Table of Content

15 October 2014 Volume 32 Issue 10

  

The importance of extraintestinal manifestations of inflammatory bowel diseases

HE Weixun, GUO Guimei

. 2014, 32(10):  901.  doi:10.3969 j.issn.1000-3606.2014.10.001

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Inflammatory bowel disease (IBD) is characterized by chronic, relapsing inflammation of the gastrointestinal (GI) tract. In recent years, the incidence is increasing in the pediatric population. Insufficient recognition of extraintestinal manifestations (EIMs) of IBD clinically may delay diagnosis. Therefore, improving the recognition of EIMs of IBD has an important significance in its early diagnosis and treatment. The pediatricians should be alerted that renal lesions of EIMs in IBD are mainly immunoglobulin A (IgA) nephropathy and interstitial nephritis.

Drug resistance of Helicobacter pylori in nodular gastritis in children　

WANG Yuhuan, HUANG Ying, WANG Chuanqing, WU Jie

. 2014, 32(10):  903.  doi:10.3969 j.issn.1000-3606.2014.10.002

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Objective　To analyze the drug resistance of Helicobacter pylori (H.pylori) in nodular gastritis in children and to further explore the rational use of antibiotics for drug-resistant H.pylori strains. Methods A total of 473 children with upper gastrointestinal symptoms undergoing gastroscopy from January 2013 to June 2014 in our hospital were enrolled. Two pieces of gastric antral mucosa in children with nodular gastritis were collected for H.pylori rapid urease test and H.pylori culture. The
resistance of H.pylori to amoxicillin, clarithromycin, metronidazole, moxifloxacin and levofloxacin was detected by agar dilution method and E-test. Results In 473 cases of nodular gastritis, 258 cases were H.pylori culture-positive. The resistance rate of H.pylori isolates to amoxicillin, clarithromycin, metronidazole, moxifloxacin, levofloxacin was 6.2%, 34.9%, 49.2%, 8.9% and 5.0%, respectively. Dual resistance to clarithromycin and metronidazole was 23.3%. Moreover, 405 cases had a family history of H.pylori infection. Conclusions Nodular gastritis is a special sign of H.pylori infection in children. H.pylori infection has obvious familial
aggregation. The low resistance rate of H.pylori to amoxicillin in children with nodular gastritis indicates that amoxicillin can be used as the main drug for eradication of H.pylori. Meanwhile, clarithromycin should be applied according to the drug sensitive test due to high resistance rate of H.pylori to clarithromycin.

Intestinal microflora changes in children with Henoch-Schönlein purpura

LUO Wen, XU Hua, CHEN Xiaoqing, ZENG Huasong, LIN Zhuoyin

. 2014, 32(10):  907.  doi:10.3969 j.issn.1000-3606.2014.10.003

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Objective　To study the intestinal microflora changes in children with Henoch-Schönlein purpura (HSP). Methods　The feces of 64 children with HSP (the experimental group) and 25 healthy children (the control group) were collected. 16S rRNA/DNA fluorescent quantitative polymerase chain reaction were performed to detect bacterial content of Lactobacillus, Bifidobacterium and Escherichia coli in feces. Results　The amounts of Lactobacillus , and Bifidobacterium in the HSP group were lower than those of the healthy group, and the amounts of E.coli were higher, but the differences were not statistically significant. The amounts of Lactobacillus and Bifidobacterium in the HSP children with gastrointestinal symptoms (abdominal pain, vomiting, e.g.) were significantly lower than those in HSP children without gastrointestinal symptoms and in the healthy group. The difference was significant (P<0.05). Conclusions　It suggests that intestinal microflora in children with HSP in acute phase may exist disorders, especially in the HSP children with gastrointestinal symptoms.

