Abstract Objective To explore the prognosis of children with idiopathic pulmonary hemosiderosis (IPH) after long-term maintenance of glucocorticoid therapy. Methods This ambidirectional observational study included children with IPH admitted to the Department of Respiratory, Beijing Children’s Hospital, Capital Medical University, between January 2010 and July 2021. Their clinical characteristics were collected from medical records and during follow-up. Results A total of 211 children (88 males,123 females, age of onset: 3.42 [0.33–12.75] years, the median time from onset to diagnosis: 2.8 months [4 days-8 years]) with IPH were included. All children received glucocorticoid therapy in the acute alveolar hemorrhage phase. After discharge, 5 (2.4%) children lost to follow-up, 11 (5.2%) died during follow-up, and a total of 195 patients survived, with a median follow-up time of 5.52 (1.43, 12.58) years, the 5-year and 10-year survival rate were 95.9% and 86.2%, respectively. Of the 195 patients, Clinical remission was achieved in 65 patients (33.3%), including 61 patients treated with glucocorticoid alone and 4 patients treated with immunosuppressive agents. 109 (55.9%) were in a stable phase with medications, and 21 (10.8%) had no clinical remission. Of the 195 patients who survived, 141(72.3%) were treated with glucocorticoid alone and 54(26.7%) with immunosuppressive agents. Four (2.05%) patients developed secondary cataracts, and 33 (16.92%) patients were short in stature. Conclusion Long-term low-dose glucocorticoid maintenance therapy might improve the prognosis of IPH children, but the side effects should be monitored.

Abstract Background Thromboembolism is less common in children than in adults, but it is frequently associated with Mycoplasma pneumoniae infection in many cases. This study aimed to investigate the clinical characteristics of pediatric M. pneumoniae pneumonia complicated with thromboembolism. Methods Hospitalized patients with M. pneumoniae pneumonia complicated by thromboembolism were enrolled from January 2012 to December 2021 in Beijing Children's Hospital, Capital Medical University, China. The data on clinical manifestations, laboratory tests, and treatment were evaluated. Results A total of 49 cases were enrolled, with a mean age of 7.9 years old, including 27 boys and 22 girls. Consolidation of pulmonary lobe or segment was observed in 95.9% (47/49) of the cases, whereas interstitial change was found only in two patients; 85.7% (42/49) of patients had pleural effusion. Pulmonary vascular thromboembolism was most common in 35 patients, whereas 13 cases had thromboembolism of multiple anatomic sites. The levels of C‐reaction protein, lactate dehydrogenase, and erythrocyte sedimentation rate were all increased, with a mean value of 54.08 ± 52.27 g/L, 451.12 ± 218.76 U/L, 43.40 ± 29.43 mm/h, respectively. Blood coagulation test showed that all 49 patients had elevated D‐dimer values (median 3.81 ng/ml, range, 0.34–48 ng/ml) and normal PT. aPTT.LA was positive in 74.3% (26/35) of the cases. aCL‐IgM was positive in 66.7% (26/39) of the cases. aβ2GPI‐IgM was positive in 79.4% (27/34) of the cases. The prognosis was generally good in this group. Conclusion Pulmonary arteriovenous thromboembolism is the most common thromboembolism complicated in MPP, and cerebral artery embolism and cardiac thrombosis are common in extrapulmonary thromboembolism. In the cases of MPP with thromboembolic complications, pulmonary consolidation with pleural effusion is the main characteristic. About two thirds of the cases are positive for antiphospholipid antibodies.

Background: Primary ciliary dyskinesia (PCD) is a rare genomic disorder. The phenotype heterogeneity depends on the genotype. Critical genes mutant like CCNO had severe respiratory disease, while limited data are available until now. Case presentation: We presented a patient with neonatal respiratory distress at birth, and had cough with wheeze for 8 years as flows. According to clinical and imaging findings, screenings of PCD related genes showed compound heterozygous mutation of CCNO. We also overviewed the literature of CCNO-related PCD and compared to our patient. A total of 43 patients from 30 families were enrolled. Approximately 57.1% (24/42) of individuals were born in the consanguineous marriage family. Most patients developed onset symptoms at neonate, accounted for 85.3%. Recurrent respiratory tract infection (83.3%), neonatal respiratory distress (69.0%), and sinusitis/rhinorrhea (50.0%) were major manifestations, the subsequents were chronic cough 15/42(35.7%), and recurrent otitis media (28.6%); hear losing, infertility, congenital heart defects and hydrocephalus were rare, but heterotaxy was never seen. Bronchiectasis was the most common radiologic findings, while the patient in our study presented with special findings of diffuses small nodular in both lungs like diffuse pan-bronchiolitis (DPB). Thirteen different CCNO variants were identified with most located in exon 1 (79.1%, 34/43). Our participant was identified previously reported c.263_267dupAGCCC and c.258_262dupGGCCC mutation from her mother and father respectively. Conclusion: CCNO variants are rare in PCD patients, but it causes more severe phenotypes than the other genes. Neonatal respiratory distress is common, and diffuse bronchiolitis could be the radiologic feature of CCNO-related PCD.

