Objective To assess the relationship between AG, GG and AG+GG genotype and G allele of glutathione S-transferase pi (GSTP1) A313G polymorphisms and childhood acute lymphoblastic leukemia (ALL).Methods PubMed, EMBASE, OVID, China Bio-Medicine Database, China National Knowledge Infrastructure and Wanfang Chinese Periodical Database were searched for the case-control study on the association of GSTP1 A313G polymorphisms with ALL from January 2000 to June 2014. According to the inclusion criteria, related articles were evaluated. Poor-quality studies were excluded. RevMan 5.3 software and Stata 12.0 software were applied for investigating the meta-analysis and calculating the pooled odds ratio (OR) and 95% confidence interval (CI).Results Nine eligible studies were included with 1 476 childhood ALL cases and 1 905 controls. Overall, the variant genotypes AG, GG, AG+GG and G allele of GSTP1 A313G polymorphisms had no association with childhood ALL risk (AG genotype: OR=1.07, 95%CI:0.93-1.24, P=0.35; GG genotype: OR=1.12, 95%CI:0.86-1.45, P=0.41; AG+GG genotype: OR=1.08, 95%CI: 0.94-1.24, P=0.28; G allele: OR=1.11, 95%CI: 0.96-1.28, P=0.16). Similarly, no significant associations were detected in any subgroups of race, source of control group, gene analysis method and sample size.Conclusion The results suggested there was no association between GSTP1 A313G polymorphisms and childhood ALL.
Objective To observe changes of palpebral fissure of non-invasive ice pack test in children with juvenile myasthenia gravis （JMG）, and evaluate the diagnostic value of ice pack test in JMG.Methods The patients with ptosis were divided into JMG group and non-myasthenic gravis (MG) group. Palpebral fissures of the patients with ptosis in two groups were measured before and 2 min and 5 min immediately after ice pack test which was performed 24 h after neostigmine test for 60 min at 20℃-25℃. The changes of palpebral fissures relative to baseline great than 2 mm were consideed positive. The sensitivity and specificity of ice pack test (2 min and 5 min) and neostigmine test to the diagnosis of JMG were compared.Results ①There were 29 cases of JMG (1 case of generalised myasthenia gravis and 28 cases of ocular myasthenia gravis) in JMG group and 6 cases of non-MG in control group. The median age of JMG group and non-MG group was 5.5 years old and 6.2 years old, respectively. ②In JMG group, 27 cases were positive at 30 min, and 2 cases were negative at 60 min during neostigmine test. The positive results of ice pack test were abserved in 18 and 21 cases at 2 min and 5 min, respectively. ③In non-MG group, there was only 1 case whose palpebral fissure changes of ice pack test for 2 min, ice pack test for 5 min was 1 mm. There were no palpebral fissure changes in 5 cases.④The sensitivities of ice pack test for 2 min, ice pack test for 5 min and neostigmine test were 62.1%, 72.4% and 93.1%, respectively. All of the specificities of ice pack test and neostigmine test were all 100%. There were significant differences between the ice pack test for 2 min and neostigmine test, the ice pack test for 5 min and neostigmine test respectively. ⑤There were 4 cases with abdominal pain, 3 cases with nausea, 3 cases with sweating and 2 cases with vomiting during the neostigmine test. The skin of the upper eyelid of 6 cases turned red slightly.Conclusion The high sensitivity and specificity of ice pack test for 5 min, as well as the rapid, simple, safe, cheap and woundless characteristics make it benefit to the diagnosis of ptosis of JMG.
