Objective:To summarize the clinical characteristics and prognosis of infectious pulmonary artery pseudoaneurysm (IPAP) in pediatric patients.Methods:This case series study summarizes the clinical data of 3 children diagnosed with IPAP at the Children′s Hospital of Chongqing Medical University from January 2015 to December 2024.A comprehensive literature review was conducted by searching Chinese databases (China National Knowledge Infrastructure, Wanfang Data Knowledge Service Platform, China VIP Database and Chinese Medical Journals Full Text Database) and the PubMed database through December 2024, using the keywords"infect""pseudoaneurysm""pulmonary artery". The clinical characteristics and prognosis of IPAP were synthesized by combining the reviewed literature.Results:The 3 cases included a 2-month-old male infant, a 6-year-3-month-old male child and a 12-year-old female child, all of whom presented with fever and various respiratory symptoms. Case 1 was complicated by a retro-orbital abscess, Case 2 had a history of chronic granulomatous disease and psoriasis, and Case 3 was diagnosed with patent ductus arteriosus. Enhanced CT imaging in all 3 cases revealed multiple IPAP, with 2 cases associated with pulmonary infection foci and 1 case linked to infective endocarditis. Accompanying complications included pleural effusion in all 3 cases, necrotizing pneumonia in 2 cases, and pulmonary embolism in 1 case. Of the 3 cases, 2 cases showed improvement after anti-infective treatment, while 1 case resulted in death. A total of 9 English-language studies were identified, while no suitable Chinese-language studies were found. Including these 3 cases, a total of 12 pediatric IPAP cases were analyzed. The predominant clinical manifestations included fever in 10 cases, cough in 6 cases and hemoptysis in 5 cases. The most frequently identified pathogen was methicillin-resistant Staphylococcus aureus found in 3 cases. Additionally, 6 cases were complicated by extra-pulmonary infections, 4 cases had a history of congenital heart disease and surgical intervention, 1 case underwent rectovestibular fistula repair and 4 cases exhibited immune dysfunction. Imaging characteristics revealed that 9 of the 12 cases were associated with pulmonary infection foci, while 1 case was related to infective endocarditis, and 2 cases had insufficient imaging data for accurate classification. A total of 7 cases presented with a single IPAP, predominantly located in the right lower lobe in 3 cases, while the remaining 5 cases had multiple IPAP. IPAP was complicated by pleural effusion in 8 cases, by necrotizing pneumonia in 8 cases (1 of which was suspected), and by lung abscess and pulmonary embolism in 3 cases each. Among the 12 cases, 5 cases received anti-infective therapy without lobectomy or embolization, of which 3 cases improved, 1 case showed no response, and 1 case died. Additionally, 6 cases underwent endovascular embolization, with 4 cases showing improvement and 2 cases resulting in death. Only 1 case underwent lobectomy and was discharged in stable condition. Conclusions:IPAP in children manifests with a broad spectrum of clinical signs, frequently accompanied by extra-pulmonary infections and immune dysfunction. Enhanced CT plays a crucial role in the diagnosis of IPAP. The prognosis of IPAP is closely linked to the treatment approach, and timely diagnosis along with personalized treatment are essential for improving survival rates.

Cystic fibrosis(CF)is an autosomal recessive genetic disorder caused by mutations of the gene encoding the cystic fibrosis transmembrane conductance regulator(CFTR),which disrupts chloride ion transport across cell membranes,leading to multi-system involvement.The respiratory system is the most affected organ and the leading cause of mortality in affected children.With increased awareness and advancements in genetic testing,the number of diagnosed cases has increased in China.Notably,the genetic mutation profiles and clinical phenotypes of Chinese CF patients are significantly different from those reported in Western populations,posing challenges for clinical diagnosis and management.Currently,significant progress has been made in the treatment of CF in the international arena,with genotype-phenotype studies laying the foundation for personalized therapy,and small-molecule therapies such as CFTR modulators offering new hope for CF patients.This review summarizes the pathogenesis,clinical features,genotype-phenotype correlations,and therapeutic advancements in CF,aiming to enhance clinicians'understanding of the disease,reduce misdiagnosis and underdiagnosis,and facilitate early diagnosis and treatment.

