological evidence that low levels of blood cobalt are negatively associated with HOMA-IR in female adults.

Cytochrome P450 Epoxygenase 2J2 Protects Against Lung Ischemia/Reperfusion Injury by Activating the P13K/Akt/GSK-3-ß/NF-kB Signaling Pathway During Deep Hypothermic Low Flow in Mice.

BACKGROUND: Cytochrome P450 epoxygenase 2J2 (CYP2J2) metabolizes arachidonic acid to epoxyeicosatrienoic acids, which exert anti-inflammatory effects and alleviate oxidative stress in the cardiovascular system. Our previous work revealed that CYP2J2 is expressed in pulmonary artery endothelial cells. It was therefore hypothesized that CYP2J2 overexpression may prevent lung ischemia/reperfusion injury (LIRI) in 3-week-old C57BL/6 mice during deep hypothermic low flow (DHLF). This study aimed to establish whether CYP2J2 protects against LIRI and the mechanisms of CYP2J2 overexpression during DHLF in mice. The aim of this study was to explore the effects of DHLF on lung tissue in mice and to find out the regularity of this process, so as to provide theoretical data for lung tissue protection in children undergoing this process in clinic. METHODS: A 3-week-old C57BL/6 mouse model was used to mimic LIRI conditions during DHLF by clamping the left pulmonary artery and left main bronchus for 120 min, followed by reperfusion for 2 h. The body temperature of the mice was maintained between 18°C and 19°C to induce DHLF. RESULTS: During DHLF, lung ischemia/reperfusion increased the left lung wet/dry weight, the left lung weight/body weight ratio, the protein concentration in bronchoalveolar lavage fluid, and the concentration of proinflammatory mediators in the lungs, including interleukin (IL)-1, IL-8, and necrosis factor (NF)-α, and decreased the concentration of the anti-inflammatory mediator IL-10. Furthermore, activation of NF-κB p65 and degradation of IKBα were remarkably increased in lung tissues after ischemia/reperfusion. The CYP2J2 overexpression group showed the opposite results (P < 0.05), and p-Akt1 and p-GSK-3ß expression were significantly higher in the CYP2J2 overexpression group (P < 0.05). Moreover, the changes in IL-1, IL-8, tumor necrosis factor-α, IL-10, p-Akt1, p-GSK-3ß, NF-κB p65, and IKBα were reversed in the Akt1 gene heterozygous knockout group, and lung damage was significantly higher in the Akt1 gene heterozygous knockout group than in the CYP2J2 overexpression group. CYP2J2 overexpression can protect against LIRI, whereas Akt1 gene heterozygous knockout in mice can abolish this protective effect. CONCLUSIONS: CYP2J2 overexpression can protect against LIRI by activating the P13K/Akt/GSK-3ß/NF-kB signaling pathway during DHLF. Thus, changing CYP2J2 expression can be a novel strategy for the prevention and treatment of LIRI during DHLF.

Clinical Treatment of Pediatric Primary Cardiac Tumors: A Single-Institute 12-Year Experience.

BACKGROUND: Primary cardiac tumors in children are extremely rare. This study aimed to analyze clinical treatment and follow-up of pediatric primary cardiac tumors. PATIENTS AND METHODS: We performed a retrospective analysis by searching the medical records of 75 patients diagnosed with pediatric primary cardiac tumors from June 2005 to August 2017 in our institution. We followed operative patients every half year in the first postoperative year and then at least every year. If the patients had no serious symptoms or hemodynamic changes, they received nonoperative management and were followed regularly every year. RESULTS: Nineteen patients underwent surgery at our department for serious symptoms and critical hemodynamic changes. Four patients had postoperative complications. Two died of low cardiac output syndrome and arrhythmia after surgery. One patient with myxomas had tumor recurrence and one had been found of another rhabdomyoma after surgery. The other 14 patients recovered well. Fifty-six patients had nonoperative management. Four were lost in follow-up. Two patients with malignant tumors died of unknown causes after discharge. The remaining patients had no severe symptoms or tumor growth during follow-up. CONCLUSIONS: Clinical treatment of pediatric primary cardiac tumors should be performed individually. Most pediatric primary cardiac tumors are benign, and spontaneous regression is possible, especially for rhabdomyomas. The principle purpose of surgical treatment is to restore normal hemodynamics and protect important structures and cardiac tissue.

Serum 25-hydroxyvitamin D is associated with obesity and metabolic parameters in US children.