Objective To analyze anemia influencing factors of children with chronic peritoneal dialysis.Methods A retrospective review of 32 patients with registration of chronic peritoneal dialysis was carried out at Children's Hospital of Fudan University from January 2006 to April 2012, the follow-up files after dialysis were established, once follow-up was taken every three months in our hospital, duration of follow-up was 12 months at least, follow-up indicators were examined. Baseline follow-up indicators of pre-dialysis were shown by the number of cases, after dialysis the follow-up indicators (medical projects, laboratory projects and infection) were showed by episodes, hemoglobin value of every three months follow-up time ± 3 days was selected to diagnose anemia. If patients met the diagnostic criteria of anemia, this stage of follow-up indicators were included in the anemia group, whereas if the patients did not meet were included in the non-anemic group. SPSS 19.0 software was applied for statistical analysis, the measurement data were compared using Student's t-test, frequency distributions were compared using the chi-square test, multivariate logistic regression analysis was used to identify factors associated with anemia.Results (1)30/32(93.75%) patients met the criteria for anemia at the baseline，57/120(47.5%) episodes still met the criteria for anemia after following up 12 months. Hemoglobin and hematocrit in the anemia group were significantly lower than those in the non-anemic group. (2)Univariate analysis showed that：①Peritoneal dialysis age and sex did not significantly differ (P>0.05). ②There were no siginificant differences in BMI, systolic pressure or diastolic pressure between the anemia group and the non-anemic group (P> 0.05).③Serum parathyroid hormone(PTH) levels in the anemia group were higher than that in the non-anemic group［(605±582) vs（386±434）pg·mL-1］，PTH levels between 500 and 1 000 pg·mL-1 was associated with higher incidence of anemia than PTH levels below 500 pg·mL-1. Residual renal Kt/V in the anemia group was lower than that in the non-anemic group［（0.35±0.42 ）vs (0.62±0.63),P＜0.05］, incidence of left ventricular hypertrophy was higher than that in the non-anemic group（62.5% vs 35.1%，χ2=5.758, P=0.016）. In the anemia group,serum albumin, total Kt/V, total CrCL and residual renal CrCL were lower than those in the non-anemic group（P＞0.05），blood urea nitrogen and serum creatinine were higher than those in the non-anemic group（P＞0.05），the differences were not statistically significant. ④There was no siginificant difference in prescibed ACEI between the anemia group and the non-anemic group (P> 0.05). ⑤In the anemia group ,elemental iron dose and EPO dose were lower than those in the non-anemic group［(3.5±1.9) vs (4.2±1.2) mg·kg-1·d-1，（134±66） vs (170±62) U·kg-1 per week, P＜0.05］. ⑥The incidence of anemia in patients with infection was higher than patients without infection（35.1% vs 19.0%, P＜0.05). (3) Multivariate logistic regression analysis showed that serum iPTH levels, residual renal Kt/V and infection were associated with anemia in chronic PD children ( P<0.05).Conclusion ①In peritoneal dialysis children, the serum iPTH levels, residual renal Kt/V and infection were associated with anemia. ②Erythropoietin and iron to individualized treatment should be emphasized, in the high levels of serum iPTH, infection, and other circumstances, it may require higher doses of erythropoietin and iron improve anemia;③ It is important to pay more attention to the early treatment of renal anemia and management of CKD children.
Objective Analyzing the symptoms and signs to improve capability of discerning critical condition in EV71 infection commitment.Methods Massive literatures of clinical manifestations of severe cases of EV71 infection were reviewed, then the occur-time of symptoms and signs was recorded in hours from onset-symptoms, as fever, when clinical diagnosis of severe cases of EV71 infection was made. The first onset-time of symptoms and signs was recorded in severe EV71 infection cases, and at 6, 12 and 24 before death, respectively. The clinical symptoms and signs were recorded and their neural localization was analyzed.Results A total of 54 death cases (31 males and 23 females) from 5 participating hospitals from January 1, 2010 to December 31, 2012 were observed. 53 cases were 5 years old. The median time from disease onset to death was 78.5 (6-432) h, 43 of which (79.6%) died within 120 h. Four general indicators were recorded (fever, rash, herpangina and flu-like symptoms), more than 89 indicators were recorded except seven unobserved. In addition to general indicators, 8 symptoms and signs were observed within 36 h, mainly as the specific indicators (1 item), movement disorders indicators (6 items) and autonomic dysfunction index (1 item); 25 signs and symptoms increased in 36-48 h, including movement disorders index (16 items), unconsciousness index (1 item) and autonomic dysfunction index (8 items). 15 signs and symptoms appeared in 49-60 h, including movement disorders index (11 items), unconsciousness index (1 item) and autonomic dysfunction index (3 items). Ultrahyperpyrexia, persistent shock, deep coma, excessive tachycardia and sustained ultra-hypertension, neuropathic positioned mainly in the medulla and part of the hypothalamus appeared at 6 h before death, eyeball ataxia, respiratory ataxia, pharyngeal reflex decreased/disappear, NPE, respiratory rhythm abnormalities and hypertension, located in the sympathetic nervous system, the brain and the medulla appeared in 12 h before death, and anxious, tired somnolence, limb shaking, startle, vomiting, rash and fever appeared in 24 h before death.Conclusion Clinical manifestations in death cases with EV71 infection have neurological positioning, timely discerning warning signs and symptoms before death can reduce mortality of EV71 infection.
