Objective:To analyze the clinical characteristics of eosinophilic lung diseases(ELD) in children to enhance pediatricians′ understanding of ELD.Methods:In this retrospective cross-sectional study, a total of 149 children with ELD were recruited from Beijing Children′s Hospital, Capital Medical University between April 1, 2007 and March 31, 2022.Chi-square test, Fisher′s exact test, Mann-Whitney U test and Kruskal-Wallis test were used to analyze data and conclude clinical characteristics.Spearman correlation was used to analyze the correlation between eosinophils in peripheral blood and bronchoalveolar lavage fluid.Chi-square test and Kappa consistency test were used to compare the differences and consistency in diagnostic results between bronchoalveolar lavage fluid or lung biopsy and eosinophil elevation with chest imaging abnormalities. Results:(1)The isolated lung involvement was mostly caused by allergic bronchopulmonary aspergillosis(9 patients), and other system involvement by idiopathic hypereosinophilic syndrome(89 patients).(2)The main respiratory manifestations included coughing(90 cases, 60.4%) and expectoration(41 cases, 27.5%), while 23.5%(35 cases) of patients had no respiratory symptoms; 50.3% had digestive system involvement, and 40.9% had skin involvement.These were the two most commonly affected organs.(3)Spearman correlation was performed between eosinophils in peripheral blood and bronchoalveolar lavage fluid( r=0.3, P<0.05).Chi-square test was performed to compare ELD diagnosed by bronchoalveolar lavage fluid or lung biopsy with peripheral blood eosinophilia accompanied by abnormal chest imaging( P<0.05).Kappa consistency test(Kappa<0.2) showed poor consistency between the two diagnostic methods. Conclusions:ELD are present in children, and multiple etiologies may be pathogenic.Among children with ELD, the isolated lung involvement is mainly caused by allergic bronchopulmonary aspergillosis.The digestive system and skin are the most commonly affected organs, except for lungs.The correlation between eosinophil levels in peripheral blood and bronchoalveolar lavage fluid is poor.

Objective:To analyze the clinical characteristics of pulmonary vein stenosis (PVS) in children, and to explore its treatment and prognostic factors.Methods:The clinical data of 19 children with PVS treated in Beijing Children′s Hospital, Capital Medical University from October 2016 to March 2022 were analyzed retrospectively.There were 16 males and 3 females.The median age at diagnosis was (2.81±1.95) years.A descriptive analysis of clinical characteristics of children was made.Results:Of the 19 children, 14 cases (73.7%) had primary PVS and 5 cases (26.3%) had secondary PVS after surgery of anomalous pulmonary venous connection (APVC). Thirteen children (68.4%) had hemoptysis.In the hemoptysis children, 5 cases had life-threatening massive hemoptysis, and 11 cases (57.9%) had a history of recurrent respiratory tract infection or pneumonia.Other manifestations of hemoptysis included failure to thrive (6 cases), cyanosis (5 cases), and dyspnea (3 cases). Complications were pulmonary hypertension (6 cases) and right heart failure (3 cases). There were 16 cases (84.2%) of unilateral PVS and 3 cases of bilateral PVS.Interlobular septal thickening, grid shadow and ground glass opacities were found on CT of all PVS cases.Ten cases underwent surgery, and 2 cases of them received angioplasty, but restenosis occurred in both of them.Eight children underwent pulmonary lobectomy, and their clinical symptoms were all relieved after operation.Nine patients were treated conservatively, and 3 cases of them died of bilateral PVS secondary to APVC.The remaining 6 alive cases still had intermittent clinical symptoms during follow-up.Conclusions:Hemoptysis and recurrent respiratory tract infection are the main clinical manifestations of PVS in children, and life-threatening massive hemoptysis can occur.Lobectomy is an effective treatment for unilateral PVS.The prognosis of secondary PVS after APVC is poorer and its mortality is higher, compared with primary PVS.

