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    06 May 2022, Volume 37 Issue 5 Previous Issue    Next Issue

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    Expert consensus on clinical practice of medical devices in pediatric respiratory allergic diseases(2022)#br# #br#
    Respirology Group of Pediatric Allergic Committee of China Maternal and Child Health Association, Editorial Committee of Chinese Journal of Practical Pediatrics
    2022, 37(5): 321-327.  DOI: 10.19538/j.ek2022050601
    Abstract ( )  
    A series of expert recommendations on glucocorticoid treatment for pediatric nervous system diseases(Ⅰ)- an introductio
    Neurology Group of Pediatric Society of Chinese Medical Association
    2022, 37(5): 328-330.  DOI: 10.19538/j.ek2022050602
    Abstract ( )  
    A series of expert recommendations on glucocorticoid treatment for pediatric nervous system diseases(Ⅱ)- treatment for anti-Nmethyl-D-aspartate receptor encephalit
    Neurology Group of Pediatric Society of Chinese Medical Association
    2022, 37(5): 330-335.  DOI: 10.19538/j.ek2022050603
    Abstract ( )  
    A series of expert recommendations on glucocorticoid treatment for pediatric nervous system diseases(Ⅲ)- treatment for neuromyelitis optica spectrum disorder
    Neurology Group of Pediatric Society of Chinese Medical Association
    2022, 37(5): 335-338.  DOI: 10.19538/j.ek2022050604
    Abstract ( )  
    A series of expert recommendations on glucocorticoid treatment for pediatric nervous system diseases(Ⅳ)- treatment for pediatric multiple sclero
    Neurology Group of Pediatric Society of Chinese Medical Association
    2022, 37(5): 338-340.  DOI: 10.19538/j.ek2022050605
    Abstract ( )  
    A series of expert recommendations on glucocorticoid treatment for pediatric nervous system diseases(Ⅴ)- treatment for myasthenia gravis
    Neurology Group of Pediatric Society of Chinese Medical Association
    2022, 37(5): 340-343.  DOI: 10.19538/j.ek2022050606
    Abstract ( )  
    A series of expert recommendations on glucocorticoid treatment for pediatric nervous system diseases(Ⅵ)- treatment for Duchenne and Becker muscular dystroph
    Neurology Group of Pediatric Society of Chinese Medical Association
    2022, 37(5): 343-346.  DOI: 10.19538/j.ek2022050607
    Abstract ( )  
    Tricuspid annular plane systolic excursion for evaluation of right ventricular function in children with high altitude heart disease
    ZHANG Qing-you, MA Yu-de, GAO Yu-meng, et al
    2022, 37(5): 347-351.  DOI: 10.19538/j.ek2022050608
    Abstract ( )  
    Objective To explore the value of tricuspid annular plane systolic excursion (TAPSE) for evaluation of right ventricular function in children with high altitude heart disease. Methods A total 21 children with high altitude heart disease (HAHD) admitted to the Peoples Hospital of Tibet Autonomous Region from August 2017 to August 2018 were divided into mild-to-moderate heart failure group (n=13) and severe heart failure group (n=8) according to modified ROSS heart failure score,and 14 healthy children receiving echocardiography because of heart murmur coming from the same district served as control group. All children underwent the routine echocardiography. Their right ventricular outflow tract (RVOT),right ventricular anterior wall (RVAW),tricuspid regurgitation jet velocity and respiration variations of inferior vena cava were determined. Tricuspid annular plane systolic excursion(TAPSE)was measured through M-mode echocardiography. Serum brain natriuretic peptide (BNP) was detected by ELISA. Results (1) Compared with control group,RVOT was significantly increased,RVAW was significantly thickened,while TAPSE significantly decreased in patients with HAHD (P<0.05,respectively). (2) There was no significant difference in RVOT,RVAW or pulmonary artery systolic pressure (PASP) between mild-to-moderate heart failure group and severe heart failure group(P>0.05). However,compared with mild-to-moderate heart failure group,serum BNP significantly increased,and TAPSE significantly decreased in severe heart failure group (P<0.05,respectively). (3)Spearman correlation analysis showed a negative correlation between TAPSE and modified ROSS heart failure score,PASP,and BNP (r=-0.517、-0.718 and -0.749,all P<0.05,respectively). Conclusion TAPSE is a simple,workable and repeatable method to evaluate right ventricular function in children with HAHD,and has a favorable clinical significance for the diagnosis and assessment of severity of HAHD in children.
