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目的探讨小儿血管迷走性晕厥(VVS)的诊断及防治方法。方法分析1999-10—2004-07上海交通大学附属儿童医院开展斜板试验(TT)(倾斜角度80°,试验时间持续30min)的状况,对不明原因晕厥组74例及正常对照组54例进行前瞻性研究,对TT阳性组加做阳转阴试验,予以饮水试验,并随访。结果基础TT在不明原因晕厥组中阳性41例,阳性率为55.4%,对照组为0(P<0.001)。阳性组表现血管抑制型21例,心脏抑制型7例,混合型13例;28/41(68.3%)例患儿经饮水后阳转阴;随访38例TT阳性患儿,总有效率86.8%。结论TT对诊断小儿血管迷走性晕厥有较高的敏感性(55.4%)和特异性(100%)。阳转阴试验为防治小儿血管迷走性晕厥提供了理论和实验依据,饮水试验是一种简单有效可行的防治措施。     

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目的评价胶囊内镜检查在儿科应用的有效性和安全性。方法对2007年9月至2010年12月福建医科大学附属漳州市医院28例临床疑诊小肠疾病的患儿进行胶囊内镜检查。观察胶囊内镜通过胃的平均时间、通过小肠的平均时间、成功率和失败率、小肠病变检出率及患儿的耐受性和并发症。结果 28例患儿均成功吞服胶囊内镜并完整排出,检查过程患儿耐受良好。胶囊内镜通过胃的时间20～280min(中位数64min),通过小肠的时间93～380min(中位数255min),检出病变20例(20/28,71.43%)。结论对疑诊小肠疾病,胶囊内镜检查是的一种有效和安全的方法。     

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(上接本刊2006年第5期400页)2神经上皮性肿瘤广义的神经上皮性肿瘤(neuroepithelial tumors)是指所有起源于神经上皮细胞的一大类肿瘤;而狭义上特指过去通称为胶质瘤(glioma)的一类肿瘤,包括星形细胞瘤、室管膜瘤和髓母细胞瘤等,是最常见的原发性脑肿瘤,约占全部颅脑肿瘤的40%~50%。在影像学检查中,平片与造影诊断价值有限,主要靠CT和MRI。2.1星形细胞瘤星形细胞瘤(astrocytoma)为神经上皮性肿瘤中最常见的一类肿瘤。占儿童颅内肿瘤的35%,男女发病比例为1·89∶1。儿童肿瘤的发生部位与成人不同,以幕下多见。星形细胞瘤分类较复杂,1993年…     

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??Abstract??Objective??To study the characteristics of mutation of TSC1 gene exan 15 in tuberous sclerosis complex. Methods??Totally 21 children with confirmed clinical manifestations of TSC and 38 parents of the children coming from 21 TSC families were included in the study. In total?? we studied 6 familial cases and 15 sporadic cases. The mutation of exon 15 in TSC1 gene was identified by denaturing high performance liquid chromatography ??DHPLC?? and further confirmed by direct sequencing. Results??After being confirmed by DNA direct sequencing?? mutations were identified in 4/21??19%??patients?? in which there were c.1708~1709delAG??p.Arg570GlyfsX17?? and c.1888~1891delAAAG??p.Lys630GlnfsX22?? two small deletion mutations and one c.1460C > G??p.Ser487Cys?? missense mutation. c.1460C > G??p.Ser487Cys?? mutation was reported the second. One family case and three sporadic cases were found. In our study?? the mutation frequency of exon 15 in TSC1 gene was 4/21??19%???? which was higher than other reports. The main clinical characters of the patients with mutation on exon 15 in TSC1 gene were brain and skin impair. We also found that the patients with the same mutation c.1888~1891delAAAG??p.Lys630GlnfsX22?? had different phenotype?? but the patients with different mutations c.1708~1709delAG??p.Arg570GlyfsX17?? and c.1888~1891delAAAG ??p.Lys630GlnfsX22?? nearly had the same phenotype. Conclusion??Totally three TSC1 gene mutations that have never been reported in China are identified.     

