CJCP
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2014 Vol.  16 No.  5
Published: 2014-05-15
OVERSEAS PEDIATRIC RESEARCH ADVANCE
CLINICAL RESEARCH
EXPERIMENTAL RESEARCH
CLINICAL EXPERIENCE
CASE REPORT
REVIEW
OVERSEAS PEDIATRIC RESEARCH ADVANCE
437 Giuseppe A. Marraro, Chengshui Chen, Maria Antonella Piga, Yan Qian, Claudio Spada, Umberto Genovese
Acute respiratory distress syndrome in the pediatric age：an update on advanced treatment
Acute respiratory distress syndrome (ARDS) is a heterogeneous syndrome that lacks definitive treatment. The cornerstone of management is sound intensive care treatment and early anticipatory ventilation support. A mechanical ventilation strategy aiming at optimal alveolar recruitment, judicious use of positive end-respiratory pressure (PEEP) and low tidal volumes (VT) remains the mainstay for managing this lung disease. Several treatments have been proposed in rescue settings, but confirmation is needed from large controlled clinical trials before they be recommended for routine care. Non-invasive ventilation (NIV) is suggested with a cautious approach and a strict selection of candidates for treatment. Mild and moderate cases can be efficiently treated by NIV, but this is contra-indicated with severe ARDS. The extra-corporeal carbon dioxide removal (ECCO2R), used as an integrated tool with conventional ventilation, is playing a new role in adjusting respiratory acidosis and CO2. The proposed benefits of ECCO2R over extra-corporeal membrane oxygenation (ECMO) consist in a reduction of artificial surface contact, avoidance of pump-related side effects and technical complications, as well as lower costs. The advantages and disadvantages of inhaled nitric oxide (iNO) are better recognized today and iNO is not recommended for ARDS and acute lung injury (ALI) in children and adults because iNO results in a transient improvement in oxygenation but does not reduce mortality, and may be harmful. Several trials have found no clinical benefit from various surfactant supplementation methods in adult patients with ARDS. However, studies which are still controversial have shown that surfactant supplementation can improve oxygenation and decrease mortality in pediatric and adolescent patients in specific conditions and, when applied in different modes and doses, also in neonatal respiratory distress syndrome (RDS) of preemies. Management of ARDS remains supportive, aimed at improving gas exchange and preventing complications. Progress in the treatment of ARDS must be addressed toward the new paradigm of the disease pathobiology to be applied to the disease definition and to predict the treatment outcome, also with the perspective to develop predictive and personalized medicine that highlights new and challenging opportunities in terms of benefit for patient's safety and doctor's responsibility, with further medico-legal implication.
2014 Vol. 16 (5): 437-447 [Abstract] ( 9973 ) [HTML 1KB] [PDF 1078KB] ( 5099 )
448 Matthias C. Hütten, Boris W. Kramer
Patterns and etiology of acute and chronic lung injury：insights from experimental evidence Hot!

Adequate pulmonary function is pivotal for preterm infants. Besides being structurally immature, the preterm lung is susceptible to injury resulting from different prenatal conditions and postnatal insults. Lung injury might result in impaired postnatal lung development, contributing to chronic lung disease of prematurity, bronchopulmonary dysplasia (BPD). This review focuses on lung injury mediated by and related to inflammatory changes in the lung. We give an overview on experimental models which have helped to elucidate mechanisms of pulmonary inflammation in prematurity. We describe experimental data linking acute and chronic chorioamnionitis with intrapulmonary inflammation, lung maturation and surfactant production in various animal models. In addition, experimental data has shown that fetal inflammatory response is modulated by the fetus himself. Experimental data has therefore helped to understand differential effects on lung function and lung maturation exerted by maternal administration of potentially anti-inflammatory substances like glucocorticosteroids (GCS). New approaches of modulation of pulmonary inflammation/injury caused by postnatal interventions during resuscitation and mechanical ventilation have been studied in animal models. Postnatal therapeutic interventions with widely used drugs like oxygen, steroids, surfactant, caffeine and vitamin A have been experimentally and mechanistically assessed regarding their effect on pulmonary inflammation and lung injury. Carefully designed experiments will help to elucidate the complex interaction between lung injury, lung inflammation, repair and altered lung development, and will help to establish a link between lung alterations originating in this early period of life and long-term adverse respiratory effects.