The serum levels of galactose-deficient IgA1 in children with Henoch-Schönlein purpura and its clinical significance

YUAN Fang,HU Xiao,WANG Feifei,LIU Lanbo,YIN Lei,MO Xi,JIN Yanliang

. 2014, 32(10):  912.  doi:10.3969 j.issn.1000-3606.2014.10.004

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Objective　To explore the association of galactose-deficient IgA1 levels with clinical features, and further to provide guidance for individualized treatment of HSP. Methods According to the clinical symptoms and curative effect, 57 children with HSP were divided into four groups: non-HSPN group (n=26), HSPN group (n=7), refractory HSP group (n=7) and remission group (n=17). In non-HSPN group, 12 cases received glucorticoid therapy and 14 cases did not. Serum galactose-deficient IgA1 (Gd-IgA1) concentrations were detected using a Helix aspersa-lectin-based enzyme-linked immunosorbent assay (ELISA), and the total IgA1 levels were measured by ELISA. Results The serum Gd-IgA1 level was significantly higher in 40 HSP children who were not cured than that in remission group and control group (P<0.05). However, there was no difference in Gd-IgA1 level between remission group and control group (P>0.05). Compared with the control group, the serum Gd-IgA1 level was significantly higher in HSPN group, non-HSPN group and refractory HSP, and children with refractory HSP had significantly higher Gd-IgA1 level than children in non-HSPN group (P<0.05). No significant difference in Gd-IgA1 level was found either between HSPN group and refractory HSP group or between HSPN group and non-HSPN group (P>0.05). Furthermore, in non-HSPN group, the serum Gd-IgA1 level in HSP children who were not treated with glucorticoid was significantly higher than that in HSP children treated with glucorticoid (P<0.05). Conclusions The serum Gd-IgA1 level is associated with the disease activity and curative effect of HSP, especially in children with refractory HSP, and it is thus likely to be a new non-invasive disease activity marker for guiding the proper usage of glucocorticoid and immunosuppressants in HSP children.

The role of urinary kidney injury molecule-1 in pediatric Henoch-Schönlein purpura

XIA Hui, MAO Youying, ZHOU Wei, CHEN Tongxin

. 2014, 32(10):  918.  doi:10.3969 j.issn.1000-3606.2014.10.005

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Objective　To study the role of urinary kidney injury molecule-1 (KIM-1) in pediatric Henoch-Schönlein purpura (HSP). Methods Urinary levels of KIM-1 were examined using ELISA in 48 children with HSP including 23 HSPN children (HSPN group) and 25 non-HSPN children (HSP group), and 20 healthy children. The levels of urinary creatinine and 24-hour urine protein were also detected. The results were analyzed and compared among groups. Results The ratio of urinary KIM-1/creatinine (Cr) in HSPN children was significantly higher than that in the other two groups (P<0.05). There was no significant difference in the ratio of urinary KIM-1/Cr between HSP group and the control group (P>0.05). The ratio of urinary KIM-1/Cr had no correlation with 24-hour urine protein in all HSP children (r=0.239, P=0.590). Conclusions Urinary KIM-1 may play a role in the pathogenesis of pediatric HSPN.

Distribution of Th17 cells in peripheral blood of children with Henoch-Schönlein purpura and its clinical significance

LI Yanling, WANG Yongqing, ZHU Diqing, ZHOU Yiping

. 2014, 32(10):  921.  doi:10.3969 j.issn.1000-3606.2014.10.006

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Objective　To observe the percentage of Th17 cells in peripheral blood mononuclear cells (PBMCs) and the serum level of interleukin-17 (IL-17) in children with Henoch-Schönlein purpura (HSP). Methods A total of 30 HSP children and 20 healthy children were selected. The percentage of Th17 cells in PBMCs was measured by flow cytometry analysis and the serum level of IL-17 was measured by ELISA method. The results were compared between HSP children and healthy children. Results The percentage of Th17 cells in PBMCs in HSP and healthy children was (2.14 ± 0.90)% and (0.84 ± 0.41)%, respectively, and the difference was significant between two groups (P<0.05). The serum levels of IL-17 in HSP and healthy children were (38.36 ± 13.44) pg/ml and (10.59 ± 4.17) pg/ml, respectively, and the difference was also significant (P<0.05). Conclusions The percentage of Th17 cells in PBMCs was obviously increased in HSP children. It is suggested that Th17 cells may play a role in the pathology of HSP.