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患儿男,3岁,因发热5d伴发绀、气促半天,呕吐、抽1次于2004年7月27日入院。8h前无明显诱因出现呕1次,为胃内容物,非喷射状。排黄色稀水样便1次,量多未见黏液脓血,随即全身发绀,呼吸急促,在当地医院对治疗后无好转。1h前出现抽搐1次,使用鲁米那后10min全身抽搐缓解,但仍有四肢小抽动,神志不清,口白沫转入我院。2岁时曾因发热12d,嗜睡8d在外院诊病毒性脑炎、败血症,行脑脊液生化检查2次,血糖均降低分別为2.71mmol/L和2.61mmol/L。血浆白蛋白30.0L,丙氨酸转氨酶44.00IU/L,α羟丁酸脱氢酶356.00IU患儿生后逐渐出现精神运动发育迟缓,1岁10个月…     

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??Abstract?? Objective To investigate the occurrance of DKA in established T1DM children. Methods According to the registration system in the following-hospitals??Beijing Children’s Hospital of Capital Medical University?? Children’s Hospital of Shanghai?? Nanjing Children’s Hospital??Children’s Hospital of Zhengzhou?? Children’s Hospital of Jiangxi?? the First Affiliated Hospital of Xi’an Jiaotong University??First Affiliated Hospital of Kunming Medical University?? Children’s Hospital of Wuhan?? SooChow University Affiliated Children’s Hospital?? Children’s Hospital of Liaocheng?? Children’s Hospital of Fuzhou?? Chengdu Women & Children’s Central Hospital???? we investigated the frequency and cause of DKA in children with established T1DM from December 1995 to June 2014. After the diagnosis of T1DM?? the first time DKA was for group 1A?? the second DKA for group 1B. We conducted a cross-sectional survey of blood glucose control status for patients with T1DM from December 2011 to May 2012 in Beijing Children’s Hospital. Patients who did not have DKA episode in the course of T1DM were selected as control group ??group 2??. Results Totally 1676 children were newly diagnosed with T1DM by 12 hospitals?? and 89 patients occurred 100 DKA after T1DM diagnosed. The incidence and frequency of DKA was 5.3% ??89/1676?? and 5.9% ??100/1676??. The frequency was different in 12 hospitals?? fluctuating between 1.1% and 24.1%. Compared with group 2?? group 1A had high level of HbA1c ???11.31±3.03??% vs. ??8.26±1.53??%?? P??0.01?? and insulin dosage ???0.85±0.42?? IU vs. ??0.71±0.31?? IU?? P??0.01??. There were more patients with insulin bump in group 1A than group2 ??25.0% vs. 11.2%?? P??0.01???? and few patients reached the standard of blood glucose monitoring ??12.1% vs.40.1%?? P??0.01?? and follow-up ??21.2% vs. 46.6%?? P??0.01??. The main reasons of DKA in group 1A were infection ??33.7%???? interrupting insulin therapy ??21.3%?? and eating disorder ??20.2%???? one patient had DKA after islet stem cell transplantation. Infection was also the major cause of DKA in group 1B ??4/10???? and 1 patient had DKA because of insulin bump failure. For DKA which occurred within different course?? the distribution of causes was different ??P??0.01??. Within 1 year of T1DM duration?? the major reason was interrupting insulin injection ??39.3%??. For patients more than 1 year?? it only accounted for 13.1%??8/61???? the major causes were infection ??22/61?? and eating disorder ??16/61??. The major cause in mutiple hospitals with high DKA frequency was infection ??50.0%???? while in other hospitals 28.1% of patients had DKA because of infection ??P??0.01??. Conclusion The frequency of DKA is 5.3%?? which is different in 12 hospitals?? with the highest up to 24.1%. Patients with DKA have poor glycemic control?? and they can not regularly monitor blood glucose and follow-up. We should emphasize the education of diabetes. Patients with insulin pump and islet stem cell transplantation must also become a new focus of education. Hospitals with high DKA frequency should give patients information how to deal with other diseases.     