Objective To describe the trends of growth level ,obesity and obesity related chronic diseases among school-age children in Beijing from 1985 to 2013, and analyze the prevalence of obesity related chronic diseases.Methods Using the data from the six investigations of Physical Fitness and Health Surveillance of Chinese School Students (1985, 1991, 1995, 2000, 2005, 2010) and four studies on school-age children led by Department of Epidemiology, Capital Institute of Pediatrics in Beijing (2004, 2007, 2010, 2013), the trend of growth among school-age children was charted, and the change of children's physique and obesity with the development of economy and growth was observed.Results Since 1985 to 2010, with the increase of GDP per capital, height, and chest circumference of school-age children kept a steady increase. Especially among 12-years old male children, heights (cm) of urban and suburban children increased from 150.2 and 144.7 to 159.6 and 156.1, respectively. The annual average increasing speed was 0.24% and 0.30%. Chest circumferences (cm) of urban and suburban children increased from 69.6 and 69.0 to 81.1 and 78.1, respectively. The annual average increasing speed was 0.62% and 0.49%. During 2005 and 2010, the growth of weight and BMI among children in urban areas changed into declining.The prevalence of obesity among suburban school-age children still showed an upward trend. Especially among 12-years old male children, BMI (kg·m-2) of urban and suburban children decreased from 19.6 and 18.1 to 21.6 and 20.6, respectively. The annual average decreased speed was 1.62% and 2.23%.The physical fitness of suburban children have been declining, however, the physical fitness of urban children began to be improved, compared with 2005.Conclusion With the rapid development of economy, the growth level of school-age children shows an upward trend. However, suburban children are facing continuous physical decline not as we expected. Obesity and obesity related chronic diseases are in high prevalence, simultaneously and more attention should be paid to the prevention of obesity .
Objective To conduct a systematic review to identify the diagnostic value of procalcitonin（PCT） for detecting serious bacterial infections in children having different thermal process with fever without source (FWS).Methods A comprehensive electronic search was performed to retrieve relevant studies on PCT in diagnosis of serious bacterial infections in children with FWS. From the initiation of the database from establishment to July 2014, QUADAS items were used to evaluate the quality of included studies. Pooled sensitivity, specificity, positive likelihood ratio, negative likelihood ratio, summary receiver operating characteristic curve (SROC), and the heterogeneity of included studies with different thermal process were analyzed by using Meta-Disk software. Finally, the sensitivity and analysis for heterogeneity cause were performed and Stata 12.0 software was used to assess the publication bias with funnel plot.Results Eleven studies were included for the review, including 10 English studies and 1 Chinese study. The pooled sensitivity and specificity of PCT test for thermal process under 24 h were 0.75(95%CI: 0.69-0.80) and 0.80(95%CI: 0.77-0.83), SROC area under the curve (AUC) was 0.870(95%CI: 0.817-0.923); the pooled sensitivity and specificity of WBC were 0.48(95%CI: 0.41-0.55) and 0.54(95%CI: 0.51-0.58), AUC was 0.484(95%CI: 0.440-0.663); sensitivity and specificity of ANC were 0.30(95%CI: 0.21-0.40) and 0.78(95%CI: 0.73-0.83), respectively. The pooled sensitivity and specificity of PCT test for thermal process between 24 and 48 h were 0.86(95%CI: 0.79-0.91) and 0.63(95%CI: 0.60-0.67), AUC was 0.857(95%CI: 0.761-0.953); the pooled sensitivity and specificity of WBC were 0.54(95%CI: 0.44-0.65) and 0.46(95%CI: 0.41-0.51), AUC 0.558(95%CI: 0.479-0.636); sensitivity and specificity of ANC were 0.47(95%CI: 0.28-0.66) and 0.12(95%CI: 0.08-0.17), respectively. The pooled sensitivity and specificity of PCT test for thermal process over 48 h were 0.83(95%CI: 0.75-0.90) and 0.55(95%CI: 0.50-0.59), AUC was 0.816(95%CI: 0.596-0.996). The sensitivity of WBC in 2 studies were 0.69(95%CI: 0.41-0.89) and 0.34(95%CI: 0.28-0.41), respectively; the specificity of WBC in 2 studies was 0.81(95%CI: 0.69-0.91) and 0.29(95%CI: 0.24-0.35), respectively; sensitivity and specificity of ANC were 0.87(95%CI: 0.75-0.95), 0.40(95%CI: 0.34-0.46).Conclusion When children having different thermal process with FWS, PCT provides a good specificity in the thermal process under 24 h, and provides a good sensitivity in the thermal process between 24 and 48 h. Since the heterogeneity among studies, it should be applied in combination with clinical features and other tests.