While most missense mutations of the IKBKG gene typically result in Ectodermal Dysplasia with Immunodeficiency, there have been rare reported instances of missense mutations of the IKBKG gene causing both Incontinentia Pigmenti (IP) and immunodeficiency in female patients. In this study, we described an atypical IP case in a 19-year-old girl, characterized by hyperpigmented and verrucous skin areas over the entire body. Remarkably, she experienced recurrent red papules whenever she had a feverish upper respiratory tract infection. Immunohistochemical staining unveiled a substantial accumulation of CD68 + macrophages alongside the TNF-α positive cells in the dermis tissue of new pustules, with increased apoptotic basal keratinocytes in the epidermis tissue of these lesions. Starting from the age of 8 years old, the patient suffered from severe and sustained chronic respiratory mucous membrane scar hyperplasia and occluded subglottic lumen. In addition to elevated erythrocyte sedimentation rate values, inflammatory cells were observed in the pathologic lesions of endobronchial biopsies and Bronchoalveolar Lavage Fluid (BALF) smear. Further histological analysis revealed a destructive bronchus epithelium integrity with extensive necrosis. Simultaneously, the patient experienced recurrent incomplete intestinal obstructions and lips contracture. The patient’s BALF sample displayed an augmented profile of proinflammatory cytokines and chemokines, suggesting a potential link to systemic hyperinflammation, possibly underlying the pathogenic injuries affecting the subglottic, respiratory, and digestive systems. Furthermore, the patient presented with recurrent pneumonias and multiple warts accompanied by a T + B low NK low immunophenotype. Next generation sequencing showed that the patient carried a novel de novo germline heterozygous missense mutation in the IKBKG gene (c. 821T>C, p. L274P), located in the highly conserved CC2 domain. TA-cloning sequencing of patient’s cDNA yielded 30 mutant transcripts out of 44 clones. In silico analysis indicated that the hydrogen bond present between Ala270 and Leu274 in the wild-type NEMO was disrupted by the Leu274Pro mutation. However, this mutation did not affect NEMO expression in peripheral blood mononuclear cells (PBMCs). Moreover, patient PBMCs exhibited significantly impaired TNF-α production following Lipopolysaccharide (LPS) stimulation. X-chromosome inactivation in T cells and neutrophils were not severely skewed. Reduced levels of IκBα phosphorylation and degradation in patient's PBMCs were observed. The NF-κB luciferase reporter assay conducted using IKBKG -deficient HEK293T cells revealed a significant reduction in NF-kB activity upon LPS stimulation. These findings adds to the ever-growing knowledge on female IP that might contribute to the better understanding of this challenging disorder.

目的:探讨儿童延髓肿瘤相关的睡眠呼吸障碍的临床特征。方法:回顾分析3例延髓肿瘤伴随的睡眠呼吸障碍患儿的临床资料，并检索国内外相关文献报道进行病例总结。结果:3例患儿中，例1、例2均以睡眠呼吸障碍为表现发现延髓肿瘤，例3为延髓肿瘤术后发现睡眠呼吸障碍，3例患儿均表现为中枢性睡眠呼吸暂停，例2伴有低通气；例2及例3均给予双水平正压通气治疗后症状有所改善，长期随访预后不佳。总结国内外报道的延髓肿瘤合并睡眠呼吸障碍的9例病例，以中枢性呼吸暂停为最常见表现(8例)，其他表现包括阻塞性睡眠呼吸暂停(4例)，中枢性低通气(2例)和睡眠结构紊乱(2例)；延髓肿瘤术后随访显示睡眠呼吸障碍持续存在，需要长期的双水平呼吸支持治疗。结论:延髓肿瘤可引起儿童严重的睡眠呼吸障碍，以中枢性呼吸暂停为主，但症状容易隐匿不易识别；延髓肿瘤引起的中枢性呼吸暂停即使在手术后仍可能持续存在。

