ObjectiveTo report a case of severe Mycoplasma pneumoniae pneumonia complicated with necrotizing laryngotracheobronchitis, and to detail its clinical manifestations, auxiliary examinations and treatment process, so as to enhance clinicians’ understanding of necrotizing laryngotracheobronchitis and emphasize the indispensable role of bronchoscopy in the diagnosis and treatment of necrotizing laryngotracheobronchitis.MethodsReport on a child with severe Mycoplasma pneumoniae pneumonia complicated by necrotizing laryngotracheobronchitis; the child was a 7 - year - old boy; the clinical features included recurrent high fever, cough and hoarseness; physical examination revealed fair spirits, pharyngeal congestion, grade I tonsillar enlargement, coarse breath sounds in both lungs, and no rales heard; auxiliary examinations confirmed Mycoplasma pneumoniae infection; under bronchoscopy, necrosis of the bronchial mucosa and exposure of cartilage were observed, and the pathological examination of the lesion showed inflammatory necrosis; after anti - infection treatment and multiple bronchoscopic lavage treatments, the child's condition improved and he was discharged from the hospital.ConclusionNecrotizing laryngotracheobronchitis can present with diffuse necrosis of the airway mucosa, has an acute onset and a severe disease progression, and can lead to death; in this case, the child developed necrosis of the tracheal and bronchial mucosa after Mycoplasma pneumoniae infection, and there is currently no report of necrotizing laryngotracheobronchitis occurring after Mycoplasma pneumoniae infection; in treatment, anti - infection treatment should be combined with bronchoscopic lavage treatment.

Background:This study aimed to investigate characteristic changes in the upper respiratory tract (URT) microbiome and metabolome in children with asthma and explore their associations with lung function. Methods:Children with asthma aged 6 years and above admitted to the Children's Hospital of Soochow University from December 2022 to December 2023 comprised the study group. Age-matched healthy children undergoing physical examinations in the Department of Child Health were recruited as controls. Throat swabs were collected for microbiome detection using 16S rDNA sequencing and metabolomics analysis using liquid chromatography-mass spectrometry (LC-MS). Results:(1) Significant differences in alpha and beta diversity were observed among the control group (H), chronic persistent asthma group (CA), and acute exacerbation group (AA). In both CA and AA groups, FVC% predicted (FVC%/Pred) and FEV1% predicted (FEV1%/Pred) were negatively correlated with URT microbiota abundance. Actinobacillus abundance was positively correlated with FEV1%/Pred, FEV1/FVC, FEF25%/Pred, FEF50%/Pred, and FEF75%/Pred. (2) Metabolite differences between CA and AA groups were analyzed, and the top 5 differential metabolites were evaluated for their accuracy as asthma assessment biomarkers. L-carnitine showed an AUC > 0.9, with a sensitivity of 85.7% and specificity of 85%. Other differential metabolites, including monoisobutyl phthalate, 4-hexyl-2,5-dimethyloxazole, and dibutyl phthalate, correlated with several lung function indices. The most relevant differential metabolic pathways included arginine biosynthesis, alanine-aspartate-glutamate metabolism, central carbon metabolism in cancer, and D-amino acid metabolism. Conclusion:The URT microbiota in asthmatic children exhibits alterations in composition, structure, and diversity, with lower diversity in acute asthma compared to chronic persistent asthma. At the genus level, some microbiota (Actinobacillus, Fusobacterium) were correlated with FEV1%/Pred, FEV1/FVC, FEF25%/Pred, FEF50%/Pred, FEF75%/Pred. The differential metabolite L-carnitine may be a potential biomarker for asthma assessment.

Background: Neutrophilic asthma is characterized by the predominant infiltration of neutrophils in airway inflammation. Objective: To explore the therapeutic potential of an antibody against the inducible T cell co-stimulator ligand (ICOSL) in a mouse model of neutrophilic asthma. Methods: Female BALB/c mice were randomly assigned to different groups. They were then injected with ovalbumin (OVA)/lipopolysaccharides (LPS) to induce neutrophilic asthma. The mice were then treated with either anti-ICOSL (the I group), control IgG (the G group), or no treatment (the N group). Additionally, a control group of mice received vehicle PBS and was labeled as the C group (n=6 per group). One day after the last allergen exposure, cytokine levels were measured in plasma and bronchoalveolar lavage fluid (BALF) using ELISA. After analyzing and categorizing BALF cells, the lung tissues were examined histologically and immunohistochemically. Results: Administering anti-ICOSL resulted in a significant decrease in the total number of inflammatory infiltrates and neutrophils found in BALF. Moreover, it led to a decrease in the levels of interleukin (IL)-6, IL-13, and IL-17 in both BALF and plasma. Additionally, there was an increase in IFN-gamma levels in the BALF of asthmatic mice (p<0.05 for all). Treatment with anti-ICOSL also reduced lung interstitial inflammation, mucus secretion, and ICOSL expression in asthmatic mice. Conclusion: The treatment of anti-ICOSL effectively improved lung interstitial inflammation and mucus secretion in mice with neutrophilic asthma by restoring the balance of Th1/Th2/Th17 responses. These findings indicate that blocking the ICOS/ICOSL signaling could be an effective way to manage neutrophilic asthma.