Value of B-cell activating factor expression in the diagnosis and prognosis of children with idiopathic thrombocytopenic purpura

ZHONG Yuqiang, WANG Qing

. 2014, 32(10):  924.  doi:10.3969 j.issn.1000-3606.2014.10.007

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Objective　To study the value of B-cell activating factor (BAFF) expression levels in the diagnosis and prognosis of children with idiopathic thrombocytopenic purpura. Methods Children with idiopathic thrombocytopenic purpura (ITP) admitted to our hospital from September 2011 to September 2013 were enrolled in observation group, and healthy check-up children during the same period were enrolled as controls. The serum levels of B-cell activating factor, platelet antibodies (PAIgG, PAIgM, PAIgA) and platelet count were detected, and curative effect in children with ITP was observed. Results The BAFF, PAIgG, PAIgM and PAIgA levels were higher and platelet count were less in both acute and chronic ITP children than those in normal controls. The difference was significant (P<0.05). The results of linear regression analysis indicated that BAFF levels were positively correlated with PAIgG, PAIgM and PAIgA levels (β=1.87~2.25, P<0.05), and were negatively correlated with platelet count (β=-2.42, P<0.05). Meanwhile, BAFF levels were negatively correlated with curative effect in ITP children (β=-1.88, P<0.05). When the value of BAFF was 1.35, the sensitivity for predicting effective treatment was 0.84 and the specificity was 0.75. The area under the ROC curve was 0.85 (95%CI: 0.73~0.98, P=0.000). Conclusions The serum BAFF expression was increased and closely related to the level of antiplatelet antibody, platelet count and treatment effect in ITP children, and therefore, was a good indicator for the diagnosis and prognosis of ITP.

Expression of 4-1BB and lymphocyte subsets in peripheral blood of children with acute infectious lymphocytosis

FU Qiang, FENG Qihua, YU Konggui, TANG Linfei, LI Aimin, SONG Xiaoxiang

. 2014, 32(10):  928.  doi:10.3969 j.issn.1000-3606.2014.10.008

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Objective　To investigate expression of 4-1BB and lymphocyte subsets in peripheral blood of children with acute infectious lymphocytosis. Methods Flow cytometry (FCM) was applied to detect the expression of 4-1BB and lymphocyte subsets in peripheral blood of 15 cases of acute infectious lymphocytosis and 20 cases of acute upper respiratory infection, and 20 healthy children. Results The expression of 4-1BB and CD3+, CD4+ and CD8+ lymphocytes were higher in acute infectious lymphocytosis group than those in acute upper respiratory infection group and healthy control group (P<0.05). There was no significant difference of CD19+CD23+ lymphocytes among three groups (P>0.05). A positive correlation was found between 4-1BB expression and CD3+ lymphocytes expression in acute infectious lymphocytosis group (r=0.73, P<0.05). Conclusions The abnormal expression of 4-1BB may play a pathological role in the development of acute infectious lymphocytosis. T cells in children with acute infectious lymphocytosis may not function to activate B cells.