Neuroendocrine cell hyperplasia of infancy（NEHI）is an pulmonary dysmaturation disorder of interstitial lung disease in infants. The etiology of NEHI is unknown. The clinical features of NEHI are tachypnea,retraction,crackles and hypoxemia. The diagnosis mainly depends on immunohistochemistry of bombesin. The number of neuroendocrine cells（NECs）increases in the distal airway.NECs exist in at least 75% of the airways; NECs account for10% in each airway; there is lack of the features diagnosed as the other airway and interstitial diseases. High resolution computed tomography（HRCT）of lungs shows central ground glass opacities,mainly involving right middle lobe and left lingula,and air trapping in other areas. There is no specific treatment for NEHI, mainly oxygen therapy and basic nutritional support treatment. NEHI is a benign process,no death has been reported,and it is relieved with age.

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:The etiology of diffuse alveolar hemorrhage (DAH) in childhood is often unknown, and it may be an early manifestation of rheumatic disease. Juvenile idiopathic arthritis (JIA) is one of the most common rheumatic diseases in children, but DAH as an onset manifestation of JIA is rare. This study summarizes the clinical characteristics of patients with JIA presenting as DAH.METHODS:We retrospectively analyzed the age of onset, clinical manifestations, imaging features, treatments, and prognosis of five cases of JIA presenting as DAH.RESULTS:Themedian age at DAH onset was 6 months (range, 2 months-3 years). Pallor was the most common manifestation of onset (5/5). Other symptoms included cough (2/5), tachypnea (2/5), hemoptysis (1/5), cyanosis (1/5), and fatigue (1/5). Imaging showed ground-glass opacity (GGO) (5/5), subpleural or intrapulmonary honeycombing (4/5), consolidation (3/5), interlobular septal thickening (2/5), and nodules (1/5). Anticitrullinated protein antibodies (ACPA) and rheumatoid factor (RF) were positive in five children (5/5), and antinuclear antibody (ANA) was positive in four children (4/5). ANA in three children and ACPA/RF in one child were positive before the onset of joint symptoms. The median age at the onset of joint symptoms was 3 years and 9 months (2 years and 6 months-8 years). Joint symptoms were mainly characterized by joint swelling, pain, and difficulty walking, and the most commonly affected joints were the knees, ankles, and wrists. After the diagnosis of DAH, the five patients were treated with glucocorticoids. Alveolar hemorrhage was effectively controlled in three cases, but the other two patients still had anemia and poor improvements in chest imaging. After joint symptoms, the patients were treated with glucocorticoids combined with diclofenac, disease-modifying antirheumatic drugs, and biological agents. Alveolar hemorrhage was in remission, and joint symptoms were relieved in the five cases.CONCLUSION:DAH can be the first clinical manifestation of JIA, and joint involvement occurs 1-5 years later. Children with DAH who are positive for RF, ACPA, and/or ANA and have GGO accompanied by honeycombing on imaging should be concerned about their joint involvement in future.

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.

目的 研究北京地区儿童肺炎支原体(MP)感染情况和流行病学特征,为新冠肺炎疫情下本地区MP感染的防治提供理论依据.方法 选择2018年1月至2021年12月在首都医科大学附属北京儿童医院就诊的5951例社区获得性肺炎患儿为研究对象.所有患儿采集咽拭子标本,采用实时荧光核酸恒温扩增技术(SAT)检测MP靶标RNA.结果 5951例患儿的MP总检出率为36.62％(2179/5951).4年中MP出现一次暴发流行(2018年至2019年),流行年检出率可高达42.81％.新冠肺炎疫情爆发后,MP感染率明显降低;不同季节MP检出率差异具有统计学意义(P<0.0001),总检出率最高季节为秋季(47.48％)和夏季(41.78％);新冠肺炎疫情爆发后,MP的季节流行特点发生改变;各年龄组间MP检出率差异具有统计学意义(P<0.0001),学龄组患儿MP总检出率最高(60.24％),其次为青春组(49.47％),婴儿组MP总检出率最低(5.97％),且新冠肺炎疫情前后,MP感染高发人群未发生改变;女性患儿MP总检出率(38.75％,1037/2676)高于男性患儿(34.87％,1142/3275),差异具有统计学意义(χ2=9.56,P=0.002);新冠肺炎疫情爆发后,不同性别患儿间MP检出率差异无统计学意义(P>0.05).结果 MP是北京地区儿童社区获得性肺炎的主要病原体,其分布特点具有季节、年龄和性别差异.新冠肺炎疫情下,MP感染率明显降低,但无显著性别差异,且高发季节发生改变.

