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Table of Content

    15 March 2014 Volume 32 Issue 3
      
    Original Article
    Evaluation of the therapies for neonatal posthaemorrhagic hydrocephalus 
    YU Lijun,LIN Zhenlang 
    . 2014, 32(3):  201-205. 
    Abstract ( 224 )   PDF (722KB) ( 393 )  
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    Although the percentage of preterm infants developing intraventricular haemorrhage (IVH) has been greatly reduced in the past three decades, increased survival of extremely preterms has meant that severe IVH with subsequent posthae-morrhagic hydrocephalus is still one of the serious unsolved problems for managements of preterms. Therapeutic interventions for posthaemorrhagic hydrocephalus include serial lumbar punctures, repeated aspiration through a ventricular access device, ventriculoperitoneal shunting, drainage, irrigation and fibrinolytic therapy, diuretic therapy treatment to reduce cerebrospinal fluid production, intraventricular fibrinolytic therapy, etc. This review summarizes the current concepts on the pathophysiology, intervention indications, and the effectiveness and safety of different interventions of subsequent posthaemorrhagic hydrocephalus.
    The reference ranges of oxygen saturation and heart rate in healthy infants during the first ten minutes after birth 
     WANG Huijuan,YANG Yun,LU Chengqiu,JIANG Hong,ZHANG Zheng,MENG Yongqin,WANG Jimei 
    . 2014, 32(3):  206-209. 
    Abstract ( 515 )   PDF (908KB) ( 760 )  
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     Objective To establish the reference ranges of preductal oxygen saturation (SpO2) and heart rate (HR) for healthy neonates in 10 minutes after birth. Methods SpO2 and HR recordings of 203 term neonates (vaginal group: n=97 and cesarean group: n=106) with regular respiratory pattern were evaluated. 10th-95th percentile charts of SpO2 and HR from 1 minute to 10 minutes after birth were drawn. Results The SpO2 of P10, P50 and P95 at 1 minute after birth was 62%, 71% and 85% respectively. The heart rate of P10, P50 and P95 at 1 minute after birth was 66 bpm, 98 bpm and 126 bpm respectively. The median time for SpO2 to reach 90% was 5 minutes. The rising trend of HR was evident during 1-5 minutes after birth, and then the HR leveled off. Conclusions The status of newborn can be assessed using the charts of SpO2 and HR combined with clinical manifestations. The oxygen intervention should be used with care to avoid damage caused by hyperoxemia and hypoxemia.
    Analysis of pathogen distribution and antibiotic resistance in 1606 blood cultures from neonates 
     TANG Xiaojuan,FENG Xing
    . 2014, 32(3):  210-213. 
    Abstract ( 206 )   PDF (735KB) ( 443 )  
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     Objective To determine the pathogen profile and antibiotic resistance in aerobic isolates from blood cultures of neonates. Methods All blood culture reports (n=28120) from newborns admitted to the Department of Neonatology during 2002-2012 were analyzed, and the sensitivity patterns were recorded. Results A total of 1665 bacteria were isolated from 1606 blood culture-positive samples and the positive rate of blood cultures was 5.7% (1606/28120). Gram-positive bacteria were isolated in 1336 cases, with Staphylococcus epidermidis (902 cases) and Staphylococcus haemolyticus (206 cases) being the common bacteria. Klebsiella pneumoniae (108 cases), followed by Escherichia coli (73 cases), were the major Gram-negative bacteria (235 cases). The determination of the antibiotic resistance of aerobic isolates was performed in 2012. Most Gram-positive isolates were sensitive to vancomycin and moxifloxacin, and more than 90% were resistant to penicillin while most of Gram-negative isolates were sensitive to amikacin and imipenem. Conclusions Staphylococcus epidermidis, Staphylococcus haemolyticus, Klebsiella pneumoniae and Escherichia coli remain to be the principal organisms responsible for neonatal sepsis.
    Analysis of risk factors of late preterm infants with hyperbilirubinemia
     YANG Shujie,YI Haiying,WANG Zhenzhen,HUANG Jie,HE Yuan,WEI Xian,WANG Yanhua
    . 2014, 32(3):  214-217. 