2014 Vol. 16 (5): 448-459 [Abstract] ( 7878 ) [HTML 1KB] [PDF 1288KB] ( 2511 )
CLINICAL RESEARCH
460 FU Chun-Hua, XIA Shi-Wen
Clinical application of nasal intermittent positive pressure ventilation in initial treatment of neonatal respiratory distress syndrome
Objective To study the clinical effectiveness and safety of nasal intermittent positive pressure ventilation (NIPPV) in the initial treatment of neonatal respiratory distress syndrome (NRDS) and the initial setting of NIPPV parameters. Methods One hundred neonates with NRDS were divided into NIPPV group (n=50) and nasal continuous positive airway pressure (NCPAP) group (n=50). A randomized controlled study was conducted to compare the effectiveness of NIPPV versus NCPAP in the initial treatment of NRDS from the following aspects: reducing CO2 retention, improving oxygenation, reducing second endotracheal intubation and second use of pulmonary surfactant (PS), reducing the duration of invasive respiratory support, reducing the duration of oxygen use, and reducing the incidence of air leak, abdominal distension and ventilator-associated pneumonia. Results After 1 and 6 hours of noninvasive respiratory support, the NIPPV group was superior to the NCPAP group with respect to the reduction in CO2 retention and improvement in oxygenation (P<0.05); in addition, compared with the NCPAP group, the NIPPV group had significantly lower rates of second endotracheal intubation and second PS use, significantly shorter duration of invasive respiratory support and time of FiO2 >0.21, and significantly lower incidence of apnea and ventilator-associated pneumonia (P<0.05); there were no significant differences in the incidence of air leak and abdominal distention between the two groups. Conclusions NIPPV is effective and safe in the initial treatment of NRDS and holds promise for clinical application.
2014 Vol. 16 (5): 460-464 [Abstract] ( 6195 ) [HTML 1KB] [PDF 1193KB] ( 2040 )
465 FENG Jin-Xing, LIU Xiao-Hong, HUANG Hui-Jun, YU Zhen-Zhu, YANG Hui, HE Liu-Fang
Correlation between end-tidal carbon dioxide and partial pressure of arterial carbon dioxide in ventilated newborns
To study the correlation between end-tidal carbon dioxide (PetCO2) and partial pressure of arterial carbon dioxide (PaCO2) in ventilated newborns. Methods Thirty-one ventilated newborn underwent mainstream PetCO2 monitoring; meanwhile, arterial blood gas analysis was performed. The correlation and consistency between PetCO2 and PaCO2 were assessed. Results A total of 85 end-tidal and arterial CO2 pairs were obtained from 31 ventilated newborns. The mean PetCO2 (41±10 mm Hg) was significantly lower than the corresponding mean PaCO2 (46±11 mm Hg) (P<0.01). There was a significant positive correlation between PetCO2 and PaCO2 (r=0.92, P<0.01). The overall PetCO2 bias was 5.1±4.3 mm Hg (95% limits of consistency, -3.3 to 13.6 mmHg), and 5% (4/85) of the points were beyond the 95%CI. When the oxygenation index (OI) was less than 300 mm Hg (n=48), there was a significant positive correlation between PetCO2 and PaCO2 (r=0.85, P<0.01); the PetCO2 bias was 5.9±4.3 mm Hg (95% limits of consistency, -2.6 to 14.5 mm Hg), and 4.2% (2/48) of the points were beyond the 95%CI. When the OI was more than 300 mm Hg (n=37), there was also a significant positive correlation between PetCO2 and PaCO2 (r=0.91, P<0.01); the PetCO2 bias was 4.1±4.1 mm Hg (95% limits of consistency, -3.9 to 12.1 mm Hg), and 5% (2/37) of the points were beyond the 95%CI. Conclusions There is a good correlation and consistency between PetCO2 and PaCO2 in ventilated newborns.