Objective:To analyze the clinical characteristics, treatment course and prognosis of children with intermediate-high risk pulmonary embolism.Methods:The clinical data of 48 children with pulmonary embolism treated in Beijing Children′s Hospital, Capital Medical University from January 2017 to December 2021 were analyzed retrospectively.Including 12 intermediate-high risk cases and 36 low-risk cases.The clinical manifestations, laboratory results, treatment and prognosis were compared between groups by the t-test, rank sum test and Chi- square test with the yates continuity correlation or Fisher′ s exact test. Results:There were no significant differences in the sex and age between the intermediate-high risk group and the low-risk group.The proportions of patients with shortness of breath, dyspnea, cyanosis or hypoxemia were higher in the intermediate-high risk group than those of in low-risk group.Twelve children in the low-risk group did not have specific symptoms of pulmonary embolism.There were no significant differences in the D-dimer level, and the distribution of pulmonary embolism between the two groups (all P>0.05). However, the proportion of children with other thromboembolism in the intermediate-high risk group was significantly higher than that of the low-risk group, among which heart thrombosis was the most common (7 cases). There were no significant differences in the underlying diseases and thrombophilia between the two groups (all P>0.05). The treatment of the intermediate-high risk group was more active: 6/12(50.00%) patients in the intermediate-high risk group received reperfusion treatment, including 3 cases of systemic thrombolysis, 1 case of catheter thrombolysis, and 2 cases of thrombectomy.In the low-risk group, only 1 case was treated with systematic thrombolysis.Unfavorable outcomes were reported in 3/48 (6.25%) patients, including 1 death of massive bleeding after catheter-directed thrombolysis in the acute phase, 1 case of recurrent pulmonary embolism after self-decided withdrawal and 1 case of progression of pulmonary embolism that was managed by surgical thrombectomy, all of whom were in the intermediate-high risk group. Conclusions:Shortness of breath, dyspnea, cyanosis or hypoxemia and co-existed venous thromboembolism were more common in intermediate-high risk cases.The treatment regimen of was more aggressive, but the incidence of unfavorable outcomes was higher in intermediate-high risk group; further research is needed to determine the risk factors for intermediate-high risk pulmonary embolism in children.

Objective:To investigate the etiology of pleural effusion in hospitalized children in Beijing Children′s Hospital.Methods:Clinical information of children with pleural effusion admitted to Beijing Children′s Hospital Affiliated to Capital Medical University from January 2016 to December 2018 was retrospectively analyzed.According to the etiology, the children were divided into infection group (parapneumonic pleural effusion, tuberculous pleurisy and empyema) and non infection group.According to the age, the children were further divided into ≤ 3 years old, >3-7 years old and > 7 years old groups.Classification of statistics was performed, and the etiology of pleural effusion were retrospectively analyzed.Results:Among the 1 165 children with pleural effusion, 746 cases(64.0%) were infected with pleural effusion, 697 cases (697/746, 93.4%) of who were parapneumonic effusion.In patients with parapneumonic effusion, 457 cases (61.3%) had Mycoplasma pneumonia (MP) infection.Infectious pleural effusion was more common in children >7 years old(339/479 cases, 70.8%), while non-infectious pleural effusion was prevalent in children under 3 years old(188/324 cases, 58.0%). The difference was statistically significant ( χ2=96.33, P<0.05). Among the patients with non-infectious pleural effusion, 239 cases (239/419 cases, 57.0%) had multi-system diseases and 97 cases (97/419 cases, 23.2%) had malignant pleural effusion.All the 18 deaths were non-infectious pleural effusion. Conclusions:The leading reason for pleural effusion in children is infection.The most prevalent symptom is parapneumonic effusion, which is mainly caused by MP.

