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Primary lung cancer in childhood is extremely rare, with an incidence rate of less than 2/100,0000, and pulmonary mucoepidermoid carcinoma (PMEC), is even rarer. Their symptoms are usually not specific, and there are no guidelines for their management, which makes their clinical management a challenge for pediatricians. The purpose of this report is to discuss the clinical presentation, positive signs, examinations, pathological characteristics, surgical modalities, chemotherapy regimens, and prognosis in children. The clinical data of four patients diagnosed with PMEC at the Children's Hospital of Chongqing Medical University from June 2021 to November 2022 were retrospectively analyzed, and their clinical features, treatment, and prognosis were summarized. Among them, two were male and two were female; their ages ranged from 3 years and 10 months to 10 years and 11 months, and all were staged according to tumor node metastasis classification (TNM). Immunohistochemical tests were performed in all children, among which four cases were positive for cytokeratin (CK), two cases were positive for CK7, four cases were positive for p63, about 5–10% of tumor cells were positive for Ki67. Among the four children, three had surgery alone and one had surgery + chemotherapy. All four children are presently living, with no evidence of tumor recurrence or metastasis. PMEC in children is very rare, and its age of onset and symptoms are not specific, and there is no obvious correlation with gender. Its diagnosis mainly relies on pathomorphological diagnosis, and immunohistochemical detection has no specific performance. The prognosis of children with PMEC is related to the clinical stage and whether surgery is performed. Whether further chemotherapy or radiotherapy is needed for patients who cannot undergo surgical resection and for those who have a combination of distant metastases requires further clinical studies. Plain Language Summary: Clinical presentation and treatment of 4 children with pulmonary mucoepidermoid carcinoma Lung cancer in childhood is extremely rare, occurring at a rate of less than 2/1000000, and a type of lung cancer called pulmonary mucoepidermoid carcinoma (PMEC), is even rarer. The symptoms are usually not specific, and there are no guidelines for its management, which is a challenge for doctors. The purpose of this report is to discuss the signs and symptoms medical examinations, disease characteristics, surgical procedures, chemotherapy regimens and prognosis in children with pulmonary mucoepidermoid carcinoma. The clinical data of four patients diagnosed with pulmonary mucoepidermoid carcinoma at the Children's Hospital of Chongqing Medical University from June 2021 to November 2022 were analyzed, and their clinical features, treatment and prognosis were summarized. All four children are currently alive, and there is no recurrence or spread of the tumor after treatment. We have discussed various aspects of the disease, such as the rate of occurrence, causes, signs and symptoms, the way in which it might be diagnosed and treated, and the survival rate after operation, hoping to provide some insights for future work. [ABSTRACT FROM AUTHOR]

Background: Pediatric bronchiectasis is a common respiratory disease in children. The use of video-assisted thoracoscopic surgery (VATS) for its treatment remains controversial. Objectives: The objective of our study was to compare and analyze the clinical efficacy of thoracoscopic surgery and thoracotomy in the treatment of pediatric bronchiectasis and summarize the surgical treatment experience of VATS in children with bronchiectasis. Design: Retrospective single-center cohort study. Methods: A retrospective analysis was conducted on the clinical data of 46 pediatric patients who underwent surgery with bronchiectasis at the Children's Hospital of Chongqing Medical University from May 2015 to May 2023. The patients were divided into two groups: the VATS group (25 cases) and the thoracotomy group (21 cases). Comparative analysis was performed on various parameters including basic clinical data, surgical methods, operation time, intraoperative blood loss, transfusion status, postoperative pain, postoperative mechanical ventilation time, chest tube drainage time, length of hospital stay, incidence of complications, and follow-up information. Results: There were no statistically significant differences between the two groups of patients in terms of age, weight, gender, etiology, duration of symptoms, site of onset, and comorbidities (p > 0.05). The operation time in the VATS group was longer than that in the thoracotomy group (p < 0.001). However, the VATS group had better outcomes in terms of intraoperative blood loss, transfusion status, postoperative pain, postoperative mechanical ventilation time, chest tube drainage time, and length of hospital stay (p < 0.05). The incidence of postoperative complications in the VATS group was lower than that in the thoracotomy group, although the difference was not statistically significant (p = 0.152). Follow-up data showed no statistically significant difference in the surgical treatment outcomes between the two groups (p = 0.493). Conclusion: The incidence of complications and mortality in surgical treatment of bronchiectasis is acceptable. Compared with thoracotomy surgery, VATS has advantages such as smaller trauma, less pain, faster recovery, and fewer complications. For suitable pediatric patients with bronchiectasis, VATS is a safe and effective surgical method. [ABSTRACT FROM AUTHOR]