Effect of clinical pathway management on pediatric capillary bronchitis

YIN Lijuan, PENG Donghong, YANG Yang, HUANG Ying, DAI Jihong

. 2014, 32(10):  931.  doi:10.3969 j.issn.1000-3606.2014.10.009

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Objective　To evaluate the effect of clinical pathway management on pediatric capillary bronchitis. Methods Infants with capillary bronchitis admitted to our hospital were selected. Several indices were compared between the infants with and without clinical pathway management including hospital stay, costs of hospitalization, satisfaction in parents of children, cure rate, readmission rate one week after discharge, hospital infection and variation in the process of clinical pathway management. Results A total of 204 eligible infants were divided into research group (n=96) and control group (n=108). There were no significant differences in sex, age, respiratory rate, heart rate and temperature, and detection rate of respiratory syncytial virus in nasopharyngeal secretion and sputum culture (P>0.05). Compared with the infants in control group, the total drug costs, the antibiotics costs and the average length of stay were significantly decreased in infants with clinical pathway management (P<0.05). There were no significant differences in such indices as satisfaction in parents of children, cure rate, readmission rate one week after discharge and hospital infection rate between two groups (P>0.05). In research group, 49 infants (51.04%) completed the clinical pathway management. Positive variance was found in 43 infants (44.79%) and negative variance in 4 infants (4.17%). Two infants (2.08%) dropped out. Conclusions For capillary bronchitis in infants, clinical pathway management has an effect on controlling and reducing the medical expenses, and meanwhile improving the medical quality and satisfaction of patients.

Disseminated Staphylococcus aureus infection in children: a clinical analysis of 14 cases

WU Hujun, WEN Shunhang, LIN Li, LIU Liu, GUO Shuzhen, LI Changchong

. 2014, 32(10):  936.  doi:10.3969 j.issn.1000-3606.2014.10.010

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Objective To study the disseminated Staphylococcus aureus infection (DSAI) in children. Method Clinical features, treatment and prognosis data of 14 children with DSAI admitted to Yuying Children’s Hospital Affiliated to Wenzhou Medical University from January 2006 to December 2013 was retrospectively reviewed. Results 14 children with DSAI occurred in community, median age: 15m (range 6d–13y); 50% male (7 cases). All patients presented with fever. Addition to fever, the first symptom was skin and soft tissue infections (SSTIs，6 cases) as well as limb and/or joint pain (5 cases). Among children with DSAI, white blood cell count and C-reactive protein values increased significantly. Pyogenic infection site were skin and soft tissue in 12 cases (85.7%), pulmonary (12 cases), bone (4 cases), joint (3 cases), central nervous system (3 case), and pericardium (1 case). SSTIs concurrent with pulmonary infection was found in 10 cases (71.4%). Incision and drainage of skin and soft tissue absc sses were performed in 9 cases, joint debridement and vacuum sealing drainage (VSD) in 3 cases, osteomyelitis debridement and VSD in 3 cases, and closed chest drainage in 3 cases. All cases received vancomycin and/or linezolid treatment, 5 cases supplemented by rifampicin, and intravenous immune globulin therapy was administered in 11 cases. Clinical manifestations were cured or improved in 12 cases (85.7%). Conclusions Clinical diagnosis of DSAI in children needs to be vigilant. SSTIs, bone and joint infections were major precipitating factors. Intravenous immune globulin therapy was supplemented to the application of antibiotics, which might get better clinical outcomes in children.

The effect of standardized usage of antibiotics and hand hygiene on carbapenem-resistant Enterobacteriaceae infection in neonates

CHEN Juan, HE Huayun, JU Yan, HUA Ziyu

. 2014, 32(10):  940.  doi:10.3969 j.issn.1000-3606.2014.10.011

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Objective　To investigate the effect of standardized usage of antibiotics and hand hygiene on carbapenem-resistant Enterobacteriaceae (CRE) infection in neonates. Methods According to the situation of standardized management of antibiotics in our hospital, the study period, from January 2011 to December 2013, was divided into pre-intervention, transition and post-intervention. Retrospective study was employed to analyze the antibiotics use density (AUD), the hand hygiene compliance rate among the medical staff, the positive rate of CRE and the nosocomial infection percentage of the neonatology department. Results From pre-intervention to post-intervention period, the AUD was decreased from 12.93 DDDs to 8.10 DDDs; hand hygiene compliance rate of medical staff was increased from 53.49% to 83.33%; the detection rate of CRE was decreased from 3.71% to 2.27%. The differences were all significant among different periods (P<0.01). The nosocomial infection percentage decreased from 0.22% to 0.17% and there was no significant difference (P=0.441). Conclusions Standardized usage of antibiotics and hand hygiene can be served as economical yet effective approaches to controlling CRE infection in neonates.

