CJCP
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2009 Vol.  11 No.  08
Published: 2009-08-15

CLINICAL RESEARCH
EXPERIMENTAL RESEARCH
CLINICAL EXPERIENCE
CASE REPORT
REVIEW
CLINICAL RESEARCH
609 WANG Tong, YE Jun, HAN Lian-Shu, QIU Wen-Juan, ZHANG Hui-Wen, ZHANG Ya-Fen, GAO Xiao-Lan, WANG Yu, GU Xue-Fan
Diagnosis,treatment and gene mutation analysis in children with holocarboxylase synthetas deficiency

OBJECTIVE: To report the clinical diagnosis, treatment and follow-up of children with holocarboxylase synthetas(HCS) deficiency and explore the gene mutation spectrum of the disease. METHODS: Eleven children with HCS deficiency were enrolled. Mass spectrometry analysis and biotinidase activity determination were used for diagnosis of HCS deficiency. HCS gene mutations were analyzed by PCR directed sequencing methods. Ten patients received oral biotin treatment (10-40 mg/d). Clinical effects of biotin treatment were observed. RESULTS: All 11 cases developed apathetic, lethargy and metabolic acidosis at different degrees, and 10 cases presented with skin lesions. The average blood 3-hydroxyisovaleryl-carnitine concentrations and urinary 3-methylcrontonylglycine and methylcitrate concentrations increased significantly. The biotinidase activity increased, being higher over 30% of the normal reference value. Four mutations in HCS gene were identified, and they were c.1522C>T (R508W), c.1088T>A (V363D), c.126G>T (E42D) and c.1994G>C (R665P) (a new variant) and the frequency was 50%, 29%, 7% and 14% respectively. The symptoms disappeared in 10 cases 1-2 weeks after biotin treatment, and blood and urinary abnormal metabolites were gradually reduced to normal 2-6 months after treatment. CONCLUSIONS: HCS deficiency is characterized by nervous system damage, skin lesions and metabolic acidosis. Mass spectrometry analysis, biotinidase activity determination and gene mutation analysis may be helpful in the definite diagnosis of this disorder. The effect of early biotin treatment is satisfactory. The mutations R508W and V363D might be hot-spots in Chinese children with HCS deficiency.[Chin J Contemp Pediatr, 2009, 11 (8):609-612]

2009 Vol. 11 (08): 609-612 [Abstract] ( 6350 ) [HTML 1KB] [PDF 1187KB] ( 1847 )
613 CHEN Xiao-Wen, LI Chang-Gang, YUE Li-Jie, ZHANG Min, CHEN Yun-Sheng, MAI Hui-Rong, WANG Ying, LI Cheng-Rong
Genetic diagnosis for female carriers of glucose-6-phosphate dehydrogenase deficiency by RT-PCR-DGGE

OBJECTIVE: To study the feasibility of genetic diagnosis for female carriers of human glucose-6-phosphate dehydrogenase (G6PD) deficiency by reverse transcriptase-PCR-denaturing gradient gel electrophoresis (RT-PCR-DGGE). METHODS: Blood samples were collected from suspected 54 female carriers of G6PD deficiency. Total RNAs of peripheral blood were prepared and reverse-transcripted into cDNA. Design of 6 primer pairs for DGGE was based on 17 mutation sites of G6PD cDNA described in the Chinese population. Mutations in the coding region of G6PD gene were screened and genotyped by combination of PCR-DGGE and DNA sequencing. RESULTS: One case of 1024C/T, 20 cases of 1376G/T and 12 cases of 1388G/A were detected in the 54 samples. The total detection rate was 66.1% (33/54). CONCLUSIONS: Heterozygous mutation rate in female carriers of G6PD deficiency detected by RT-PCR-DGGE is high. RT-PCR-DGGE is value of clinical diagnosis for G6PD-deficiency female carriers.[Chin J Contemp Pediatr, 2009, 11 (8):613-616]

2009 Vol. 11 (08): 613-616 [Abstract] ( 5301 ) [HTML 1KB] [PDF 1175KB] ( 1477 )
617 GAO Xue-Ping, SU Lin-Yan, ZHAO Ai-Ling, LUO Xue-Rong, XIA Kun
Association of 14 polymorphisms in the five candidate genes and attention deficit hyperactivity disorder

OBJECTIVE: Attention deficit hyperactivity disorder (ADHD) is one of the most common behavior disorders in childhood and adolescent. The etiology of ADHD is unknown. The aim of this study was to investigate the relationship between each of the 14 polymorphisms in the five candidate genes and ADHD, and between the combination of some polymorphisms in those genes and ADHD, in attempting to examine whether combinations of genotypes would confer a significant susceptibility to ADHD. METHODS: One hundred and thirty-nine children with ADHD and one hundred and nineteen normal children were enrolled. Eight single nucleotide polymorphisms (SNP) of three candidate genes were examined with PCR and RFLP techniques. 48 bp VNTR in DRD4 gene was examined with PCR, nondenaturing polyacrylamide gel electrophoresis and silver staining. Five microsatellites (MS) of three candidate genes were examined with genotyping. The relationship between the combinations of 12 polymorphisms and ADHD was examined with logistic regression analysis. RESULTS: 1.The frequency of 1065T/1065T genotype and the 1065T allele were significantly higher in ADHD children than that in normal controls (P<0.05). The frequency of -48G/-48G genotype of the A-48G polymorphism of DRD1 gene was significantly lower in ADHD children than that in normal controls (P<0.05). 2. A specific combination of three polymorphisms in the two genes showing an association with ADHD gave a prediction level of 77.5%.ConclusionsThe T1065G polymorphism in the SNAP-25 may be associated with ADHD. The 1065T/ 1065T genotype and the 1065T allele may be a risk factor for ADHD. The A-48G polymorphism of DRDI may be associated with ADHD. The-48G/-48G genotype may be a protective factor for ADHD. The specific combination of three sites of SNP in SNAP-25 gene and DRDI gene is found and shows an association with ADHD in 12 polymorphisms of the five candidate genes on glutamatergic/dopaminergic pathway. [Chin J Contemp Pediatr, 2009, 11 (8):617-622]