[Display omitted] • The specific mechanisms of PS-NP-induced pulmonary injury are unclear. • A mouse model of lung injury is created based on the real-world NP exposure in human. • PS-NP exposure induces ferroptosis in lung tissues and bronchial epithelial cells. • HIF-1α inhibition rescues PS-NP-induced ferroptosis in bronchial epithelial cells. • PS-NP exposure induces ferroptosis via the HIF-1α/HO-1 signaling pathway in lungs. It has been shown that polystyrene nanoplastic (PS-NP) exposure induces toxicity in the lungs. This study aims to provide foundational evidence to corroborate that ferroptosis and abnormal HIF-1α activity are the main factors contributing to pulmonary dysfunction induced by PS-NP exposure. Fifty male and female C57BL/6 mice were exposed to distilled water or 100 nm or 200 nm PS-NPs via intratracheal instillation for 7 consecutive days. Hematoxylin and eosin (H&E) and Masson trichrome staining were performed to observe the histomorphological changes in the lungs. To clarify the mechanisms of PS-NP-induced lung injury, we used 100 μg/ml, 200 μg/ml and 400 μg/ml 100 or 200 nm PS-NPs to treat the human lung bronchial epithelial cell line BEAS-2B for 24 h. RNA sequencing (RNA-seq) of BEAS-2B cells was performed following exposure. The levels of glutathione, malondialdehyde, ferrous iron (Fe2+), and reactive oxygen species (ROS) were measured. The expression levels of ferroptotic proteins were detected in BEAS-2B cells and lung tissues by Western blotting. Western blotting, immunohistochemistry, and immunofluorescence were used to evaluate the HIF-1α/HO-1 signaling pathway activity. H&E staining revealed substantial perivascular lymphocytic inflammation in a bronchiolocentric pattern, and Masson trichrome staining demonstrated critical collagen deposits in the lungs after PS-NP exposure. RNA-seq revealed that the differentially expressed genes in PS-NP-exposed BEAS-2B cells were enriched in lipid metabolism and iron ion binding processes. After PS-NP exposure, the levels of malondialdehyde, Fe2+, and ROS were increased, but glutathione level was decreased. The expression levels of ferroptotic proteins were altered significantly. These results verified that PS-NP exposure led to pulmonary injury through ferroptosis. Finally, we discovered that the HIF-1α/HO-1 signaling pathway played an important role in regulating ferroptosis in the PS-NP-exposed lung injury. PS-NP exposure caused ferroptosis in bronchial epithelial cells by activating the HIF-1α/HO-1 signaling pathway, and eventually led to lung injury. [ABSTRACT FROM AUTHOR]

Purpose: To predict the International Neuroblastoma Pathology Classification (INPC) in neuroblastoma using a computed tomography (CT)-based radiomics approach. Methods: We enrolled 297 patients with neuroblastoma retrospectively and divided them into a training group (n = 208) and a testing group (n = 89). To balance the classes in the training group, a Synthetic Minority Over-sampling Technique was applied. A logistic regression radiomics model based on the radiomics features after dimensionality reduction was then constructed and validated in both the training and testing groups. To evaluate the diagnostic performance of the radiomics model, the receiver operating characteristic curve and calibration curve were utilized. Moreover, the decision curve analysis to assess the net benefits of the radiomics model at different high-risk thresholds was employed. Results: Seventeen radiomics features were used to construct radiomics model. In the training group, radiomics model achieved an area under the curve (AUC), accuracy, sensitivity, and specificity of 0.851 (95% confidence interval (CI) 0.805–0.897), 0.770, 0.694, and 0.847, respectively. In the testing group, radiomics model achieved an AUC, accuracy, sensitivity, and specificity of 0.816 (95% CI 0.725–0.906), 0.787, 0.793, and 0.778, respectively. The calibration curve indicated that the radiomics model was well fitted in both the training and testing groups (p > 0.05). Decision curve analysis further confirmed that the radiomics model performed well at different high-risk thresholds. Conclusion: Radiomics analysis of contrast-enhanced CT demonstrates favorable diagnostic capabilities in distinguishing the INPC subgroups of neuroblastoma. Critical relevance statement: Radiomics features of contrast-enhanced CT images correlate with the International Neuroblastoma Pathology Classification (INPC) of neuroblastoma. Key points: Radiomics features derived from contrast-enhanced CT images are strongly associated with the INPC subgroups of neuroblastoma. Radiomics analysis of contrast-enhanced CT images can effectively distinguish between unfavorable and favorable histology of neuroblastoma. The decision curve analysis has validated the clinical utility of the radiomics model. [ABSTRACT FROM AUTHOR]

Pulmonary sequestration (PS) is a rare congenital malformation, which mainly contains two variants involving extralobar and intralobar sequestrations. Extralobar sequestrations (ELS) are isolated from the remaining lung tissue and have their visceral pleura. Herein, we report the first case of a torsed ELS associated with congenital lobar emphysema. We described a boy who mainly presented with abdominal and chest pain. A contrast-enhanced computed tomography (CT) scan revealed a mildly enhanced posterior mediastinal mass with left lower lobar emphysema. Thoracoscopic surgery identified a dark and hemorrhagic mass that was connected to the thoracic aorta by a twisted feeding vessel and had its visceral pleura. Pathological findings were consistent with an ELS that had undergone torsion and infarction. The postoperative recovery was uneventful. For the left lower lobar emphysema, he was required for a regular outpatient follow-up. In conclusion, although extremely rare, physicians should maintain a high index of suspicion for torsed ELS when a posterior mediastinal mass with abdominal or chest pain is presented in children. [ABSTRACT FROM AUTHOR]

