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AMA may cause inhibition of proliferation, survival, and migration of hippocampal neural stem cells. AMA may also affect their differentiation into neurons and astrocytes, which will eventually lead to developmental disorders of hippocampal neural stem cells in offspring rats.To study the clinical effect of oral sirolimus in the treatment of children with blue rubber bleb nevus syndrome (BRBNS) in the gastrointestinal tract, a retrospective analysis was performed on the clinical data and follow-up results of two children with BRBNS treated by sirolimus. The two children with BRBNS had gastrointestinal bleeding and anemia and were treated with sirolimus at a dose of 1 mg/day as part of treatment. The plasma concentration of the drug was maintained between 2.5-12.0 ng/mL. The children showed disappearance of gastrointestinal bleeding and improvements in anemia and coagulation function, and blood transfusion could be stopped during treatment, with no obvious adverse drug reactions. PubMed, Wanfang Data, and CNKI were searched for related articles on sirolimus in the treatment of BRBNS. A total of 26 cases of children with BRBNS, aged 0-18 years, were obtained. With the addition of the 2 cases in this study, sirolimus treatment achieved a satisfactory clinical effect in all 28 cases. Sirolimus may be effective and safe in the treatment of children with BRBNS, and further prospective studies are needed to evaluate the long-term efficacy of this drug.

To evaluate the value of capsule endoscopy in children with small intestinal diseases with hematochezia as the chief complaint.

A retrospective analysis was performed on the clinical data and capsule endoscopy findings of 93 children with hematochezia who were admitted to Children's Hospital of Fudan University from May 2015 to January 2019 and underwent capsule endoscopy. According to the capsule endoscopy findings of the jejunum and the ileum, they were divided into a positive lesion group with 39 patients and a negative lesion group with 54 patients. Related clinical data and the features of lesion on capsule endoscopy were analyzed for the two groups.

There were no significant differences in age, sex, duration of capsule endoscopy, gastric transit time, and small intestinal transit time between the positive lesion and negative lesion groups (P>0.05). The positive lesion group had a significantly lower level of hemoglobin than the negative lesion group (P<0.05). Hemoglobin level was negatively correlated with the rate of positive lesions on capsule endoscopy (r=-0.342, P=0.001). Among the 39 patients with positive lesions on capsule endoscopy, the detection of Meckel's diverticulum was the highest (41%), followed by inflammatory bowel disease (21%).

Capsule endoscopy has a certain value in detecting small intestinal diseases, especially diseases in the jejunum and the ileum, in children with lower gastrointestinal hemorrhage.

Capsule endoscopy has a certain value in detecting small intestinal diseases, especially diseases in the jejunum and the ileum, in children with lower gastrointestinal hemorrhage.

To study the clinical screening and genetic diagnosis of children suspected of Prader-Willi syndrome (PWS), as well as the differences in the scores of clinical diagnostic criteria among the children with a confirmed diagnosis of PWS.

A total of 94 children suspected of PWS who were admitted from July 2016 to December 2018 were enrolled as subjects. Methylation-specific multiplex ligation-dependent probe amplification (MS-MLPA) was performed to confirm the diagnosis. For the children with a confirmed diagnosis of PWS, the scores of clinical diagnostic criteria were determined, and the perinatal characteristics were analyzed.

A total of 11 children with PWS were confirmed by MS-MLPA, with a detection rate of 12%, among whom there were 7 boys and 4 girls, with a median age of 3 years and 4 months (range 25 days to 6 years and 8 months) at the time of confirmed diagnosis. Among the 11 children with PWS, only 5 children (45%) met the criteria for clinical diagnosis. The main perinatal characteristics of the children with PWS were decreased fetal movement (9 cases, 82%), cesarean section birth (11 cases, 100%), hypotonia (11 cases, 100%), feeding difficulties (11 cases, 100%), and weak crying (11 cases, 100%).

Gene testing should be performed as early as possible for children suspected of PWS by clinical screening. PWS may be missed if only based on the scores of clinical diagnostic criteria.

Gene testing should be performed as early as possible for children suspected of PWS by clinical screening. PWS may be missed if only based on the scores of clinical diagnostic criteria.

To study the value of cleaved lymphocytes in peripheral blood smear in assisting the early diagnosis of pertussis.

Nasopharyngeal swabs and peripheral blood samples were collected from 107 children with pertussis-like disease. PCR-flow fluorescent hybridization was used to detect the nucleic acids of Bordetella pertussis. Based on the detection results, the children were divided into two groups pertussis (n=52) and non-pertussis (n=55). According to age, the pertussis group was divided into two subgroups <1 year old (n=42) and ≥1 year old (n=10). According to disease severity, the pertussis group was divided into another two subgroups mild (n=45) and severe (n=7). An automatic blood cell analyzer was used to determine peripheral blood cell counts. Wright's staining and peroxidase staining were used to observe and count cleaved lymphocytes under a microscope.