2014 Vol. 16 (5): 465-468 [Abstract] ( 5970 ) [HTML 1KB] [PDF 1155KB] ( 1719 )
469 CHEN You, MA Li-Ya, HUANG Yan, HUANG Shang-Ming, XIAO Shan-Qiu, LU Guang-Jin
Association of Ureaplasma urealyticum infection with bronchopulmonary dysplasia in very low birth weight infants with respiratory distress syndrome
Objective To study the relationship between Ureaplasma urealyticum (UU) infection in the lower respiratory tract and the incidence of bronchopulmonary dysplasia (BPD) in very low birth weight (VLBW) infants with respiratory distress syndrome (RDS). Methods Seventy-three VLBW infants diagnosed with neonatal RDS, who had received at least one dose of pulmonary surfactant, as well as mechanical ventilation, and were hospitalized for over 28 days, were recruited. Endotracheal aspirates were obtained from the lower respiratory tract and examined by real-time PCR to detect UU DNA. The infants were divided into UU infection and non-UU infection groups according to examination results. Clinical characteristics and the incidence of BPD were compared between the two groups. Results Compared with the non-UU infection group, the UU infection group had a higher rate of maternal vaginal delivery, higher incidence of recurrent nosocomial pulmonary infection and premature rupture of membranes (PROM), and longer durations of PROM, oxygen supplementation, and hospital stay; in addition, the UU infection group had higher plasma IgM level, leukocyte count, and neutrophil count within 3 hours after birth. Among 73 VLBW infants, 45 developed BPD; the incidence of BPD in the UU infection group was 90% (19/21), versus 50% (26/52) in the non-UU infection group (P<0.01). Conclusions UU infection in the lower respiratory tract increases the incidence of BPD in VLBW infants with RDS.
2014 Vol. 16 (5): 469-472 [Abstract] ( 5882 ) [HTML 1KB] [PDF 1177KB] ( 1624 )
473 WEI Hong-Ling, LI Zai-Ling
Clinical analysis of twin-twin transfusion syndrome complicated by cardiac abnormalities in preterm infants
Objective To investigate the complications of twin-twin transfusion syndrome (TTTS) in preterm infants and to analyze the clinical conditions and prognosis of cardiac abnormalities in TTTS recipients. Methods A retrospective analysis was performed on the clinical data of 17 pairs of preterm infants with TTTS born between June 2009 and December 2012. Results Compared with the recipients, the donors had significantly lower body weights (1.4±0.6 kg vs 1.9±0.6 kg; P<0.05). With treatment during pregnancy, cardiac complications were found in 14 cases, and brain injuries in 12 cases. The proportion of recipients with cardiac abnormalities (60%) was higher than that of donors (24%). Among 10 recipients who had cardiac complications, cardiac abnormalities mainly included valve thickening, stenosis, or atresia (50%). Conclusions Among preterm infants with TTTS, the recipients are more susceptible to complications of valvular heart disease and cardiomyopathy. Fetal echocardiography, evaluation of cardiac function, and treatment should be performed for recipients as early as possible to improve the prognosis.
2014 Vol. 16 (5): 473-477 [Abstract] ( 5318 ) [HTML 1KB] [PDF 1174KB] ( 1216 )
478 LIU Rui-Ke, LIU Fang, LI Jian-Ying, WU Xiu-Fang
A contrastive study of corpus callosum area in very preterm and full-term infants
Objective To compare the differences between full-term and VLBW premature infants at term equivalent for the whole and sub-regional corpus callosum areas in order to provide reference for monitoring the extrauterine development of corpus callosum in VLBW premature infants. Methods Brain MR image data of 24 term infants with a gestational age of 39 weeks were collected within 24 hours after birth. Brain MR image of 30 VLBW neonates at 39 weeks' gestational age equivalent were successfully obtained. Routine T1WI, T2WI and DWI were applied. T1-weighted images on the mid-sagittal slice were selected, analyzed and measured. Forty-nine eligible MR images of them were chosen, 21 cases from the full-term infant group and 28 cases from the premature infant group. Corpus callosum and brain MR images were then sketched by two radiographic doctors. All data were analyzed by the Image Processing Function of MATLAB R2010a, and the whole corpus callosum and six sub-regions were obtained. Results The whole corpus callosum, anterior mid-body, posterior mid-body, isthmus and splenium area in the premature infant group were smaller than those in the full-term infant group (P<0.05), but the differences of Genu and rostral body area between the two groups was not statistically significant (P>0.05). Conclusions The areas of the whole corpus callosum, anterior mid-body, posterior mid-body, isthmus and splenium in VLBW preterm infants at term are reduced, suggesting that the posterior end of the corpus callosum is probably most vulnerable to insults following pathogenic factors.