Background: The optimal timing of surgery for pectus excavatum (PE) is controversial. A large proportion of children will not undergo surgery before puberty. However, untimely surgery may lead to a decline in the children's social adaptation and competitiveness because the children have already developed psychological and physiological impairments due to PE at an early age. The study retrospectively compared the academic performance in PE children undergoing the Nuss procedure versus nonsurgical observation. Methods: This retrospective real-world research study included 480 PE patients with definite surgical indications, in whom it was first recommended that they undergo surgery between the ages of 6 and 12 years old. Academic performance was collected at baseline and 6 years later. A generalized linear regression was calculated to screen the factors affecting the performance. A propensity score matching (PSM) analysis was conducted to reduce the potential for confounding factors between surgical and nonsurgical PE patients. Results: Haller index (HI) and pulmonary function were recognized as factors affecting baseline performance according to the generalized linear regression. For PE children with surgical indications, their academic performance significantly declined after 6 years of nonsurgical observation (52.1% ± 17.1% versus 58.3% ± 16.7%, p = 0.042). The academic performance in the surgery group was better than that in the nonsurgery group 6 years after PSM (60.7% ± 17.7% versus 52.1% ± 17.1%, p = 0.008). Conclusions: The severity of PE will affect the academic performance of children. For PE children with definite surgical indications between the ages of 6 and 12 years old, surgical intervention rather than nonsurgical observation is more conducive to the development of children's academic performance. [ABSTRACT FROM AUTHOR]

Dendritic fibromyxolipoma (DFML) is an uncommon benign tumor. We report the first DFML in the right thorax of a child. An 11-year-old girl was admitted because of a giant tumor in the right thorax. An enhanced chest CT scan indicated a thoracic mass with mild enhancement. Thoracoscopic biopsy revealed that the tumor was composed of stellate and spindle cells embedded within abundant myxoid stroma. Additionally, mature adipocytes, cytoplasmic dendritic processes, short strands of keloidal-type collagen, and plexiform blood vessels were observed. Immunohistochemical staining indicated positive for CD34 and BCL-2. DDIT3 alteration or MDM2 amplification were not observed. The diagnosis of DFML was considered, and complete tumorectomy was performed. In conclusion, definite diagnosis of DFML should be made according to the pathologic features. Accurate diagnosis is crucial to avoid overtreatment because DFML potentially can be mistaken for more aggressive neoplasms. [ABSTRACT FROM AUTHOR]

To investigate the feasibility of quantitative susceptibility mapping in children with attention‐deficit hyperactivity disorder (ADHD), 53 children with ADHD aged 5–16 years were prospectively selected as the study group and 49 healthy children matched with age and gender were selected as the control group. All children underwent magnetic resonance imaging conventional sequence, 3D‐T1, and enhanced T2*‐weighted magnetic resonance angiography (ESWAN) sequence scanning. The iron content of brain regions was obtained through software postprocessing, and the iron content of brain regions of children with ADHD and healthy children was compared and analyzed to find out the characteristics of the iron content of brain regions of children with ADHD. The iron content in frontal lobe, globus pallidus, caudate nucleus, substantia nigra, putamen, and hippocampus of children with ADHD was lower than that of healthy children (p <.05). There was no significant difference in the content of iron in the left and right brain regions of children with ADHD (p >.05). The volume of frontal lobe and hippocampus of children with ADHD was lower than that of healthy children (p <.05). Iron content in brain areas such as globus pallidus, caudate nucleus, hippocampus, and putamen could distinguish children with ADHD (Area under curve [AUC] > 0.5, p <.05). Quantitative susceptibility mapping showed decreased iron content in some brain regions of children with ADHD. [ABSTRACT FROM AUTHOR]

Objective: To explore the application of three-dimensional pseudocontinuous arterial spin labeling (3D-PCASL) perfusion imaging in the brains of children with autism and to understand the characteristics of cerebral blood perfusion in children with autism. Methods: A total of 320 children with autism (160 men and 160 women) aged between 2 and 18 years and 320 age- and sex-matched healthy children participated in the study. All children were scanned by 3.0 T magnetic resonance axial T1 fluid-attenuated inversion recovery (FLAIR), T2 FLAIR, 3D-T1, and 3D-PCASL sequences. After postprocessing, cerebral blood flow (CBF) values in each brain region of children with autism and healthy children at the same age were compared and analyzed. Furthermore, CBF characteristics in each brain region of autistic children at various ages were determined. Results: The CBF values of the frontal lobe, hippocampus, temporal lobe, and caudate nucleus of children with autism are lower than those of healthy children (P < 0.05). Additionally, as the ages of children with autism increase, the number of brain regions with decreased CBF values gradually increases. A receiver operating characteristic (ROC) analysis results show that the CBF values of the frontal lobe, hippocampus, temporal lobe, and caudate nucleus can distinguish children with autism [area under the ROC curve (AUC) > 0.05, P < 0.05]. Conclusion: The 3D-PCASL shows lower brain CBF values in children with autism. Clinical Trial Registration: www.ClinicalTrials.gov, identifier: ChiCTR2000034356. [ABSTRACT FROM AUTHOR]