Objective:In order to explore the impact of corona virus disease 2019(COVID-19)on the hospitalization of children with bronchiolitis and to improve clinicians′ understanding of the characteristics of bronchiolitis during the COVID-19 epidemic.Methods:This was a multicenter clinical study, and the data have been collected from 23 children′s medical centers in China.All the clinical data were retrospectively collected from children with bronchiolitis who were hospitalized at each study center from January 1, 2019 to December 31, 2021.The results included gender, age at hospitalization, length of stay, respiratory syncytial virus(RSV) test results, severity rating, ICU treatment, and the total number of children hospitalized with respiratory tract infection during the same period.The clinical data of children with bronchiolitis in 2019 before COVID-19 epidemic and in 2020、2021 during COVID-19 epidemic were statistically analyzed and compared.Results:According to a summary of data provided by 23 children′s medical centers, there were 4 909 cases of bronchiolitis in 2019, 2 654 cases in 2020, and 3 500 cases in 2021.Compared with 2019, the number of bronchiolitis cases decreased by 45.94% in 2020 and 28.70% in 2021.In 2019, 2020 and 2021, there were no significant differences in gender ratio, age, and duration of hospitalization.Compared with 2019, the ratio of bronchiolitis to the total number of hospitalizations for respiratory tract infection decreased significantly in 2020 and 2021( χ2=12.762, P<0.05; χ2=84.845, P<0.05).The proportion of moderate to severe bronchiolitis cases in both 2020 and 2021 was lower than that in 2019, and the difference was statistically significant ( χ2=4.054, P<0.05; χ2=8.109, P<0.05).There was no statistically significant difference in the proportion of bronchiolitis cases requiring ICU treatment between 2019, 2020, and 2021 ( χ2=1.914, P>0.05).In 2019, a total of 52.60%(2 582/4 909) of children with bronchiolitis underwent RSV pathogen testing, and among them, there were 708 cases with RSV positive, accounting for 28.00%.In 2020, 54.14%(1 437/2 654) of children with bronchiolitis underwent RSV pathogen testing, and there were 403 cases with RSV positive, accounting for 28.04%.In 2021, 66.80%(2 238/3 500) of children with bronchiolitis underwent RSV pathogen testing, and there were 935 cases with RSV positive, accounting for 41.78%.Compared with 2019 and 2020, the RSV positive rate in 2021 showed a significant increase( χ2=99.673, P<0.05; χ2=71.292, P<0.05). Conclusion:During the COVID-19 epidemic, the implementation of epidemic prevention and control measures reduced the hospitalization rate and severity of bronchiolitis, but did not reduce the positive rate of RSV detection.

Abstract Objective To investigate the etiological characteristics of plastic bronchitis (PB) caused by pulmonary infections in children and to identify any differences in the clinical features of PB cases caused by different pathogens. Method We collected data on children diagnosed with PB and admitted to the Respiratory Department at Soochow University Children’s Hospital between July 2021 and March 2023 utilizing electronic bronchoscopy. We analyzed clinical characteristics and the species of pathogens causing the illness in these children. Result A total of 45 children were enrolled. The main clinical symptoms observed were cough (100%), fever (80%), shortness of breath (28.9%), and wheezing (20.0%). Pathogens were identified in 38 (84.4%) patients. Mycoplasma pneumoniae (MP) had the highest detection rate at 53.3%, followed by the Boca virus at 26.7%. MP-induced PB typically occurs in older children with an average age of 7.46 ± 2.36 years, with the main symptoms including high fever (85.7%) and local hyporespiration (42.9%). In contrast, Boca virus-induced PB tends to occur in younger children, with the main symptoms of moderate fever (54.5%), and wheezing (54.5%). The MP group exhibited a higher incidence of both internal and external pulmonary complications, including pleural effusion (42.9%), elevated aspartate aminotransferase (52.4%), lactic dehydrogenase (76.2%), and D-D dimer (90.5%). Conversely, the Boca virus group primarily showed pulmonary imaging of atelectasis (81.8%), with no pleural effusion. The average number of bronchoscopic interventions in the MP group was 2.24 ± 0.62, which was significantly higher than that required in the Boca virus group (1.55 ± 0.52). During the second bronchoscopy, 57.1% of children in the MP group still had visible mucus plugs, while none were observed in the Boca virus group. Conclusion MP and Boca virus are the primary pathogens responsible for PB among children. The clinical manifestations of PB typically vary significantly based on the pathogen causing the condition.

