Abstract: Objective To explore the clinical data of children with Talaromyces marneffei (TM) infection admitted to the pediatric intensive care unit (PICU) to improve the diagnosis and treatment of TM infection in children. Methods The clinical data of 23 children with TM infection treated in four PICUs in southern China from January 2013 to December 2022 were retrospectively analyzed, including clinical characteristics, laboratory tests, treatment regimens, and outcomes. Results Among the 23 children, there were 14 boys and 9 girls, with a median age of 24 months. The time from onset to admission was 15 (range 10-30) days, and one patient had immunodeficiency disease. Common symptoms included fever (91.3%, 21 cases), cough (78.3%, 18 cases), and hepatosplenomegaly (78.3%, 18 cases). Severe clinical complications included multiorgan dysfunction (69.6%, 16 cases), septic shock (65.2%, 15 cases), and acute respiratory distress syndrome (65.2%, 15 cases). All patients elevated CRP levels, and 69.2% (9/13) had a positive G test. Elevated IgE levels were observed in 53.3% (8/15) cases, CD4/CD8 inversion in 17.6% (3/17) cases, and reduced NK cells in 84.2% (16/19) cases. HIV tests were negative in all cases. TM was most frequently detected by blood and bone marrow cultures. Seven cases were diagnosed with immunodeficiency by genetic testing. With monotherapy or combination of amphotericin B or/and voriconazole, followed by oral medication, 13 (56.5%) children died. Conclusions TM infection is clinically atypical in HIV-negative children, and patients admitted to PICU have rapidly deteriorated, with severe complications and a high mortality rate. Early use of multiple samples, and multiple methods to detect TM, combined with immune function and genetic test, is helpful to early diagnosis. The antifungal treatment strategies still need further study.

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 investigate the abnormal expression of proto-oncogene YES-associated protein (YAP) in gastric cancer tissues in the elderly and its correlation with poor prognosis.Methods Clinical data of 80 elderly patients with gastric cancer treated in our hospital from March 2011 to October 2014 were statistically analyzed.Results The positive expression rate of YAP was significantly higher in gastric carcinoma than in adjacent tissues [71.3％ (57/80) vs.13.8％ (11/80),P＜0.05].The positive expression of YAP were significantly associated gastric tumor size,tumor stage,invasion depth and lymph node metastasis (all P＜0.05),but had no correlation with tumor differentiation (P＞0.05).The 5-year survival rate was significantly lower in patients with YAP-positive expression than in patients with YAP-negative expression (P ＜ 0.05),but the differences in 1-year,3-year survival rates were not significant between the two groups (all P＞0.05)The YAP expression,tumor stage,lymph node metastasis were significantly associated with the prognosis of gastric cancer in patients (all P＜0.05).Conclusions YAP-positive expression rate is significantly higher in gastric cancer tissues than in adjacent tissues in the elderly,which indicates poor prognosis of patients with gastric cancer.

Adenovirus pneumonia is a common infectious disease of the respiratory tract in children, and severe cases developed post﹣infectious bronchiolitis obliterans (PIBO),which is chronic airflow obstruc﹣tion syndrome caused by small airway inflammatory injury. The major clinical manifestations of PIBO are persistent cough,wheezing and dyspnea after acute stage of adenovirus pneumonia,and nowadays there is no specific treatment. PIBO has a prolonged course and very poor prognosis without early intervention,and it in﹣fluences patients on health and life seriously. The article reviewed the early recognition and the therapeutic progress of adenovirus pneumonia complicated with PIBO.

Human adenoviruses (HAdVs) are the common infectious pathogens in children, which mainly infect the respiratory tract, digestive tract and eyes.Severe HAdVs may even be life-threatening.HAdVs enter the host and cause infection by binding to the host epithelial cells.At the same time, they are recognized by various immune cells and then activate the immune defense response.This study aims to review the interaction between HAdVs and the host, and the immune defense mechanism, thus improving the understanding of the immune response to HAdVs.

BACKGROUND: Despite the progress in perinatal-neonatal medicine, complications of extremely preterm infants continue to constitute the major adverse outcomes in neonatal intensive care unit. Human umbilical cord Wharton’s Jellyderived mesenchymal stem cells (HUMSCs) may offer new hope for the treatment of intractable neonatal disorders. This study will explore the functional differences of HUMSCs between extremely preterm and term infants. METHODS: UMSCs from 5 extremely preterm infants(weeks of gestation: 22+5 w,24+4 w,25+3 w,26 w,28 w) and 2 term infants(39 w,39+2 w) were isolated, and mesenchymal markers, pluripotent genes, proliferation rate were analyzed.HUVECs were injured by treated with LPS and repaired by co-cultured with HUMSCs of different gestational ages. RESULTS: All HUMSCs showed fibroblast-like adherence to plastic and positively expressed surface marker of CD105,CD73 and CD90, but did not expressed CD45,CD34,CD14,CD79a and HLA-DR; HUMSCs in extremely preterm exhibited significant increase in proliferation as evidenced by CCK8, pluripotency markers OCT-4 tested by RT-PCR also showed increase. Above all, in LPS induced co-cultured inflame systerm, HUMSCs in extremely preterm were more capable to promote wound healing and tube formation in HUVEC cultures, they promoted TGFb1 expression and inhibited IL6 expression. CONCLUSIONS Our results suggest that HUMSCs from extremely preterm infants may be more suitable as candidates in cell therapy for the preterm infants.

Hyper-IgE syndrome (HIES) comprises a group of rare primary immunodeficiencies, which are characterized by extremely high serum IgE levels, eczema, recurrent skin and pulmonary infections.Signal transduction and activator of transcription 3( STAT3)-HIES is the most common type, which is caused by dominant-negative mutations in STAT3.STAT3-HIES confers broad innate and acquired immune defects, defects in skeletal, connective tissue, and vascular functions, causing a clinical phenotype including eczema, staphylococcal and fungal skin and pulmonary infections, scoliosis and minimal trauma fractures, vascular tortuosity and aneurysm.In this article, the advance in diverse clinical manifestations and management strategies of STAT3-HIES was summarized.

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.

Primary immunodeficiencies (PIDs) are caused by one or more defects of the immune system.Children with PIDs are more likely to experience recurrent and/or severe infections and tend to develop a wide range of complications.Respiratory diseases are the main and initial manifestation in most cases.Pulmonary complications have significant morbidity and mortality in children with PIDs.The spectrum of pathogens usually varies among different types of PID.Early diagnosis and appropriate therapy can prevent or at least slow down the development and course of respiratory complications of PIDs.In this study, the main respiratory manifestations of common PIDs in children are reviewed.

Penicillium marneffei (PM) is the only thermally dimorphic species as a Penicillium that causes the transmitted penicilliosis marneffei (PSM). PM infection is more common in immunodeficient children.Due to the lack of specific clinical manifestations, PSM is easily misdiagnosed as pulmonary plasmacycosis, tuberculosis or pulmonary aspergillosis.Because of the rapid progress and multiple complications of transmitted PSM, its mortality remains high without timely and effective treatment.Therefore, early diagnosis and treatment are of great significance, and the diagnosis of PSM mainly relies on the immune function examination, measurement of serum markers, pathogen detection and imaging examinations.There is no standard antifungal regimen, and Amphoterus B and Itraconazole are used as the first-line treatment.This study aims to review the progress of diagnosis and treatment of PM infection in children.