Objective:  To summarize experience in the treatment of pentalogy of Cantrell (POC) in our hospital and explore the effect of artificial materials in repairing sternal defects.Materials and Methods:  A retrospective analysis was performed on treatment of five children with POC treated by using the Gore-Tex patch and titanium mesh in the Department of Cardio-Thoracic Surgery, Children's Hospital of Chongqing Medical University, from January 2010 to January 2019.Results:  The concurrent conditions included double outlet of right ventricle (n = 2), ventricular septal defect (VSD) and atrial septal defect (ASD) (n = 1), VSD and ASD and patent ductus arteriosus (n = 1), and VSD and left ventricular diverticulum (n = 1) in five cases with POC. Color Doppler echocardiography and computed tomography (CT) + three-dimensional (3D) reconstruction of the thorax and abdomen were performed preoperatively. The cardiac malformation was corrected according to color Doppler echocardiography, and a Gore-Tex patch was used to repair the pericardial defect. Titanium mesh was made according to CT 3D reconstruction with a 3D printing mold to repair sternal defects. All patients underwent a one-stage operation, all hearts were eventually repositioned, no deaths occurred after the operation, and follow-up was performed for 6 months to 2 years. The patients recovered well, and the exterior thorax was normal.Conclusion:  The diagnosis of POC is not difficult. The priority of surgical treatment for POC is to obtain satisfactory corrections of cardiac malformation. The repair of the pericardial defect with the Gore-Tex patch and the sternal defect with the titanium mesh can make the heart return to the mediastinum, reduce the pressure on the heart, reduce the surgical trauma, reduce the difficulty of repairing the sternal defect, and optimally restore the exterior thorax. [ABSTRACT FROM AUTHOR]

Download slide Download slide OBJECTIVES Surgical repair of pectus excavatum is typically carried out immediately prior to puberty. However, at the time of surgery, some psychosocial issues, such as behavioural disorders may already have developed and the likelihood of these psychosocial disorders resolving after surgery is unclear. For this reason, some surgeons choose to perform surgical repair at an earlier age in some children. The study retrospectively compares the rate of behavioural disorders in children undergoing the Nuss procedure at 4 vs. 10 years of age. We also attempted to develop a model to predict the risk of behavioural disorders in 10 year-old patients. METHODS The current study included children receiving Nuss procedure for pectus excavatum at either 4 or 10 years of age. The presence/absence of behavioural disorder was assessed preoperatively, and in the third year, after removal of the bar. A propensity score matching (PSM) analysis was conducted to reduce the potential for confounding by baseline factors. Multivariable logistic regression was conducted to establish a model to predict the risk of behavioural disorders in the third year after the removal of the bar. The model was accessed by discrimination and calibration. A formula and a nomogram were developed based on the results. RESULTS The number of patients in each group was 45 after PSM. The rate of behavioural disorders at the baseline was significantly higher in the children undergoing Nuss procedure at 10 years of age [36% vs 20%, odds ratio (OR) 2.21, 95% confidence interval (CI) 0.85–5.72; P  = 0.157]. The rate of behavioural disorders in the third year after the removal of the bar was 36% and 18% in children undergoing surgery at 10 and 4 years of age, respectively (OR 2.55, 95% CI 0.96–6.79; P  = 0.094). The rate of persistent behavioural disorders, defined as continuing to have behavioural disorders in the third year after the removal of the bar in those with behavioural disorders at the baseline, was 88% vs 56% (OR 3.47, 95% CI 0.56–21.36; P  = 0.204). Two patients (4%) relapsed in each group. A risk prediction model by variables of gender, Haller index, pulmonary function and score of Child Behaviour Checklist at the baseline was provided. CONCLUSIONS The rate of behavioural disorders was considerably lower in children who underwent the Nuss procedure at 4 years of age than at 10 years of age. Behavioural disorders may not readily resolve after surgery. Performing surgery at an early age rather than just before puberty may be better for psychosocial development. Psychosocial aid is necessary in addition to surgery to address behavioural disorders. [ABSTRACT FROM AUTHOR]

