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 explore the influencing factors of fetal growth restriction (FGR) in patients with preeclampsia (PE) and construct a Nomogram prediction model.Methods:From Aug. 2021 to May. 2023, 273 PE patients admitted to our hospital were regarded as the study subjects, and grouped into a modeling group (n=191) and a validation group (n=82). Multivariate logistic regression analysis was applied to determine the influencing factors of FGR in PE patients. R4.3.1 was applied to construct a Nomogram model for predicting FGR in PE patients. Receiver operating characteristic (ROC) curve and the Hosmer Lemeshoe (H-L) goodness of fit test were applied to evaluate the discrimination and consistency of the Nomogram model in predicting FGR in PE patients.Results:There was no statistically obvious difference in gestational age, blood pressure, hemoglobin, urinary protein (UP), uric acid, umbilical artery systolic/diastolic blood pressure (S/D), D-dimer (D-D), or birth frequency between the modeling group and the validation group ( P>0.05). Compared with no concurrent FGR group, the onset of pregnancy in the concurrent FGR group was earlier, the levels of UP, S/D, and D-D, and the proportion of oligohydramnios were obviously higher, and the platelet count (PLT) was obviously lower ( t/χ 2=2.588, 1.437, 6.262, 5.464, 9.881, 3.326, P<0.05). Multivariate Logistic regression analysis showed that UP, S/D, D-D, and oligohydramnios were risk factors for FGR in PE patients ( OR=1.004, 3.807, 1.006, 4.348, P<0.05), while PLT was a protective factor ( OR=0.980, P<0.05). Nomogram model showed that when the total score of the above 5 influencing factors in PE patients was 149, the probability of concurrent FGR was 60%; when the total score was 167, the probability of concurrent FGR was 90%, and the probability of exceeding 167 was over 90%. Modeling group H-L test χ 2=6.736, P=0.565, validation group χ 2=5.812, P=0.668. The area under the ROC curve (AUC) of the modeling group and the validation group was 0.924 (95% CI: 0.883-0.965) and 0.932 (95% CI: 0.880-0.984), respectively. The sensitivity was 83.93% and 90.48%, and the specificity was 89.63% and 81.97%, respectively. Decision curve analysis (DCA) was applied to evaluate the clinical application value of the Nomogram model in predicting FGR in PE patients. Conclusion:The Nomogram model constructed based on the five indicators of UP, S/D, D-D, PLT, and oligohydramnios for predicting the risk of FGR in PE patients has high discrimination and consistency.

Adenovirus pneumonia is a common respiratory tract infection in children.Severe cases of adenovirus pneumonia have the characteristics of rapid onset, rapid progression and a panel of complications.Therefore, early recognition, diagnosis and treatment are particularly important.In recent years, studies on the pathogens, hosts, laboratory tests and biomarkers have provided new clinical clues for the early recognition and diagnosis of severe adenovirus pneumonia in children.There are currently no specific antiviral drugs for severe adenovirus pneumonia.Therefore, the main therapeutic strategies for adenovirus pneumonia are immune therapy and respiratory support.A large number of clinical studies have provided new ideas for standardizing therapeutic strategies and improving the prognosis of children.

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.

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.

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.

Objective:To analyze the clinical features of bronchiectasis in children after severe adenovirus pneumonia and to provide clinical clues for the early diagnosis of bronchiectasis in children after severe adenovirus pneumonia.Methods:A retrospective study was made to analyze the clinical data of 26 children with bronchiectasis after severe adenovirus pneumonia treated in Guangzhou Women and Children′s Medical Center, Guangzhou Medical University from May 2016 to May 2021.Results:A total of 26 cases were reported, including 18 males and 8 females.The median onset age of severe adenovirus pneumonia was 23.0 (15.0, 48.0) months.A total of 23 cases suffered concurrent infections, and bacterial co-infection was the most common (16 cases). High resolution computed tomography (HRCT) showed multiple lobar solids in the lung with/without pleural effusion.During the acute phase, most of the cases were treated with intravenous immunoglobulin (21 cases), mechanical ventilation (20 cases), and systemic glucocorticoids (19 cases). The median age at diagnosis of bronchiectasis was 29.5 (21.0, 56.8) months, and the median time that the patients took to develop into acute adenovirus pneumonia was 6.0 (3.3, 13.0) months.Six cases suffered bronchiectasis alone, and 20 cases had bronchiectasis combined with post-infectious bronchiolitis obliterans (PIBO). Of these 20 cases, 3 cases developed bronchiectasis and PIBO simultaneously, and the remaining 17 cases developed bronchiectasis after PIBO.In the included 26 cases, diffuse bronchiectasis predominated (24 cases), most frequently involving the left lower lobes (21 cases) and right lower lobes (21 cases). Cylindrical bronchiectasis was the most common type (23 cases). All the patients had recurrent cough and wheezing during follow-up, and only 3 cases coughed up pus sputum without hemoptysis.All children had acute exacerbations, which were mostly caused by bacteria (21 cases). Nineteen cases combined with PIBO and 1 case with only bronchiectasis were rehospitalized.There was no cases of surgical resection or death.Conclusions:Bronchiectasis after severe adenovirus pneumonia mostly occurs in patients with or without PIBO.Multiple lobe involvement and co-infection may be a risk factor for PIBO patients to develop bronchiectasis.The clinical manifestations are mostly recurrent cough and wheezing, while sputum and hemoptysis are less common.Pediatricians should promptly perform chest HRCT for early diagnosis of the disease.

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.

It is of great significance to use biosynthesis to transform the inorganic substance formaldehyde into organic sugars. Most important in this process was to find a suitable catalyst combination to achieve the dimerization of formaldehyde. In a recent report, an engineered glycolaldehyde synthase was reported to catalyze this reaction. It could be combined with engineered D-fructose-6-phosphate aldolase, a "one-pot enzyme" method, to synthesize L-xylose using formaldehyde and the conversion rate could reach up to 64%. This process also provides a reference for the synthesis of other sugars. With the increasing consumption of non-renewable resources, it was of great significance to convert formaldehyde into sugar by biosynthesis.
Biocatalysis ; Formaldehyde ; chemistry ; Fructose-Bisphosphate Aldolase ; metabolism ; Xylose ; chemical synthesis

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.