2009 Vol. 11 (08): 617-622 [Abstract] ( 5590 ) [HTML 1KB] [PDF 1056KB] ( 1652 )
623 HUANG Yong, WAN Gen-Ping, ZHOU Zhen-Wen, DENG Qiu-Lian, HUANG Xu-Qiang, DENG Li, ZHAO Chang-An
Antimicrobial resistance and penicillin resistance-associated genes of Streptococcus pneumoniae isolated from children with respiratory tract infection

OBJCTIVE: To investigate the antimicrobial resistance and penicillin resistance-associated genes (TEM and pbp2B) of Streptococcus pneumoniae (S. pneumoniae) isolated from sputum specimens of Guangzhou children with respiratory tract infection. METHODS: E-test and Kirby-Bauer methods were applied to detect the antibiotic susceptibility of 44 strains of S. pneumoniae. PCR was used to detect resistance genes pbp2B and TEM, followed by DNA sequence analysis of pbp2B gene. The sequence results were compared to those of penicillin-susceptible S. pneumoniae R6. RESULTS: Of the 44 isolates of S. pneumoniae, only 5 (11.4%) were susceptible to penicillin. All strains were resistant to erythromycin but susceptible to ofloxacin and vancomycin. The resistance rate of the isolates to clindamycin and trimoxazole was more than 90%. The S. pneumoniae isolates showed a high susceptibility to amoxicillin, imipenem and ceftriaxone, with a resistance rate of 0, 2.6% and 3.9%, respectively. The sequence analysis showed that more than 99% nucleotide sequence of pbp2B gene of five penicillin-susceptible isolates was the same as penicillin-susceptible S. pneumoniae R6, without any amino acid replacement. Site mutation was found in the remaining 39 penicillin-nonsusceptible isolates with a nucleotide mutation rate ranging from 13.2% to 23.1% and amino acid replacement rate from 6.5% to 10.9%. The 39 penicillin-nonsusceptible isolates were classified into 4 types according to the mutation site between Ser391 and Thr492 of pbp2B: type I (n=30), type II (n=7), type III (n=1) and type IV (n=1). No TEM gene was detected in all the 44 S. pneumoniae isolates. CONCLUSIONS: The S.pneumoniae isolates from Guangzhou children with respiratory tract infection are resistant to penicillin and erythromycin. Amoxicillin and the third generation cephalosporin may be recommended for treating S. pneumoniae infection. The mutation of pbp2B gene plays an important role in the development of S. pneumoniae resistance to penicillin.[Chin J Contemp Pediatr, 2009, 11 (8):623-626]

2009 Vol. 11 (08): 623-626 [Abstract] ( 6087 ) [HTML 1KB] [PDF 1247KB] ( 1420 )
627 YANG Xi-Chen, WANG Feng-Ming, ZHAO Nai-Zheng, QIN Yu-Ming
Risk factors for acute fulminant myocarditis in children

OBJECTIVE: To investigate the risk factors for fulminant myocarditis by analyzing clinical symptoms/signs or laboratory findings in children with viral myocarditis. METHODS: The medical data of 71 children with acute viral myocarditis from March 2005 to September 2008 were retrospectively studied. They were classified into fulminant (n=16) and non-fulminant myocarditis groups (n=55). Chi-square and Student's t-test were used to analyze the clinical presentations, laboratory data, EEG and cardiac ultrasound findings on admission. The multiple regression analysis was used to identify the independent risk factors for fulminant myocarditis. RESULTS: Eight children (50%) died in the fulminant myocarditis group, but none in the non-fulminant group. The following factors were closely related to the fulminant course of myocarditis: lower blood pressure, higher serum CK-MB level, positive cTnI, complete atrioventricular block and left bundle branch block, ST segment alterations, prolonged QRS complex, and decreased left ventricular ejection fraction and short axis fractional shortening. Multiple regression analysis revealed that prolonged QRS complex (OR=1.139; CI=1.014-1.279, P<0.05) and decreased left ventricular ejection fraction (OR= 0.711; CI=0.533-0.949, P<0.05) were independent risk factors for fulminant myocarditis. CONCLUSIONS: The mortality of fulminant myocarditis is high in children. Prolonged QRS complex and decreased left ventricular ejection fraction on admission are independent risk factors for fulminant myocarditisin in children.[Chin J Contemp Pediatr, 2009, 11 (8):627-630]

2009 Vol. 11 (08): 627-630 [Abstract] ( 6280 ) [HTML 1KB] [PDF 1030KB] ( 1985 )
631 LIN Xiao-Mei, WEN Fei-Qiu, CHEN Ting, DING Lu, ZHANG Xiao-Yuan, ZHANG Wei
Value of serum brain natriuretic peptide in the diagnosis of hyperthyroid heart disease in children