To summarize the etiology and treatment of 119 patients with chylothorax in our hospital. A total of 119 patients with chylothorax, divided into a neonate group, an infant group, and an older-than-1-year group, were included in our study and analyzed from January 2000 to July 2017 in the Children's Hospital of Chongqing Medical University. A total of 90 males and 29 females were included in our study. In the neonate group, 21 chylothorax cases were due to idiopathic factors, constituting 72.4% of the cases; 8 were related to cardiothoracic surgery, constituting 27.6%. In the infant group, 15 chylothorax cases were due to cardiothoracic surgery, constituting 55.6% of the cases; 11 were related to idiopathic factors, constituting 40.7%; and 1 was related to lymphoma, constituting 3.8%. In the older-than-1-year group, 33 chylothorax cases were due to idiopathic factors, constituting 52.4% of the cases; 25 were cardiothoracic surgery, constituting 39.7%; 2 were related to another internal medicine disease, constituting 3.2%; 2 were due to injury, constituting 3.2%; and 1 was related to lymphoma, constituting 1.6%. All the patients sequentially underwent thoracic drainage therapy, followed by fasting, thoracic injection of an adhesion-promoting agent, and thoracic duct ligation surgery. Among the neonates, 23 patients (79%) with fasting therapy improved, and 5 patients with fasting + intrapleural injection improved (17%). In the infant group, fasting promoted recovery in 14 patients, accounting for 51%, and fasting + thoracic injection improved the conditions of 10 patients, accounting for 37%. In the older-than-1-year group, fasting was effective in 35 patients, accounting for 55%; fasting + thoracic injection was effective in 22 patients, accounting for 34%; and fasting + thoracic injection + thoracic duct ligation surgery enabled the recovery of 2 patients, accounting for 3.2%. In our center, the main causes of chylothorax in the neonates group are idiopathic factors but may also include a history of unspecified birth trauma; the proportions of idiopathic factors in the infant and older-than-1-year groups are also higher, but the iatrogenic factors are significantly increased in the latter 2 groups. All the patients underwent thoracic drainage therapy, and fasting promoted the recovery of most children. When fasting was ineffective, subsequent thoracic injections were effective. If the above two methods failed, surgery was a method of choice, but it was not always effective. Level IV. Retrospective study. [ABSTRACT FROM AUTHOR]

Abstract Purpose To describe the diagnosis and treatment of 34 cases of congenital tracheobronchial cartilage remnants of esophagus. Methods The diagnosis and treatment of 34 cases of congenital tracheobronchial cartilage remnants of esophagus were analyzed retrospectively in our hospital. Results Congenital tracheobronchial cartilage remnants of esophagus could be specifically diagnosed by clinical situation and esophageal barium meal examination. The anterior wall of the esophagus was cut longitudinally with the posterior wall of the esophagus preserved. And the cartilage was removed and the open anterior wall of the esophagus was sutured horizontally with full layer. In our study, 34 patients who underwent the operation had a satisfactory outcome. However, one patient underwent submucosal cartilage stripping, which resulted in a complication involving fistulae from the esophagus to the abdominal cavity that were healed after gastrostomy and nutritional support. Conclusion Congenital tracheobronchial cartilage remnants of esophagus caused symptoms when the infants were started on adjunct foods. Vomiting the food without gastric fluid and bile was the leading clinical manifestation. Distinguishing signs on esophageal barium contrast could be used as preoperative diagnosis evidence. Surgically removing the cartilage and horizontally suturing the esophagus provides a reliable outcome. Level of Evidence Level IV. Type of Study Retrospective study. [ABSTRACT FROM AUTHOR]

Abstract Background Pectus excavatum, the most common congenital chest wall deformity in pediatric patients, leads to pulmonary dysfunction. There is no consensus regarding the effectiveness of the Nuss procedure for recovering pulmonary function. In this meta- analysis, we focused on the changes that occur in pulmonary function after the Nuss procedure. Methods We performed a literature search in the MEDLINE, Embase, Cochrane library and PubMed databases. The included studies were required to contain pulmonary function tests with results adjusted to predicted values both before and after the Nuss procedure. The key outcomes of interest in this analysis were pulmonary function measured as forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC). Subgroup analyses were performed based on time since surgery and the mean ages of the patients when they underwent surgery by forest plots and meta-regressions. Results Thirteen studies involving 465 participants were included in this review. The standard mean difference (SMD) observed in FEV1 and FVC after surgery were 0.17 (95% CI, 0.01–0.33, p = 0.04) and − 0.18 (95% CI, − 0.41–0.06, p = 0.14), respectively. The overall meta-regression SMD of FEV1 and FVC by time since surgery were 1.21 (95% CI, 1.04–1.41, p = 0.020) and 1.38 (95% CI, 1.05–1.83, p = 0.027), respectively. We found evidence of a temporal relationship between time at which pulmonary function tests were performed after surgery and predicted FEV1 and FVC values. The SMD of FEV1 (0.26, p = 0.012) was slightly higher in group evaluated more than 2 year after initial surgery. Conclusions Abnormal resting pulmonary functions tests performed prior to surgery showed an initial depression after surgery. The FEV1 of patients slightly increased at 2 year post surgery compared with the baseline. Further studies with longer term follow-up are still needed to determine if pulmonary function could improve to normal after surgery. Levels of Evidence Level of evidence: 4 (based on lowest level of article analyzed in meta-analysis/systematic review). [ABSTRACT FROM AUTHOR]