    Abstract ( 268 )   PDF (640KB) ( 321 )  
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     Objective To explore the risk factors of hyperbilirubinemia in late preterm infants. Methods Clinical data of 211 cases of late preterm infants with hyperbilirubinemia and 246 cases of late preterm infants without hyperbilirubinemia were retrospectively analyzed between 2011 and 2012. The risk factors of hyperbilirubinemia were filtered. Results Twenty-seven cases of late premature infants with hyperbilirubinemia were severe. Hospital stay less than 3 days, birth asphyxia history, small for gestatio-nal age, head hematoma, delivery injury, hypoalbuminemia, polycythemia, infection, hemolytic disease, feeding intolerance, and fetal excretion delay were associated with hyperbilirubinemia (P<0.05). Rural origin, pregnancy-induced hypertension syndrome and premature rupture of membrane were also associated with hyperbilirubinemia (P<0.05). Multivariate logistic regression analysis showed the history of birth asphyxia , fetal excretion delay, hypoalbuminemia, pregnancy-induced hypertension syndrome were risk factors of hyperbilirubinemia in late preterm infants (OR=2.35-4.05). Pregnancy-induced hypertension syndrome and hemolytic disease were risk factors of severe hyperbilirubinemia in late preterm infants (OR=5.74, 73.64). Conclusions Neonatal asphyxia, fetal excretion delay, hypoalbuminemia and pregnancy-induced hypertension syndrome are risk factors of hyperbilirubinemia in late preterm infants. Strengthening the management of pregnancy-induced hypertension syndrome and the treatment of newborn hemolytic disease can reduce the occurrence of severe hyperbilirubinemia in late preterm infants.#br#Key words: hyperbilirubinemia; preterm; risk factor#br#
    Clinical analysis of Langerhans cell histiocytosis in one newborn
     WANG Ying,ZHAO Zhiguang,SUN Zhongmin 
    . 2014, 32(3):  218-219. 
    Abstract ( 276 )   PDF (658KB) ( 302 )  
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     Objective To understand the clinical features and laboratory examination and diagnosis of Langerhas cell histiocytosis (LCH) in newborns. Methods Retrospective analysis of the clinical data of a neonatal case of LCH was performed. Results A one-day-old girl was admitted with chief complaint of eating less, less crying and skin rash for one day. The unequal sized rash was on the head, limbs, trunk, toe and oral mucosa. The skin rashes were protruding from the skin and were partially broken and crusted. Biopsy of the skin tissue showed the expression of S-100 and CD1α, consistent with the diagnosis of LCH. Conclusions When newborns have special rash after birth, LCH should be considered. To avoid misdiagnosis, skin biopsy should be performed.
    Distribution and drug resistance of pathogens in 132 cases of neonatal sepsis 
    CHEN Ting,LU Qin,YANG Li,XU Wenjun,SHI Mingxia 
    . 2014, 32(3):  220-223. 
    Abstract ( 271 )   PDF (707KB) ( 333 )  
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     Objective To investigate the distribution and antibiotic resistance of isolated pathogens in neonatal sepsis. Methods The results of blood culture and drug susceptibility test in neonates sepsis from January 2012 to June 2013 were retrospectively analyzed. Results One hundred and thirty-two strains were detected in the blood samples, with 100 (75.76%) Gram-positive bacteria, 30 (22.73%) Gram-negative bacteria and 2 (1.52%) fungus. Staphylococcus epidermidis, Escherichia coli and Staphylococcus aureus were the three most common pathogens. Gram-positive cocci was strongly resistant to penicillin (100.00%), erythromycin, selectrin and ampicillin/sulbactam (62.50%-100.00%), but still sensitive to vancomycin and teicoplanin. The resistance rate of Gram-negative bacilli to ampicillin was 100.00%, and the resistance rate to cefatriaxone, selectrin and cefuroxime was 61.54%-100.00%. The resistance rate to imipenem and piperacillin/tazobactam was lower. Conclusions The selection of sensitive antibiotics should be based on the pathogens and drug resistance testing for the treatment of neonatal sepsis.