OBJECTIVE: To evaluate the value of serum brain natriuretic peptide (BNP) in the diagnosis of hyperthyroid heart disease in children. METHODS: Fifty-eight children with hyperthyroidism were assigned to two groups according to their cardiac functions: hyperthyroid heart disease (n=28) and hyperthyroidism alone (n=30). Thirty healthy children served as the control group. Serum BNP level, left ventricular ejection fraction (LVEE) and E/A ratio were measured before and after treatment. The diagnostic value of BNP was evaluated in children with hyperthyroid heart disease. RESULTS: The serum BNP level in the hyperthyroid heart disease and the hyperthyroidism alone groups before treatment was significantly higher than that in the control group (P<0.05), while the LVEF and the E/A ratio were significantly lower than those in the control group (P<0.05). Serum BNP level was positively correlated with the TT3 (r=0.801, P<0.05) and TT4 levels (r=0.578, P<0.05) and negatively with the LVEF (r=-0.48, P<0.05) and the E/A ratio (r=-0.35, P<0.05) in the hyperthyroid heart disease group. The serum BNP, TT3 and TT4 levels in the hyperthyroid heart disease and the hyperthyroidism alone groups were reduced and the LVEF and the E/A ratio increased significantly three months after treatment (P<0.05). When serum BNP level of >323.62 pg/mL was proposed as a cutoff point, the sensitivity, specificity, positive predictive value and negative predictive value were 92.86%, 90.00%, 89.66% and 93.10% respectively for the diagnosis of hyperthyroid heart disease. CONCLUSIONS: BNP may serve as a reliable marker for the diagnosis of hyperthyroid heart disease in children. Serum BNP level along with the LVEF and the E/A ratio may be useful in the evaluation of the severity and the cardiac function in children with this disease.[Chin J Contemp Pediatr, 2009, 11 (8):631-634]

2009 Vol. 11 (08): 631-634 [Abstract] ( 4940 ) [HTML 1KB] [PDF 1024KB] ( 1284 )
635 LI Jiu-Jun, LIU Chun-Feng, YUAN Zhuang, DAI Bing
Treatment of congenital heart disease complicated by severe bronchopneumonia in infants

OBJECTIVE: To study the treatment and the treatment outcome in infants with congenital heart disease complicated by severe pneumonia and heart failure. METHODS: The clinical data of 24 infants with congenital heart disease (left to right shunt) complicated by severe pneumonia and heart failure between January 2007 and December 2007 were retrospectively reviewed. RESULTS: Twenty-two infants recovered and 2 died. Severe pneumonia and heart failure were refractory even after 1-2 months medical treatment in 6 infants at ages of <6 months. They then underwent an open heart surgery under the mechanical ventilation and tracheal intubations and were successfully cured. The other 18 infants underwent a selective heart surgery after pneumonia and heart failure had been improved. Sixteen infants were successfully cured and 2 died of postoperative low cardiac output syndrome and diffuse intravascular clotting. CONCLUSIONS: The heart surgery should be performed early when the medical treatment does not work in infants with congenital heart disease complicated by severe pneumonia and heart failure. This may improve their outcome.[Chin J Contemp Pediatr, 2009, 11 (8):635-637]

2009 Vol. 11 (08): 635-637 [Abstract] ( 8088 ) [HTML 1KB] [PDF 1006KB] ( 2264 )
638 ZHANG Qing, MENG Bao-Ying, PENG Le, WANG Tao, MA Chao, TAO Jing
Myocardial protection of cold autoblood cardioplegia in infants with congenital heart disease

OBJECTIVE: To study the effects of cold autoblood cardioplegia on oxygen free radicals in the myocardium in infants who underwent cardiopulmonary bypass and to explore the possible mechanism of myocardial protection of autoblood cardioplegia. METHODS: Thirty infants with acyanotic congenital heat disease (CHD) (weight≤8 kg) were randomized to receive cold crystalloid, cold blood or cold autoblood cardioplegia (n=10 each group) during cardiopulmonary bypass. The biopsy samples were taken from the right atrium just before heart arrest and after heart self-recovery for the measurement of malonaldehyde (MDA) and superoxide dismutase (SOD) contents. The time and the rate of the heart self-recovery to sinus rhythm, and the incidence of ventricular fibrillation were recorded during operation. The cardiac index (CI) and the dependence of positive inotropic drugs were monitored after operation. RESULTS: Before the operation, there were no significant differences in myocardial MDA (0.87±0.14, 0.88±0.11 and 0.86±0.15 nmol/mg prot, respectively) and SOD contents (61.3±3.4, 69.2±3.1 and 64.4±4.2 U/g, respectively) among the crystalloid, the blood and the autoblood cardioplegia groups. After operation, the myocardial MDA content increased (3.12±0.21, 2.93±0.27 and 1.67±0.15 nmol/mg prot, respectively) and SOD content (42.6±2.3, 44.6±3.1 and 57.7±2.1 U/g, respectively) decreased significantly in the three groups (P<0.05 or 0.01). The autoblood cardioplegia group had lower myocardial MDA content and higher SOD content than the crystalloid and the blood cardioplegia groups (P<0.05). The time of heart self-recovery was shortened and the dependence of positive inotropic drugs were reduced in the autoblood cardioplegia group compared with the crystalloid and the blood cardioplegia groups (P<0.05). Post-operational CI in the autoblood cardioplegia group was significantly higher than that in the blood and the crystalloid cardioplegia groups. There were significant differences in the time of heart self-recovery, the dependence of positive inotropic drugs and the CI between the blood and the crystalloid cardioplegia groups (P<0.05 or 0.01). CONCLUSIONS: Cold autoblood cardioplegia reduces oxygen free radicals in the myocardium, thus providing myocardial protections in infants undergoing cardiopulmonary bypass.[Chin J Contemp Pediatr, 2009, 11 (8):638-640]

2009 Vol. 11 (08): 638-640 [Abstract] ( 5024 ) [HTML 1KB] [PDF 1074KB] ( 1415 )
641 HE Xiao-Ri, CHEN Ping-Yang, WANG Tao, XIE Zong-De, HU Jin-Tao, BO Tao, GAI Jian-Fang
Comparison of therapeutic effect of different doses of ganciclovir for neonatal congenital cytomegalovirus infection

