Objective To analyze the clinical and anatomic characteristics of bridging bronchus (BB) malformation in children,and to explore its diagnostic strategy,treatment and risk factors.Methods A retrospective study was performed in 23 pediatric patients with BB who were admitted to Guangzhou Women and Children's Medical Center form May 2010 to October 2016.All their clinical features,imaging examination,treatment and prognosis were reviewed and analyzed.Results (1) Among the 23 patients with BB,15 were males and 8 were females.The age range of onset was 0 to 4 years old with a median age of 3.17 months.(2)The main clinical manifestations were cough (23/23 cases,100.0％),recurrent wheezing (20/23 cases,87.0％),cyanosis (8/23 cases,34.8％) and feeding diffficulty/slow body weight growth (6/23 cases,24.6％).(3) There were 17 cases of type Ⅰ and 4 cases of type Ⅱ according to Wells,and the other 2 cases were anterior BB.There were 2 cases with atypical anatomical morphology in type Ⅰ and type Ⅱ respectively.(4) Twenty-one cases were associated with bronchial stenosis in which the origin of left main bronchus complicated with BB stenosis was the most common(5 cases).Bronchoscopy revealed bronchomalacia in 5 of the 16 patients.Sixteen patients associated with cardiac or vascular anomalies,11 of whom were compound anomalies,and the most common type was sling left pulmonary artery (SLPA) (12 cases).(5) Nine of 12 patients with SLPA received surgical management,ages ranging from 1 month to 8 years old.The respiratory symptoms remitted gradually or disappeared from 4 to 17 months after surgical management.Two died and one has been lost to follow-up among the remaining 3 patients with SLPA.Conclusions BB is a rare tracheobronchial malformation that is often associated with stenosis or bronchomalacia,and cardiac or vascular anomalies.The severity of the disease mainly depends on whether there is airway stenosis or malacia and its extent.The definitive diagnosis of BB requires the combination of different imaging modalities.The combination with simple SLPA could get better curative effect.

Objective:To analyze the clinical and imaging characteristics of pulmonary artery sling (PAS) in children and to explore its diagnosis and treatment strategies and risk factors.Method:s A retrospective study was performed in 98 pediatric patients with PAS who were admitted to Guangzhou Women and Children′s Medical Center, Guangzhou Medical University, from January 2011 to October 2018.All their clinical features, imaging examination, treatment and prognosis were reviewed and analyzed.Result:s (1) Among the 98 patients with PAS, 56 cases were male and 42 cases were female.The age range of onset was 0-19 months with the median age of 2 months, and the age range of diagnosis was 0-84 months with the median age of 4 months.(2) The main clinical manifestations were cough (61/98 cases, 62.24%), recurrent wheezing(47/98 cases, 47.96%), shortness of breath (43/98 cases, 43.88%), cyanosis (19/98 cases, 19.39%), and laryngeal stridor (8/98 cases, 8.16%) and so on.(3) Ninety-six patients underwent cardiac ultrasonography, and the diagnostic rate was 87.50%(84/96 cases), and 62 cases (62/96 cases, 64.58%) of them were found to be associated with other congenital cardiovascular anomalies, among which atrial septal defect was the most common (32/96 cases, 33.33%). (4) Chest CT and three-dimensional reconstruction were performed in 92 PAS children, and the diagnostic rate was 100.00%.Airway stenosis was mainly in the middle and lower sections of the main bronchus (76/92 cases, 82.61%), and bronchobridge was found in 13 cases (13/92 cases, 39.13%). Bronchoscopy was performed in 77 children with PAS, showing complete tracheal cartilage ring in 43 cases (43/77 cases, 55.84%), and tracheobronchomalacia in 13 cases (13/77 cases, 16.88%). (5) Among 67 cases who had received left pulmonary artery (LPA) reimplantation, 5 cases died after operation because of airway stenosis and weaning failure (LPA reconstruction was performed in 1 case, and both LPA reconstruction and tracheal intervention were performed in the other 4 cases), 24 cases lost to follow-up, 38 cases survived, and the respiratory symptoms were improved in 2 to 96 months of follow-up.There were 31 cases of non-surgical treatment, of which 18 cases died of respiratory failure, 7 cases lost to follow-up, and the rest 6 cases showed respiratory symptoms of different degrees in the follow-up period.(6) Multivariate regression analysis showed that conservative treatment was an independent risk factor affecting the prognosis of PAS ( OR=7.45, 95% CI: 1.23-48.68). Conclusions:The combination of cardiac ultrasound, chest CT and bronchoscopy is important to the diagnosis of PAS.LPA reconstruction is the main method to treat PAS, which can improve respiratory symptoms, but its fusion with tracheal intervention poses a high risk of death.Conservative treatment is an independent risk factor that affects the prognosis of PAS children.