Objective:To investigate the etiology, incidence and clinical characteristics of pediatric prolonged and chronic diarrhea.Methods:The clinical data of children with prolonged and chronic diarrhea were collected and analyzed, which were hospitalized in the Department of Gastroenterology of the Children′s Hospital Affiliated to the Capital Institute of Pediatrics from January 2017 to June 2020.Results:A total of 190 children with prolonged and chronic diarrhea were collected, with a male-to-female ratio of 1.64∶1(118/72) and a median age of 11.2(5.0, 48.0)months.Among them, 74.3%(141/190) were infants aged 0-3 years, and 54.3%(103/190) were infants aged 0-1 years.The overall cure and improvement rate was 83.7%(159/190). Gastrointestinal concomitant symptoms were dominated by abdominal pain, and vomiting, bloating, and extraintestinal concomitant symptoms were mainly fever, weight loss, and growth and development disorders.Common comorbidities included malnutrition (46.3%), anemia (35.3%), and electrolyte abnormalities (20.5%). The lesion detection rate of electronic gastrointestinal endoscopy and capsule endoscopy reached 93.1%(122/131). The detection rate of genetic testing was 60.0%(9/15). In this group of studies, 169 patients had a clear cause, and the confirmed diagnosis rate was 88.9%.The main causes of childhood prolonged and chronic diarrhea were food allergy(36.8%), inflammatory bowel disease(12.6%), and irritable bowel syndrome(9.5%), but 11.1% of the children did not identify the cause after comprehensive examination.Conclusion:Children with prolonged and chronic diarrhea are mainly infants and young children, especially infants, with diverse etiology, mainly non-infectious factors, food allergy and inflammatory bowel disease are important causes.Malnutrition, anemia and other complications are easy to occur.Endoscopy is helpful in diagnosing and differentiating the cause, if necessary, genetic testing could help to determine the cause.

Objective:To analyze the clinical characteristics and genetic variants of children with hepatic Wilson disease (WD).Methods:The clinical data and genetic test results of 35 children, who were diagnosed as WD with primary hepatic manifestation in the Department of Gastroenterology, Children′s Hospital of Capital Institute of Pediatrics from March 2018 to March 2022, were retrospectively analyzed. The relationship between phenotype and genotype of patients was analyzed.Results:Among 35 children, there were 24 males and 11 females with a median age at diagnosis of 5.5 (4.0, 7.5) years. All patients had elevated transaminases. The elevated transaminases was found during routine physical examination in 33 cases (94.3%), in whom there was no fever, cough, recurrent vomiting, abdominal pain, diarrhea, jaundice, limb tremor, gait instability and other discomfort 2 weeks before admission, except 1 case with nausea; abdominal ultrasonography showed that 5 cases (15.2%) had no abnormality, and others had different degrees of hepatomegaly, splenomegaly, and echo enhancement in liver parenchyma. Among the remaining 2 cases, one 11-year-old child presented with edema, and had cirrhosis portal hypertension with esophageal varices; another 7-year-old child was diagnosed as acute liver failure manifested with nausea and jaundice. Thirty three patients(94.3%)had decreased serum ceruloplasmin levels (<100 mg/L); 24-h urinary copper concentration was>100 μg in 16 cases (45.7%) and<40 μg in 2 cases (5.7%). The tests of hepatitis B virus, hepatitis C virus, cytomegalovirus and EB virus were all negative in 35 children, and the autoimmune hepatitis antibodies were also negative. A total of 34 different ATP7B gene mutations were detected; the most frequent mutation was c.2333G>T (P.R778L) at exon 8, followed by c.2621C>T(p.A874V)at exon 11 and c.2621C>T(p.A874V)at exon 13. There was no significant difference in clinical phenotype between patients with nonsense mutation, frameshift mutation or splicing mutation and those with only missense mutations( Z=-1.00, t=-0.16, Z=-1.14, Z=-1.03,all P>0.05). Conclusions:The onset of WD in children is obscure, and clinicians should consider this disease in patients presenting with elevated transaminase. Ceruloplasmin and urine copper should be tested timely, the early diagnosis and treatment can improve the prognosis. And there is no significant correlation between genotype and clinical phenotype.