Cystic fibrosis(CF)is an autosomal recessive genetic disorder caused by mutations of the gene encoding the cystic fibrosis transmembrane conductance regulator(CFTR),which disrupts chloride ion transport across cell membranes,leading to multi-system involvement.The respiratory system is the most affected organ and the leading cause of mortality in affected children.With increased awareness and advancements in genetic testing,the number of diagnosed cases has increased in China.Notably,the genetic mutation profiles and clinical phenotypes of Chinese CF patients are significantly different from those reported in Western populations,posing challenges for clinical diagnosis and management.Currently,significant progress has been made in the treatment of CF in the international arena,with genotype-phenotype studies laying the foundation for personalized therapy,and small-molecule therapies such as CFTR modulators offering new hope for CF patients.This review summarizes the pathogenesis,clinical features,genotype-phenotype correlations,and therapeutic advancements in CF,aiming to enhance clinicians'understanding of the disease,reduce misdiagnosis and underdiagnosis,and facilitate early diagnosis and treatment.

Objective:To summarize the clinical characteristics and prognosis of infectious pulmonary artery pseudoaneurysm (IPAP) in pediatric patients.Methods:This case series study summarizes the clinical data of 3 children diagnosed with IPAP at the Children′s Hospital of Chongqing Medical University from January 2015 to December 2024.A comprehensive literature review was conducted by searching Chinese databases (China National Knowledge Infrastructure, Wanfang Data Knowledge Service Platform, China VIP Database and Chinese Medical Journals Full Text Database) and the PubMed database through December 2024, using the keywords"infect""pseudoaneurysm""pulmonary artery". The clinical characteristics and prognosis of IPAP were synthesized by combining the reviewed literature.Results:The 3 cases included a 2-month-old male infant, a 6-year-3-month-old male child and a 12-year-old female child, all of whom presented with fever and various respiratory symptoms. Case 1 was complicated by a retro-orbital abscess, Case 2 had a history of chronic granulomatous disease and psoriasis, and Case 3 was diagnosed with patent ductus arteriosus. Enhanced CT imaging in all 3 cases revealed multiple IPAP, with 2 cases associated with pulmonary infection foci and 1 case linked to infective endocarditis. Accompanying complications included pleural effusion in all 3 cases, necrotizing pneumonia in 2 cases, and pulmonary embolism in 1 case. Of the 3 cases, 2 cases showed improvement after anti-infective treatment, while 1 case resulted in death. A total of 9 English-language studies were identified, while no suitable Chinese-language studies were found. Including these 3 cases, a total of 12 pediatric IPAP cases were analyzed. The predominant clinical manifestations included fever in 10 cases, cough in 6 cases and hemoptysis in 5 cases. The most frequently identified pathogen was methicillin-resistant Staphylococcus aureus found in 3 cases. Additionally, 6 cases were complicated by extra-pulmonary infections, 4 cases had a history of congenital heart disease and surgical intervention, 1 case underwent rectovestibular fistula repair and 4 cases exhibited immune dysfunction. Imaging characteristics revealed that 9 of the 12 cases were associated with pulmonary infection foci, while 1 case was related to infective endocarditis, and 2 cases had insufficient imaging data for accurate classification. A total of 7 cases presented with a single IPAP, predominantly located in the right lower lobe in 3 cases, while the remaining 5 cases had multiple IPAP. IPAP was complicated by pleural effusion in 8 cases, by necrotizing pneumonia in 8 cases (1 of which was suspected), and by lung abscess and pulmonary embolism in 3 cases each. Among the 12 cases, 5 cases received anti-infective therapy without lobectomy or embolization, of which 3 cases improved, 1 case showed no response, and 1 case died. Additionally, 6 cases underwent endovascular embolization, with 4 cases showing improvement and 2 cases resulting in death. Only 1 case underwent lobectomy and was discharged in stable condition. Conclusions:IPAP in children manifests with a broad spectrum of clinical signs, frequently accompanied by extra-pulmonary infections and immune dysfunction. Enhanced CT plays a crucial role in the diagnosis of IPAP. The prognosis of IPAP is closely linked to the treatment approach, and timely diagnosis along with personalized treatment are essential for improving survival rates.