OBJECTIVE: Ganciclovir is a first-line drug for treatment of cytomegalovirus (CMV) infection. However, some ganciclovir treatment-related side-effects can be found. This study aimed to compare the efficacy and side effects of relatively low and high doses of ganciclovir in the treatment of neonatal congenital CMV infection. METHODS: One hundred and sixty-seven neonates with congenital CMV infection were randomly assigned to high-dose (n=79) and low-dose ganciclovir groups (n=88). The high-dose ganciclovir group was injected with ganciclovir of 7.5 mg/kg in the inducement phase and of 10 mg/kg in the maintaining phase. The low-dose ganciclovir group was injected with ganciclovir of 5 mg/kg in the inducement and the maintaining phases. The efficacy and side effects were observed in the two groups. RESULTS: After treatment the clinical symptoms and signs were obviously improved in both groups. CMV-IgM became negative in 93.8% of neonates in the high-dose ganciclovir group and 93.1% of neonates in the low-dose ganciclovir group (P>0.05). CMV-DNA became negative in 80.8% of neonates in the high-dose ganciclovir group and in 86.7% in the low-dose ganciclovir group (P>0.05). The low-dose ganciclovir group had lower incidence of side effects than the high-dose ganciclovir group: vomiting 2.3% vs 11.4%; anemia 8.0% vs 20.3%; reduction of neutrophilic granulocytes 5.7% vs 16.5%; increase in platelet count 8.0% vs 18.9% (P<0.05).ConclusionsLow-dose ganciclovir has the same clinical efficacy to high-dose ganciclovir for treatment of neonatal congenital CMV infection, but fewer side effects occur in the low-dose group.[Chin J Contemp Pediatr, 2009, 11 (8):641-644]

2009 Vol. 11 (08): 641-644 [Abstract] ( 5427 ) [HTML 1KB] [PDF 1024KB] ( 1696 )
645 XIA Yao-Fang, LIU Cui-Qing, SHI Hui-Ju, MA Li
Interleukin-4 and interleukin-13 concentrations in bronchoalveolar lavage fluid in neonates with respiratory distress syndrome and concurrent ventilator-associated pneumonia

OBJECTIVE: To study the concentrations of IL-4 and IL-13 in bronchoalveolar lavage fluid (BALF) in neonates with respiratory distress syndrome (RDS) and concurrent ventilator-associated pneumonia (VAP). METHODS: Sixty-eight neonates with RDS undergoing mechanical ventilation for over 48 hrs were enrolled. IL-4 and IL-13 levels in BALF were measured using ELISA 1, 72 and 96 hrs after mechanical ventilation. The results were compared between the neonates with concurrent VAP (n=37) and without (n=31). RESULTS: The levels of BALF IL-4 96 hrs after ventilation in the VAP group (35.34±1.78 ng/mL) were significantly higher than those in the non-VAP group (13.69±2.47ng/mL, P<0.05). The levels of BALF IL-13 96 hrs after ventilation in the VAP group (33.74±2.74 ng/mL) also increased significantly compared with those in the non-VAP group (13.50±3.81 ng/mL) (P<0.05). There were significant differences in BALF IL-4 and IL-13 levels between 1 hr and 96 hrs in the VAP group (P<0.05).ConclusionsBALF IL-4 and IL-13 levels increase in neonates with RDS and concurrent VAP. IL-4 and IL-13 may involve in the regulation of the inflammatory immue response.[Chin J Contemp Pediatr, 2009, 11 (8):645-648]

2009 Vol. 11 (08): 645-648 [Abstract] ( 4833 ) [HTML 1KB] [PDF 1033KB] ( 1393 )
649 WU Zhi-Jun, HUANG Shang-Ming, CHEN Rui, HU Bin, CHEN You, ZHU Yuan-Peng, LU Guang-Jin, HAN Yu-Kun
Value of blood apoH gene expression and urinary NAG and RBP in early diagnosis of renal function damage in neonates

OBJECTIVE: To study the value of apolipoprotein H (apoH) gene expression in peripheral blood mononuclear cell (PBMC) and urinary N-Acetyl-beta-D-Glucosaminidase (NAG) and retinal-binding protein (RBP) in the early diagnosis of renal function damage in neonates. METHODS: Sixty sick neonates who renal function damage probably occurred were enrolled. The blood and urinary samples were collected twice within 48 hrs following admission, with an interval of 12-24 hrs. Expression of apoH gene in PBMC was determined with RT-PCR. The levels of blood urea nitrogen (BUN) and creatinine, and urinary activities of NAG and RBP were measured with enzymatic reaction. RESULTS: The abnormal rates of blood apoH and urinary NAG and RBP were 73.3%, 83.3% and 76.7%, respectively in the first detection. The second detection for blood apoH and urinary NAG and RBP showed abnormal rates of 70.0%, 66.7% and 76.7%, respectively. There were no significant differences in the abnormal rates between the three markers either in the first or the second detection (P>0.05). Beside there were no significant significances in the abnormal rates between urinary NAG and blood BUN in the second detection, the abnormal rates of blood apoH and urinary NAG and RBP in both detections were significantly higher than those of BUN or creatinine (P<0.01 or 0.05). CONCLUSIONS: There are identical values of blood apoH gene expression and urinary NAG and RBP in the early diagnosis of renal function damage in neonates. The above three markers are more sensitive to early renal function damage than blood BUN and creatitine.[Chin J Contemp Pediatr, 2009, 11 (8):649-652]

2009 Vol. 11 (08): 649-652 [Abstract] ( 4470 ) [HTML 1KB] [PDF 1022KB] ( 1391 )
653 HUANG Tie-Shuan, ZHU Jin-Lan, LI Bing, HU Yan, CHEN Li, LIAO Jian-Xiang
Valproic acid versus lamotrigine as a monotherapy for absence epilepsy in children