Objective This study sought to summarize the diagnostic and treatment aspects of congenital diaphragmatic eventration (CDE) in children by retrospectively analyzing their medical records to identify and understand the complications of CDE, its treatment, and to evaluate the long-term outcomes of diaphragmatic plication. Methods The medical records of children who received treatment for CDE from January 2000 to December 2011 at the Children's Hospital of Chongqing Medical University, China were analyzed. Data analyzed included the following: age, sex, symptom, location of eventration, associated anomalies, surgical procedures, complications, and survival and follow up details after diaphragmatic plication. Results The medical records of 177 children (boys: 128, girls: 49, mean age: 10.28 ± 2.35 months) with CDE were included in this study. Specific symptoms of eventration of the diaphragm were reported for 86 cases; and the typical symptoms included rapid breathing, vomiting, and recurrent respiratory infections. Except for a bilateral case, all the other patients had unilateral CDE. Associated malformations were observed in 31 cases (17.5%), hypoplastic lung (10 cases) was the most common followed by congenital heart disease (9 cases), and cryptorchidism (3 cases). Interestingly, 91 patients were asymptomatic. Diaphragmatic plication was performed in all symptomatic patients (86 cases, 48.5%) and none had recurrence. Conclusions Clinical symptoms of CDE varied in severity, ranging from asymptomatic conditions to life-threatening respiratory distress. Timely accurate diagnosis and treatment of symptomatic CDE could effectively resolve respiratory morbidity and reduce complications. The diaphragm plication surgery provided good results among the study population with no recurrence. [ABSTRACT FROM AUTHOR]

Osteosarcoma (OS) is the most common type of bone cancer in children and adolescents. LncRNA differentiation antagonizing nonprotein coding RNA (DANCR) has been reported to be aberrant expression in osteosarcoma and contribute to proliferation, migration and invasion of cancer cells. Here, we further explore the exacted molecular mechanism of DANCR in OS. The expression of DANCR, microRNA-216a-5p (miR-216a-5p) and SOX5 was analyzed by quantitative real-time polymerase chain reaction (qRT-PCR). Cells proliferation and apoptosis were analyzed by WST-1assay or flow cytometry, respectively. The migratory and invasion abilities were measured by transwell assay. Western blot was used to detect the level of SOX5 and autophagy-related protein of Beclin1, LC3-I and LC3-II. The interaction among DANCR, miR-216a-5p and SOX5 was explored by luciferase reporter assay, RIP assay or Pull-down assay. Murine xenograft model was established using 143B cells transfected with sh-DANCR. We found that a significantly elevated of DNACR was detected in osteosarcoma tissue and cell lines. Functional experiments suggested that down-regulation of DANCR inhibited cells proliferation, migration, invasion and autophagy but induced apoptosis in osteosarcoma in vitro. Additionally, we also determined knockdown of DANCR inhibited the growth and autophagy of osteosarcoma in vivo. DANCR was a sponge of miR-216a-5p activity. DANCR regulated survival of osteosarcoma through targeting miR-216a-5p. Additionally, SOX5 was a direct target of miR-216a-5p, overexpression miR-216a-5p exerted inhibition effects via down-regulating SOX5 expression. Furthermore, DANCR regulated SOX5 expression by sponging to miR-216a-5p. In conclusion, LncRNA DANCR silence inhibits SOX5-medicated progression and autophagy in osteosarcoma via regulating miR-216a-5p which indicating DANCR may act as a potential prognostic biomarker and therapeutic target for osteosarcoma. [ABSTRACT FROM AUTHOR]

Objectives: Double aortic arch (DAA) is a rare congenital vascular malformation. This study aims to summarize the experience of diagnosis and surgical treatment for congenital double aortic arch.Methods: The clinical data of 24 cases with double aortic arch (DAA) from January 2008 to January 2018 in our hospital was reviewed retrospectively.Results: A total of 24 cases, including 12 patients with isolated DAA and 12 patients with DAA and associated intracardiac defects were identified. There were 14 males and 10 females, with an average age of 11 months. The associated intracardiac malformations included ventricular septal defect (VSD), atrial septal defect (ASD), patent ductus arteriosus (PDA), tetralogy of Fallot (TOF), transposition of the great arteries (TGA), pulmonary stenosis (PS), and patent foramen ovale (PFO). Of the 12 patients with DAA and intracardiac malformations, 7 patients underwent intracardiac repair simultaneously, however, 3 patients underwent isolated double aortic arch correction. One patient with DAA and TGA underwent surgical correction of congenital vascular ring at the first stage, and the arterial switch operation was performed at the second stage. The clinical outcomes of 23 patients were promising, however, in one patient, parents decided not to do the surgery due to personal reasons. The average follow-up time was 35 months.Conclusions: Tracheal and esophageal compression are commonly seen in patients with DAA, however could be relieved significantly after surgery. In particular cases, the simultaneous intracardiac defects repair could be performed. Misdiagnosis was easily established with isolated echocardiography. Fortunately, the correct diagnosis of DAA and associated intracardiac defects could be established with the use of combined chest computed tomography. [ABSTRACT FROM AUTHOR]

Mitochondria, pivotal organelles governing cellular biosynthesis, energy metabolism, and signal transduction, maintain dynamic equilibrium through processes such as biogenesis, fusion, fission, and mitophagy. Growing evidence implicates mitochondrial dysfunction in a spectrum of respiratory diseases including acute lung injury/acute respiratory distress syndrome, bronchial asthma, pulmonary fibrosis, chronic obstructive pulmonary disease, and lung cancer. Consequently, identifying methods capable of ameliorating damaged mitochondrial function is crucial for the treatment of pulmonary diseases. Extracellular vesicles (EVs), nanosized membrane vesicles released by cells into the extracellular space, facilitate intercellular communication by transferring bioactive substances or signals between cells or organs. Recent studies have identified abundant mitochondrial components within specific subsets of EVs, termed mitochondrial extracellular vesicles (mitoEVs), whose contents and compositions vary with disease progression. Moreover, mitoEVs have demonstrated reparative mitochondrial functions in injured recipient cells. However, a comprehensive understanding of mitoEVs is currently lacking, limiting their clinical translation prospects. This Review explores the biogenesis, classification, functional mitochondrial cargo, and biological effects of mitoEVs, with a focus on their role in pulmonary diseases. Emphasis is placed on their potential as biological markers and innovative therapeutic strategies in pulmonary diseases, offering fresh insights for mechanistic studies and drug development in various pulmonary disorders.