Infantile cholestasis (IC) is the most common hepatobiliary disease in infants, resulting in elevated direct bilirubin levels. Indeed, hepatointestinal circulation impacts bile acid and bilirubin metabolism. This study evaluates changes in the gut microbiota composition in children with IC and identifies abnormal metabolite profiles associated with microbial alterations. The gut microbiota in the IC group exhibits the higher abundance of Veillonella, Streptococcus and Clostridium spp. (P < 0.05), compared to healthy infants (CON) group. Moreover, the abundance of Ruminococcus, Vibrio butyricum, Eubacterium coprostanogenes group, Intestinibacter, and Faecalibacterium were lower (P < 0.05). In terms of microbiota-derived metabolites, the levels of fatty acids (palmitoleic, α-linolenic, arachidonic, and linoleic) (P < 0.05) increased and the levels of amino acids decreased in IC group. Furthermore, the abundances of Ruminococcus, Eubacterium coprostanoligenes group, Intestinibacter and Butyrivibrio are positively correlated with proline, asparagine and aspartic acid, but negatively correlated with the α-linolenic acid, linoleic acid, palmitoleic acid and arachidonic acid. For analysis of the relationship between the microbiota and clinical index, it was found that the abundance of Veillonella and Streptococcus was positively correlated with serum bile acid content (P < 0.05), while APTT, PT and INR were negatively correlated with Faecalibalum and Ruminococcus (P < 0.05). Microbiota dysbiosis happened in IC children, which also can lead to the abnormal metabolism, thus obstructing the absorption of enteral nutrition and aggravating liver cell damage. Veillonella, Ruminococcus and Butyrivibrio may be important microbiome related with IC and need further research.

BackgroundWithin the past 3-5 years, Mycoplasma pneumoniae has become a major pathogen of community-acquired pneumonia in children. The pathogenic mechanisms involved in M. pneumoniae infection have not been fully elucidated.MethodsPrevious protein microarray studies have shown a differential expression of CXCL9 after M. pneumoniae infection. Here, we conducted a hospital-based study to explore the clinical significance of the type 1 immune response inflammatory factors interferon (IFN)-γ and CXCL9 in patients with M. pneumoniae pneumonia (MPP). Then, through in vitro experiments, we explored whether CARDS toxin stimulated F-DCs (dendritic cells incubated with Flt3L) to promote Th-cell differentiation; we also investigated the IFN-γ-induced CXCL9 secretion pathway in macrophages and the role of CXCL9 in promoting Th1 cell migration.ResultsThe CXCL9 expression level was upregulated among patients with a higher fever peak, fever duration of greater than 7 days, an imaging manifestation of lobar or segmental, or combined pleural effusion (P<0.05). The peripheral blood levels of IFN-γ and CXCL9, which were higher in patients than in the healthy control group, were positively correlated with each other (r=0.502, P<0.05). In patients, the CXCL9 expression level was significantly higher in the bronchoalveolar lavage fluid (BALF) than in the peripheral blood, and the BALF CXCL9 expression level was higher than that in the healthy control group (all P<0.05). Our flow cytometry analysis revealed that M1-phenotype macrophages (CD16+CD64+CD163−) were predominant in the BALF from children with MPP. In in vitro experiments, F-DCs stimulated with CARDS toxin promoted the differentiation of CD4+IFN-γ+ Th (Th1) cells (P<0.05). Moreover, IFN-γ induced high levels of CXCL9 expression in M1-type macrophages in a dose-dependent and time-dependent manner. Additionally, macrophages transfection with STAT1-siRNA-1 downregulated the expression of CXCL9 (P<0.05), and CXCL9 promoted Th1 cell migration (P<0.05).ConclusionsOur findings suggest that CARDS toxin induces a type 1 immune response positive feedback loop during M. pneumoniae infection; this putative mechanism may be useful in future investigations of immune intervention approaches for M. pneumoniae pneumonia.

目的 探讨晚发型多种酰基辅酶A脱氢酶缺乏症(multiple acyl-CoA dehydrogenase deficiency,MADD)临床特征和基因特点,加强对MADD的认识.方法 回顾性分析2020年2月就诊于首都儿科研究所附属儿童医院的1例MADD患儿的临床资料,并复习相关文献.结果 患儿,女,12岁,以反复呕吐5个月、双下肢乏力2个月为主要表现,伴体重显著减轻.血生化检查提示转氨酶升高,乳酸脱氢酶、肌酸激酶同工酶升高;氨基酸肉碱质谱分析提示多种酰基辅酶A脱氢酶缺乏症;腹部计算机断层扫描(computed tomography,CT)提示脂肪肝;肌肉磁共振成像(magnetic resonance ima-ging,MRI)提示肌炎.基因检测发现ETFDH基因存在7号外显子c.736G>A和3号外显子c.389A>T复合杂合突变,确诊为MADD.予核黄素、左卡尼汀治疗后好转.随访6个月无呕吐,肌力恢复正常,血生化指标正常.结论 晚发型MADD临床表现多样,基因检查可以帮助确诊MADD,核黄素对晚发型MADD治疗有效.