    Predictive value of the ratio of venous-to-arterial carbon dioxide partial pressure difference to arteriovenous oxygen content difference for peritonitis - associated septic shock in children
    WANG Ying, TIAN Jia-hao, WANG Yi, et al
    2022, 37(5): 352-356.  DOI: 10.19538/j.ek2022050609
    Abstract ( )  
    Objective To investigate the prediction of the ratio of venous-to-arterial carbon dioxide partial pressure difference to arteriovenous oxygen content difference in peritonitis-associated septic shock in children. Methods The clinical data of 63 cases of peritonitis-associated septic shock treated in the Paediatric Intensive Care Unit of the Childrens Hospital Affiliated to Xian Jiaotong University from December 2016 to December 2021 were collected and analyzed retrospectively. The data were analyzed by SPSS21.0 statistical software. According to different prognosis,the children were divided into survival group and death group.  An independent sample t-test was performed to compare the differences in continuous quantitative variables with normal distribution between the two groups. Categorical variables were compared using a Pearsons Chi-square test. Binary Logistic regression was used to evaluate all variables for prognosis prediction,and the receiver operating characteristic (ROC)curve was used to analyze the predictive value of risk factors. The area under the curve (AUC)being >0.5 was considered as being with predictive value. Results A total of 63 children were enrolled,including 30 males and 33 females. There were no significant differences in gender,age,weight or pathogen distribution between the two groups. Compared with the survival group,the death group was higher in proportion of ventilation,surgical intervention and vasoactive agents use,procalcitonin,lactate,the ratio of venous-to-arterial carbon dioxide partial pressure difference to arteriovenous oxygen content difference,pediatric sequential organ dysfunction score,activated partial thromboplastin time,and score of the pediatric risk of mortality,but was lower in platelets,fibrinogen and mean arterial pressure All the differences were statistically significant (all P<0.05). Binary Logistic regression analysis was used to screen the variables and it was found that serum lactate and the ratio of venous-to-arterial carbon dioxide partial pressure difference to arteriovenous oxygen content difference were independent risk factors that might affect prognosis. ROC curve showed that lactate AUC was 0.745 with a sensitivity of 75% and specificity of 71%;AUC of the ratio of venous-to-arterial carbon dioxide partial pressure difference to arteriovenous oxygen content difference was 0.876,with sensitivity being 85% and specificity 87%.The combined AUC was 0.923,sensitivity being 88% and specificity being 91%. Conclusion The ratio of venous- arterial carbon dioxide partial pressure difference to arterial-venous oxygen content difference has a good predictive value for the prognosis of peritonitis-associated septic shock in children.
    Analysis of clinical phenotypes of epilepsy related to CDKL5 deficiency disorder:A report of 20 cases
    MA Rong, LU Yan-li, ZHANG Xi-lian, et al
    2022, 37(5): 357-365.  DOI: 10.19538/j.ek2022050610
    Abstract ( )  
    Objective Totally 20 cases of CDKL5 gene mutation positivity,i. e. CDKL5 deficiency disorder(CDD),were found through gene detection in children with recurrent seizures of unknown etiology to summarize the phenotypic characteristics of epilepsy. Methods A total of 20 children with CDD,who were admitted to the Department of Pediatrics,the First Affiliated Hospital of Tianjin University of Chinese Medicine from April 2016 to December 2021,were included in the study. Their seizure characteristics and evolution were summarized. The whole exon group was sequenced by high-throughput sequencing technology,and the phenotypic analysis was conducted after the gene detection results were obtained. Results Twenty children with CDD presented with recurrent seizures,including one male and 19 females;17 cases had obvious intellectual and motor development delay. The onset age of epileptic seizure was 2 days to 1 year after birth,and the common types of first seizure were generalized tonic-clonic seizure,focal seizure and tonic seizure. Fourteen children successively experienced epileptic seizure,all of which occurred before 8 months old. In the course of 12 childrens diseases,the electroencephalogram recorded the peak ataxia. All 20 children were treated with antiepileptic drugs. Except for one patient who was treated with sodium valproate alone,the rest of the cases received combination therapy. A total of 19 CDKL5 gene mutation types were found,5 of them have been reported,and the remaining 14 were new mutations. In this study,CDKL5 mutations were divided into eight groups according to the analysis of protein structural regions involved by gene mutation,of which one case was in Group H,with a good prognosis. Conclusion The phenotypic characteristics of epilepsy in children with CDD are summarized and it is analyzed that its characteristics are related to the mutation types and function of CDKL5 gene.