Introduction: It has been shown that polystyrenenanoplastic (PS-NP) exposure induces toxicity in the lungs., Objectives: This study aims to provide foundational evidence to corroborate that ferroptosis and abnormal HIF-1α activity are the main factors contributing to pulmonary dysfunction induced by PS-NP exposure., Methods: Fifty male and female C57BL/6 mice were exposed to distilled water or 100 nm or 200 nm PS-NPs via intratracheal instillation for 7 consecutive days. Hematoxylin and eosin (H&E) and Masson trichrome staining were performed to observe the histomorphological changes in the lungs. To clarify the mechanisms of PS-NP-induced lung injury, we used 100 μg/ml, 200 μg/ml and 400 μg/ml 100 or 200 nm PS-NPs to treat the human lung bronchial epithelial cell line BEAS-2B for 24 h. RNA sequencing (RNA-seq) of BEAS-2B cells was performed following exposure. The levels of glutathione, malondialdehyde, ferrous iron (Fe 2+ ), and reactive oxygen species (ROS) were measured. The expression levels of ferroptotic proteins were detected in BEAS-2B cells and lung tissues by Western blotting. Western blotting, immunohistochemistry, and immunofluorescence were used to evaluate the HIF-1α/HO-1 signaling pathway activity., Results: H&E staining revealed substantial perivascular lymphocytic inflammation in a bronchiolocentric pattern, and Masson trichrome staining demonstrated critical collagen deposits in the lungs after PS-NP exposure. RNA-seq revealed that the differentially expressed genes in PS-NP-exposed BEAS-2B cells were enriched in lipid metabolism and iron ion binding processes. After PS-NP exposure, the levels of malondialdehyde, Fe 2+ , and ROS were increased, but glutathione level was decreased. The expression levels of ferroptotic proteins were altered significantly. These results verified that PS-NP exposure led to pulmonary injury through ferroptosis. Finally, we discovered that the HIF-1α/HO-1 signaling pathway played an important role in regulating ferroptosis in the PS-NP-exposed lung injury., Conclusion: PS-NP exposure caused ferroptosis in bronchial epithelial cells by activating the HIF-1α/HO-1 signaling pathway, and eventually led to lung injury., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024. Production and hosting by Elsevier B.V.)

Background: Nuss procedure is the most common method of surgical treatment to pectus excavatum (PE). A significant percentage of surgeons choose to use thoracoscopic assistance during the Nuss procedure (TNP) to avoid cardiac injury. However, our previous findings confirm the safety of single incision Non-thoracoscopic Nuss Procedure (SINTNP). Hence, Further studies, particularly prospective randomized controlled trials, are necessary to assess the value of SINTNP for PE., Methods: This study is a prospective, superiority, multicenter, non-masked, randomized controlled trial that investigates the outcome and hospitalization medical expense of SINTNP compared to TNP for PE. A total of 320 eligible patients according to sample size calculation by retrospective data will be randomly assigned to the SINTNP group or the TNP group at a 1:1 ratio using stratified blocked randomization and the zone length was set as four. Patients aged between 3 and 18 years old for the first surgery and without combination of complex anomalies such as Marfan syndrome and congenital heart disease will be considered for the study. The co-primary endpoint is thoracic related complications and medical expense during hospitalization. Thoracic related complications were defined as pneumothorax, pleural effusion, pneumonia and incision infection. The secondary endpoints include surgery duration and length of hospital stay.The registration number for this study protocol is ChiCTR230073081 (Chinese Clinical Trial Registry, A Primary Registry of International Clinical Trial Registry Platform, World Health Organization)., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Wang, Pan, Wu, Li, Wang, Dai, Ren, Xie, Xiong, Zhang and Li.)