Objective:To analyze the clinical features and follow-up results of children with acute exogenous lipoid pneumonia (ELP), and to investigate the diagnosis strategy, treatment and risk factors of ELP.Methods:The clinical features, imaging manifestations, results of bronchoalveolar lavage fluid (BALF), treatment methods and prognosis of 41 ELP inpatients in Guangzhou Women and Children′s Medical Center, Guangzhou Medical University from May 2013 to May 2018, were summarized and analyzed, and the related literature was reviewed.Results:(1) Among 41 patients, 29 cases were male and 12 cases were female.The age of onset ranged from 4 to 53 months.Most of them (35/41 cases, 85.4%) were less than 3 years old, and the majority were aged 1 to 2 years (23/41 cases, 56.1%). (2) The grease taken by children by accident included perfume oil (11 cases), white electric oil (7 cases), electric mosquito oil (5 cases), lubricating oil (5 cases), paraffin oil (4 cases), gasoline/diesel oil (3 cases), kerosene (2 cases), cod-liver oil (2 cases), sewing machine oil (1 case), and unclear oil (1 case). (3) The main clinical manifestations were cough (26/41 cases, 63.4%), shortness of breath (23/41 cases, 56.1%), fever (17/41 cases, 41.5%), cyanosis with hypoxemia (15/41 cases, 36.6%), wheezing (9/41 cases, 22.1%), respiratory distress (9/41 cases, 22.1%), pulmonary hemorrhage (6/41 cases, 14.7%), feeding difficulties and transient vomiting (4/41 cases, 9.8%). Some cases showed no symptoms (3/41 cases, 7.3%). (4) Chest X-ray mainly showed bilateral pulmonary exudative changes or pulmonary consolidation.The most common sign of high-resolution computerized tomography (HRCT) was local or extensive exudation, often accompanied by pulmonary consolidation (18/41 cases, 43.9%). The most common affected sites were bilateral lower lobes (8/41 cases, 19.5%), followed by right lower lobes (3/41 cases, 7.3%), right middle lobes (2/41 cases, 4.8%) and left lower lobes (2/41 cases, 4.8%). Other signs included alveolar changes, hyperventilation, ground glass changes, and nodule formation.(5) Seven children needed mechanical ventilation due to respiratory failure, and all patients underwent bronchoalveolar lavage (BAL). All patients recovered without death, with (11.67±4.90) days of hospitalization, and the hospitalization time of ELP patients with adenovirus infection was (19.25±5.93) days.Nine of them were lost to follow-up (8 cases were mild and 1 case was severe). The symptoms of the remaining children disappeared within 1 week to 1 month.The lung images of 21 cases returned to normal within 1 month after discharge, 7 cases returned to normal within 3 months after discharge, and 4 cases returned to normal after 6 months.Conclusion:The clinical manifestations and lung imaging of children with acute ELP have no specificity.Pulmonary imaging of severe patients shows multiple lobes involved, and consolidation and vacuolar changes may occur.Most patients have a favorable prognosis.Co-infection, especially adenovirus infection, may be a risk factor for acute ELP in children.BAL can play a role in both diagnosis and treatment, and its combination with local/systemic glucocorticoid therapy is effective for severe acute ELP.