Objective:To analyze the patterns of antibacterial agents in Chinese children surveyed by the China multi-center monitoring network for the application of antibacterial agents in children and neonates in 2019 by using World Health Organization (WHO) Access, Watch, Reserve and Not-recommended (AWaRe) and typical anatomical/therapeutic/chemical (ATC) in this study.Methods:The cross-sectional method was adopted.A multi-center cross-sectional survey was conducted on one day from September to December 2019.The information of all inpatients taking antibiotics was uploaded to the network-based data collection system (https: //garpec-31.mobilemd.cn/login.aspx? relogin=true). This study covered 13 hospitals from 10 provinces and cities in China.All hospitalized children in the Respiratory Department, Infectious Disease Department, General Surgery Department, Pediatric Intensive Care Units, Neonatal Intensive Care Units and Neonatology joined in this survey.The clinically used antibacterial agents were classified by AWaRe and ATC, and the AWaRe and ATC distributions of antibacterial agents prescribed for Chinese children and neonates were described.Results:Of the 2 644 antibiotic prescriptions included from 13 hospitals, 2 134 (80.71%) were for children and 510 (19.29%) were for neonates.Of all antibiotic prescriptions, there were 368 (13.92%) Access antibiotics prescriptions, 1 973 (74.62%) Watch prescriptions, 60 (2.27%) Reserve prescriptions and 243 (9.19%) Not-recommended prescriptions.The top-five antibiotics prescribed for children and neonates were third-generation cephalosporins (1 056, 39.94%), macrolides (492, 18.61%), carbapenems (275, 10.40%), beta lactam-beta lactamase inhibitors (246, 9.30%), and second-generation cephalosporins (136, 5.14%). The use ratios of Access, Watch, Reserve and Not-recommended antibiotics in each center ranged from 0 to 30.00%, 36.67% to 97.20%, 0 to 17.02% and 0 to 33.33%, respectively.In 1 360 antibiotic prescriptions for children and neonates with pneumonia, there were 152 (11.18%) Access antibiotics, 1 051 (77.28%) Watch antibiotics, 37 (2.72%) Reserve antibiotics, and 120 (8.82%) Not-recommended antibiotics.The top-five antibiotics prescribed for children with pneumonia were third-generation cephalosporins (522, 38.38%), macrolides (388, 28.53%), beta lactam-beta lactamase inhibitors (141, 10.37%), carbapenems (117, 8.6%) and penicillins (49, 3.60%).Conclusions:Watch antibiotics and broad spectrum antibiotics such as third-generation cephalosporins and macrolides prone to induce resistance are the main antibacterial agents used in Chinese children and neonates with pneumonia.Broad-spectrum antibiotics may be overused in Chinese children and neonates.

Objective:To analyze the cystic fibrosis transmembrane conductance regulator (CFTR) gene variations and phenotypes in 7 Chinese children.Methods:In this retrospective study, the data of 7 children with CFTR gene variations admitted to Children′s Hospital of Chongqing Medical University from December 2013 to October 2020 were extracted. The general information, clinical manifestations, gene variations, diagnosis and treatment were summarized.Results:Among the 7 children, 2 were males and 5 were females, aged 5.2(0.5-11.3) years. Main clinical manifestations included malnutrition (5 cases), recurrent respiratory infection (4 cases), bronchiectasis (3 cases), steatorrhea (3 cases), vomiting in infancy (2 cases), liver cirrhosis (2 cases), meconium ileus (1 case), metabolic alkalosis and hypochloremia (1 case). A total of 15 variations were found by whole exon sequencing and Sanger sequencing, among which 3 were newly discovered, and 7 were missense mutations. Four children were diagnosed as CF, and the other 3 were diagnosed as CFTR related disease (CFTR-RD). Compared with CF patients, the pancreatic insufficiency and typical CF lung disease were relatively mild in CFTR-RD patients. After treatment, 6 children were clinically improved, while the rest one withdrew treatment due to critical pulmonary infection and disturbance of water-electrolyte metabolism.Conclusions:The loci and phenotypes of CFTR gene variants vary hugely and the pathogenicity of some variations are not clear. Whole exon sequencing can facilitate the identification of CF-and CFTR-RD-causing variaions. For the cases not compatible with CF, CFTR-RD should be considered and evaluated by timely gene detection, so as to carry out appropriate long term management.