The association between serum albumin levels and prognosis in children with hand-foot-mouth disease

SHEN Huaiyun, ZHU Hongwei, ZHOU Rui, YANG Xiaoyun, XU Jiali

. 2014, 32(10):  945.  doi:10.3969 j.issn.1000-3606.2014.10.012

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Objective　To observe levels of the serum albumin and their association with prognosis in children with hand-foot-and-mouth disease (HFMD) . Methods A total of 122 HFMD children admitted from 2011 to 2013 were selected and divided into three groups: common group (n=57), severe group (n=52) and critical group (n=13). Serum albumin levels, blood glucose levels, peripheral blood white cell counts of the children in the three groups were compared within 24 hours of admission. Moreover, the incidence of hypoalbuminemia and mortality of the children were analyzed in each group. Results In critical group, serum albumin levels were significantly decreased while blood glucose levels and peripheral blood white cell counts were significantly increased than those in the severe group and common group, and the differences were all statistically significant (all P<0.05). There were no significant differences in serum albumin levels, blood glucose levels and peripheral blood white cell counts between severe group and common group (all P>0.05). There was no hypoalbuminemia and death in severe group and common group. The percentage of hypoalbuminemia and mortality of children in critical group were higher than those in the other two groups (all P<0.01). Conclusions The mortality was extremely high in children with critical HFMD. The decrease of serum albumin levels was a high risk factor for death in children with critical HFMD. Early and dynamic monitoring serum albumin levels may be helpful in evaluation of disease condition and prognosis of HFMD.

The expressions of Clara cell secretory protein and IL-17 in serum of children with Mycoplasma pneumoniae pneumonia and its significance

LI Yuhong, WANG Jinrong

. 2014, 32(10):  948.  doi:10.3969 j.issn.1000-3606.2014.10.013

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Objective　To explore the serum expression levels of Clara cell secretory protein (CCSP) and interleukin17 (IL-17) in children with Mycoplasma pneumoniae pneumonia (MPP) and its clinical significance. Methods A total of 72 children with MPP admitted to our hospital from May 2013 to November 2013 were enrolled including 38 cases in acute phase (20 severe cases, 18 mild cases) and 34 cases in recovery phase (18 severe cases, 16 mild cases). Meanwhile, 22 healthy children were selected as normal controls. The serum levels of CCSP and IL-17 were measured by ELISA. Results In acute phase of MPP, serum levels of CCSP decreased while IL-17 levels elevated compared with convalescent MPP children and healthy controls, the differences were statistically significant (P<0.05). There were no significant differences in serum levels of CCSP and IL-17 between convalescent MPP children and healthy controls (P>0.05). The level of CCSP were negatively correlated with IL-17 in acute phase of MPP (r=-0.75, P<0.05), but this correlation disappeared in recovery phase. In the acute phase of MPP, compared with mild cases, lower serum level of CCSP and higher level of IL-17 were observed in severe cases, and the differences were statistically significant (P<0.05). In recovery phase of MPP, no difference in level of either CCSP or IL-17 was found between severe cases and mild cases (P>0.05). Conclusions Both CCSP and IL-17 are involved in the pathogenesis and development of MPP, and also related to the severity of illness.

Analysis of deceleration capacity of rate and heart rate varibility in children with precardial distress of unknown origin

YI Lanfen, WEN Hongxia, CAO Xiaoxiao, ZHANG Jingyang, QIU Mei, LI Si, WANG Wenjuan