Objective:To clarify the clinical characteristics and related fators of children with delayed antibody production of mycoplasma pneumoniae pneumonia(MPP).Methods:Two hundreds and eithty-five cases of children hospitalized at Children′s Hospital of Soochow University with MPP(positive for nucleic acid testing of respiratory secretion)were chosen from January 1st, 2019 to September 31st, 2019.Delayed antibody production group included 36 cases, who were tested for negative IgM antibody meanwhile the titer of IgG antibody changed less than 4 folds within 14 days.Positive group included 249 cases who were tested for positive IgM antibody or the titer of IgG antibody changed over 4 folds within 14 days.The characteristics of clinical manifestation, immunology and radiology were comparatively analyzed.Results:The medium age of delayed antibody production group was 0.75(0.30, 2.78)years old, which was obviously younger than that from positive group[5.50(3.73, 7.20)years old]( P<0.001). Low level of serum immunoglobulin IgG was the independent effect factor of delayed production for Mycoplasma pneumoniae antibody( P=0.037). When the serum immunoglobulin IgG level was lower than 7.155mmol/L, the sensitivity of predicting delayed production for mycoplasma pneumoniae antibody would be 0.819 and the specificity was 0.833.The underlying diseases associated with delayed antibody production were hospitalization history during neonatal period( P=0.007)and congenital heart disease( P=0.001). There were 11.11%(4/36)of children appearing spasmodic cough, 41.67%(15/36)of children showing wheezing and 33.33%(12/36)showing diarrhea in delayed antibody group, which were significantly higher than those in positive group[0.40%(1/249), 24.50%(61/249)and 9.64%(24/249), respectively, P<0.05]. The incidence of fever in delayed antibody group were 63.89%(23/36), which was lower than that in positive group[92.37%(230/249)]( P<0.001), meanwhile, the fever last time was 2.50(0, 4.75)days in delayed antibody group, which was shorter than that in positive group[ 7(5.00, 8.50)days]( P<0.001). In the delayed antibody group, there was 19.44%(7/36)of children sufferring from lobar pneumonia, and no extrapulmonary manifestations occurred, which were significantly lower than those in positive group[75.50%(188/249), 14.86%(37/249)]( P<0.05). Conclusion:Delayed antibody production in children with MPP is more common when serum immunoglobulin IgG level is lower than 7.155 mmol/L, especially in the presence of neonatal hospital history and congenital heart disease.The clinical manifestations of these children are mainly characterized by spasmodic cough and wheezing, with low probability of fever, lobular pneumonia and extrapulmonary manifestations.

目的 探讨常染色体隐性遗传病Shwachman-Diamond综合征(Shwachman-Diamond syndrome,SDS)患儿的临床特点和基因学特征.方法 收集2019年4-10月首都儿科研究所附属儿童医院收治的以肝损害为主要表现的2例SDS患儿的临床资料,对患儿的临床特点、影像学表现、基因测序结果、治疗情况及随访结果进行系统性分析,并查阅相关文献.结果 2例患儿均以肝功能异常为主要表现起病并就诊,ALT为129～291 U/L.起病隐匿,均在2岁之内发病,表现为体质量和身高明显落后,低于正常同龄儿相应参考值的第3百分位点.例2有反复呼吸道感染,均不伴有脂肪泻.2例均伴有持续性/间歇性中性粒细胞减少(最低值为0.5×109/L和0.28×109/L).例2存在动脉导管未闭,腹部CT示胰腺脂肪化,双股骨X线片示右侧髋关节间隙稍增宽.2例患儿SBDS基因检测确诊,例1为c.183_184delinsCT及c.258+2T＞C的复合杂合突变,例2为c.183_184delinsCT杂合突变及c.258+2T＞C纯合突变.治疗主要为胰酶替代治疗及营养支持治疗.治疗后2例患儿身高、体重均稳步增长,监测中性粒细胞计数维持稳定.以"Shwachman-Diamond""SBDS"为关键词,查阅文献并复习,已报道病例中以肝损害起病者较少,首发表现多以生长发育迟缓、血象异常及脂肪泻被重视并得以就诊.结论 SDS患儿常见临床表现为胰腺脂肪化和外分泌不足、外周血象中至少一系下降,尤其中性粒细胞减少和骨骼异常.部分患儿以肝功能异常隐匿起病,需提高对本疾病的认识,SBDS基因检测有助于确诊.

OBJECTIVE:To explore the characteristics of SLC25A13 gene variants in 16 infants with neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD).METHODS:The infants were subjected to high-throughput DNA sequencing for coding exons and flanking regions of the target genes. Suspected variants were verified by Sanger sequencing and bioinformatic analysis.RESULTS:Among the 16 NICCD cases, 15 were found to harbor pathogenic variants. Among these, IVS14-9A>G, c.1640G>A, c.762T>A, c.736delG, c.1098Tdel and c.851G>A were previously unreported.CONCLUSION:Six novel SLC25A13 variants were found by high-throughput sequencing, which has enriched the spectrum of SLC25A13 gene variants and provided a basis for genetic counseling and prenatal diagnosis.