2014 Vol. 16 (5): 478-482 [Abstract] ( 5059 ) [HTML 1KB] [PDF 1354KB] ( 1109 )
483 WU Xiao-Jing, ZHONG Dan-Ni, YE De-Zhi, ZHONG Yong, XIE Xiang-Zhi
Mutations in UGT1A1 gene in neonates with hyperbilirubinemia of Guangxi Heiyi Zhuang nationality
Objective To study the distribution of mutations of UDP-glucuronosyltransferase 1A1 (UGT1A1) gene and its relationship with hyperbilirubinemia among neonates with hyperbilirubinemia of Guangxi Heiyi Zhuang nationality. Methods Total genomic DNA was extracted from the blood of 100 neonates with hyperbilirubinemia (case group) and 100 neonates without hyperbilirubinemia (control group), all of whom were selected from Guangxi Heiyi Zhuang population. TATA box and all exons of UGT1A1 gene were amplified by PCR and directly sequenced. Results (TA)7 insertion mutation in TATA box, G71R missense mutation in exon 1, and 4 single nucleotide polymorphisms (SNPs) (rs199539868, rs114982090, rs1042640 and rs8330) in exon 5 were observed. The allele frequency of G71R mutation in the case group was significantly higher than that in the control group (P<0.01). There were no significant differences in the genotype distribution and allele frequency of TATA box mutation and SNPs (rs1042640 and rs8330) between the two groups (P>0.05). The logistic regression analysis showed that the odds ratios (95% confidence intervals) of UGT1A1 TATA box mutation, G71R mutation, and SNPs (rs1042640 and rs8330) associated with the development of neonatal hyperbilirubinemia were 0.846 (0.440, 1.629), 3.932 (1.745, 8.858), 0.899 (0.364, 2.222), respectively. Conclusions (TA)7 insertion mutation and G71R missense mutation of UGT1A1 gene are common mutation types in neonates with hyperbilirubinemia of Guangxi Heiyi Zhuang nationality. Four SNPs (rs199539868, rs114982090, rs1042640, and rs8330) was first reported in China. UGT1A1 G71R missense mutation is a risk factor for hyperbilirubinemia in neonates of Guangxi Heiyi Zhuang nationality.
2014 Vol. 16 (5): 483-488 [Abstract] ( 5937 ) [HTML 1KB] [PDF 1432KB] ( 1268 )
489 JIANG Min, LI Xiao-Qing, HU Dong, ZHANG Zhi-Quan, YANG Yu-Yao, BAI Lu-Chao, JIN Run-Ming
Clinical characteristics of childhood leukemia with EVI1 gene and BCR/ABL gene co-expression
Objective To study the clinical characteristics of ecotopic viral integration site-1 (EVI1) and BCR/ABL positive childhood leukemia. Methods Clinical data of four children with EVI1 and BCR/ABL positive leukemia and eight children with BCR/ABL positive but EVI1 negative chronic myeloid leukemia (CML) were retrospectively analyzed. Results In the four children with EVI1 and BCR/ABL positive leukemia, two were initially diagnosed with chronic phase of CML, one with accelerated phase of CML and one with high-risk acute lymphoblastic leukemia (ALL). There were no significant differences in clinical characteristics at diagnosis between the patients with EVI1 and BCR/ABL positive leukemia and BCR/ABL positive but EVI1 negative leukemia. CD33 and CD38 were highly expressed and t(9;22) abnormality was present in all patients with EVI1 and BCR/ABL positive leukemia. Two of the 3 children with EVI1 and BCR/ABL positive CML achieved complete remission one or three months after treatment. Acquired negative status conversion occurred for EVI1 but not BCR/ABL in one CML case. The 3 children with EVI1 and BCR/ABL positive CML survived 20, 13 and 14 months, respectively, without recurrence. The child with EVI1 and BCR/ABL positive ALL failed to achieve complete remission after the first course of treatment and discontinued further treatment. Conclusions Co-expression of EVI1 and BCR/ABL fusion gene can be found in childhood CML and ALL. The relatively rare leukemia has not significant difference respect to clinical characteristics. Prognosis of the disease needs to be determined by clinical studies with a larger sample size.
2014 Vol. 16 (5): 489-493 [Abstract] ( 5374 ) [HTML 1KB] [PDF 1265KB] ( 1242 )
494 HUANG Shan-Ya-Mei, JIA Yue-Ping, LIU Gui-Lan, ZHANG Le-Ping, LU Ai-Dong, WANG Bin
A comparison of minimal residual disease in children with acute lymphoblastic leukemia of different genetic abnormalities
Objective To study the changes of minimal residual disease (MRD) in children with B cell acute lymphoblastic leukemia (B-ALL) of different genetic abnormalities. Methods Between February 2004 and April 2013, 271 newly diagnosed B-ALL pediatric patients who had finished the induction chemotherapy were enrolled in the study. The characteristics of changes in MRD in patients with different genetic abnormalities on the 15th day and at the end of the induction therapy were analyzed. Results On the 15th day of the induction chemotherapy, the MRD positive proportion in patients with hyperdiploid was higher on all the three cut-off levels of MRD≥0.1%, 1% and 10% compared to patients without hyperdiploid (P<0.05), but there was no significant difference in the MRD positive proportion on the three levels of MRD between the TEL-AML1-positive and TEL-AML1-negative groups (P>0.05). On the end of induction chemotherapy, there was no significant difference in the MRD positive proportion on the three levels of MRD between the patients with and without hyperdiploid (P>0.05), neither between the BCR-ABL-positive and negative groups. The MRD positive proportion in TEL-AML1-negative patients was significantly higher than in TEL-AML1-positive patients on all three levels of MRD (P<0.05). The MRD positive proportion on two levels of MRD≥0.01% and 0.1% in E2A-PBX1-negative patients was significantly higher than in E2A-PBX1-positive patients (P<0.05). Conclusions Children with B-ALL of different genetic abnormalities have different MRD levels during, and at the end of, induction therapy. The prognostic significance of MRD may be related to the genetic abnormalities.