. 2014, 32(10):  951.  doi:10.3969 j.issn.1000-3606.2014.10.014

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　Objectives To explore the clinical application of deceleration capacity of rate (DC), acceleration capacity of rate (AC) and heart rate variability (HRV) in children with precardial distress of unknown origin. Methods A total of 56 children with precardial distress of unknown origin and 63 healthy children aged 6 to 17 years were examined by 24 h dynamic electrocardiogram, and the indexes of DC and HRV were compared between these two groups. Results DC value of children with precardial distress is less than that of the control group (P<0.05), AC value is greater than that of the control group (P<0.05), and heat rate (HR) is greater than that of the control group (P<0.05). No statistical differences were observed in the indexes of HRV between the two groups. The indexes of DC show a significant positive correlation with HRV in children with precardial distress(r=0.27~0.40, P<0.05), while appear a negative relation with HR (r=-0.46, P=0.000). In contrast, the indexes of AC show a significant negative correlation with HRV (r=-0.57~-0.34, P<0.05), and appears a positive relation with HR(r=0.61, P=0.000). HR valu  is higher in male children less than 12 years old with precardial distress than that of age-matched males in control group, and RMSSD is lower than the latter. DC value of male children more than 12 years with precardial distress is lower than that of age-matched males in control group, while AC value is higher than that of the latter; DC value is significant lower in female children more than 12 yeares with precardial distress than that of age-matched females in the control group (P<0.05). Conclusions The activity of vagus nerve in children with precardial distress of unknown origin is decreased. DC value is significantly lower than that of control group, and shows correlation with indexes of HRV. There is no significant difference in DC and HRV value between male and female children with precardial distress. DC value is lower in children aged 12 or older with precardial distress than that of age-matched children in the control group, which indicates adolescents are vulnerable to autonomic nerve functional disorder.

Analysis of complications during and post to interventional therapy of common congenital heart disease in children

　PANG Chengcheng, ZHANG Zhiwei, QIAN Mingyang, LI Yufen

. 2014, 32(10):  956.  doi:10.3969 j.issn.1000-3606.2014.10.015

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Objective To analyze the incidence of complications during and after interventional therapy for common congenital heart disease (CHD) in children. Methods From January 2011 to December 2013, interventional therapy of common congenital heart disease which include ventricular septal defect (VSD), atrial septal defect (ASD), patent ductus arteriosus (PDA) and pulmonary valve stenosis (PS) were performed in 2356 patients. Among them, 159 patients who developed complications during and post to interventional therapy were retrospectively analyzed. Results The overall complication rate was 6.75% (159/2356) (11.40% post VSD occlusion, 7.50% post ASD occlusion, 3.09% post PDA occlusion, 1.63% post percutaneous balloon pulmonary valvuloplasty (PBPV) ).The rate of arrhythmia was 4.41%(102/2356). The severe complication rate was 2.71% (64/2356) (3.62% post VSD occlusion, 2.21% post ASD occlusion, 2.53% post PDA occlusion, 1.63% post PBPV). The intraoperative severe complication rate was 0.51%(12/2356); the early severe complication rate was 1.99% (47/2356); the late severe complication rate was 0.21% (5/2356). Interventional therapy rate was 0.13% (3/2356); cardiovascular surgery rate was 0.64% (15/2356); conservative treatment rate was 1.95%(46/2356). The mortality rate was 0.08% (2/2356). Conclusions The complications and mortality rate of interventional therapy for CHD in children are relatively low, but cannot be ignored. The complication could be reduced by choosing proper indications, following the operational procedures and careful operative follow-up.

Urinary screening and follow-up analysis of 10 795 school children in Lanzhou city

LEI Xiaoyan, WANG Sanping, SAI Yipa, GAO Xia

. 2014, 32(10):  961.  doi:10.3969 j.issn.1000-3606.2014.10.016

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Objective　To investigate the prevalence of urine abnormalities for school children in Lanzhou and evaluate the significance of urine screening. Methods During 2012-2013, urine from 10795 students were collected, and screened by urine reagent paper. Two weeks later, repeated screening was conducted in those who had abnormal result in the first screening. Children with both results being abnormal were submitted to urine routine tests in local hospitals. Results In the first screening of school children, 1653 (15.31%) children were urinary occult blood positive, 516 (4.78%) had urinary protein, 112 (1.04%) had occult blood with proteinuri and 743 (6.88%) had white cells in urine. In the second urine screening, the positive rate of occult blood was 7.55% (815 ), urinary protein was 2.13% (230), occult blood with proteinuria was 0.69% (74) and white cells in urine was 2.51% (271). Four hundred and fifty-five students in 1359 were submitted to urine routine tests. Thirty-two children had pyuria, 147 had hematuria, 23 had proteinuria and 13 had occult blood with proteinuria. Thirteen cases of chronic kidney disease were found in the follow-up. The incidence of urine abnormalities was significantly higher in junior middle school than in primary school. Conclusions Urine screening is an effective way to find asymptomatic kidney disease and urinary tract infection. Follow-up should be strengthened.