OBJECTIVE: To compare the efficacy of valproic acid (VPA) and lamotrigine as a monotherapy for absence epilepsy in children.MethodsA randomized, open-label design was used. Childhood absence epilepsy was diagnosed based on the presence of typical seizures and video-EEG findings. Eligible patients were randomly treated with VPA or lamotrigine. All patients were followed up for 12 months. RESULTS: Forty-five out of 48 eligible children completed the study. There were 23 children in the VPA group and 22 children in the lamotrigine group. Seventeen children were seizure-free in the VPA group 12 months after treatment. Fifteen out of the 17 children showed normal EEG (no epileptic-formed discharge). Twelve children were seizure-free in the lamotrigine group 12 months after treatment. The proportion showing normal EEG in the camotrigine group (6/22, 27.3%) was significantly lower than that in the VPA group (15/23, 65.2%) (P<0.05). Severe adverse effects were not found in both groups. CONCLUSIONS: Both VPA and lamotrigine are safe and efficacious for treatment of absence seizures in children. VPA appears to be better than lamotrigine in tapering epileptic-formed discharge.[Chin J Contemp Pediatr, 2009, 11 (8):653-655]

2009 Vol. 11 (08): 653-655 [Abstract] ( 5692 ) [HTML 1KB] [PDF 1017KB] ( 2001 )
656 LIU Jian-Hua, XIE Cai-Jiao, LI Li
Effects of ambroxol on renal function in children undergoing cardiopulmonary bypass

OBJECTIVE: Some research has shown that ambroxol can alleviate lung injury induced by cardiopulmonary bypass (CPB). However, whether ambroxol has protective effects against CPB-induced renal injury remains unknown. This study investigated the effect of ambroxol on renal function in children undergoing CPB. METHODS: Forty children at ages of 3-8 years with cardiac function classⅠor Ⅱand weighing 12-25kg, underwent repair of ventricular septal defect (VSD) under CPB. They were randomly divided into two groups (n=20 each): control and ambroxol-treated. The children in the ambroxol-treated group received ambroxol of 4.5 mg/kg in 10 mL normal saline by intravenous injection after skin incision. The control group received 10 mL of normal saline instead. Serum concentrations of urea nitrogen (BUN), β2-microglobulin (β2-MG) and creatinine (Cr) and urinary β2-MG, retinol-binding-protein (RBP) and N-acetyl-β-D-glucosaminidase (NAG) were measured before operation, and 2, 12, 24 and 48 hrs after operation. RESULTS: Serum Cr and urinary β2-MG concentrations 2 hrs after operation, serum β2-MG concentration 2 and 12 hrs after operation, urinary RBP concentration 2, 12, 24 and 48 hrs after operation, and urinary NAG concentration 2, 12 and 24 hrs after operation in the control and the ambroxoltreated groups increased significantly as compared with their baseline values (before operation) (P<0.05). Serum Cr concentration 2 hrs after operation, serum β2-MG and urinary β2-MG concentrations 2 and 12 hrs after operation, urinary RBP concentration 2, 12, 24 and 48 hrs after operation, and urinary NAG concentration 12 and 24 hrs after operation in the ambroxol-treated group were significantly lower than those in the control group (P<0.05). CONCLUSIONS: Ambroxol administration before CPB is effective in reducing CPB-induced renal injury in children undergoing repair of VSD. Further research is required to understand the mechanism.[Chin J Contemp Pediatr, 2009, 11 (8):656-658]

2009 Vol. 11 (08): 656-658 [Abstract] ( 5909 ) [HTML 1KB] [PDF 1017KB] ( 1695 )
659 PAN Ci, TANG Jing-Yan, CHEN Jing, XUE Hui-Liang, DONG Lu, ZHOU Min, JIANG Hua, XIE Qi-Dong, GU Long-Jun
Clinical observation on the treatment outcome of 14 children with hepatoblastoma in a single medical center

OBJECTIVE: To evaluate the efficacy and safety of the ICE regimen (iphosphamide + carboplatin+etoposide) used in treating children with hepatoblastoma in the Shanghai Children's Medical Center. METHODS: From June 2000 to June 2008, 14 children with newly diagnosed hepatoblastoma (7 males and 7 females) were enrolled. Their median age on diagnosis was 1.33 years (range: 0.25-8.25 years). Six patients had stage I disease, 1 had stage II, 5 had stage III, and 2 had stage IV diseases. Thirteen children had markedly increased serum AFP level, and 1 had normal serum AFP level. Multidisciplinary co-operation treatment was performed. Eight patients had primary surgery while 3 patients had pre-operation chemotherapy before surgery. ICE chemotherapy regimen was used. Totally, 73 courses of chemotherapy were administered for the 14 children and 25 out of the 73 courses were performed before operation. RESULTS: Twelve patients responded to the treatment (85.7%) and 2 failed. Ten patients (71.4%) achieved complete remission after treatment, and two had partial remission. By July 31st, 2008, 9 patients survived without any event, with a median follow-up duration of 35 months (range: 16-96 months). The 5-year overall survival rate was 70.71±12.37%, and the 5-year event-free survival rate was 64.29±12.81%. One patient had disease relapse and two patients were lost to follow-up after they achieved partial remission. CONCLUSIONS: The ICE regimen combined with operation is effective and safe in treating children with hepatoblastoma.[Chin J Contemp Pediatr, 2009, 11 (8):659-662]

2009 Vol. 11 (08): 659-662 [Abstract] ( 5550 ) [HTML 1KB] [PDF 1055KB] ( 1486 )
663 LIU Zhi-Xiong, LIU Yun-Sheng, YUAN Xian-Rui, LIU Jin-Fang
Surgical treatment of childhood craniopharyngioma: clinical analysis of 31 cases