2014 Vol. 16 (5): 494-498 [Abstract] ( 5208 ) [HTML 1KB] [PDF 1328KB] ( 1125 )
499 XIE Cai, YUE Li-Jie, DING Hui, REN Yan-Fei, YANG Chun-Lan, ZHENG Miao-Miao
Correlations between 6-mercaptopurine treatment-related adverse reactions in children with acute lymphoblastic leukemia and polymorphisms of thiopurine methyltransferase gene
Objective To explore 6-mercaptopurine (6-MP) treatment-related adverse reactions in children with acute lymphoblastic leukemia (ALL), and to assess the association between the polymorphisms of thiopurine methyltransferase (TPMT) gene and these 6-MP related toxicities. Methods Total RNA was extracted from bone marrow samples of 46 children with ALL and was then reversed to cDNA. TPMT*1S and *3C were screened by denaturing gradient gel electrophoresis (DGGE) combining with DNA sequencing. Drug toxicities were classified according to national cancer institute-common toxicity criteria version 3.0 (NCI CTC 3.0). The relationship between TPMT gene polymorphisms and the adverse reactions of 6-MP treatment was analyzed. Results During the maintenance treatment period, 22% (10/46) of children discontinued 6-MP treatment because of serious adverse reactions. Two children with TPMT*3C genotypes presented severe adverse reactions, including 1 child with homozygotic mutation who had 6-MP dose-related myelosuppression and hepatotoxicity. The main side effects of 6-MP were myelosuppression, hepatotoxicity and gastrointestinal reaction. And there were no significant differences between TPMT*1S genotypes and severe myelosuppression or hepatotoxicity caused by 6-MP (P>0.05).Conclusions TPMT*3C may correlate with severe adverse reactions caused by 6-MP.
2014 Vol. 16 (5): 499-503 [Abstract] ( 5581 ) [HTML 1KB] [PDF 1544KB] ( 1619 )
504 GUO Jia, WANG Lu, HOU Wei-Na, BAI Song-Ting, SUN Su-Ke, SHENG Guang-Yao
Prognostic significance of coagulation disorders in children with hemophagocytic syndrome
Objective To study the prognostic significance of coagulation disorders in children with hemophagocytic syndrome (HPS). Methods Thirty-five children with HPS were retrospectively studied to analyze the etiology, clinical characteristics, laboratory results and treatment outcomes. Results After treatment, 27 of the 35 HPS patients survived, and the other 8 cases died. All cases were treated according to the HLH-2004 protocol, but etoposide (VP-16) was not used in 10 of them. The response rate in patients who received VP-16 (22/25, 88%) was significantly higher than that in those not receiving VP-16 (5/10, 50%) (P<0.05). Compared with the survival group, the dead group had significantly lower platelet count, fibrinogen level, and VP-16 utilization rate (P<0.05) but significantly longer activated partial thromboplastin time and prothrombin time (P<0.05). Conclusions Coagulation function can be used as an indicator of disease outcome. It is essential for improving the clinical outcome of HPS to monitor the coagulation function during treatment, detect and correct abnormalities in time, and provide treatment strictly according to the HLH-2004 protocol.