The relationship between behavioral problems and family environment in preschool-age children　

GU Liping, CHEN Ang, DENG Cheng, HE Shuhua, CHEN Xin, YE Chunhong, LIAO Yan, FENG Huajun, YAN Xueqin, GAO Jianhui, SHI Yuqi

. 2014, 32(10):  965.  doi:10.3969 j.issn.1000-3606.2014.10.018

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　Objective　To study the relationship between behavioral problems and family environment in preschool-age children. Methods Children between 4-6 years of age in the kindergartens of Zhongshan city were randomly selected. Child Behavior Checklist (CBCL) was used to assess the behavioral problems in children, and self-designed general situation questionnaire and Family Environment Scale-Chinese Version (FES-CV) were applied to evaluate the family environment. Results A total of 3445 children were enrolled and 29.87% children showed behavioral problems. There were significant differences in the scores of feeling expression, family conflict, intelligence, recreation, organization and control between children with and without behavior problems (P<0.001). The scores of feeling expression, intelligence, recreation, organization and control were significantly higher and the score of family conflict was lower in healthy children in comparison with children with behavior problems. Through multivariate logistic regression, it was suggested that female (OR=2.04), non-urban population (township OR=1.45, rural OR=1.51), non-local Zhongshan resident (OR=1.39), father’s low educational level (technical secondary school OR=1.77, junior high school OR=2.17, primary school and below OR=3.24), discipline styles (indulgent OR=1.47, mixed OR=1.37), family conflict (OR=1.13) were independent risk factors for behavioral problems (P<0.05). Mother’s age over 30 (OR=0.74) or over 40 (OR=0.46), feeling expression (OR=0.94) and intelligence (OR=0.87) were independent protective factors for behavioral problems (P<0.05). Conclusions Behavioral problems in preschool-age children are closely related to family environment, which needs to be paid more attention.

Chinese expert consensus on the management of pediatric monosysntptomatic enuresis

Chinese Cooperative Group for the Management of Pediatric Enuresis

. 2014, 32(10):  970.  doi:10.3969 j.issn.1000-3606.2014.10.019

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The clinical and genetic features of early-onset globoid cell leukodystrophy in one boy

Zhang Yao, Ding Yuan, Li Xiyuan, Wang Qiao, Song Jinqing, Liu Yupeng, Yang Yanling

. 2014, 32(10):  976.  doi:10.3969 j.issn.1000-3606.2014.10.020

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Objective　To investigate the clinical, biochemical and genetic features of a Chinese boy with early-onset globoid cell leukodystrophy (GLD). Methods The clinical and genetic data of a rare case of early-onset GLD were retrospectively analysed. Results At 2 months after birth, the boy showed progressive psychomotor regression. At 4 months of age when the boy was taken to a doctor, the pyramidal sign was positive. The cranial MRI showed that the body of the lateral cerebral ventricles was slightly enlarged and the brain ditch crack of frontal-temporal-parietal lobe was widened and deepened. On his brain CT scan, high signals in bilateral basal ganglia, thalami, cerebellar hemisphere were observed. β-galactosylceramidase (GALC) activity in the peripheral leucocytes was significantly decreased (3.9 nmol/g protein.h). On his GALC gene, one homozygous novel mutation c.868C>T on exon 8 was found, which resulted in the amino acid change on p.R290C proteins. Conclutions Early-onset GLD is a rare autosomal-recessive hereditary lysosomal storage disease with a terrible prognosis, in which beta-galactose glucoside enzyme deficiency is induced by GALC gene mutation. The diagnosis of early-onset GLD is difficult and should depend on enzyme assay and gene testing.