Objective: To investigate the risk factors for pneumonia in children with Epstein-Barr virus (EBV) infectious mononucleosis(IM). Methods: The clinical data of children with EBV-IM from March 2015 to February 2018 in Children′s Hospital of Chongqing Medical University were retrospectively analyzed.The patients were divided into a pneumonia group and a non-pneumonia group.The difference between 2 groups was analyzed, including gender, age, duration of fever, the maximum temperature in disease duration, the size of liver, the size of spleen, tonsillopharyngitis, cervical lymphadenopathy, edema of the eyelids, white blood cell (WBC), lymphocyte, atypical-lymphocytes, C-reactive protein (CRP), procalcitonin (PCT), alanine transaminase (ALT), glutamic oxalacetic transaminas (AST), lactic dehydrogenase (LDH), cytomegalovirus (CMV) antibody, the titer of mycoplasma (MP) antibody, EBV DNA, length of stay, and hospitalization expenses.The single factor analysis was performed to analyze the above data between 2 groups, and the data with statistical significance were analyzed by the multifactor Logistic regression.The recei-ver operator characteristic (ROC) curve was drawn to evaluate the predicting ability of the indicators for IM combined with pneumonia. Results: Among 923 cases, 133 cases(14.4%) EBV-IM patients were complicated with pneumonia.The findings of single factor analysis indicated that the risk factors were the duration of fever, the size of liver, the size of spleen, and the titer of MP antibody (all P<0.05). The multifactor Logistic regression showed that the duration of fever, the size of liver, the titer of MP antibody were the risk factors for EBV-IM children with pneumonia (P=0.013, 0.028, 0.014). The area under curve (AUC) of the duration of fever was 0.624, and the critical value was 7.5 d (P=0.000); the AUC of the size of liver was 0.590, and the critical value was 2.65 cm (P=0.003). Conclusions The incidence rate of EBV-IM children combined with pneumonia was high.With the presence of the titer of MP antibody ≥1∶160, the duration of fever≥7.5 days, and the size of liver>2.65 cm, it may be independent risk factors for pneumonia in IM children with EBV infection, which requires special attention clinically and earlier chest imageological examination are needed.

Objective To investigate the effect of Brahma-related gene 1 (Brg1) on mucus hypersecretion in the airway of asthmatic mice and explore the mechanism. Methods Female C57bl/6 mice aged 6-8 weeks were randomized into wild-type control group, wild-type asthma group, Brg1-/-group with Brg1 gene knockdown in type II alveolar epithelial cells, and Brg1-/-+asthma group (n=10). The mice in asthma group and Brg1-/-+asthma group were sensitized with ovalbumin (OVA) to establish asthmatic models. PAS staining was used to determine the number of goblet cells and mucus secretion in the airway. Real-time PCR was used to detect the expression of MUC5AC mRNA in the lung tissues. The levels of mucin MUC5AC and interleukin-13 (IL-13) in the bronchoalveolar lavage fluid (BALF) were detected with ELISA and immunohistochemistry, and the expressions of STAT6 and p-STAT6 in the lung tissue were detected using Western blotting. Results Compared with the control mice, wild-type asthmatic mice showed obvious mucus hypersecretion and increased MUC5AC mRNA in the airway with significantly increased IL-13 and MUC5AC levels in the BALF and activation of p-STAT6 in the lung tissues (P<0.05). In the transgenic mice with Brg1 gene knockdown, airway mucus secretion and MUC5AC mRNA expression was significantly reduced following OVA challenge compared with those in the wild-type asthmatic mice; IL-13 and MUC5AC levels in the BALF and p-STAT6 expression in the lung tissues were also significantly decreased in the transgenic mice (P<0.05). Conclusion Brg1 gene knockdown in type II alveolar epithelial cells alleviates OVA-induced airway mucus hypersecretion and reduces the expression of MUC5AC in C57bl/6 mice possibly by inhibiting STAT6 activation, suggesting the role of Brg1 in promoting asthmatic airway mucus hypersecretion.