Objective:To summarize the clinical features of children with autosomal dominant hyper-IgE syndrome (AD-HIES) and the differential diagnosis of hyper-IgE syndrome and allergic diseases as well.Methods:All clinical data, including general information, clinical features, and genetic changes, from 7 children with AD-HIES who were diagnosed in Beijing Children′s Hospital Affiliated to Capital Medical University from April 2016 to June 2020 were analyzed retrospectively.The diagnostic criteria are based on the National Institutes of Health′s (NIH)′s hyper-IgE syndrome score and combined with the results of gene detection, shown as follows: (1) NIH score over 40, with signal transducer and activator of transcription 3 gene ( STAT3) pathogenic mutation; (2) NIH score between 20 and 40, with reported STAT3 pathogenic mutation; (3) NIH score less than 20 points was excluded. Results:There were 3 males and 4 females.The onset age of 7 cases was within 2 months after birth, and the mean age at diagnosis was 3 years old.All seven cases had recurrent skin or lung infections, with 4 cases having skin and lung infections, 1 case of skin abscesses at the BCG vaccination site, and 2 cases without skin infection suffering from recurrent pneumonia.The mean onset age of skin abscess in 5 cases was 1.5 years, and pus culture of 3 cases were Staphylococcus aureus.Four cases developed bullae and 6 cases had lung infections.Four cases had otitis media, and oral thrush was seen in 4 cases.One case of skin and lung infection developed liver abscess and sepsis.Seven cases had eczema, which was disco-vered in the neonatal period for 6 cases.Four cases had the symptoms of eczema for the first visit.Two cases had food allergy, and 1 case had recurrent wheezing within 1 year old.The serum IgE level and blood eosinophil count in 7 children were elevated.All children had heterozygous pathogenic mutations in STAT3.Six patients had de novo mutations.There were 6 different mutation sites.The 4 mutation sites were reported: c.1145G>A, c.1144C>T, and c. 1699A>G were missense mutations, and c. 1139+ 5G>A was splicing mutation.Two mutation sites had not been reported: c.1031A>C was missense mutation, and c. 2050G>T was nonsense mutation.The pathogenic grade of them were likely pathogenic, and the NIH score of 2 cases were above 40 score, which was consistent with the clinical diagnosis of hyper-IgE syndrome. Conclusions:Eczema is a common and early clinical manifestation of hyper-IgE syndrome, along with elevated IgE levels and eosinophil counts that need to be differentiated from allergic diseases.On the contrary, it often had recurrent skin abscesses or pneumonia, which was prone to bullae.The clinical manifestations of young children were atypical, and genetic testing was helpful for early diagnosis.

Objective: To analyze the practical value of D-dimer in diseases condition judgment and prognosis evaluation of childhood Mycoplasma pneumoniae pneumonia (MPP). Methods: Retrospective analysis was performed on clinical data of 606 MPP at Department of Respiratory, Beijing Children′s Hospital, Capital Medical University from January 2009 to July 2017, and the subjects were divided a severe group (298 cases) and a moderate group (308 cases) according to severity.By comparing clinical characteristics, laboratory tests and imaging findings, multivariate Logistic regression analysis for significant single factors was accomplished, which was to find out the independent factors affecting the severity of childhood MPP in acute phase.Receiver operating characteristic (ROC) curves were drawn in the area under the curve (AUC) and the diagnosis threshold value was calculated, which could be used to judge the predicators affecting the severity judgment of childhood MPP in acute phase.And the prognosis was judged according to the convalescent fiberoptic bronchoscopic indicators in recovery phase. Results: The levels of white blood cells (WBC)[(10.25±3.76)×10⁹/L], neutrophil(Neu)[(7.31±3.76)×10⁹/L], platelet (PLT)[(334.66±143.80)×10⁹/L], C-reactive protein(CRP)[(69.00±80.50) mg/L], erythrocyte sedimentation (ESR)[(39.38±26.29) mm/1h], lactate dehydrogenase (LDH)[(436.61±248.96) IU/L], fibrinogen(Fib)[(4.61±1.36) g/L] and D-dimer [(2.09±1.66) mg/L]in the severe group were higher than those in the moderate group[(7.55±3.14)×10⁹/L, (4.77±2.54)×10⁹/L, (291.60±109.19)×10⁹/L, (23.40±42.50) mg/L, (30.25±16.18) mm/1 h, (318.05±116.97) IU/L, (4.18±0.88) g, (0.58±0.72) mg/L], and the differences were statistically significant (all P<0.01). The levels of Neu, PLT, CRP, LDH and D-dimer were independent and relevant factors for the severity of acute MPP.The area under each ROC curve was Neu 0.719, PLT 0.592, LDH 0.675, CRP 0.749, D-dimer 0.848, and each diagnostic threshold was 6.5× 10⁹/L, 265.5×10⁹/L, 417.5 IU/L, 28.9 mg/L, 0.73 mg/L, respectively.Obviously, D-dimer had the highest sensitivity and specificity for the severe MPP.There was a significant difference in D-dimer level between the endobronchial inflammation group and the subbronchial stenosis, poor ventilation and occlusion group of fiberoptic bronchoscopy [(1.11±0.26) mg/L vs.(2.14±1.84) mg/L, t=-5.870, P<0.05]. Conclusion D-dimer levels can be used as one of the most sensitive indicator for determining the severity and prognosis of MPP.