OBJECTIVE: To explore the relationships of serum 25-hydroxyvitamin D (25(OH)D) with obesity and metabolic parameters in US children. DESIGN: Cross-sectional analysis. We evaluated the associations between serum 25(OH)D and multiple measurements of adiposity, serum lipid concentrations, fasting glucose and insulin resistance in children aged 6-18 years with adjustments for multiple covariates. SETTING: The National Health and Nutrition Examination Survey, 2001-2006. PARTICIPANTS: A nationally representative sample of 6311 children and adolescents aged 6-18 years. RESULTS: Among US children and adolescents, the prevalence of vitamin D deficiency has been especially high in older children, girls and the non-Hispanic Black population. Higher odds of obesity were found at a 25(OH)D concentration of <30 nmol/l (deficiency) than at >50 nmol/l under both criteria for obesity in children (OR = 3·27, Ptrend ≤ 0.001). Moreover, increased odds of having abnormal HDL-cholesterol (OR = 1·71, Ptrend ≤ 0.001) and impaired insulin resistance (OR = 4·15, Ptrend ≤ 0·001) were found for children deficient in 25(OH)D compared with those with normal 25(OH)D concentrations. When the children and adolescents were stratified by gender, we found stronger associations between serum 25(OH)D concentration and both HDL-cholesterol and insulin resistance in girls. No association of 25(OH)D with any other metabolic parameter was found. CONCLUSIONS: Our results suggest a potential harmful association between low serum 25(OH)D concentration and the risk of obesity among children. However, the underlying mechanisms require further investigation.

Effects of Balanced Ultrafiltration During Extracorporeal Circulation for Children with Congenital Heart Disease on Postoperative Serum Inflammatory Response.

Objectives: We studied inflammatory marker changes with ultrafiltration extracorporeal techniques and compared these levels to those occurring during conventional techniques. Methods: Seventy-four children undergoing extracorporeal circulation during congenital heart surgery were divided into two groups-conventional (control) and balanced ultrafiltrations (study). Serum interleukin-6 (IL-6), tumor necrosis factor-α (TNF-α), and soluble intercellular adhesion molecule-1 (sICAM-1), static lung compliance (Cstat), alveolar-arterial oxygen partial pressure difference (A-aDO2), and oxygenation index (OI) were measured. Results: Thirty minutes after extracorporeal circulation (T1), IL-6 and TNF-α levels significantly increased (p < .05), which plummeted 3 h after surgery (T2). The levels in the study group were lower (p < .05). sICAM-1 levels at T1 and T2 significantly exceeded those at T0, and study group had lower levels (p < .05). At T1, Cstat and OI dropped (p < .05), which increased at T2 (p < .05), and study group had less decreases (p < .05). A-aDO2 at T1 surpassed that before intervention (p < .05), and study group had less increase (p < .05). Conclusion: Balanced ultrafiltration exerts protective effects on children with congenital heart disease undergoing extracorporeal circulation.

Exploring the associations of serum concentrations of PCBs, PCDDs, and PCDFs with walking speed in the U.S. general population: Beyond standard linear models.

Studies have shown that persistent organic pollutants (POPs) can have various health effects. However, little is known about the effects of multiple chemicals with possible common sources of exposure on walking speed, a proxy index reflecting lower limb neuromuscular function and physical function. We simultaneously applied multiple linear and nonlinear statistical models to explore the complex exposure-response relationship between a mixture of 22 selected POPs and walking speed. A total of 14 polychlorinated biphenyls (PCBs), 3 polychlorinated dibenzo-p-dioxins (PCDDs), and 5 polychlorinated dibenzofurans (PCDFs) were measured in the serum of participants in the National Health and Nutrition Examination Survey (NHANES) from 1999 to 2002. Walking speed was measured during a physical examination. Linear regression (LR), least absolute shrinkage and selection operator (LASSO), and group LASSO were used to evaluate the linearity of mixtures, while restricted cubic spline (RCS) regression, random forest (RF), and Bayesian kernel machine regression (BKMR) models were used to evaluate the nonlinearity of mixtures. Potential confounders were adjusted in the above models. A total of 436 subjects were included in our final analysis. The results of the LR model did not identify any POP exposure that was significantly associated with walking speed. The LASSO results revealed an inverse association of one PCDD congener and two PCDF congeners with walking speed, while the group LASSO analysis identified PCDFs at the exposure level and at the group level. In the RCS analysis, two PCB congeners presented significant overall associations with walking speed. The PCB congener PCB194 showed statistically significant effects on the outcome (P = 0.01) when a permutation-based RF was used. The BKMR analysis suggested that PCBs and PCDFs (probabilities = 0.887 and 0.909, respectively) are potentially associated with walking speed. Complex statistical models, such as RCS regression, RF and BKMR models, can detect the nonlinear and nonadditive relationships between PCBs and walking speed, while LASSO and group LASSO can identify only the linear relationships between PCDFs and walking speed. Fully considering the influence of collinearity in each method during modelling can increase the comprehensiveness and reliability of conclusions in studies of multiple chemicals.