Background: Gastric adenocarcinoma (GA) is a heterogeneous tumor, and the accurate classification of GA is important. Previous classifications are based on molecular analysis and have not focused on GA with the primitive enterocyte phenotype (GAPEP), a unique subtype with a poor prognosis and frequent liver metastases. New substituted molecular (SM) classifications based on immunohistochemistry (IHC) are needed.Methods: According to the IHC staining results, we divided 582 cases into six types: mismatch repair deficient (dMMR), Epstein-Barr virus associated (EBVa), the primitive enterocyte phenotype (PEP), the epithelial mes-enchymal transition (EMT) phenotype, not otherwise specified/P53 mutated (NOS/P53m) and not otherwise specified/P53 wild-type (NOS/P53w). We analyzed the clinicopathological features, the immune microenviron-ment (PD-L1, CD8) and expression of HER2 and VEGFR2 of those types.Results: There were 31 (5.3%) cases of the dMMR type, 13 (2.2%) cases of the EBVa type, 44 (7.6%) cases of the PEP type, 122 (21.0%) cases of the EMT type, 127 (21.8%) cases of the NOS/P53m type and 245 (42.1%) cases of the NOS/P53w type. Patients with the dMMR type had the best survival ( P < 0.001). Patients with the EBVa type were younger ( P < 0.001) and had higher PD-L1 and CD8 expression ( P < 0.001) than other patients. Patients with the EMT type exhibited poor differentiation and a higher rate of abdominal metastasis. Patients with the NOS/P53m and PEP types had the worst survival rates and the highest PD-L1/HER2/VEGFR2 expression levels among all patients ( P < 0.001).Conclusion: Different SM classifications have different clinicopathological features and expression patterns, which indicate the probable clinical treatment strategies for these subtypes.

间质性肺疾病(interstitial lung disease,ILD)是一组原因不同的慢性肺疾病,先天性表面活性物质功能缺陷是婴幼儿ILD的重要原因之一,在法国和英国分别占婴幼儿ILD的17.5％[1]和7.5％[2].编码肺表面活性物质蛋白(surfactant protein,SP)的基因(SFTP)及其相关代谢通路的基因,包括STPB、SFTPC、ATP结合盒转运子A3 (ATP-binding cassette transporter A3,ABCA3)出现突变,则会引起表面活性物质功能障碍,不仅可引起足月新生儿出生后即出现呼吸窘迫综合征(respiratory distress syndrome,RDS),也可引起儿童期ILD并出现持续性低氧血症等表现[3].现对2018年6月在首都医科大学附属北京儿童医院确诊的ABCA3基因单亲二倍体纯合突变导致的1例婴幼儿ILD患儿临床特点进行总结,并对ABCA3基因突变所致ILD的临床表型与基因型关系进行文献复习.

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 explore the application value of treatment-related markers PD-L1, PD-L2, CD30, CD23, BCL-2, BCL-6, MUM1 and GATA3 in the diagnosis and prognostic evaluation of primary mediastinal B-cell lymphoma(PMBL). Methods A retrospective study was conducted on 34 patients diagnosed with PMBL, and 31 patients with DLBCL-NOS which was not primary in the mediastinum were taken as control group. The expressions of 8 proteins were detected by IHC staining. Results The median percentages of tumor cells with PD-L1, PD-L2 and CD30 expression in PMBL group were 70% (30%, 90%), 25% (0, 70%) and 17.5% (0, 60%) respectively, which were significantly higher than those in the DLBCL-NOS group (P < 0.05). The positive rates of CD30 and CD23 in PMBL group were 61.76% (21/34) and 76.47% (26/34) respectively, significantly different with those in the DLBCL-NOS group (P=0.000). The survival curve of PMBL patients with CD30 or BCL-6 expression showed a trend of poor prognosis, despite the P value was > 0.05. Conclusion The high expression levels of PD-L1, PD-L2 and CD30 in PMBL are helpful to accurately identify more patients who may respond to immune or targeted therapy. Immunohistochemical staining of PD-L1, PD-L2, CD30 and CD23 is helpful for the differential diagnosis of PMBL and DLBCL-NOS. As candidate prognostic indicators of PMBL, CD30 and BCL-6 should be further studied in a larger number of samples.