    Expression of HLA-DRB1 alleles in children with Helicobacter pylori infection and immune thrombocytopenia
    GUO Jing, WU Run-hui, WU Jie, et al
    2022, 37(5): 366-371.  DOI: 10.19538/j.ek2022050611
    Abstract ( )  
    Objective To investigate the HP (Helicobacter pylori) infection and ITPs (Immune thrombocytopenia) susceptibility genes and protective genes by detecting HLA-DRB1*03、*04、*07、*11、*14 alleles in ITP patients,HP patients,the patients with HP infection and ITP,and healthy children. Methods Collected the whole blood of ITP patients,HP patients,the patients with HP infection and ITP,and healthy children treated in BCH from May 2014 to January 2015. Extracted DNA from samples and tested the HLA-DRB1*03、*04、*07、*11、*14 allele frequencies by PCR-SSP. Compared the gene frequency among different groups. Results There are 136 Han nationality children in this study. The distribution frequency of HLA-DRB1*04 was 21.32%,HLA-DRB1*07 was 19.12%,HLA-DRB1*14 was 15.44%,HLA-DRB1*11 was 11.76%,HLA-DRB1*03 was 8.82%. There was no statistically significant difference between genders. Compared to the above HLA alleles frequency in a different group,the frequency of HLA-DRB1*07 in ITP children was 30.5%,in healthy controls was 9.7%(OR=4.1,χ2=4.393,P=0.036). The difference was statistically significant. The frequency of HLA-DRB1*14 in patients with HP infection and ITP was significantly higher than in the HP infection group (OR=4.8,χ2=5.435,P=0.02). Compared the same group but with different gender,the HLA-DRB1*04 alleles frequency of the girls with HP infection(40%) was higher than the boys with HP infection(4.8%). According to statistical analysis,there was a significant difference between the two groups. Conclusion HLA-DRB1*07 allele may be the susceptibility gene of ITP. HP infected children with the HLA-DRB1*14 allele may have a higher risk to develop ITP. HLA-DRB1*04 allele may be the susceptibility gene of girls infected with HP. 
    Study on the ageand genderspecific reference intervals of trace elements and the relationship among the trace elements in children aged 10 to 12 years in Chongqing
    ZHONG Hai-ying, WANG Yu-wei, TANG Xian, et al
    2022, 37(5): 372-378.  DOI: 10.19538/j.ek2022050612
    Abstract ( )  
    Objective This study aims to discuss the reference intervals of trace elements and the relationships among trace elements in children aged 10-12 years in Chongqing. Methods Stratified cluster sampling was used to include 2058 children aged 10-12 years from urban and rural areas of Chongqing. Venous blood was collected and trace element levels in whole venous blood were measured. Non-parametric reference value estimation method was used to calculate the reference value of trace elements,and generalized linear regression model was used to analyze the relationships among trace elements. Results The study showed that total reference intervals of Ca,Zn,Mg,Cu and Fe were 1.35-2.13 mmol/L,56.42-107.0 μmol/L,1.23-1.81mmol/L,8.25-22.37 μmol/L and 6.14-9.45 mmol/L,respectively. The levels of Mg,Cu and Fe in girls were lower than those in boys(P<0.05);the levels of Zn and Cu in urban were higher than those in rural children,and their levels of Ca,Mg and Fe were lower than those in rural children (P<0.01).The levels of Ca and Cu decreased with the increase of age,while the levels of Zn,Mg and Fe increased with the increase of age. The generalized linear regression model showed that there were significant relationships among Ca,Zn,Mg,Cu and Fe (P<0.01). Conclusion The study has established the age- and gender-specific reference intervals of Ca,Zn,Mg,Cu and Fe of adolescent. Besides,age,gender and urban-rural areas have significant impact on trace element levels,which should be taken into account in the prevention and treatment of childrens nutritional diseases.