Objective To investigate the effect of Brahma-related gene 1 (Brg1) on mucus hypersecretion in the airway of asthmatic mice and explore the mechanism. Methods Female C57bl/6 mice aged 6-8 weeks were randomized into wild-type control group, wild-type asthma group, Brg1-/-group with Brg1 gene knockdown in type II alveolar epithelial cells, and Brg1-/-+asthma group (n=10). The mice in asthma group and Brg1-/-+asthma group were sensitized with ovalbumin (OVA) to establish asthmatic models. PAS staining was used to determine the number of goblet cells and mucus secretion in the airway. Real-time PCR was used to detect the expression of MUC5AC mRNA in the lung tissues. The levels of mucin MUC5AC and interleukin-13 (IL-13) in the bronchoalveolar lavage fluid (BALF) were detected with ELISA and immunohistochemistry, and the expressions of STAT6 and p-STAT6 in the lung tissue were detected using Western blotting. Results Compared with the control mice, wild-type asthmatic mice showed obvious mucus hypersecretion and increased MUC5AC mRNA in the airway with significantly increased IL-13 and MUC5AC levels in the BALF and activation of p-STAT6 in the lung tissues (P<0.05). In the transgenic mice with Brg1 gene knockdown, airway mucus secretion and MUC5AC mRNA expression was significantly reduced following OVA challenge compared with those in the wild-type asthmatic mice; IL-13 and MUC5AC levels in the BALF and p-STAT6 expression in the lung tissues were also significantly decreased in the transgenic mice (P<0.05). Conclusion Brg1 gene knockdown in type II alveolar epithelial cells alleviates OVA-induced airway mucus hypersecretion and reduces the expression of MUC5AC in C57bl/6 mice possibly by inhibiting STAT6 activation, suggesting the role of Brg1 in promoting asthmatic airway mucus hypersecretion.

Objective To investigate the etiology,clinical charactieristics,treatment and prognosis in pediatric hemoptysis to provide reference for its diagnosis and treatment to pediatric doctors .Methods The retrospective analysis was performed on the clinical manifestations ,laboratory tests ,imagiological and bronchoscopic manifestations ,treatment methods and outcomes in the children patients with hemoptysis as the chief complaint admitted to our hospital from April 2009 and December 2014 .Results A-mong 102 children cases of hemoptysis ,55 cases were male and 47 cases were female .The median age was (7 .05 ± 1 .2) years old . Mild hemoptysis was in 87 cases(85 .3% ) ,moderate hemoptysis was in 10 cases(9 .8% ) and massive hemoptysis was in 5 cases (4 .9% ) .The relatively exact causes of hemoptysis were found in 88 cases ,in which the lower respiratory tract infection was most common ,the top 3 causes were pneumonia ,pulmonary tuberculosis and bronchitis .In the non-infection causes ,idiopathic pulmonary hemosiderosis(IPH) was most common ,while connective tissue diseases ,pulmonary vascular malformation and airway foreign body were rare .85 cases showed different pathological lesions on chest CT scan .Among the 35 cases underwent the bronchoscopy ,3 ca-ses of bronchial stenosis caused by granulation proliferation was improved after bronchoscopic cryotherapy ,5 cases of active bleed-ing under endoscopic stopped bleeding by topical hemostasis ,among 10 cases of suspected tuberculosis with negative sputum bacte-ria ,acid-fast bacilli was found in 8 cases by bronchofiberscopic lavage .The treatment of pediatric hemoptysis was dominated by the medical etiological and symptomatic therapy .Conclusion Chest CT scan can provide a very important basis for the etiological diag-nosis of hemoptysis and the bronchoscopy examination also has an important value in the diagnosis and treatment of hemoptysis . The etiological and symptomatic treatment is predominat in pediatric hemoptysis ,and the majority have good prognosis .