    The relationships of antithrombin Ⅲ and D-dimer with pediatric critical illness score in children with sepsis QI Yingzheng (Department of Pediatrics, The Fifth Affiliated Hospital of Xinjiang Medical University, Urumqi 830011, Xinjiang, China) 
    . 2014, 32(3):  224-227. 
    Abstract ( 251 )   PDF (731KB) ( 321 )  
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    Objective To explore the relationships between antithrombin Ⅲ (AT-Ⅲ) and D-dimer (DD) level with pediatric critical illness score (PCIS) in children with sepsis, and to evaluate the values in assessing the severity of illness. Methods Sixty-one children with sepsis were included in this study. Within 24 hours after admission, blood samples were tested for the activity of AT-Ⅲ and DD level. The PCIS was calculated. According to PCIS, the patients were divided into extremely critical group (<70), critical group (71-80) and non-critical group (80-100). According to the prognosis, the patients were divided into survival and death groups. The differences of the activity of AT-Ⅲ and DD were compared and the relationship with PCIS were analyzed. Results The activity of AT-Ⅲ was lower and DD level was higher in critical group than in non-critical group (P<0.01) and the changes in extremely critical group were more evident than those in critical group. The activity of AT-Ⅲ and PCIS were positively correlated (r=0.548, P<0.01).The DD level and PCIS was negatively correlated (r=-0.657, P<0.01). Compared with survival group, the level of DD was significantly higher in death group (P<0.01), and PCIS and the activity of AT-Ⅲ were significantly lower in death group (P<0.05). Conclusions The patients with sepsis have dysfunctions of coagulation. The activity of AT-Ⅲ, DD level are correlated with illness severity, and can be useful for assessing the severity of sepsis.
    Analysis on clinical manifestations and drug resistance of Escherichia coli pneumonia in infants
     LIU Sheng,ZHANG Yali,CAI Haifang 
    . 2014, 32(3):  228-231. 
    Abstract ( 287 )   PDF (711KB) ( 550 )  
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     Objective To investigate the clinical features of Escherichia coli pneumonia, the positive rate of extend-spectrum β-lactamase (ESBLs) produced by Escherichia coli and antimicrobial resistance of Escherichia coli. Methods Three hundred and thirty-six infants with Escherichia coli pneumonia were divided into community acquired infection group and hospital acquired infection group from Jun 2003 to Jun 2013. The clinical data of those patients were collected and analyzed. The ESBLs were examined, and drug susceptibility results were analyzed. Results Infants under 6 months had higher infective rate of Escherichia coli. The Escherichia coli pneumonia had similar clinical manifestations as Gram negative bacterial pneumonia. ESBLs were found in most strains (58.3%, 196/336), positive rate of ESBLs of hospital acquired infection group (84.00%) was higher than that of community acquired infection group (50.96%) ( χ2=26.17, P<0.05). There was no difference of ESBLs positive rate between the community acquired infection groups during Jun 2003-May 2008 and those during Jun 2008-Jun 2013 (χ2=0.30, P>0.05). The ESBLs positive rate of hospital acquired infection group was significantly increased from 76.74% (Jun 2003-May 2008) to 93.75% (Jun 2008-Jun 2013) (χ2=3.95, P<0.05). The most sensitive antibiotic was carbapenem. Conclusions Escherichia coli pneumonia mainly occurs in infants, usually with severe clinical situations and more persistent. The hospital acquired infection is the high risk factor of acquiring ESBLs. Antibiotics should be prescribed depending on community acquired infection or hospital acquired infection.
    Value of 99mTc-EC dynamic renography in diagnosis of renal scars in children 
    FENG Yang,SU Xiao, LI Jianing 
    . 2014, 32(3):  232-234. 