2014 Vol. 16 (5): 504-507 [Abstract] ( 5882 ) [HTML 1KB] [PDF 1376KB] ( 1440 )
508 LIAO Jia-Yi, ZHANG Tao
Influence of OM-85 BV on hBD-1 and immunoglobulin in children with asthma and recurrent respiratory tract infection
Objective To observe the clinical therapeutic effect of OM-85 BV(OM-85 Broncho-Vaxom) in children with asthma and recurrent respiratory tract infection, and the effect of OM-85 BV on human β-defensins 1 (hBD-1) and immunoglobulin levels. Methods Sixty-two children with asthma and recurrent respiratory infection treated between 2011 January and December were divided into two groups by the randomized, double blind method: a treatment group and a control group. With inhaling corticosteroids, the treatment group was given OM-85 BV, and the control group was given a placebo. Clinical curative effects and adverse reactions were observed. Serum levels of hBD-1, IgA, IgG, IgM and urea and alanine aminotransferase (ALT) were measured before treatment and 6 months and 12 months after treatment. Results Compared with the control group and before treatment, the frequency of respiratory tract infection was reduced in the treatment group 6 months and 12 months after treatment (P<0.05), and serum levels of hBD-1, IgA and IgG in the treatment group increased significantly (P<0.05). There was no significant difference in serum levels of urea, ALT and IgM between the control and treatment groups (P>0.05). Mild adverse reactions occurred in 3 cases in the two groups, 2 cases of abdominal pain and 1 cases of constipation. Conclusions OM-85 BV can improve serum levels of hBD-1, IgA and IgG, reduce the occurrence of acute respiratory tract infection and cause mild adverse reactions, suggesting its satisfactory therapeutic effect and safety in the treatment of asthma combined with recurrent respiratory tract infection in children.
2014 Vol. 16 (5): 508-512 [Abstract] ( 7132 ) [HTML 1KB] [PDF 1376KB] ( 1970 )
513 ZHU Deng-Na, XIE Meng-Meng, WANG Jun-Hui, WANG Jun, MA De-You, SUN Li, LI Lin-Chen, WANG Ming-Mei
Therapeutic effect of ketogenic diet for refractory epilepsy in children：a prospective observational study
Objective To study the clinical efficiency, electroencephalogram (EEG) changes and cognitive improvements of ketogenic diet (KD) in children with refractory epilepsy. Methods Twenty pediatric patients (7-61 months in age) with refractory epilepsy were recruited between August 2012 and August 2013. KD therapy was performed on all participants for at least 3 months based on a fasting initiation protocol with the lipid-to-nonlipid ratio being gradually increased to 4:1. Seizure frequency, type and degree were recorded before and during KD therapy. A 24 hours video-electroencephalogram (V-EEG) examination and Gesell Developmental Scale assessment were performed prior to KD therapy, and 3, 6, 9 months after KD therapy. Results Six patients became seizure free after KD therapy, with a complete control rate of 30%. Seizure frequency reduction occurred in 13 (65%) patients, EEG improvement in 8 (40%) patients, and improvement in Gesell Developmental Scales (gross motor and adaptability in particular) in 6 (30%) patients. The KD therapy-related side effects were mild. Conclusions KD therapy is safety and effective in reducing seizure frequency and improving EEG and cognitive function in children with refractory epilepsy.
2014 Vol. 16 (5): 513-517 [Abstract] ( 6729 ) [HTML 1KB] [PDF 1340KB] ( 2187 )
518 LIU Shuang, WEI Min, XIAO Juan, WANG Chang-Yan, QIU Zheng-Qing
Three PHEX gene mutations in Chinese subjects with hypophosphatemic rickets and literature review
The clinical data of three Chinese children who had been definitely diagnosed with X-link dominate hypophosphatemic rickets (XLH) by gene mutation analysis of phosphate-regulating gene with homologies to endopeptidases on the X chromosome (PHEX) were retrospectively studied and the relevant literature was reviewed. PHEX gene mutations were detected in all 3 XLH children; a nonsense mutation (c.58C>T) in one case and splicing mutations (c.1645+1G>A, c.436+1G>A) in the other two cases. Among these mutations, c.436+1G>A was novel. As of January 2014, a total of 329 PHEX gene mutations were reported, primarily within three mutation hot spots, throughout the world. Missense mutations accounted for the highest proportion (24%) among all mutations. There is literature showing geographic differences in the total number of XLH subjects and PHEX mutation types across the world. In the current literature, 89 cases of XLH with 28 types of PHEX mutations have been reported in the population of mainland China. Exon 22 is the most frequent mutation site (18%) and missense mutations are the most common type of mutations (61%). It is concluded that exon 22 is the mutation hot spot and missense mutation is the most common type of mutation in the PHEX gene in Chinese XLH patients and that c.436+1G>A detected in this study is a novel PHEX gene mutation in Chinese with XLH.