Objective To explore changes of postnatal gut microbiota in preterm infants in NICU and its association with neonatal sepsis.Methods Preterm infants hospitalized in NICU of Children's Hospital of Fudan University were enrolled in the study. Fecal samples were collected at day 1, 7, 14 and 21 after birth, respectively. Illumina high-throughput sequencing techno-logy was used to sequence 16S rRNA-V3 hypervariable region of all microbes in 7 fecal samples of 3 recruited subjects. MG-RAST V3.3.6 was used to analyze and calculate the numbers of sequences and operational taxonomic units （OTUs） for each sample, then the species abundance and distribution were analyzed and followed by cluster analysis.Results Seven samples had been analyzed. Three subjects had an average gestational age of (31.3±0.8) weeks and an average birth weight of (1 540±144) g. They all received antibiotic administration after birth. Maternal antibiotic exposure occured in 1 case. Another one case developed SIRS during hospitalization. ①The rarefaction curves showed that adequate sequencing depth was achieved. Analysis of species abundance showed that the OTUs number ranged from 381 to 608 indicating high microbial diversity. Meanwhile, the microbial diversity had a positive correlation with the postnatal age. ②Eighteen phyla were detected from all samples. Actinobacteria, Bacteriodetes, Firmicutes and Proteobacteria were predominant in all samples. However, the dominant phylum of case 1 belonged to Proteobacteria, with a percentage of 97.5％(day 14) and 49.1％(day 21) , while Actinobacteria predominated in day-7 sample of case 2, with a percentage of 99.5％. ③172 families were detected altogether, 63 of them were detected in all samples. Ten families were in higher relative abundance. In case 2, Corynebacteria occupied 97.9％ in day-7 sample, and Staphylococcaceae occupied a higher proportion in day-21 sample(27.2％) than the other two samples. ④The cluster analysis showed high similarity of intestinal microflora in different time points of one subject.Conclusion Gut microbial colonization and development in preterm infants in NICU were altered by various factors. Prolonged broad-spectrum antibiotics at prenatal and early postnatal age might profoundly decrease microbial diversity and affect microbial colonisation. Microbiota was less diverse from birth in infants who developed sepsis. There may be a microbiome predominanted of pathogens that may be associated with sepsis in preterm infants.