Objective: To study the differences in computed tomography angiography (CTA) imaging of gothic arches, crenel arches, and romanesque arches in children with Aortic Coarctation (CoA), and to apply computational fluid dynamics (CFD) to study hemodynamic changes in CoA children with gothic arch aorta., Methods: The case data and CTA data of children diagnosed with CoA (95 cases) in our hospital were retrospectively collected, and the morphology of the aortic arch in the children was defined as gothic arch ( n  = 27), crenel arch ( n  = 25) and romanesque arch ( n  = 43). The three groups were compared with D1/AOA, D2/AOA, D3/AOA, D4/AOA, D5/AOA, and AAO-DAO angle, TAO-DAO angle, and aortic arch height to width ratio (A/T). Computational fluid dynamics was applied to assess hemodynamic changes in children with gothic arches., Results: There were no significant differences between D1/AOA and D2/AOA among gothic arch, crenel arch, and romanesque arch ( P  > 0.05). The differences in D3/AOA, D4/AOA, and D5/AOA among the three groups were statistically significant ( P  < 0.05), D4/AOA, D5/AOA of the gothic arch group were smaller than the crenel arch group, and the D3/AOA and D5/AOA of the gothic arch group were smaller than the romanesque arch group ( P  < 0.05). The difference in AAO-DAO angle among the three groups was statistically significant ( P  < 0.05), and the AAO-DAO angle of gothic arch was smaller than that of romanesque arch and crenel arch group ( P  < 0.05). There was no significant difference in the TAO-DAO angle between the three groups ( P  > 0.05). The difference in A/T values among the three groups was statistically significant ( P  < 0.05), and the A/T values: gothic arch > romanesque arch > crenel arch ( P  < 0.05). The CFD calculation of children with gothic arch showed that the pressure drop between the distal stenosis and the descending aorta was 58 mmHg, and the flow rate at the isthmus and descending aorta was high and turbulent., Conclusion: Gothic aortic arch is common in CoA, it may put adverse effects on the development of the aortic isthmus and descending aorta, and its A/T value and AAO-DAO angle are high. CFD could assess hemodynamic changes in CoA., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Qin, Ouyang, Yan, Wang, Guo, Jin, Pan and He.)

Background: The incidence of clinical adverse events after tetralogy of Fallot (TOF) repair remains high. This study was performed to explore risk factors for adverse events and develop a prediction model through machine learning (ML) to forecast the incidence of clinical adverse events after TOF repair., Methods: A total of 281 participants who were treated with cardiopulmonary bypass (CPB) at our hospital from January 2002 to January 2022 were included in the study. Risk factors for adverse events were explored by composite and comprehensive analyses. Five artificial intelligence (AI) models were used for ML to build prediction models and screen out the model with the best performance in predicting adverse events., Results: CPB time, differential pressure of the right ventricular outflow tract (RVOTDP or DP), and transannular patch repair were identified as the main risk factors for adverse events. The reference point for CPB time was 116.5 minutes and that for right ventricular (RV) outflow tract differential pressure was 70 mmHg. SPO 2 was a protective factor, with a reference point of 88%. By integrating the results for the training and validation cohorts, we confirmed that, among all models, the logistic regression (LR) model and Gaussian Naive Bayes (GNB) model were stable, showing good discrimination, calibration and clinical practicability. The dynamic nomogram can be used as a predictive tool for clinical application., Conclusions: Differential pressure of the RV outflow tract, CPB time, and transannular patch repair are risk factors, and SPO 2 is a protective factor for adverse events after complete TOF repair. In this study, models developed by ML were established to predict the incidence of adverse events., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://tp.amegroups.com/article/view/10.21037/tp-22-246/coif). The authors have no conflicts of interest to declare., (2023 Translational Pediatrics. All rights reserved.)

Objective: To explore the application of quantitative magnetic resonance imaging in the diagnosis of autism in children., Methods: Sixty autistic children aged 2-3 years and 60 age- and sex-matched healthy children participated in the study. All the children were scanned using head MRI conventional sequences, 3D-T1, diffusion kurtosis imaging (DKI), enhanced T2*- weighted magnetic resonance angiography (ESWAN) and 3D-pseudo continuous Arterial Spin-Labeled (3D-pcASL) sequences. The quantitative susceptibility mapping (QSM), cerebral blood flow (CBF), and brain microstructure of each brain area were compared between the groups, and correlations were analyzed., Results: The iron content and cerebral blood flow in the frontal lobe, temporal lobe, hippocampus, caudate nucleus, substantia nigra, and red nucleus of the study group were lower than those in the corresponding brain areas of the control group ( P < 0.05). The mean kurtosis (MK), radial kurtosis (RK), and axial kurtosis (AK) values of the frontal lobe, temporal lobe, putamen, hippocampus, caudate nucleus, substantia nigra, and red nucleus in the study group were lower than those of the corresponding brain areas in the control group ( P < 0.05). The mean diffusivity (MD) and fractional anisotropy of kurtosis (FAK) values of the frontal lobe, temporal lobe and hippocampus in the control group were lower than those in the corresponding brain areas in the study group ( P < 0.05). The values of CBF, QSM, and DKI in frontal lobe, temporal lobe and hippocampus could distinguish ASD children (AUC > 0.5, P < 0.05), among which multimodal technology (QSM, CBF, DKI) had the highest AUC (0.917) and DKI had the lowest AUC (0.642)., Conclusion: Quantitative magnetic resonance imaging (including QSM, 3D-pcASL, and DKI) can detect abnormalities in the iron content, cerebral blood flow and brain microstructure in young autistic children, multimodal technology (QSM, CBF, DKI) could be considered as the first choice of imaging diagnostic technology., Clinical Trial Registration: [http://www.chictr.org.cn/searchprojen.aspx], identifier [ChiCTR2000029699]., Competing Interests: LN was employed by GE Healthcare. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Tang, Nie, Liu, Chen, Zhou, Pan and He.)