2014 Vol. 16 (5): 518-523 [Abstract] ( 5711 ) [HTML 1KB] [PDF 1908KB] ( 1794 )
524 DENG Fang-Ming, GONG Xue-Min, CUI Hong-Yan, YANG Yu-Jia, HU Ping-Cheng
Risk factors for unintentional injury among children in rural areas of Liling, Hunan Province, China
Objective To evaluate the risk factors for unintentional injury among children in the rural areas of Liling, Hunan Province, China, as a basis for developing prevention and intervention measures for unintentional injury in rural children. Methods A total of 3 257 students, aged between 5 and 16 years from 4 middle schools and 2 primary schools in eastern and western rural areas of Liling were recruited in October 2013 by stratified sampling and cluster sampling. The general personal information and data on family backgrounds, living environment, and incidence of unintentional injury were collected from all subjects through a self-designed questionnaire. The risk factors for childhood unintentional injury were assessed by an unconditional multiple logistic regression analysis. Results Out of the 3 257 subjects, 356 (10.93%) were injured during the 12-month period prior to the study. The univariate analysis showed that unintentional injury in these subjects was related to sex, left-behind status, times of internet surfing in internet bars per week, parent companion or not, age of guardian, degree of harmony of parents’ marital relationship, employment status of one or both parents as a migrant worker, storage of fireworks and firecrackers at home or not, violence in residential areas, and participation or not in violence in residential areas. The unconditional multiple logistic regression analysis showed that the major risk factors for unintentional injury in these subjects were male gender (OR=0.751, P=0.013), left-behind status (OR=1.779, P<0.001), storage of fireworks and firecrackers at home (OR=1.337, P=0.028) and violence around residential areas (OR=1.517, P<0.001). Conclusions Risk for unintentional injury is multifactorial among children in the rural areas of Liling, Hunan. To reduce the incidence of unintentional injury in children in Liling, particular attention should be paid to boys, left-behind children, children who have home storage of fireworks and firecrackers and children who are living in areas with frequent violence.
2014 Vol. 16 (5): 524-528 [Abstract] ( 5354 ) [HTML 1KB] [PDF 1453KB] ( 1199 )
EXPERIMENTAL RESEARCH
529 CAI Na, FENG Zhi-Chun, WANG Rui-Juan
Effects of recombinant human interleukin-11 on LPS-induced intestinal epithelial cell injury in rats
Objective To study the effects of recombinant human interleukin-11 (rhIL-11) on the proliferation and apoptosis of rat intestinal epithelial cell line (IEC-6). Methods IEC-6 cells were treated with LPS to establish necrotizing enterocolitis (NEC) model in vitro. rhIL-11 (100 ng/mL) was administered following LPS treatment and these cells were used as the IL-11 treatment group. The cells treated with normal saline only served as the control group. MTT assay was used to determine an optimal concentration (5-200 μg/mL) and time (1-24 h). MTT assay was used to measure the proliferation of IEC-6 cells at 3, 6, 9 and 12 hours after rhIL-11 treatment. Flow cytometry was used to evaluate the apoptosis of IEC-6 cells. Results IEC-6 cells treated with various concentrations of LPS at various time points showed a lower proliferation than the control group (P<0.05). After 9 hours of rhIL-11 treatment, the proliferation activity of IEC-6 cells in the IL-11 treatment group significantly increased compared with the NEC model group without rhIL-11 treatment (P<0.05), reaching to the level of the control group. The total apoptotic and necrotic rate of IEC-6 cells in the IL-11 treatment group decreased significantly compared with the NEC model group without rhIL-11 treatment (P<0.01), but were still higher than the control group (P<0.05). Conclusions rhIL-11 can promote proliferation and reduce apoptotic and necrotic rates of IEC-6 cells treated with LPS.