Objective: We sought to compare the clinical characteristics of pediatric respiratory tract infection and respiratory pathogen isolations during the coronavirus disease (COVID-19) pandemic to those of cases in 2018 and 2019.Methods: Our study included all children from 28 days to 15 years old with respiratory tract infections who were admitted to the Department of Respiration, in the Children's Hospital of Soochow University, between January 2018 and December 2020. Human rhinovirus (HRV) and human metapneumovirus (hMPV) were detected by reverse transcription polymerase chain reaction (RT-PCR). Mycoplasma pneumoniae (MP) and human bocavirus (HBoV) were detected by real-time fluorescence quantitative polymerase chain reaction (qPCR); In parallel, Mycoplasma pneumoniae was detected by enzyme-linked immunosorbent assays, and bacteria were detected by culture in blood, bronchoalveolar lavage specimen, and pleural fluid.Results: Compared to 2018 and 2019, the pathogen detection rate was significantly lower in 2020. With regard to infections caused by single pathogens, in 2020, the detection rates of MP were the lowest and those of HRV were the highest when compared to those in 2018 and 2019. Meanwhile, the positive rates of respiratory syncytial virus (RSV) and hMPV reported in 2020 were less than those recorded in 2018 but similar to those recorded in 2019. Also, the 2020 rate of adenovirus (ADV) was lower than that recorded in 2019, but similar to that recorded in 2018. There were no statistical differences in the positive rates of HBoV and PIV III over the 3 years surveyed. Infections in infants were significantly less common in 2020, but no significant difference was found among children aged 1 to 3 years. The detection rate of pathogens in children old than 5 years in 2020 was significantly lower than those recorded in the previous 2 years. Notably, the pathogen detection rates in the first and second quarters of 2020 were similar to those recorded in the previous 2 years; however, the rates were reduced in the third and fourth quarters of 2020. As for co-infections, the positive rate was at its lowest in 2020. In the previous 2 years, viral-MP was the most common type of mixed infection. By contrast, in 2020, viral-viral infections were the most common combination.Conclusion: The pathogen detection rate was significantly reduced in Suzhou City during the COVID-19 pandemic. Public interventions may help to prevent respiratory pathogen infections in children.

Plastic bronchitis (PB) is a rare respiratory condition which can result in severe respiratory complications such as respiratory failure and death. Mycoplasma pneumoniae infection is a main etiology cause of plastic bronchitis. However, the pathogenesis of plastic bronchitis complicated by Mycoplasma pneumoniae pneumonia (MPP) has not yet been fully elucidated. Our article aims to explore biomarkers for early prediction of MPP cases complicated with plastic bronchitis. We utilized a protein chip to screen for significantly different proteins among the groups of healthy, general Mycoplasma pneumoniae pneumonia (GMPP) and refractory Mycoplasma pneumoniae pneumonia (RMPP) patients, where layilin exhibited a potent change across biology information technology. Next, we demonstrated the high expression of MUC5AC, MUC5B, and layilin in bronchoalveolar lavage fluid (BALF) of MPP cases complicated with plastic bronchitis. Further study suggested that the level of layilin had a positive correlation with both MUC5AC and MUC5B. A receiver operating characteristic (ROC) analysis was performed to assess the diagnostic values of MUC5AC, MUC5B, and layilin in MPP cases with PB. Data show that the three indicators have similar diagnostic ability for MPP children with plastic bronchitis. Then, we used different concentrations of community-acquired respiratory distress syndrome (CARDS) toxin or lipid-associated membrane proteins (LAMPs) to simulate an in vitro experiment. The in vitro assay revealed that CARDS toxin or LAMPs induced A549 cells to secrete MUC5AC, MUC5B, layilin, and proinflammatory factors. These findings suggest that MUC5AC, MUC5B, and layilin are correlated with MPP. The high expression of MUC5AC, MUC5B, and layilin play an essential role in prediction in the development of plastic bronchitis caused by MPP. The high expression of MUC5AC, MUC5B, and layilin may be relevant to the severity of illness.