OBJECTIVE To investigate and analysis of hearing status and characteristics of China civil aviation air traffic controllers(ATC). METHODS With cluster random sampling, air conduction threshold data of 1498 ATC, who had finished the class Ⅲa medical assessment this year in a certain area were studied. The subjects were tested by pure tone audiometry, the prevalence rate of speech and high frequency hearing loss between gender groups were compared; After age correction, the threshold of different frequencies were compared between age groups. RESULTS The prevalence rate of hearing loss at speech frequency was 6.68％ in male and 1.97％ in female. The result of high frequency was 7.87％ and 1.23％ respectively. Both the threshold and prevalence rate of hearing loss of every frequency were higher in male(P<0.05); The threshold of 3000 Hz

Objective To study the genetic mutation of surfactant dysfunction in children with interstitial lung disease.Methods The surfactant protein B (SFTPB),surfactant protein (SFTPC),ATP binding cassette transporter A3 (ABCA3) gene sequence detection were conducted for 26 patients with interstitial lung disease who were onset before 2 years old or without specific etiology after the biopsy during January 2012 to December 2017 in Beijing Children's Hospital Affiliated to Capital Medical University,then the result of gene sequence detection and the clinical data were analyzed.The method of gene analysis is PCR amplify and Sanger sequencing.Results (1) In total,4 cases of abnormal gene mutations had been found,of which 3 cases were pathogenic SFTPC gene mutations,such as c.218T ＞ C,IVS4,+ 1G ＞ C,c.115G ＞ T,1 case was ABCA3 compound heterozygous mutations,such as C.3913 T ＞ C and heterozygous deletion in Exon 13-18.(2)There were also 7 uncertain or suspected cases.Four cases were undefined pathogenic SFTPC mutations,such as c.406G ＞ C,IVS4,+ 12 G ＞ G,c.364T ＞ C,c.68G ＞ A.Three cases had been found with two ABCA3 heterozygous gene mutations,which were not confirmed by parents.The lung pathology of the patient with SFTPC (IVS4,+ 12 T ＞ G) and heterozygous ABCA3 (c.737C ＞ T)gene mutations was amyloidosis,and there was similar history in his family.(3) No pathogenic gene mutation was found in the 15 cases.No pathogenic SFTPB gene mutation was found in all the patient.Lung biopsy was performed in 2 cases of SFTPC c.115G ＞ T and ABCA3 compound heterozygous mutation,the lung tissue of this 2 cases were nonspecific interstitial pneumonia (NSIP).One case of SFTPC c.115G ＞ T had died at the age of 14 years old and 1 case of IVS4,+ 1G ＞ C had died at the age of 11 months old.Only 1 case of SFTPC c.218T ＞ C gene mutation with the similar family history,had improved significantly after glucocorticoid treatment,another case of ABCA3 compound heterozygous mutation was mildly improved after the glucocorticoid treatment.The chest CT/high resolution computed tomography displayed diffuse ground glass opacity in 4 cases,and cystic in 2 cases,all 2 cases with cystic were cases of SFTPC mutation and were dead.Conclusions The gene mutation of surfactant dysfunction is associated with interstitial lung disease in children.The pathology feature can be NSIP,and the prognosis may be poor in some cases,and the treatment of the corticosteroids may be effective in few case.

Objective: To analyze the clinical characteristics, diagnosis and treatment of bronchopulmonary foregut malformation(BPFM). Method: The clinical manifestations, imaging findings and treatment of 8 patients with BPFM were analyzed retrospectively from January 2006 to May 2016 in Beijing Children′s Hospital. Result: The age of children varied from 2 months to 7 years and 3 children were male while 5 female. Symptoms showed cough in 6 cases, fever in 4 cases, bucking when intaking of fluids or foods in 3 cases, tachypnea in 1 case, wheezing in 1 case, vomiting in 1 case, haematemesis in 1 case Pulmonary signs were decreased breath sounds in 4 cases, phlegm rale in 3 cases, shortness of breath in 2 cases, wheeze in 1 case, and retraction in 1 case. The upper gastrointestinal series showed abnormal fistulous tracts arising from the esophagus or the gastric fundus and extending into the mass in the lung. CT showed pulmonary sequestration and prompted the tube between lung and esophagus. Six children underwent pneumonectomy and esophageal fistula repair. They were discharged and their symptoms were improved. Two cases of children were discharged from a hospital without surgery. Conclusion Bronchopulmonary foregut malformation usually has its onset in early stage of life. The most common symptoms include recurrent pneumonia or bucking when intaking of fluids or foods. CT can demonstrate the bronchopulmonary sequestration and evaluate the communication with the gastrointestinal tract. The upper gastrointestinal series can demonstrate the abnormal tract directly. Pneumonectomy and esophageal fistula repair are the treatment of this disease.