2014 Vol. 16 (5): 529-533 [Abstract] ( 5585 ) [HTML 1KB] [PDF 1495KB] ( 1144 )
534 YU Ren-Qiang, WU Xiao-You, ZHOU Xiang, ZHU Jing, MA Lu-Yi
Cyanidin-3-glucoside attenuates body weight gain, serum lipid concentrations and insulin resistance in high-fat diet-induced obese rats
Objective Cyanidin-3-glucoside (C3G) is the main active ingredient of anthocyanidin. This study aimed to evaluate the effects of C3G on body weight gain, visceral adiposity, lipid profiles and insulin resistance in high-fat diet-induced obese rats. Methods Thirty male Sprague-Dawley rats were randomly divided into a control group (n=8) and a high fat diet group (n=22), and were fed with standard diet or high fat diet. Five weeks later, 17 high-fat diet-induced obese rats were randomly given C3G [100 mg/(kg·d)] or normal saline via intragastric administration for 5 weeks. Five weeks later, body weight, visceral adiposity and food intake were measured. Blood samples were collected for detecting fasting glucose, serum insulin, lipid profiles and adiponectin. Insulin resistance index, atherosclerosis index and average feed efficiency ratio were calculated. Results C3G supplementation markedly decreased body weight, visceral adiposity, average feed efficiency ratio, triglyceride, total cholesterol, low density lipoprotein cholesterol, fasting glucose, serum insulin, insulin resistance index and atherosclerosis index in high-fat diet-induced obese rats. C3G supplementation normalized serum adiponectin and high density lipoprotein cholesterol levels in high-fat diet-induced obese rats. Conclusions Cyanidin-3-glucoside can reduce body weight gain, and attenuate obesity-associated dyslipidemia and insulin resistance in high-fat diet-fed rats via up-regulating serum adiponectin level.
2014 Vol. 16 (5): 534-538 [Abstract] ( 6292 ) [HTML 1KB] [PDF 1562KB] ( 1389 )
CLINICAL EXPERIENCE
539
No abstract available
2014 Vol. 16 (5): 539-540 [Abstract] ( 4046 ) [HTML 1KB] [PDF 1055KB] ( 1041 )
CASE REPORT
541
No abstract available
2014 Vol. 16 (5): 541-543 [Abstract] ( 5047 ) [HTML 1KB] [PDF 1854KB] ( 1395 )
544
No abstract available
2014 Vol. 16 (5): 544-545 [Abstract] ( 3791 ) [HTML 1KB] [PDF 1147KB] ( 986 )
546
No abstract available
2014 Vol. 16 (5): 546-547 [Abstract] ( 3911 ) [HTML 1KB] [PDF 1068KB] ( 1043 )
REVIEW
548 TANG Mao-Zhi, DOU Ying, ZHAO Xiao-Dong
Pathogenesis of human metapneumovirus infection and research on attenuated live vaccine
Numerous studies have indicated that human metapneumovirus (hMPV) is an important viral pathogen in acute respiratory infections in children, presenting similar manifestations with respiratory syncytial virus (RSV). HMPV infection peaks in the winter-spring season and is more prevalent in younger ages, especially in children less than 1 year old. Host innate immune response has been implicated in recognition of pathogen-associated molecular patterns (PAMPs) of the virus. This recognition occurs through host pattern recognition receptors (PRRs). Toll like receptors (TLRs) are one of the largest class of PRRs which initiate and regulate adaptive immune responses. Some studies have indicated that TLR 3 and TLR 4 may play critical roles in hMPV infection. Construction of recombinant mutant viruses lacking one or two N-linked glycosylation sites in the F protein by using site-directed mutagenesis and reverse genetics may be helpful for developing attenuated live vaccines.
2014 Vol. 16 (5): 548-554 [Abstract] ( 5452 ) [HTML 1KB] [PDF 1666KB] ( 1782 )
555 ZHAO Lan, ZHONG Yan
Effects of central precocious puberty on physical and sexual development in children
As abnormal pubertal development, central precocious puberty (CPP) is manifested by the secondary sexual characteristics, skeletal maturation, and physical development in advance. It ultimately affects the adult height of children, even producing some psychological and behavioral problems such as fear and anxiety. Currently, gonadotropin releasing hormone analogue (GnRHa) is recognized as the best medicine worldwide for treating CPP in order to increase children’s final adult height; however, it has some adverse effects on the growth and development of children. This paper reviews the effects of CPP and GnRHa on children's physical and sexual development, with the aim of increasing physicians awareness of this disease and the safety of medication.
2014 Vol. 16 (5): 555-559 [Abstract] ( 8044 ) [HTML 1KB] [PDF 1487KB] ( 1897 )
560 LIANG Su-Gai, SI-Tu Ming-Jing, HUANG Yi
Research advance in autistic traits in non-affected population of autism spectrum disorder
Autistic traits including social reciprocal deficits, communication deficits and stereotyped behaviors, are manifested not only in patients with autism spectrum disorders (ASD) and their families, but also in general population. In recent years, there has much research related to autistic traits. This ariticle summarizes research advance of autistic traits in ASD relations and general population.
2014 Vol. 16 (5): 560-565 [Abstract] ( 5474 ) [HTML 1KB] [PDF 1610KB] ( 1266 )
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