Objective:To investigate the clinical and genetic characteristics of genetic and metabolic infantile cholestatic hepatopathy (ICH), and to provide evidence for its diagnosis and treatment.Methods:Clinical data and follow-up outcomes of hospitalized children diagnosed with ICH in the Department of Gastroenterology, Children′s Hospital, Capital Institute of Pediatrics from January 2014 to December 2019 were retrospectively analyzed.Among the 80 children, 27 were female and 53 were male, with a mean age of onset of (39±18) days old.Children with confirmed etiology by high-throughput sequencing analysis were included in the genetic metabolic group (44 cases), and those with idiopathic neonatal cholestasis(INC) of unknown etiology after the systematic examination were included in the INC group (36 cases). The t-test or independent sample rank sum test was used to compare the laboratory test results and biochemical indexes.The infection rate of cytomegalovirus was compared by the Chi- square test. Results:(1) A total of 80 cases were included, and 44 cases (55.0%)were confirmed as INC by high-throughput sequencing.Among those with a positive molecular diagnosis, there were 23 cases of citrin deficiency (CD), 10 cases of Alagille syndrome (ALGS), 6 cases of progressive familial intrahepatic cholestasis (PFIC), 2 cases of congenital bile acid synthesis defect, 2 cases of Nieman Pick disease, and 1 case of cystic fibrosis.(2) Serum total bile acid (TBA) and activated partial prothrombin time (APTT) levels in the genetic metabolic group were significantly higher than those in the INC group (all P<0.05). TBA and APTT levels in genetic metabolites were 180.6 (115.5, 271.6) μmol/L and 40.6 (37.1, 45.2) s, respectively, which were 123.3 (98.8, 163.4) μmol/L and 34.8 (31.7, 40.1) s in INC group, respectively.There was no significant difference in the cytomegalovirus infection rate between the 2 groups ( P>0.05). (3)The pathological examination of liver tissue in the genetic metabolic group was worse than that in the INC group, with spot-like and fusion focal-like necrosis, and 5 cases (4 cases of ALGS and 1 case of CD) showed a reduced number of bile ducts in the portal area and lumen stenosis. Conclusions:CD, ALGS and PFIC are the common causes of genetic and metabolic ICH.Fundamental cause of cholestasis should be actively examined in children with cytomegalovirus infection.High-throughput sequencing is of great significance in the accurate diagnosis of ICH.

Objective To explore the clinical characteristics and genetic findings of patients with infantile intrahepatic cholestasis. Methods The clinical data were collected in children who were admitted to the Department of Gastroenterology in Children's Hospital, Capital Institute of Pediatrics from June 2017 to June 2019 and were suspected of inherited metabolic diseases. Next generation sequencing based on target gene panel was used for gene analysis in these children. Sanger sequencing technology was used to verify the genes of the members in this family. Results Forty patients were enrolled. Pathogenic gene variants were identified in 13 patients (32%), including SLC25A13 gene variation in 3 patients who were diagnosed with citrin deficiency, JAG1 gene variation in 3 patients who were diagnosed with Alagille syndrome, ABCB11 gene variation in 3 patients who were diagnosed with progressive familial intrahepatic cholestasis type 2, HSD3B7 gene variation in 1 patient who was diagnosed with congenital bile acid synthesis defect type 1, AKR1D1 gene variation in 1 patient who was diagnosed with congenital bile acid synthesis defect type 1, NPC1 gene variation in 1 patient who was diagnosed with Niemann-Pick disease, and CFTR gene variation in 1 patient who was diagnosed with cystic fibrosis. Conclusions The etiology of infantile intrahepatic cholestasis is complex. Next generation sequencing is helpful in the diagnosis of infantile intrahepatic cholestasis.

目的:探讨百日咳综合征住院患儿的临床特点及影响住院危险因素，提高临床诊治水平。方法:回顾性分析2014年至2019年在苏州大学附属儿童医院呼吸科住院的489例百日咳综合征患儿的病史、症状、体征、实验室检查及病原学等资料，总结其临床特点及影响住院危险因素。结果:年龄<3个月占53.17%，3～6个月占24.13%，>6个月占22.70%。患儿均有痉挛样咳嗽，其他症状发生率依次为喘息32.11%、咳嗽后青紫7.16%、呼吸困难3.27%、咳嗽后吸气性回声2.04%。实验室检查中白细胞升高占75.05%，淋巴细胞比例升高占73.62%，CRP升高占13.29%，血小板计数升高占85.89%。病原学检测阳性率61.15%，病毒检出率为35.58%，细菌检出率为30.47%，肺炎支原体检出率为13.70%，单一病原排序前5位是鼻病毒(24.57%)、肺炎支原体(16.46%)、肺炎链球菌(10.57%)、呼吸道合胞病毒(8.85%)、流感嗜血杆菌(8.11%)；混合感染占17.79%，其中病毒混合细菌占62.07%，病毒混合肺炎支原体占19.54%，细菌混合肺炎支原体占13.79%，病毒、细菌及肺炎支原体混合占4.60%。季节以春、夏季最为多见，占73.00%，其次为秋季19.02%，冬季7.98%。住院≥14 d的患儿中重症肺炎、先天性气道发育畸形、先天性心脏病、胃扭转、喘息发生率均明显高于住院<14 d患儿，差异均有统计学意义( χ 2＝54.920、13.986、4.770、19.966、6.891， P均<0.05)。 结论:百日咳综合征多见于6个月以下患儿，以春夏季多见，痉挛样咳嗽是特征性临床表现，血常规提示以淋巴细胞为主的白细胞升高，感染病原依次是病毒、细菌和肺炎支原体，前3位病原体依次是鼻病毒、肺炎支原体和肺炎链球菌，年龄<1岁易发生混合感染。有喘息症状、病情重、有先天性气道发育异常、先天性心脏病、胃扭转是住院时间延长的危险因素。