Cleaved lymphocytes in peripheral blood were round with small cytoplast, less cytoplasm and cleaved or lobulated nuclei. According to the negative peroxidase staining results, these cells were confirmed as lymphocytes. Compared with the non-pertussis group, the pertussis group had significantly higher leukocyte count, lymphocyte percentage, platelet count, and percentage of cleaved lymphocytes (P<0.001). For the children with pertussis, the <1 year old subgroup had significantly higher lymphocyte percentage, platelet count, and percentage of cleaved lymphocytes than the ≥1 year old subgroup (P<0.05). The severe subgroup had slightly higher leukocyte count, lymphocyte percentage, platelet count, and percentage of cleaved lymphocytes than the mild subgroup (P>0.05).

The detection of cleaved lymphocytes combined with peripheral blood cell counts provides a new option for the early diagnosis of pertussis in children.

The detection of cleaved lymphocytes combined with peripheral blood cell counts provides a new option for the early diagnosis of pertussis in children.

To study the value of anti-neutrophil cytoplasmic antibody (ANCA) in assessing the severity of bronchiolitis obliterans (BO) in children.

A prospective analysis was performed on 59 children who were diagnosed with BO from June 2009 to October 2014. ELISA was used to measure the concentrations of myeloperoxidase (MPO)-ANCA and proteinase 3 (PR3)-ANCA in serum. According to the results of ELISA, the children were divided into three groups double-negative ANCA (n=22), single-positive ANCA (n=17), and double-positive ANCA (n=20). The three groups were compared in terms of the scores of BO risk factors, clinical symptoms, chest high-resolution computed tomography (HRCT), and lung pathology on admission, as well as the changes in the expression level of ANCA and the scores of clinical symptoms and chest HRCT over time.

Compared with the double-negative ANCA group, the double-positive ANCA group had a significantly higher score of BO risk factors (P<0.05), and the single-positive ANCA group and the double-positive ANCA group had significantly higher scores of clinical symptoms, chest HRCT, and lung pathology (P<0.05). The children were followed up for 6 months after discharge, and there were significant reductions in MPO-ANCA and PR3-ANCA titers from admission and discharge to the end of follow-up (P<0.05), as well as a significant reduction in the score of clinical symptoms from admission to the end of follow-up (P<0.05), while there was no significant change in the score of chest HRCT from admission to the end of follow-up (P>0.05). The single-positive ANCA and double-positive ANCA groups still had a significantly higher score of clinical symptoms than the double-negative ANCA group (P<0.05).

The expression level of ANCA is correlated with the severity of BO in children and thus has certain clinical significance in disease evaluation.

The expression level of ANCA is correlated with the severity of BO in children and thus has certain clinical significance in disease evaluation.

To study the influencing factors for the clinical effect of bronchoalveolar lavage (BAL) in children with Mycoplasma pneumoniae pneumonia (MPP) and atelectasis.

A total of 75 children with MPP and atelectasis were divided into a good response group with 51 children and a poor response group with 24 children according to the clinical effect of BAL treatment. LASSO logistic regression analysis was used to investigate the factors influencing the clinical effect of BAL treatment. The receiver operating characteristic (ROC) curve and restricted cubic spline model analysis were used to evaluate the value of the course of the disease at the time of BAL treatment in predicting the clinical effect of BAL treatment.

Compared with the good response group, the poor response group had a significantly lower percentage of lymphocytes in bronchoalveolar lavage fluid, a significantly higher proportion of children with atelectasis of two or more lung lobes or stenosis of the bronchial cavity or opening caused by inflammainflammation-induced stenosis of the bronchial cavity or opening shown under bronchoscope may indicate a poor clinical effect of BAL treatment.

Early BAL treatment may have a good clinical effect in children with MPP and atelectasis. Atelectasis of two or more lung lobes and inflammation-induced stenosis of the bronchial cavity or opening shown under bronchoscope may indicate a poor clinical effect of BAL treatment.

To study the expression level of cAMP response element-binding protein (CREB) in children with recurrent wheezing under three years of age and its effect on the expression of the serum orosomucoid 1-like protein 3 (ORMDL3) gene.

Thirty-six children with recurrent wheezing under three years of age who visited the hospital from June 2017 to June 2019 were selected as the recurrent wheezing group. Twenty-four healthy children from physical examination were selected as the control group. The CREB expression level in peripheral blood was measured by quantitative real-time PCR. Human bronchial epithelial cells (BEAS-2B) were cultured, and dual-luciferase reporter assay and quantitative real-time PCR were used to investigate the effects of overexpression and siRNA interference of CREB on the promoter activity and mRNA expression of the ORMDL3 gene in the BEAS-2B cells.

The expression level of CREB in the recurrent wheezing group was significantly higher than that in the control group (P<0.001). TPX0046 In BEAS-2B cells, overexpression of CREB significantly up-regulated the promoter activity and mRNA expression of the ORMDL3 gene (P<0.05), while siRNA interference of CREB significantly reduced the promoter activity and mRNA expression of the ORMDL3 gene (P<0.05).

The expression of CREB is increased in children with recurrent wheezing, and CREB may be involved in the pathogenesis of recurrent wheezing by regulating expression of the ORMDL3 gene.

The expression of CREB is increased in children with recurrent wheezing, and CREB may be involved in the pathogenesis of recurrent wheezing by regulating expression of the ORMDL3 gene.

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