Feasibility and efficacy of home rectal irrigation in neonates and early infancy with Hirschsprung disease.

PURPOSE: A single-stage pull-through (SSPT) is the most commonly performed procedure for Hirschsprung disease (HSCR) and has been shown to be better than multi-stage procedures. However, performing a SSPT in the neonatal period or early in infancy is a risk factor for an inaccurate diagnosis, post-operative enterocolitis, and a protracted post-operative recovery. The present study was primarily designed to evaluate the feasibility and efficacy of home rectal irrigation in the neonatal period and early in infancy, followed by a delayed and planned SSPT in a prospective cohort with HSCR. METHODS: Between January 2014 and December 2016, a total of 147 neonates diagnosed with HSCR were enrolled in the study. Six patients were excluded as a result of ganglion cells found in second rectal biopsies after the neonatal period. One hundred twenty-two patients successfully underwent 2-4 months of home rectal irrigation during the neonatal period, followed by a SSPT procedure after the neonatal period (group A, n = 122). Nineteen patients were not candidates for home rectal irrigation, and thus, colostomies were performed during the neonatal period followed by multi-stage procedures after the neonatal period (group B, n = 19). One hundred twenty-two healthy children, age- and gender-matched to group A were enrolled as the healthy control group for assessment of nutrition status (group C, n = 122). The birth weight, gender ratio, aganglionic segment, age, and Hirschsprung-associated enterocolitis (HAEC) score at the time of HSCR diagnosis were measured to evaluate the feasibility of home rectal irrigation in neonates and early in infancy. The nutritional indices, including weight, body length, serum albumin, serum prealbumin, serum retinol-binding protein, and incidence of HAEC after 2-4 successful home rectal irrigation, were used to assess the efficacy of home rectal irrigation. Anastomotic strictures or leakage, perianal excoriation, frequency of defecation, and morbidity of post-operative HAEC were recorded to evaluate the beneficial effects to pull through (PT), which were facilitated by home rectal irrigation. RESULTS: Higher HAEC scores and older age at the time of diagnosis of HSCR were associated with group B, compared to group A (4.34 ± 1.25 vs. 11.0 ± 2.56 [t = 18.20, p < 0.05] and 2.8 ± 1.46 days vs. 12.1 ± 5.3 days [t = 16.10, p < 0.05], respectively). The ratio of rectosigmoid HSCR to non-rectosigmoid HSCR was higher in group A than group B (104/18 vs. 4/15 [χ2 = 34.29, p < 0.05]). There were no differences in birth weight, weight at the time of diagnosis of HSCR, and gender ratio between groups A and B. There were no differences in birth weight, birth length, post-home rectal irrigation age, post-home rectal irrigation weight, post-home rectal irrigation length, and post-home rectal irrigation serum albumin between groups A and C (3.47 ± 0.42 kg vs. 3.48 ± 0.40 kg [t = 0.10, p > 0.05], 50.02 ± 0.49 cm vs. 50.05 ± 0.46 cm [t = 0.61, p > 0.05], 98.59 ± 13.34 days vs. 97.83 ± 13.58 days [t = 0.44, p > 0.05], 6.77 ± 0.66 kg vs. 6.97 ± 0.87 kg [t = 1.95, p > 0.05], 61.55 ± 2.14 cm vs. 61.70 ± 2.07 cm [t = 0.59, p > 0.05], and 41.78 ± 2.42 g/L vs. 41.85 ± 2.37 g/L [t = 0.22, p > 0.05], respectively). The rate of HAEC in the period of home rectal irrigation in group A was low; however, the post-home rectal irrigation serum prealbumin level and retinol-binding protein were significantly lower in group A than group C (0.15 ± 0.04 g/L vs. 0.17 ± 0.05 g/L [t = 3.50, p < 0.05] and 22.51 ± 7.53 g/L vs. 30.57 ± 9.26 g/L [t = 7.46, p < 0.05], respectively). There were no anastomotic strictures or leakage after definitive PT performed in group A. The frequency of defecation ranged from 2-6 times per day, 10 patients had perianal excoriation 3 months after PT, and 11 patients had post-operative HAEC during 6 months of follow-up after PT. CONCLUSION: Home rectal irrigation in neonates and early in infancy, followed by a delayed and planned SSPT is feasible and effective in patients with HSCR, and could be beneficial to definitive PT. However, for patients with an extended aganglionic segment, older age, or high HAEC score at the time of diagnosis of HSCR, rectal irrigation maybe not suitable. TRIAL REGISTRATION: This was a prospective comparative study designed to evaluate the effects of home rectal irrigation for facilitating and enhancing recovery after PT, and was registered at Clinical Trials.gov as NCT02776176.