    Abstract ( 284 )   PDF (699KB) ( 364 )  
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     Objective To evaluate the diagnostic value of 99mTc-EC dynamic renography for renal scars in children with urinary tract infection. Methods The 99mTc-EC and 99mTc-DMSA renographic results of 67 children diagnosed with urinary tract infection were retrospectively studied. In comparison with 99mTc-DMSA cortical images, the value of 99mTc-EC dynamic renography for the diagnosis of urinary tract infection, hydronephrosis and renal scars was analyzed. Results The sensitivity and specificity of the initial 2 minutes summed images in the 99mTc-EC images for the diagnosis of renal scars was 80.28% and 88.89% respectively, and the likelihood ratio was 7.23. Renal scars were more likely to be formed in patients with obstructed upper urinary tracts, as compared with patients with unobstructed upper urinary tracts and unsmooth upper urinary tracts (P<0.05). The incidence of renal scar formation was not significantly different among groups with hydronephrosis of varying degrees (P>0.05). Conclusions For children with urinary tract infection, the diagnostic sensitivity and specificity of the initial summed images of 99mTc-EC dynamic renography are high. Furthermore, the excretion of upper urinary tract showed by 99mTc-EC dynamic renography is valuable for the diagnosis of renal scars.
    Clinical pathological analysis of children with medulloblastoma 
    CHENG Xianghua,CHEN Jianhua,CHEN Kuisheng
    . 2014, 32(3):  235-237. 
    Abstract ( 268 )   PDF (604KB) ( 344 )  
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     Objectives To explore the pathological characteristics, treatment and prognosis of medulloblastoma (MB) in children. Methods Pathological characteristics, treatment outcomes and other clinical data were retrospectively analyzed in 60 children with MB. Results The MB in all 60 children were high-grade tumor. The pathological type was mainly classic (45 cases, 75.00%) and other subtypes were seen in 15 cases (25.00%). Forty-eight (80.00%) children had total resection by microsurgery, 9 children (15.00%) subtotal resection, and 3 children (5.00%) partial resection. Twenty-seven children were treated by radiotherapy after surgery, 6 children were treated by chemotherapy after surgery, and 5 children were treated with radiotherapy combined chemotherapy after surgery. Thirty-eight cases were followed up. The 2-year survival and disease-free survival rates were 63.16% and 55.26%. The 5-year survival and disease-free survival rates were 26.32% and 18.42%. The children with desmoplastic/nodular type survived significantly longer than the children with large cell anaplastic. The children with total resection survived significantly longer than the children with partial resection. The children with radiotherapy or chemotherapy survived significantly longer than the children without chemotherapy (P<0.05). Conclusions MB in most of children is high-grade tumors and the prognosis is poor. The pathological type is related with prognosis. Microsurgical total resection is the first choice of treatment. Postoperative radiotherapy and chemotherapy can increase the survival rate.
    Value of bronchofibroscopy in etiological diagnosis and treatment of hemoptysis in children 
    LI Linlin,LIANG Hui,WANG Quan,ZHAO Deyu
    . 2014, 32(3):  238-241. 
    Abstract ( 270 )   PDF (688KB) ( 314 )  
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     Objective To evaluate the value and safety of flexible bronchoscopy in etiological diagnosis and treatment of hemoptysis in children. Methods The flexible bronchoscopy findings and clinical data of 22 children with hemoptysis were analyzed retrospectively. Results The causes of hemoptysis were as follows, bronchiolitis and pneumonia in 8 cases (2 cases with bronchiectasis), foreign body in bronchus in 3 cases , idiopathic pulmonary hemosiderosis in 3 cases, neoplasms in airway in 2 cases, suspi-ciion of bronchial pulmonary vessel dysplasia in 4 cases (requiring angiogram) and unknown causes in 2 cases. Two cases suffered hypoxemia. Active bleeding was found in 6 cases and topical hemostasis treatment was effective. The bronchial foreign body was removed from right lower lobe bronchus in 3 cases. One case with atelectasis caused by granulation was treated with bronchoscopic cryotherapy 2 times, no granulation was found during the third bronchoscopy and chest CT showed the atelectasis was cured 2 weeks later. Conclusions Flexible bronchoscopy is one of the necessary and safe examinations in children with hemoptysis and is of important value in clinical diagnosis and treatment.