OBJECTIVE: To study the diagnosis, surgical treatment and outcome of craniopharyngioma in 31 children.MethodsThe clinical data of 31 children (aged 7-14 years) with craniopharyngioma were studied retrospectively. RESULTS: Headache, visusal disorder and growth retardation were main manifestations in the 31 children. The 31 children were definitely diagnosed with craniopharyngioma by CT and MRI. In the 31 cases, 19 (61.3%) underwent total tumor removal, 5 (16.1%) subtotal removal, and 7 (22.6%) partial removal. After tumor removal, transient diabetes insipidus occurred in 19 cases (61.3%) and long-term diabetes insipidus in 3 cases. Six cases (19.4%) presented hypothalamic injuries after surgery. No patient died after surgery. Five patients (16.1%) had recurrent tumor in a mean follow-up of 32.5 months.ConclusionsThe diagnosis of childhood craniopharyngioma may be based on clinical manifestations and CT/MRI examinations. Craniotomy is a preferred surgical treatment. Proper extent of tumor resection should be determined in order to reduce the tumor recurrence and the incidence of postoperative complications.[Chin J Contemp Pediatr, 2009, 11 (8):663-665]

2009 Vol. 11 (08): 663-665 [Abstract] ( 5244 ) [HTML 1KB] [PDF 1012KB] ( 1407 )
666 LUO Yan-Hong, YOU Jie-Yu, LIU Li, TANG Shuo, CHEN Zhi-Yong, XU Bin
Application of electronic gastroscopy in 177 infants at ages of 0-3 months

OBJECTIVE: To study the clinical application and the safety of electronic gastroscopy in infants at ages of 0-3 months.MethodsAn Olympus electronic gastroscope GIF 260 or GIF 230 was applied in 177 infants at ages of 0-3 months with upper gastrointestinal symptoms. An ECG Monitor II was used for monitoring heart rate, cardiac rhythm and pulse transcutaneous oxygen saturation in 65 infants during the whole process of gastroscopy. Some related treatments were performed under the electronic gastroscope, such as removal of foreign body, topical administration of anthemorrhagic drugs, reduction of volvulus of stomach and bougienage of oesophagus.ResultsAll 177 infants were examined successfully and they all well tolerated. Forty-two cases demonstrated positive findings in 68 cases of upper gastrointestinal bleeding. Ninety-two cases demonstrated positive findings in 104 cases of recurrent vomiting. Under the electronic gastroscope, removal of foreign body in the upper gastrointestinal tract was performed in 5 cases; topical administration of anthemorrhagic drugs or electrocoagulation for stopping bleeding in 22 cases; reduction of volvulus of stomach in 3 cases. CONCLUSIONS: As a safe and visual means for diagnosis of digestive tract diseases, the electronic gastroscopy may be recommended for wide application in infants at ages of 0-3 months.[Chin J Contemp Pediatr, 2009, 11 (8):666-668]

2009 Vol. 11 (08): 666-668 [Abstract] ( 4827 ) [HTML 1KB] [PDF 1012KB] ( 1192 )
669 HUANG Jian-Jun, TONG Zhi-Jie, WEN Hui-Hong, YANG Jie-Wen, ZHONG Li-Ping
Pulmonary function of children with right lung middle lobe syndrome

OBJECTIVE: To study the changes of pulmonary function in children with right lung middle lobe syndrome before and after treatment. METHODS: Thirty children with right lung middle lobe syndrome were classified into two age groups: ≤4 years old and >4 years old. Pulmonary function was tested by the 2600-type and the MIR-type pulmonary function spirometry in the ≤4 years and the >4 years age groups, respectively before and after treatment. Terminal flows/peak expiratory flow (25/PF) and the percentage of tidal volume to peak tidal expiratory flow (% V-PF) were measured in the <4 years age group. Forced vital capacity (FVC), forced expiratory volume in one second (FEV1) and peak expiratory flow (PEF) were measured in the >4 years age group. RESULTS: The values of 25/PF and %V-PF in the ≤4 years age group were 0.42±0.08 and 0.28±0.03, respectively before treatment. The values were improved after treatment (0.58±0.12 and 0.39±0.06 respectively) (P<0.05). The values of FVC, FEV1 and PEF were 1.75±0.32, 1.36±0.52 and 2.56±0.78, respectively in the >4 years age group before treatment. The values were also improved after treatment (2.37±0.78, 2.08±0.65 and 3.68±0.80 respectively) (P<0.05). CONCLUSIONS: There are significant differences in the pulmonary function before and after treatment in children with right lung middle lobe syndrome. The pulmonary function can return to normal after treatment.[Chin J Contemp Pediatr, 2009, 11 (8):669-671]

2009 Vol. 11 (08): 669-671 [Abstract] ( 5469 ) [HTML 1KB] [PDF 1007KB] ( 1421 )
CASE REPORT
648 LI Jun, LI Gui-Na, SUN Zheng-Xiang, LIU Xin-Hui, LI Qiang
Malignant histiocytosis in a neonate
2009 Vol. 11 (08): 648-648 [Abstract] ( 3622 ) [HTML 1KB] [PDF 977KB] ( 1166 )
697 XIN Ying, TONG Ya-Jie, YANG Min, GUAN Jun-Hong
Primary hypothyroidism in a child mimicking a pituitary nacroadenoma on MR imaging
2009 Vol. 11 (08): 697-698 [Abstract] ( 3659 ) [HTML 1KB] [PDF 1093KB] ( 1155 )
699 GUO Jun-Bin, LI Zhao-Zhu
A case report of congenital colonic stenosis
2009 Vol. 11 (08): 699-700 [Abstract] ( 4647 ) [HTML 1KB] [PDF 1132KB] ( 1296 )
700 PENG Wei, YANG Xiao, ZHU Li-Na, MA Ning, LIU Xin, WANG Wei
A case report of 13q partial trisomy syndrome
2009 Vol. 11 (08): 700-701 [Abstract] ( 4237 ) [HTML 1KB] [PDF 1243KB] ( 1317 )
CONFERENCE
672 SHENG Xiao-Yang, XU Ji-De, SHEN Xiao-Ming
Key parameters and formulas for approximate average height and weight of normal Chinese children under 7 years of age