[Research progress on transcription factors and signal pathways involved in congenital esophageal atresia].

Congenital esophageal atresia is one of the serious birth defects. Identifying the etiology and mechanism of esophageal development can provide clues for the effective prevention and treatment of congenital esophageal atresia. Recent studies have shown that a variety of transcription factors and signaling pathways (including Wnt signaling pathway, bone morphogenetic protein signaling pathway, SHH signaling pathway, vascular endothelial growth factor signaling pathway) are involved in esophageal proliferation, differentiation and other processes, promoting the normal development of esophagus. Understanding the regulatory mechanisms during the normal development of esophagus can give reference for the prevention and treatment of congenital esophageal atresia. This article reviews the research progress on related transcription factors and signaling pathways in esophageal development.

[Application of thoracoscopic surgery in repairing esophageal atresia type â ¢ with tracheoesophageal fistula in neonates].

OBJECTIVE: To compare the efficacy of thoracoscopic surgery versus thoracotomy in repairing esophageal atresia type Ⅲ with tracheoesophageal fistula (EA/TEF) in neonates. METHODS: A retrospective analysis was conducted in 97 neonates who underwent EA/TEF repair between January 2012 and December 2017 in the Affiliated Children's Hospital of Nanjing Medical University, including 75 patients receiving thoracotomy and 22 patients receiving thoracoscopic surgery. The perioperative data and the incidence of early postoperative complications were compared between two groups. RESULTS: The operations were completed in all patients. One child (4.5%) in thoracoscopic surgery group was converted to thoracotomy with modified Livaditis procedure due to the long distance of two blind ends (>4 cm) and thinner distal end. The operation time was longer in thoracoscopic surgery group[(143±48) min vs. (120±40) min, P<0.05], but the postoperative ventilation time was shorter[(55±22) h vs. (65±19) h, P<0.05] and the first oral feeding was earlier in thoracoscopic surgery group[(3.2±1.1) d vs (3.9±1.3) d, P<0.05]. No statistical difference was observed in the ratio of red blood cell transfusion, length of hospital stay and drainage tube indwelling time between two groups (all P>0.05). The incidence of lung complications in thoracotomy group was higher than that in thoracoscopic surgery group (20.0% vs. 9.1%, P<0.01), while there were no significant differences in the incidence of other postoperative complications between two groups. There was no death in thoracoscopic surgery group, while 2 patients died in thoracotomy group. CONCLUSIONS: Thoracoscopic repair is a preferred surgical procedure for EA/TEF in neonates.

Molecular and cellular insights into Zika virus-related neuropathies.

Zika virus (ZIKV), a relatively elusive Aedes mosquito-transmitted flavivirus, had been brought into spotlight until recent widespread outbreaks accompanied by unexpectedly severe clinical neuropathies, including fetal microcephaly and Guillain-Barré syndrome (GBS) in the adult. In this review, we focus on the underlying cellular and molecular mechanisms by which vertically transmitted microorganisms reach the fetus and trigger neuropathies.

Environmental factors in the etiology of isolated and nonisolated esophageal atresia in a Chinese population: A case-control study.

BACKGROUND: Esophageal atresia (EA) is a common birth defect that occurs with tracheoesophageal fistula (TEF), although etiological studies on EA/TEF have produced inconsistent results. METHODS: The aim of this study was to examine the association between environmental factors during pregnancy and the risk of EA/TEF in a Chinese population. Cases of isolated EA and nonisolated EA and unaffected controls were identified between July 2005 and November 2015, and face-to-face questionnaires concerning exposure to environmental factors were administered to the birth mothers of 130 cases and 400 controls. The adjusted odds ratio (OR) and 95% confidence interval (CI) were calculated to assess the association between environmental factors and the risk of EA/TEF. RESULTS: The results of this case-control study suggest that lower maternal education (p < 0.0001), maternal binge drinking (OR = 2.63; 95% CI, 1.05-6.6) and pickled food consumption (OR = 2.04; 95% CI, 1.31-3.71) during pregnancy increase the risk of EA in offspring, while maternal folic acid supplementation (OR = 0.45; 95% CI, 0.29-0.71) is significantly associated with a decreased risk of EA. CONCLUSION: These results suggest a role for environmental exposures in the etiology of EA/TEF; however, further studies are needed to replicate the observed associations. Birth Defects Research (Part A) 106:840-846, 2016. © 2016 Wiley Periodicals, Inc.