Objective:To explore the immunological characteristics of peripheral blood and genetic variations of 11 immunodeficiency virus(HIV)-negative children with Talaromyces marneffei(TM) infection, thus enhancing the diagnostic and therapeutic levels of TM infection in children. Methods:Clinical data of 11 HIV-negative children with TM infection who presented to Guangzhou Women and Children′s Medical Center, Guangzhou Medical University from January 2010 to December 2022 were retrospectively analyzed, including clinical characteristics, peripheral immune profile and genetic test results.Results:A total of 11 HIV-negative children with TM infections were recruited, involving 9 males and 2 females with a median age of 19 months.The main clinical manifestations were fever (10/11, 90.91%), cough (10/11, 90.91%) and hepatomegaly (7/11, 63.64%). Common severe complications included acute respiratory distress syndrome (7/11, 63.64%) and septic shock (5/11, 45.45%). Finally, 2 children died.Transient neutropenia occurred in 6 cases (6/11, 54.55%), and lymphocytopenia combined with serum immunoglobulin (Ig) G decrease was observed in 4 cases (4/11, 36.36%). IgA decrease, IgM decrease, IgE decrease, IgM increase and IgE increase were observed in 6 cases, 3 cases, 5 cases, 3 cases, and 2 cases, respectively.Both T-lymphocyte and B-lymphocyte counts decreases was observed in 1 case.Genetic testing was performed in all recruited children, and genetic variations were detected in all of them.Inborn errors of immunity (IEIs) were diagnosed in 8 cases, including 4 diagnosed as CD 40 ligand deficiency with CD40LG variation, 1 of severe combined immunodeficiency with IL2RG variation, 1 of Signal transduction and activator of transcription 3(STAT3)-hyper-IgE syndrome with STAT3 variation and 1 of familial candidiasis type 2 with CARD9 compound heterozygous mutations.In the other 3 cases, 2 carried genetic variations that were likely pathogenic, and 1 case was considered uncertain. Conclusions:The clinical manifestations of HIV-negative children with TM infection are atypical, which is characterized as serious complications and high mortality.Early identification and gene testing to detect potential IEIs can improve the prognosis of TM infection.

Objective:To observe the therapeutic timing and dosage of intravenous immunoglobulin (IVIG) in children with severe adenovirus pneumonia.Methods:Clinical data of children with severe adenovirus pneumonia treated with IVIG at the Department of Respiratory, Guangzhou Women and Children′s Medical Center, Guangzhou Medical University from January 2019 to January 2020 were retrospectively analyzed.Participants were classified as early presenters (5-10 days of illness course) and later presenters (11-15 days of illness course) according to the timing of IVIG treatment.They were further subdivided into plan 1 group[1 g/(kg·d) IVIG for 2 days] and plan 2 group [0.4-0.5 g/(kg·d) IVIG for 3-5 days]. Continuous variables and categorical variables between groups were analyzed by the nonparametric Mann- Whitney U test and the Fisher′ s exact test, respectively. Results:A total of 202 patients with the median age of 12 (12, 36) months were recruited, involving 128 early presenters (63.37%) and 74 later presen-ters (36.63%). Later presenters had a longer duration of fever [18.00(14.00, 23.25) days vs.11.00(9.00, 14.00) days], more demands for mechanical ventilation (33.78% vs.20.31%), and higher incidence of bronchiectasis (9.46% vs.1.56%) than those of early presenters (all P<0.05). For early presenters, no significant differences were detected in the demand for advanced life support, outcomes and sequelae between plan 1 group and plan 2 group (all P>0.05). For later presenters, a shorter duration of fever [18.00(14.00, 21.00) days vs.21.00(15.50, 30.75) days] and lower usage of extracorporeal membrane oxygenation (ECMO, 2.13% vs.18.52%) were observed in the plan 1 group than that of plan 2 group (all P<0.05). The incidence of post-infectious bronchiolitis obliterans and bronchiectasis as pulmonary sequelae was comparable between plan 1 group and plan 2 group ( P>0.05). The incidence of adverse events was 5.77% during IVIG infusion, showing no significant difference between plan 1 group and plan 2 group ( P>0.05). Conclusions:Early treatment of IVIG are very important to improve the prognosis of children with severe adenovirus pneumonia.For later presenters, a high dosage of IVIG is effective in reducing the ECMO use and shortening the duration of fever, thus providing clinical benefits.