Objective To explore the clinical features, diagnosis and treatment of pulmonary artery sling in infants.Methods From September 2009 to July 2014, patients who were diagnosed as pulmonary artery sling at Children's Hospital of Chongqing Medical University were recruited and analyzed. The clinical data, imaging examination (X-ray, echocardiography and multi-detector computed tomography scan), bronchoscopy, treatment and surgery were retrospectively reviewed.Results Twenty-nine cases were diagnosed as pulmonary artery sling, including sixteen males and thirteen females with the age at diagnosis ranging from two to forty-two months（median age was seven months）. Twenty-six cases manifested with recurrent wheezing and stridor. The chest auscultation of twenty-six patients had wheeze sound. Fifteen cases of children were with simple pulmonary artery sling and the diagnosis of pulmonary artery sling median age was 6 months, the other fourteen cases were accompanied by other cardiovascular diseases, with median age of 8 months with no statistical difference between the two groups（P> 0.05）. Twenty-six patients underwent multi-detector CT vascular and airway reconstruction which indentified the diagnosis of pulmonary artery sling and the combinating malformation of heart, another three patients only underwent multi-detector CT airway reconstruction. The CT results showed tracheal stenosis in all patients and found seven bridging bronchus. Twenty-five patients received echocardiography, 3 of them were performed before CT scan, but only five patients were indentified with pulmonary artery sling. Thirteen patients received bronchoscopy and were found with tracheal stenosis, six of which were combined with cartilaginous rings. Sixteen patients underwent surgery, but four of them died. After operation, the respiratory symptom of twelve patients was improved. The duration of mechanical ventilation >72 hours, operation age ≤ 5 months or cardiopulmonary bypass time >100 minutes significantly influenced mortality (P<0.05).Conclusion The infants who had respiratory symptoms such as recurrent wheezing, stridor and respiratory infection with poor effective medical treatment should be evaluated by multi-detector CT to identify pulmonary artery sling and airway abnormalities. Combined with cardiovascular abnormality could help early detection of pulmonary artery sling. Patients with respiratory symptom should be treated with surgery to improve tracheal stenosis as early as possible, and a significant portion of patients may not need tracheal surgery. The duration of mechanical ventilation >72 hours, operation age≤ 5 months or cardiopulmonary bypass time >100 minutes were significantly associated with mortality.
Objective To evaluate the safety and efficacy of transcatheter closure of post-surgery residual ventricular septal defect (VSD) and its mid- to long-term follow-up results.Methods The clinical data of patients with post-surgery residual VSD who underwent an attempt of percutaneous device closure from September 2002 to December 2013 in Children's Hospital of Fudan University were summarized. Results 21 patients (11 males and 10 females) with post-surgery residual VSD underwent an attempt of percutaneous device closure in our center. The mean age was 8.8 years (2.7-21 years) and the mean weight was 26.3 kg (10.5-53.5 kg). Among these 21 patients, their previous surgery procedures were VSD repair in 13 cases, repair of tetralogy of Fallot (TOF) in 5 cases, repair of double outlet right ventricle (DORV) in 2 cases, and repair of complete transposition of great arteries (TGA) with VSD in 1 case. In addition, 1 patient of post-TOF repair was associated with frequently premature ventricular contraction and ventricular tachycardia, 2 patients of post VSD repair were associated with recoarctation of the aorta. The duration between previous surgery and transcathter device closure was 11-80 months. The mean diameter of VSDs in right ventricle (RV) side was 3.73 mm (1.6-6.3 mm) by angiogram. There were multiple shunts in RV side in 4 patients, and one patient was left ventricle to right atrium shunt. The QP/Qs ratio was 1.53±0.23, and there was no pulmonary hypertension more than moderate. Amplatzer devices were successfully deployed in all cases except for 1 failure case due to a repeat complete block during the arterial-venous loop establishing, and 2 devices were used in 1 patient. The median device size was 6.5 mm (4-12 mm). A radio-frequency ablation and 2 balloon dilatations were successfully finished for the associated defects in the same procedure. There was no procedure-related adverse events. Mild residual shunt was showed by angiogram and transthoracic echocardiography (TTE) immediate after closure in 3 patients. Three patients with moderate to severe tricuspid regurgitation before device closure improved significantly after closure. All patients were doing well clinically in a mean 22.2 months (1- 79 months) follow up. Minor arrhythmia was observed in 2 patients in the first year follow-up ECG including incomplete right bundle branch and first-degree atrioventricular block, and remained the same in another 6 months to 1 year follow-up. All 3 residual shunts disappeared in the follow-up echocardiography except for a 3.4 mm residual shunt still existed in 1 patient with normal heart size and function. No new onset tricuspid or aortic regurgitation was showed. There was no device malposition or embolization, or thrombosis formation.Conclusion Transcatheter closure of post-surgery residual ventricular septal defect is safe, effective and less trauma. The mid- to long-term follow-up results of these cases are promising.