    Clinical analysis of 21 cases of pediatric bridging bronchus 
    ZHANG Shulan,ZHAO Deyu
    . 2014, 32(3):  242-245. 
    Abstract ( 283 )   PDF (645KB) ( 535 )  
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     Objective To explore the clinical diagnosis and treatment of pediatric bridging bronchus deformity and brid-ging bronchus with pulmonary artery sling. Methods The clinical data of the 21 cases of diagnosed bronchial bridge deformity and bronchial bridge with pulmonary artery sling, the imaging findings of all the echocardiography, MSCT chest scan, enhanced CT and reconstructive image were retrospectively analyzed. Results In 21 cases, 10 were diagnosed as bronchial bridge only, among which three cases had other types of congenital heart disease; 11 cases were diagnosed of pulmonary artery sling with pulmonary artery sling, among which 8 cases had other types of congenital heart disease; their average age of the 8 cases were all younger than 2 years old, except one case; most of them had the symptoms of recurrent respiratory tract infections and paroxysmal dyspnea. According to Wells, bronchial bridge was divided into type I and typeⅡ; 15 cases were type I, 6 cases were typeⅡ, 10 patients with bronchial bridge only were all type I, 5 cases of typeⅡwere associated with pulmonary artery sling. All of the 6 cases were diagnosed as typeⅡassociated with pulmonary artery sling. Conclusions Infants and young children with clinical recurrent respiratory tract infections should be considered the possibility of bronchus bridge deformity, and should be paid more attention to the possibility of pulmonary artery sling. MSCT plain chest scan, enhanced CT and airway reconstructive image are the important ways to diagnose the disease.
    Clinical and immunological features of 45 children with lupus nephritis
     CHENG Xueqin,BAO Huaying,ZHANG Aihua,DING Guixia,ZHAO Fei
    . 2014, 32(3):  246-249. 
    Abstract ( 173 )   PDF (679KB) ( 273 )  
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     Objective To analyze the clinical and immunological features of 45 pediatric patients with lupus nephritis (LN). Methods Forty-five LN patients were included in this study. Clinical, pathological data and immunological parameters were retrospectively analyzed. Results Forty-five LN patients had 6 males and 39 females, with the mean onset age of (10.9 ± 2.8) years. Acute nephritis was the most common type, accounting for 42.2%. Nephrotic syndrome accounted for 31.1%. Renal biopsy showed class II (17.8%), III (4.4%), IV (48.9%), V (2.2%), V+III (6.7%) and V+IV (13.3%) in 42 cases. The remission rate reached 91.1% in the early therapeutic stage, and 15.0% patients recurred after 24-month follow-up. Conclusions The clinical manifestations of LN children are diverse. The renal pathology is complex. The clinical manifestations in part of the children are not consistent with renal pathology.
    Effect of lipid emulsions on the IL-1β and IL-6 expressions in acute lung injury induced by endotoxin in rats 
    SHI Jipeng,HUANG Limi,QIAN Yan,SHANG Yun
    . 2014, 32(3):  250-253. 