Background Bronchiolitis is a clinical syndrome commonly encountered in practice, particularly among infants and young children. To investigate the prevalence of pathogens in hospitalized infants with bronchiolitis and study the relationship between the clinical characteristics and pathogens. Methods We investigated the respiratory specimens and clinical data of 1012 children with bronchiolitis who were treated at the Children’s Hospital of Soochow University between November 2011 and December 2018. The nasopharyngeal aspirates were examined by direct immunofluorescence assay or polymerase chain reaction (PCR) to detect viruses and by PCR and enzyme-linked immunosorbent assay to detect Mycoplasma pneumoniae (MP). Results Of the 1012 children with bronchiolitis, 842 (83.2%) were detected at least a pathogen. 614 (60.7%) had single viral infections, 91 (9.0%) had MP infections, 70 (6.9%) had multiple viral infections, and 67 (6.6%) had mixed viral and MP infection. The most common pathogens detected were respiratory syncytial virus (RSV) (44.4%), MP (15.6%), and human rhinovirus (HRV) (14.4%). RSV was the most common pathogen detected in children less than 6 months. Coinfection was detected in 13.5% (137/1012) of the children, but it was less common in children less than 6 months. The age of children with single virus infection was the youngest. Children with single virus infection had a higher proportion of oxygen therapy compared with single MP infection. Conclusions The most common pathogen detected in children with bronchiolitis is RSV, followed by MP and HRV. Co-infections lead to prolonged illness and worsening of the symptoms Keywords: Etiology, Bronchiolitis, Infant, Respiratory syncytial virus, Mycoplasma pneumoniae

Objective:To analyze the clinical characteristics of patients suffering from plastic bronchitis (PB) caused by Mycoplasma pneumoniae (MP) and explore its risk factors as well. Methods:A retrospective analysis on clinical and laboratory data of PB children caused by MP and treated in Department of Respiratory in Children′s Hospital of Soochow University from January 2011 to December 2017, compared with MP pneumonia(MPP) children without PB in the same period.Meanwhile, Logistic regression analysis was performed. Results:Among the 306 MPP children, there were 50 cases in the PB group and 256 cases in the non-PB group.Compared with children in the non-PB group, children in PB group were higher in terms of age [(82.74±35.17)months vs.(66.63±35.67) months], percentage of neutrophils (0.705 8±0.139 1 vs.0.605 7±0.162 6), C reactive protein(CRP) [17.4(10.21, 42.86) mg/L vs.11.43(4.55, 23.66) mg/L], D-dimer(DD) [1 071 (279.5, 2 386.5) μg/L vs.523 (233, 1 099.5) μg/L], lactate dehydrogenase(LDH) [491.1 (342.3, 607.4) U/L vs.394.9 (319.1, 512.8) U/L], erythrocyte sedimentation rate(ESR)[25.0 (17.0, 36.0) mm/1 h vs.15.5(9.0, 28.0) mm/1 h], aspartate aminotranferase(AST) [33.5(26.1, 49.3) U/L vs.29.2(24.0, 37.2) U/L], alanine aminotransferase (ALT) [19.1(11.45, 31.50) U/L vs.13.6 (10.3, 23.15) U/L], IgA [1.46(0.98, 2.12) mg/L vs.1.15 (0.64, 1.60) mg/L], CD3 -CD (16+56)+ (0.155 0±0.088 6 vs.0.120 2±0.071 5), allergy history [44.0%(22/50 cases) vs.25.8%(65/256 cases)], mixed infection [38.0% (19/50 cases) vs.24.6%(63/256 cases)], and microscopic mucosal erosion [10.0%(5/50 cases) vs.2.3%(6/256 cases)] (all P<0.05). Logistic regression analysis displayed that allergy history ( OR= 5.604, 95% CI: 1.937-16.216), age ( OR = 3.142, 95% CI: 1.425-6.929), percentage of neutrophils ( OR=2.387, 95% CI: 1.088-5.238), CRP ( OR=3.959, 95% CI: 1.072-14.662), and DD ( OR=7.824, 95% CI: 2.824-21.673) were independent risk factors for PB caused by MP infection (all P<0.05). The cut-off values of age, percentage of neutrophils, CRP, and DD were 64 months, 0.70, 35 mg/L, and 2 000 μg/L. Conclusions:Children with PB caused by MP often develop in older and allergic children who have stronger inflammatory reactions, immune disorders, and hyperfibrinolysis.