    A multicenter clinical study of tocilizumab in the treatment of systemic-onset juvenile idiopathic arthritis
    LIANG Fang-fang, LIU Da-wei, FAN Zhi-dan, et al
    2022, 37(5): 379-384.  DOI: 10.19538/j.ek2022050613
    Abstract ( )  
    Objective To explore the critical time points of tocilizumab in treating systemic-onset juvenile idiopathic arthritis (SJIA) and evaluate the short-term and long-term efficacy and safety of early use of tocilizumab. Methods It is a multicenter prospective cohort study. We enrolled 88 children with first-onset moderate to severe active SJIA from 5 research centers,including Shenzhen Childrens Hospital,Childrens Hospital of Chongqing Medical University,Peking Union Medical College Hospital,Childrens Hospital of Fudan University,and Childrens Hospital of Nanjing Medical University,from November 1,2017,to November 1,2020. The patients were divided into tocilizumab group(n=51) and control group(n=37). After short-term (three months and six months) and long-term (one year and two years) treatment,we compared analyzed the clinical effects between the two groups. Results The two groups C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) decreased significantly after 3 and 6 months of treatment,respectively. In the tocilizumab group,the two indices in more than 90% of patients decreased to the normal level,which had significant difference (P<0.05) compared with the control group. There were significant differences between the two groups in the dosage of hormone use at the initial stage(P<0.05),as well as after three and six months of treatment (P<0.05). The hormone dosage was significantly reduced,and the proportion of the patients who successfully stopped using hormone in half a year increased significantly. The short-term compliance rates of ACRPedi 30/50/70/90 in tocilizumab group and control group were significantly different (P<0.05),being 100.0%/94.0%/70.0%/36.0% and 97.2%/77.8%/50.0%/8.3% after 3 months,and 100.0%/95.5%/84.4%/57.8% and 96.4%/64.3%/42.9%/10.7% after 6 months,respectively. Comparing the long-term efficacy between the two groups,the compliance rate of ACRPedi 70/90 in the tocilizumab group was significantly higher than that in the control group (P<0.05). The most common adverse reaction was respiratory tract infections in the two groups,and no other serious adverse events occurred. Conclusion The early use of tocilizumab in moderate and severe SJIA is effective. Tocilizumab can efficiently relieve the inflammatory index and reduce the hormone dosage,achieving the goal of rapidly reducing the dosage or even stopping the use of hormone. It provides a good effect on improving disease activity in the long time.
    Study on the efficacy and safety of Bi Dou Yan oral liquid in the treatment of acute rhinosinusitis in children
    WEI Ping, LIU Da-xin, XU Zheng-min, et al
    2022, 37(5): 385-390.  DOI: 10.19538/j.ek2022050614
    Abstract ( )  
    Objective To investigate the efficacy and safety of Bi Dou Yan oral liquid in the treatment of children with acute rhinosinusitis. Methods A multi-center,randomized,double-blind,controlled clinical research method was adopted. From September 2016 to February 2019,480 children with acute rhinosinusitis from 17 centers in China were enrolled,and were randomly divided into two groups with 240 cases in each one. The experimental group was given conventional treatment and Bi Dou Yan oral liquid;the control group was given conventional treatment and placebo;the efficacy and safety were evaluated. Results The disappearance time of main symptoms in the experimental group (5 days) was shorter than that of the control group (7 days) (P<0.01). The objective quantitative score of nasal endoscopy (P<0.01) and traditional Chinese medicine (TCM) syndrome score (P<0.01) were significantly lower in the experimental group than those in control group(P<0.01);the total effective rate of TCM syndromes in the experimental group was significantly higher than that in the control group(P<0.01). The recurrence rate of major symptoms (P>0.05) and the Visual Analogue Scale (VAS) score (P>0.05) were lower inthe experimental group than in control group,but there was no statistical difference between the two groups. There was no significant difference in the incidence of adverse events or drug-related adverse reactions between the two groups (P>0.05). Conclusion Bi Dou Yan oral liquid has significant effect and good safety in the treatment of pediatric acute rhinosinusitis,and is worthy of widespread application in pediatric clinical medication.
    Chronic granulomatous disease combined with destruction of rib bone:A report of 1 case
    LI Yu, ZHONG Li-li, XIE Liang-yi, et al
    2022, 37(5): 391-395.  DOI: 10.19538/j.ek2022050615
    Abstract ( )  
    DIGFAN syndrome caused by MORC2 gene mutation: A report of 1 case
    TAO Liang, JIANG Wei, XIAO Nong
    2022, 37(5): 396-400.  DOI: 10.19538/j.ek2022050616
    Abstract ( )