Objective To investigate the clinical effect of live combined bifidobacterium,lactobacillus and enterococcus powder (probiotics bifico) treatment in children with secondary diarrhea of pneumonia and analysis of related factors.Methods Three hundred and eighty-five pneumonia children were studied.The related factors of secondary diarrhea of pneumonia were screened.One hundred and twenty cases of children with secondary diarrhea of pneumonia were divided into observation group and control group by table of random digit with 60 cases each.The observation group was given the probiotics bifico combined with conventional treatment,the control group was given the conventional treatment.The clinical efficacy was evaluated treatment after 7 d.Results The effective rate of observation group was significantly higher than that of control group [96.7％(58/60) vs.61.7％(37/60),x2 =22.282,P＜ 0.01].Age of onset,hospitalization time,invasive procedures,combination therapy of antibiotics,therapy of hormone and therapy of probiotics bifico were related with the incidence of secondary diarrhea of pneumonia (P ＜ 0.01 or ＜ 0.05).Conclusions The younger children,long hospital stay,invasive procedures,combination therapy of antibiotics and therapy of hormone are the risk factors of secondary diarrhea of pneumonia.The probiotics bifico for treating the children with secondary diarrhea of pneumonia has exact clinical efficacy,and it is worthy of clinical application.

Objective To investigate the relationship between the level of circulating CD133+/KDR+ endothelial progenitor cells (EPCs) and outcome in patients with acute ischemic stroke.Methods Inpatients with first-ever ischemic stroke within 24 hfrom the onset and age-and sex-matched healthy subjects were enrolled in the study.The demographic and clinical data of the patients were collected.The level of CD133+/KDR+ EPCs was detected by flow cytometry.All patients were followed up at 90 d.The modified Rankin Scale was used to evaluate the clinical outcome,0-2 was defined as good outcome and >2 was defined as poor outcome.Results A total of 126 consecutive patients with first-ever ischemic stroke within 24 hfrom the onset and 60 age-and sex-matched healthy subjects were enrolled.In patients with ischemic stroke,33 (26.19%) were large artery atherosclerosis (LAA),74 (58.73%) were small artery occlusion (SAO),19 (15.08%) were cardioembolism (CE);82 (65.08%) had good outcomes and 44 (34.92%) had poor outcomes.The number of circulating EPCs at baseline in patients of the LAA subtype (0.071%±0.018%),CE subtype (0.068%±0.16%) and SAO subtype (0.118%±0.12%) was significantly lower than that in the control group (0.246%±0.052%;all P<0.05),and the CE subtype (P=0.028) and LAA subtype (P=0.037) were significantly lower than the SAO subtype;the CE subtype was lower than the LAA subtype,but the difference was not statistically significant (P=0.762).The proportions of patients with LAA subtype (40.91% vs.18.29%;χ2=7.577,P=0.006) and CE subtype (29.55% vs.7.32%;χ2=11.049,P=0.001) and atrial fibrillation (29.55% vs.10.98%;χ2=6.582,P=0.009),and age (69.64±9.62 years vs.61.12±7.31 years;t=5.570,P<0.001),and baseline NIHSS score (14.16±4.22 vs.6.96±2.04;t=12.919,P<0.001),baseline systolic blood pressure (176.06±13.42 mmHg vs.164.12±11.69 mmHg,1 mmHg=0.133 kPa;t=5.187,P<0.001),low-density lipoprotein cholesterol (2.92±0.52 mmol/L vs.2.49±0.36 mmol/L;t=5.447,P<0.001),fasting blood glucose (8.76±2.88 mmol/L vs.6.82±2.24 mmol/L;t=4.185,P<0.001),C-reactive protein (7.62±1.82 mg/L vs.4.57±1.58 mg/L;t=9.790,P<0.001),and D-dimer (1.14±0.08 mg/L vs.0.97±0.22 mg/L;t=4.946,P<0.001) levels in the poor outcome group were significantly higher than those in the good outcome group,while the proportion of the SAO subtype patients (29.55% vs.74.39%;χ2=23.759,P<0.001),high-density lipoprotein cholesterol (0.94±0.68 mmol/L vs.1.16±0.14 mmol/L;t=2.829,P=0.005),and baseline EPCs (0.069%±0.018% vs.0.098%±0.021%;t=7.755,P<0.001) were significantly lower than those in the good outcome group.Multivariate logistic regression analysis showed that the higher baseline NIHSS score (odds ratio 1.242,95% confidence interval 1.126-1.372;P<0.001),CE subtype (odds ratio 3.460,95% confidence interval 1.312-5.146;P=0.016),and the lower baseline EPCs (odds ratio 1.632,95% confidence interval 1.006-3.024;P<0.001) were the independent risk factors for poor outcome in patients.Conclusion s The level of circulating EPCs was decreased significantly in patients with acute ischemic stroke,and the lower level of baseline EPCs was an independent predictor of poor outcome in patients with ischemic stroke at 90 d.