OBJECTIVE: In order to understand the normal physical growth of Chinese children, data of children aged from 0 to 7 years from urban and rural areas of nine Chinese cities in 2005 were analyzed. METHODS: The original data of height and weight were drawn into growth curves charts by Graphpad Prism 5.0 software according to the different age groups. The children were classified into five age groups: 0-3 months, 4-6 months, 7-12 months, 13-24 months, and 2-7 years. RESULTS: The average birth weight was 3.3 kg and the height averaged 50 cm. The average monthly weight gain was 1.0-1.2 kg and the average monthly increase of height was 4 cm in the 0-3 months group. By 3 months of age, the weight and height averaged 6.6 kg and 62 cm, respectively. In the 4-6 months group, the growth rate was reduced to a half of the 0-3 months group, with an average monthly weight and height gain was 0.5-0.6 kg and 2 cm respectively. The growth rate in the 7-12 months group was a half of the 4-6 months group. By 12 months of age, the weight and height average 9.9 kg and 75 cm, respectively. The average monthly weight and height gain in the 13-24 months group was 0.2 kg and 1 cm respectively, with an average weight and height of 12 kg and 87 cm respectively by 24 months of age. A steady growth was found in the 2-7 years group, with a yearly average weight and height increment of about 2 kg and 7 cm respectively. The formulas for approximate average weight and height in children between 2 and 7 years were as follows: age (yr)×2+8 (kg) (weight); age (yr)×7+75 (cm) (height). CONCLUSIONS: The approximate weight and height of normal Chinese children under 7 years of age can be evaluated by the key parameters and formulas above mentioned.[Chin J Contemp Pediatr, 2009, 11 (8):672-674]

2009 Vol. 11 (08): 672-674 [Abstract] ( 8250 ) [HTML 1KB] [PDF 1289KB] ( 1780 )
675 YAN Qin-Qin, JIAO Wen-Yan, LIN Jing, JIAO Fu-Yong
Effect of childhood physical and emotional abuse on psychological health in undergraduate students

ObjectiveTo study the effect of childhood physical and emotional abuse on psychological health in undergraduate students.MethodsA questionnaire investigation (Bernstein, revised) on childhood abuse history was performed in 1 200 undergraduate students in Xi'an. Their psychological states were assessed by Symptom Check List-90.ResultsSix hundred and sixty-eight students (55.7%) had physical and emotional abuse experiences, including humiliation, corporal punishment, beating or activity confinement, before 16 years old. Twenty-one percent of the 1 200 students experienced beating, even serious beating with equipment. The students who experienced serious physical and emotional abuse during childhood were more prone to the development of somatization symptoms, obsession, interpersonal sensitivity, depression, anxiety, hostility, phobic anxiety and paranoia than those students without abuse experiences.ConclusionsChildhood physical and emotional abuse is common in China and this may adversely affect the psychological health of undergraduate students who have experienced it.[Chin J Contemp Pediatr, 2009, 11 (8):675-678]

2009 Vol. 11 (08): 675-678 [Abstract] ( 5489 ) [HTML 1KB] [PDF 1025KB] ( 1776 )
EXPERIMENTAL RESEARCH
679 LEI Qi, TANG Suo-Qin, WANG Tian-You
Effect of topotecan on MAGE gene expression of HPB-AM cells

OBJECTIVE: To investigate whether topotecan, a novel anti-tumor angent, down-regulates gene expression of melanoma antigen-encoding (MAGE) in HPB-AM cells. METHODS: MAGE mRNA expression of HPB-AM cells was detected by RT-PCR 4, 8, 12 and 16 hrs after different concentrations (0.05, 0.10, 0.15 and 0.20 μmol/L) of topotecan treatment. RESULTS: MAGE mRNA expression of HPB-AM cells decreased with increasing concentrations of topotecan 12 hrs after treatment. The MAGE mRNA expression of HPB-AM cells treated by 0.10, 0.15 and 0.20 μmol/L of topotecan was significantly lower than that in the blank control group (P<0.05). MAGE mRNA expression of HPB-AM cells was significantly reduced in a time-dependent manner after 0.10 μmol/L of topotecan treatment. The MAGE mRNA expression of HPB-AM cells treated by 0.10 μmol/L of topotecan was significantly lower than that in the blank control group 12 and 16 hrs after treatment (P<0.05). CONCLUSIONS: Topotecan is capable of inhibiting the expression of MAGE mRNA of HPB-AM cells in a time- and dose-dependent manner.[Chin J Contemp Pediatr, 2009, 11 (8):679-682]

2009 Vol. 11 (08): 679-682 [Abstract] ( 3911 ) [HTML 1KB] [PDF 1112KB] ( 978 )
683 HUANG Li-Xin, WU Xing-Heng
Effect of Xinmailong on hypoxia-inducible factor-1α expression in neonatal rats with asphyxia