    Abstract ( 278 )   PDF (778KB) ( 298 )  
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     Objective To compare the effect of three types of lipid emulsions on the IL-1β and IL-6 expressions in acute lung injury induced by lipopolysaccharide (LPS) in rats. Methods One hundred young SD rats were divided into control, LPS, ω-6, ω-9 and ω-3 group. Age 29 days, the control group were intravenously injected with saline, ω-6 group injected lipid emusion (C14-24), ω-9 group injected long chain fat emusion, ω-3 group injected fish oil fat emusion. Then the control group followed by intratracheal instillation of saline, and the reamining four groups were sprayed with LPS. Pathologic changes in lung tissue section were observed. The expression levels of IL-1β mRNA and IL-6 mRNA were determined by RT-PCR, and the concentrations of IL-1β and IL-6 in bronchoalveolar lavage fluid (BALF) were measured by enzyme-linked immune-specific assay. Results Infiltration and bleeding were observed in lung tissue under light microscopy in ALI rats. The lung indexes and pathological scores of ALI model groups were significantly higher than those of control group (P<0.005). The expressions of IL-1β and IL-6 mRNA in groups ω-9 and ω-3 were significantly higher than those in groups LPS and ω-6 (P<0.005). The levels of IL-1β and IL-6 in BALF in groups ω-9 and ω-3 were significantly higher than those in groups LPS and ω-6 (P<0.005). There was no significant difference (P>0.005) of the expressions of IL-1β and IL-6 mRNA between ω-9 and ω-3 group. Conclusions ω-6 PUFAs can accentuate inflammation by up-regulating the levels of IL-1β and IL-6 while ω-9 PUFAs and ω-3 PUFAs can relieve inflammation by down-regulating the levels of IL-1β and IL-6.
    The neuroprotective effect of Bax-inhibiting peptide on neonatal rats with hypoxic-ischemic brain damage 
    SHAN Jili,HAN Shuzhen,JING Ruquan,LI Xiang,XU Lijie,LI Tang
    . 2014, 32(3):  254-257. 
    Abstract ( 197 )   PDF (760KB) ( 241 )  
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    Effects of terbutaline sulfate on pulmonary fluid transport in preterm rats 
    MA Rongwei,YAO Jianhong,JI Yong,WANG Li 
    . 2014, 32(3):  258-260. 
    Abstract ( 208 )   PDF (631KB) ( 239 )  
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    Study about effects of BCG treatment on expression of stem cell factor in asthmatic mice
     LIN Xiaoliang,ZHANG Jianhua
    . 2014, 32(3):  261-264. 
    Abstract ( 203 )   PDF (739KB) ( 237 )  
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    Effects of dexamethasone on the levels of IL-12 and IL-13 in bronchoalveolar lavage fluid and serum in rats 
    WANG Zhihua,WANG Yushui,LIU Yan 
    . 2014, 32(3):  265-270. 
    Abstract ( 231 )   PDF (814KB) ( 317 )  
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    Immunogenicity and safety of the acellular pertussis, diphtheria, tetanus, inactivated poliomyelitis, Haemophilus influenza type b conjugate vaccine (DTaP-IPV/Hib combined vaccine): a meta-analysis 
    REN Sisi,WANG Dongfang,ZHONG Zhaohui 
    . 2014, 32(3):  271-277. 
    Abstract ( 272 )   PDF (786KB) ( 340 )  
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     Objective To evaluate the effectiveness and safety of DTaP-IPV/Hib combined vaccine in comparison with commercially available DTaP (diphtheria, tetanus and pertussis), Haemophilus influenzae type b (Hib), tetanus conjugate and IPV monovalent vaccine. Methods Randomized controlled trials (RCTs) on DTaP-IPV/Hib were retrieved by searching international and national databases. The pooled mean difference and relative risk and 95% CI were assessed by meta analysis with RevMan 5.0 software. Results Totally 6 studies were included for the final analysis. The seroprotection/seroconversion level of the Anti-PT (RR=0.26, 95%CI: 0.14, 0.48) in combination vaccine was higher. The antibody titer levels of Anti-PT (WMD=21.11, 95%CI: 9.36, 32.86), Anti-polio type1 (WMD=59.15, 95%CI: 2.81, 115.48), Anti-polio type 3 (WMD=169.82, 95%CI: 75.33, 264.30) were higher respectively. But the antibody titer level of Anti-PRP (WMD=-3.58, 95%CI: -5.52, -1.64) in the combination vaccine group was lower. Redness (RR=0.82, 95%CI: 0.72, 0.93) and Tenderness (RR=0.45, 95%CI: 0.30, 0.65) were lower in the combination vaccine. Swelling (RR=2.03, 95%CI: 1.02, 4.01) was more common in the patients given the combination vaccine. Conclusions This study supports the conclusion that the DTaP-IPV/Hib combination vaccine is equivalent to the separate injections based on similar antibody responses to the vaccine antigens, effectiveness and safety after primary doses.