目的 评价基于胸部X线卷积神经网络(CNN)模型诊断儿童不同病原体社区获得性肺炎(CAP)的价值.方法 纳入1769例CAP患儿,根据病原学诊断分为病毒组(n=487)、细菌组(n=496)及肺炎支原体(MP)组(n=786),对比组间胸部X线征象的差异;将患儿以7∶1∶2比例随机分为训练集、验证集和测试集,对测试集患儿根据性别和年龄分为不同亚组进行分层分析.基于胸部X线片分割全肺和病灶ROI,分别训练全肺模型和局部模型,通过混淆矩阵评估2种模型的整体效能;绘制受试者工作特征(ROC)曲线,计算曲线下面积(AUC),评价2种模型诊断不同病原CAP的效能;采用Delong检验比较模型诊断效能的差异.结果 3组病变累及范围、受累肺组织密度改变特点、肺过度通气及空洞差异均有统计学意义(P均＜0.05).全肺模型及局部模型诊断不同病原CAP的准确率分别为61.85％及58.04％,精确度分别为63.77％及54.05％.全肺模型和局部模型诊断MP性CAP的效能最佳,AUC分别为0.798及0.819;全肺模型诊断病毒及细菌性CAP的AUC均大于局部模型(P均＜0.05).全肺模型和局部模型诊断测试集中男性亚组和女性亚组不同病原CAP的AUC、诊断高年龄亚组和低年龄亚组病毒性及细菌性CAP的AUC差异均无统计学意义(P均＞0.05),诊断高年龄亚组和低年龄亚组MP性CAP的AUC差异均有统计学意义(P均＜0.05).结论 基于胸部X线片建立CNN模型诊断儿童不同病原体CAP的效能较好;全肺模型优于局部模型,2种模型均对MP性CAP诊断效能最佳.

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.

Both gastric adenocarcinoma with primitive enterocyte phenotype (GAPEP) (including hepatoid adenocarcinoma) and alpha-fetoprotein (AFP)-producing gastric adenocarcinoma have poor prognoses. However, the value of the serum AFP test and AFP/glypican-3 (GPC3)/spalt-like transcription factor 4 (SALL4) immunohistochemistry is still not clear, and these two methods have not yet been thoroughly compared. We collected 421 consecutive non-neoadjuvant surgically or endoscopically resected gastric adenocarcinoma patients with serum AFP results before surgery (group A). We divided these cases into serum AFP-high (sAFP-H) and serum AFP-normal (sAFP-N) by serum AFP levels, and into GAPEP (expressing AFP, GPC3, or SALL4) and non-GAPEP (nGAPEP) by AFP/GPC3/SALL4 immunohistochemistry results. We also collected 12 non-resected gastric adenocarcinoma patients with serum AFP ≥ 7 ng/mL before treatment (group B). We analyzed these patients’ clinicopathological characteristics and prognoses. Seventeen (4.04%) patients in group A were sAFP-H. These patients were younger and mainly had tubular adenocarcinoma with later pT (P = 0.014) and pN (P = 0.047) categories and more lymphovascular invasion (P < 0.001), perineural spread (P = 0.008), and metastases or recurrence (P < 0.001). For immunohistochemistry, 34 (8.08%) cases were GAPEP, and GAPEP cases also had later pT categories than nGAPEP cases (P = 0.001). Most group B patients with elevated serum AFP (especially > 1000 ng/mL) had simultaneous metastases, mainly liver metastases. Both the serological method and immunohistochemical method were useful for predicting prognosis (AUC sAFP = 0.625, AUC A/G/S-IHC = 0.723, z statistic = 1.726, P = 0.084). The serum AFP level (especially > 1000 ng/mL) is more specific (100%), and immunohistochemistry is more sensitive (50%). Both the serum AFP level and immunohistochemical expression of AFP/GPC3/SALL4 can be used to indicate a poor prognosis for gastric adenocarcinoma.