Objective:To investigate the quality of life in children with McCune-Albright syndrome (MAS) and its influencing factors, so as to provide scientific basis for improving their quality of life.Methods:The clinical data of 31 children with MAS diagnosed (MAS group) and followed up in Henan Children′s Hospital from June 2015 to December 2021 were retrospectively analyzed.During the same period, 37 healthy age and sex-matched children at a ratio of 1∶1 were recruited as healthy control group.The children′s Quality of Life Universal Core Scale (PedsQL?4.0) was used for the investigation and comparative analysis.Statistical analysis was performed using the independent sample t test, Chi- square test and multiple regression analysis. Results:Compared with the healthy control group, the physiological function ( t=2.092, P<0.05), emotional function ( t=2.373, P<0.05) and total score ( t=2.360, P<0.05) of MAS group significantly decreased.Multiple regression analysis showed that physiological function was negatively correlated with the annual number of vaginal bleeding ( t=-2.367, P<0.05) and the age of first fracture ( t=-2.606, P<0.05). Social function was negatively correlated with the number of fractures ( t=-2.481, P<0.05). Conclusions:The overall quality of life of MAS children is low, especially the quality of physiological function and emotional function.The annual number of vaginal bleedings, the age of the first fracture and the number of fractures are influencing factors for the reduction of the quality of life of children with MAS.

Objective:To investigate the clinical and molecular characteristics of 11β-hydroxylase deficiency(11β-OHD) to improve the understanding of this disorder.Methods:The clinical manifestation, hormone level, imaging examination, characteristics of gene variation and follow-up of five patients with 11β-OHD diagnosed in Henan Children′s Hospital from 2016 to 2021 were carefully reviewed.Results:Among the 5 children, 3 were male and 2 were female, all without positive family history. The age at diagnosis was 1 year 5 months to 7 years(average 3 years and 9 months), and the bone age was 3 years 6 months to 16 years(average 10 years and 3 months). Two cases were misdiagnosed as 21-hydroxylase deficiency(21-OHD) and treated with long-term mineralocorticoids. Three patients presented with hypertension and one patient had testicular adrenal rest tumor. Adrenal CT showed bilateral adrenal hyperplasia in five patients. ACTH, 17-hydroxyprogesterone, testosterone, and androstenedione levels were increased in 5 children, and hypokalemia occurred in 1 patient. One patient carried homozygous novel missense variant, and four patients had compound heterozygous variants. Four patients carried missence mutations, two patients had deletion and one patient harbored a chimeric CYP11B2 exon1-6/CYP11B1 exon7-9. Three novel CYP11B1 mutations, including c. 1385T>C(p.L462P), c.64C>T(p.Q22*)and c. 1354G>A(p.G452R) were identified. The final height of 2 male children were 164.4 cm and 150.2 cm, respectively, and the related hormone levels of the other 3 children were normal.Conclusion:11β-OHD is easily misdiagnosed, leading to severe impairment of final height. CYP11B1 gene variation is complex and diverse, which requires variety of gene detection methods.

Objective:To explore the clinical features and genetic variants in three children suspected for β-ketothiolase deficiency (BKTD).Methods:Clinical manifestations, laboratory examination and genetic testing of three children suspected for BKTD at Henan Children′s Hospital between January 2018 and October 2022 were collected, and their clinical and genetic variants were retrospectively analyzed.Results:The children were all males with a age from 7 to 11 months. Their clinical manifestations have included poor spirit, shortness of breath, vomiting, convulsions after traumatic stress and/or infection. All of them had severe metabolic acidosis, elevated ketone bodies in blood and urine, hypoglycemia, with increased isoprenyl-carnitine and 3-hydroxyisovalyl-carnitine in the blood, and 2-methyl-3-hydroxybutyrate and methylprotaroyl glycine in the urine. All of them were found to harbor compound heterozygous variants of the ACAT1 gene, including c. 1183G>T and a large fragment deletion (11q22.3-11q23.1) in child 1, c. 121-3C>G and c. 826+ 5_826+ 9delGTGTT in child 2, and c. 928G>C and c. 1142T>C in child 3. The variants harbored by children 2 and 3 were known to be pathogenic or likely pathogenic. The heterozygous c. 1183G>T variant in child 1 was unreported previously and rated as a variant of unknown significance (PM2_Supporting+ PP3+ PP4) based on guidelines from the American College of Medical Genetics and Genomics. The large segment deletion in 11q22.3-11q23.1 has not been included in the DGV Database and was rated as a pathogenic copy number variation. Conclusion:The variants of the ACAT1 gene probably underlay the pathogenesis of BKTD in these three children.