    Effect of delayed cord clamping in term infants: a meta-analysis
     YU Zhangbin,HAN Shuping,SHA Li,DONG Xiaoyue,CHEN Yulin,QIU Yufang 
    . 2014, 32(3):  278-283. 
    Abstract ( 227 )   PDF (782KB) ( 254 )  
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    Chiari II malformation in infant: a case report 
    HU Fenge
    . 2014, 32(3):  284-286. 
    Abstract ( 406 )   PDF (653KB) ( 249 )  
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     Objective To analyze the clinical data of an infant with Chiari II malformation so as to deepen understanding of this disease in pediatrics. Methods The clinical, laboratory and imaging data of an infant with Chiari II malformation were retrospectively analyzed and a literature review was performed. Results A 2-month-old male infant was admitted to our hospital due to a neck tumor for two months. Brain magnetic resonance imaging (MRI) showed herniation of the cerebellar tonsils into the foramen magnum. The infant was finally diagnosed with Chiari II malformation based on the result of MRI and the presence of myelomeningocele. After repair of spinal cord and meninges and decompression of pillow macroporous by surgery, the infant presented with intermittent fever and progressively enlarged anterior fontanel, with culture-positive cerebrospinal fluid for bacteria and hydrocephalus diagnosed by CT, and intracranial infection was considered. Ventriculoperitoneal shunt was not performed due to uncontrolled intracranial infection after treatment, and motor retardation was found during the follow-up. Conclusions Chiari II malformation may results in nervous system dysfunction with poor diagnosis and high case fatality rate. Early diagnosis and operation may be helpful to the improvement of prognosis.#br#Key words: Chiari malformation II type; brainstem symptoms; prognosis#br#
    Gaucher disease II: a case report 
    WANG Meijuan,ZHONG Xuemei,MA Xin,ZHANG Yanling 
    . 2014, 32(3):  287-288. 
    Abstract ( 265 )   PDF (646KB) ( 252 )  
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     Objectives To investigate the clinical characteristics and diagnosis of Gaucher disease. Methods The clinical features, enzyme activities and genetypes of an infant with Gaucher disease II were analyzed. Results The main clinical manifestations of the infant included hepatosplenomegaly, ocular dyscinesia and mental retardation. Gaucher′s cells could be seen in the bone marrow aspirates. Glucocerebrosidase activity was low (0.3 nmol/g/min). Serum chitotriosidase activity was high (87317 nmol/L/min). GBA mutations were M85T (c.371T>C) and R120W (c.475C>T). Conlusions Main features of Gaucher disease II are hepatosplenomegaly with nerve system injury. Glucocerebrosidase activity and gene analysis are important for the diagnosis of Gaucher disease.#br#Key words: Gaucher disease; glucocerebrosidase; Gaucher cell#br#
    Latest update on diagnosis and treatment of steroid-resistant nephrotic syndrome in children
      Reviewer: ZHU Hongtao; Reviser: GUO Yanfang
    . 2014, 32(3):  289-292. 
    Abstract ( 255 )   PDF (676KB) ( 419 )  
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    Steroid-restant nephrotic syndrome (SRNS) is often continuously relapsed, and even develops into end-stage renal di-sease. It has been a great difficulty in the treatment of nephritic syndrome in children. In recent years, many researchers of children SRNS on diagnosis, pathology, treatment have been carried on domestically and in abroad. This paper reviews the latest update on the diagnosis and treatment of SRNS in children.
    Research progress in macrosomia growth 
    Reviewer: FU Dongxia; Reviser:WEI Haiyan
    . 2014, 32(3):  293-294. 
    Abstract ( 254 )   PDF (534KB) ( 455 )  
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    The incidence of macrosomia was increasing year by year, which may be associated with gestational diabetes, obesity and pregnancy weight gain. The study showed that macrosomia was prone to obesity in childhood, and diabetes, hypertension and cardiovascular disease in adulthood. The growth and development status of macrosomia in childhood is worthy of attention.