Objective To explore the effect of interleukin-24(IL-24) gene transfer on glomerular mesangial cells(GMCs) apoptosis and to find out the effect of IL-24 on cell cycle regulatory protein p21,p27 and CyclinE of GMCs induced by LPS.Methods 293 cells were cultured in 10%FBS/DMEM and Ad.IL-24 and Ad.GFP were amplifycated in 293 cells. GMCs were analysed after 4 to 6 generations. ①They were divided into four groups: control group, Ad.IL-24 group, LPS group and LPS+Ad.IL-24 group.And control group and LPS group weren't infected with Ad.IL-24, Ad.IL-24 group and LPS+Ad.IL-24 group GMCs were infected with Ad.IL-24,then LPS+Ad.IL-24 group GMCs were cultured in 5%FBS/DMEM with LPS(10 mg·L-1). The apoptosis of the GMCs was examined by AnnexinV/FITC flow cytometry; ②The effect of IL-24 on cell cycle regulatory protein p21, p27 and CyclinE of GMCs induced by LPS were determined. They were divided into three groups:control group, Ad-GFP group and IL-24 group. Control group GMCs were cultured in 5%FBS/DMEM. Ad-GFP group GMCs were infected with Ad.GFP and then cultured in 5%FBS/DMEM with LPS(10 mg·L-1). GMCs were infected with Ad.IL-24. The expressions of cell cycle regulatory protein p21, p27 and cyclinE were examined by Western-blotting.Results The GMCs were cultured for 24 hours and 48 hours. The apoptosis rate was (0.86±0.15)% and (0.98±0.4)% in the control group, (1.02±0.22)% and (1.43±0.31)% in the Ad. IL-24 group, (2.19±0.81)% and (2.49±0.12)% in the LPS group, (18.01±1.17)% and (26.82±5.01)% in LPS + Ad.IL-24 group. There was no difference between control group and Ad. IL-24 group, and the apoptosis rate of LPS group was higher than control group（P＜0.05). The apoptosis rate of LPS+Ad.IL-24 group was the highest while there was no change in Ad. IL-24 group（P＜0.05). ②The expressions of p21 and p27 were down-regulated while CyclinE expression was up-regulated in GMC by LPS（P<0.05）. Adenovirus mediated IL-24 gene transfer could abolish the role of LPS on regulation of p21, p27 and CyclinE expression（P<0.05）.Conclusion Adenovirus mediated exogenous IL-24 gene transfer could effectively inhibit LPS-induced proliferation of glomerular mesangial cells but had no effect on normal GMCs, and it could abolish the role of LPS on regulation of p21, p27 and CyclinE expression then increased the apoptosis of LPS-induced proliferation of GMCs.
Aorto-Left Ventricular Tunnel is an abnormal approach from asacend arota to left ventricular. Levy gave this name to the diasease in 1963. Aorto-Left Ventricular Tunnel is a rarely congenital heart disease, which incidency is 0.05-0.12% and combined with arota valve deformation, stenosis and/or insufficiency and otherwise. Now will report 1 case which patient suffered ALVT and analyze with literatures, discussing the value of echocardiography diagnosis on ALVT.
Objective To investigate the clinical and genetic characteristics of familial hypercholesterolemia, to improve the understanding of familial hypercholesterolemia in children. Methods The clinical features, laboratory examinations, imagings, and gene sequencing results of two cases with familial hypercholesterolemia were presented and analyzed; and the related literatures were reviewed. Results The first presented symptoms of both cases are skin xanthomata and tendon xanthoma, with significantly elevated TC and LDL-C increased significantly. Analysis of LDLR gene sequencing showed both of 2 cases have LDLR gene mutation; LDLR homozygous mutation was detected in 1 case, the other had LDLR heterozygous mutation, the homozygous child presented corneal arcus, calcification of aorta and coronary arterie. Conclusion The clinical features in children with familial hypercholesterolemia include skin xanthomata and tendon xanthoma, corneal arcus, vascular lesions, elevated TC and LDL-C. Gene sequencing can find the causative mutation and confirm diagnosis. Treatment targets are extremely difficult to achieve with cholesterol-lowering drugs in homozygous FH, liver transplantation should be considered when necessary.