OBJECTIVE: Xinmailong, a compound extracted from periplaneta americana, is used for the treatment of cardiovascular diseases. This study investigated the effects of Xinmailong on myocardial hypoxia-inducible factor-1α (HIF-1α) and plasma endothelin-1(ET-1) levels in neonatal rats with asphyxia and explored the protection mechanism of Xinmailong in hypoxia-ischemic myocardial injury. METHODS: Seven-day-old Sprague-Dawley rats were randomly divided into three groups (n=30 each): sham-operated, asphyxia, Xinmailong-treated asphyxia. Each group was randomly subdivided into three groups according to the observed time points: 6 hrs, 24 hrs and 72 hrs. Xinmailong (5 mg/kg) was intraperitoneally injected to the rats in the Xinmailong-treated group five minutes before asphyxia. Myocardial HIF-1α expression, and plasma ET-1 and creatine kinase (CK) levels were measured. The histopathologic changes of the myocardium were observed by hematoxylin-eosin staining. RESULTS: Four rats died in the asphyxia group while only one died in the Xinmailong-treated group during the experiment. The plasma ET-1 and CK levels as well as myocardial HIF-1α expression increased at 6 hrs, reached a peak at 24 hrs, and declined at 72 hrs after asphyxia in the asphyxia group, being higher than that in the sham-operated group (P<0.01). Myocardial ischemia was observed in the three time points, and cell necrosis occurred at 24 hrs after asphyxia in the asphyxia group. Myocardial HIF-1α expression was positively correlated with plasma ET-1 levels (r=0.876, P<0.01). In the Xinmailong-treated group, plasma levels of CK and ET-1 as well as myocardial HIF-1α expression were significantly lower than those in the asphyxia group (P<0.01). Myocardial ischemia was alleviated and no cell necrosis was found in the Xinmailong-treated group. CONCLUSIONS: Asphyxia leads to increase in myocardial HIF-1α expression and plasma levels of ET-1 and CK. Xinmailong can reduce the myocardial expression of HIF-1α and decrease plasma ET-1 levels, thus alleviating hypoxia-ischemic myocardial injury.[Chin J Contemp Pediatr, 2009, 11 (8):683-686]

2009 Vol. 11 (08): 683-686 [Abstract] ( 4589 ) [HTML 1KB] [PDF 1321KB] ( 1227 )
687 QIAN Zhao-Xin, HUANG Han, LIN Xiao-Juan
Protective effects of tetramethylpyrazine on rat myocardial cells infected by Coxsackie virus B3 and its signal transduction mechanism

OBJECTIVE: To study the protective effects of tetramethylpyrazine (TMPZ) on rat myocardial cells infected by Coxsackie virus B3 (CVB3) and its signal transduction mechanism. METHODS: The cultured myocardial cells of neonatal Sprague-Dawley rats were randomly treated with CVB3, CVB3+TMPZ (100 μmol/L), TMPZ (100 μmol/L) (negative control) or DMEM (blank control). After treatment, the beating rate of myocardial cells and the LDH activity in the culture fluid were measured. Cell viability was ascertained with MTT assay. Western blot was used to study the expression of nuclear factor kappa-B (NF-κB) protein in myocardial cells. RESULTS: The beating rate of myocardial cells in the untreated CVB3 infection group was significantly lower than that in the TMPZ-treated CVB3 infection group (32.0±3.6 bpm vs 84.3±3.5 bpm, P<0.01). The LDH activity and NF-κB expression in the TMPZ-treated CVB3 infection group was significantly reduced when compared with untreated CVB3 infection group (P<0.05, P<0.01, respectively). Cell viability 7 days after CVB3 infection in the TMPZ-treated group was higher than that in the untreated CVB3 infection group [(86.7±2.7)% vs (35.3±3.4)%; P<0.01]. CONCLUSIONS: TMPZ can provide protective effects on rat myocardial cells infected by CVB3, possibly by an inhibition of the activity of NF-κB in myocardial cells.[Chin J Contemp Pediatr, 2009, 11 (8):687-690]

2009 Vol. 11 (08): 687-690 [Abstract] ( 4954 ) [HTML 1KB] [PDF 1118KB] ( 1325 )
691 LUO Li-Li, XIONG Ying, WANG Hui-Qing
Expression of GRP78 and caspase-12 in neonatal rats with experimental hypoxic-ischemic white matter damage

OBJECTIVE: GRP78 is a sensitive marker of endoplasmic reticulum stress. Caspase-12 is involved in apoptosis induced by endoplasmic reticulum stress. This study was designed to explore the changes of GRP78 and caspase-12 mRNA in neonatal rats with experimental hypoxic-ischemic white matter damage (WMD) and investigate the roles of endoplasmic reticulum stress in the WMD. METHODS: Two-day-old rats were randomized to WMD and control groups (n=49 each). The pups were sacrificed at 0, 2, 4, 6, 12, 24 and 72 hrs after hypoxia-ischemia (HI). The light microscope was used to observe the brain pathological changes. Real time PCR was used to detect the expression of GRP78 mRNA and caspase-12 mRNA in the white matter tissue. RESULTS: The expression of GRP78 mRNA began increasing 2 hrs after HI and peaked at 6 hrs in the WMD group, demonstrating significant differences at 2, 4, 6, 12, 24 and 72 hrs compared with the control group (P<0.05). The caspase-12 mRNA expression in the WMD group began increasing 6 hrs after HI and demonstrated significantly increased levels 6, 12 and 24 hrs after HI compared with those in the control group (P<0.05). CONCLUSIONS: GRP78 and caspase-12 mRNA expression increased significantly in neonatal rats with WMD. This suggests that endoplasmic reticulum stress may be induced following HI. Endoplasmic reticulum stress seems to be involved in the apoptosis of oligodendrocytes induced by HI in neonatal rats with WMD.[Chin J Contemp Pediatr, 2009, 11 (8):691-694]

2009 Vol. 11 (08): 691-694 [Abstract] ( 4634 ) [HTML 1KB] [PDF 1481KB] ( 1342 )
CLINICAL EXPERIENCE
695 ZHU Ming-Liang, HE Shuo
Efficacy of montelukas in the prevention of acute asthma episode in children
2009 Vol. 11 (08): 695-696 [Abstract] ( 4048 ) [HTML 1KB] [PDF 992KB] ( 2112 )
REVIEW
702 XIANG Lei, LIU Ling
Research advance in neonatal pain
2009 Vol. 11 (08): 702-705 [Abstract] ( 4170 ) [HTML 1KB] [PDF 1021KB] ( 1397 )
706 XU Xiao-Zhi, HE Ping
Heme oxygenase-1 and oxidative stress
2009 Vol. 11 (08): 706-709 [Abstract] ( 4271 ) [HTML 1KB] [PDF 1013KB] ( 1459 )
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