A Chinese girl with ethylmalonic encephalopathy and a novel mutation on ETHE1 gene

LI Xiyuan, DING Yuan, LIU Yupeng, WANG Qiao, SONG Jinqing, YE Jintang, ZHANG Yao, WU Tongfei,YANG YanLing

. 2014, 32(10):  980.  doi:10.3969 j.issn.1000-3606.2014.10.021

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Objective　To introduce a case of ethylmalonic encephalopathy which is an autosomal recessive metabolic disorder caused by mutations in the ETHE1 gene. Methods The clinical course and gene mutation in a case of ethylmalonic encephalopathy was retrospectively analysed. Results A previously healthy girl presented with intractable diarrhea from the age of 7 months. Since then, progressive psychomotor regression has been observed. When she was 23 months, her blood butyrylcarnitine was significantly increased (4.48 μmol/L vs. normal range 0.0~1.0 μmol/L), and isovalerylcarnitine (0.70 μmol/L vs. normal range 0.0~0.65 μmol/L) was also elevated. Her urine levels of ethylmalonic acid and methylsuccinate acid were markedly increased. Cranial MRI revealed bilateral basal ganglia lesions supporting the diagnosis of ethylmalonic encephalopathy. On her ETHE1 gene, a reported mutation (c.488G>A, p.R163Q) and a novel mutation (c.203T>C, p.L68P) were identified. After lactose-free dietary treatment and the supplements of L-carnitine, coenzyme Q10, vitamins B1, B2 and C, gradual improvement in general condition, intelligence and motor development has been observed. Conclusions Ethylmalonic aciduria is common in the patients with inborn errors of mitochondrial fatty acid beta-oxidation. In ethylmalonic encephalopathy, elevated blood levels of butyrylcarnitine and isovalerylcarnitine are common and ETHE1 sequencing is helpful in its diagnosis.

Energy consumption assessment by indirect calorimetry in critically ill children

SHI Huixuan

. 2014, 32(10):  985.  doi:10.3969 j.issn.1000-3606.2014.10.022

Abstract ( 381 )   PDF (1274KB) ( 493 )  

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Nutritional and metabolic disorders are the common problems in PICU. They often lead to deterioration in child nutrition and vice versa. Reasonable assessment of the energy consumption can provide theoretical basis for proper nutrition support in critically ill children. In this review, the methods of evaluating the energy consumption which included energy metabolism measurement and prediction equations were introduced, and the importance of indirect calorimetry was emphasized. Meanwhile, the research situation of energy consumption assessment at home and abroad and the current status of its clinical application were also reviewed.

Current research of hepatoblastoma in children

JIN Sheng

. 2014, 32(10):  988.  doi:10.3969 j.issn.1000-3606.2014.10.023

Abstract ( 318 )   PDF (1293KB) ( 415 )  

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　Hepatoblastoma (HB) is the most common malignant liver tumors among children. It typically begins with few symptoms, making it difficult to diagnose and treat. The number of HB cases has been on the rise in recent years, resulting in gradual development of the research on various aspects of the disease. This paper is a round-up of the etiology, pathological classification, clinical staging, prognostic factors and therapeutic principles of hepatoblastoma.

Prevention and treatment of valve-related complications induced by interventional therapy in structural heart diseases

QIU Qinghuan

. 2014, 32(10):  993.  doi:10.3969 j.issn.1000-3606.2014.10.024

Abstract ( 323 )   PDF (1257KB) ( 549 )  

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　Structural heart diseases refer to anatomic heart or great vessels abnormalities by congential or acquired factors, including congenital heart disease, acquired heart valve disease, cardiomyopathy. In recent years, interventional therapy in structural heart diseases has gradually matured and been widely applied in clinical practice. Due to the fragility of heart valves and valve related organizations, or the congenital dysplasia of valves, the complex interventional therapeutic procedures can cause valve-related complications. In this paper, the causes, prevention and treatment of valve-related complications induced by interventional therapy in structural heart diseases are introduced.