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??Abstracts?? Objective To study the epidemiology of asthmatic children from 0 to 14 years old in the city of Xiamen in a decade?? in order to find a scientific basis for future children’s asthma prevention and treatment. Methods Secondary children’s asthma epidemiology study roster?? preliminary screening and questionnaires were provided by Centre of Asthma Prevention and Education of Chinese Capital Institute of Pediatrics. All the asthma specialists who participated in this study were trained with the same content; we used cluster sampling methods?? and nearly 10??000 children were involved in this secondary investigation; 95% of children in the study roster were involved in our investigations. Quality controls were set during the present investigations. All the data were duplex entered into the software of EPI-Info3.5.1 ??America CDC??. Rate?? mean and Chi square statistics were used as statistic methods. Results The actual surveys of children included 11845 and 11508 respectively?? and the rate of lost of follow-up was 0.53% and 0.9% respectively in the year of 2000 and 2010. Average of the morbidity of asthma was 3.96% and 3.03% in the year of 2000 and 2010 respectively??P??0.05??. The rate of misdiagnosis was 35.56% and 35.5% in the year of 2000 and 2010 respectively?? ??P??0.05??.Two thirds of the children had their first episode within 3 years old. About 75.6% and 71.7% suffered from asthma within one year in the year of 2000 and 2010 respectively. Hospitalization rate of asthma was 45.78% and 34.5% in the year of 2000 and 2010 respectively. About 51.28% and 59.5% children with asthma suffered from allergic rhinitis?? and the percentage of using antibiotics was 100% and 83.4% in the year of 2000 and 2010 respectively?? ??P??0.01??. Conclusions The morbidity of asthma in children aged 0~14 years old decreased in a decade in Xiamen city?? but the misdiagnosis rate is still near one thirds; more than half of the asthmatic children have allergic rhinitis at the same time. The morbidity?? hospitalization rate and the rate of using antibiotics have decreased in this decade. We also need to pay more attention to the training of the pediatricians who don’t specialize in asthma in the battle of asthma prevention.     

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??Objective??To explore the preventive effect of regular doses of captopril on the pulmonary arterial hypertension associated with ventricular septal defect??VSD?? and its mechanism. Methods??Forty cases of children with VSD in accordance with the inclusive criteria??who were hospitalized in Chengdu Women and Children’s Central Hospital from July 2013 to July 2015 ??were collected and randomly divided into blank control group??n??20??and captopril intervention group??n??20??. Plasma MMP-9 and TIMP-1 were examined by using ELISA??and PASP??the Qp/Qs and right ventricular Tei index were measured by cardiac color Doppler ultrasound at 0 week??1 week??4 weeks??8 weeks??and 12 weeks respectively??side effects in captopril intervention group were also observed. Results??PASP??the Qp/Qs??right ventricular Tei index??plasma MMP-9 and TIMP-1 level in blank control group were increased over time??the same indexes in captopril intervention group were decreased??the changing trend of two groups had statistically significant difference??all P??0.05??. The indexes were of no difference between the two groups at 0 week??all P??0.05????PASP and right ventricular Tei index in captopril intervention group were lower than blank control group at 1 week??4 weeks??8 weeks??and 12 weeks??all P??0.05????the Qp/Qs plasma??MMP - 9 and TIMP - 1 level in captopril intervention group were lower at 4 weeks??8 weeks??and 12 weeks??all P??0.05??. No side effects were found in captopril intervention group during follow-up. Conclusion??Oral regular doses of captopril in the VSD children undergoing elective surgery may reduce pulmonary vascular remodeling and prevent pulmonary hypertension in order to achieve the best age at surgery??one of the mechanisms might be improving indexes of PASP??right ventricular Tei index and the Qp/Qs by reducing the plasma MMP 9 and TIMP - 1 level.     

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??Objective??To evaluate sleep habits and related affecting factors in children with epilepsy aged from 7 to 14 years old. Methods??By asking parents and guardians?? the children's sleep habit questionnaire was used to evaluate sleep habits of 162 children with epilepsy and 162 normal controls. Results??The mean score on the sleep questionnaire for children with epilepsy was 32.4±3.6 ??P < 0.01???? whereas for the normal control group it was 58.8±3.3 ??P < 0.01??. In children with epilepsy?? neuropsychomotor developmental delay had higher scores??49.9±7.3?? than that of appropriate development ??43.5±8.1??P < 0.01??. The group under polytherapy ??54.3±5.7?? had a higher score than that under monotherapy??42.9±6.3??. In addition?? the mean score for patients with daytime seizures was 44.1±7.6??and for those with nocturnal seizures it was 52.3±5.7 ??P < 0.01??. The mean score for children with partial seizures was 41.6±4.9??and for those with generalized seizures it was 47.4±8.7 ??P < 0.01??. Conclusion??Children with epilepsy are prone to have sleep disorders. Many factors?? such as neuropsychomotor development??seizure type??seizure frequency and therapy of seizures?? can affect their sleep habits.     

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??Objective??To study the gender and age distribution of bone metabolic markers in healthy children and adolescents??5-14 years old??. Methods??Detailed clinical documents were collected??and morning fasting 5 mL venous blood samples were drawn from 273 cases of the healthy volunteers and healthy school children. Automatic electrochemiluminescence immunoassay analyzer was applied for the tests of bone formation markers??Procollagen type 1 nitrogenous propeptides??P1NP????Osteocalcin??OC???? and bone resorption marker??β-Cross-linked C-terminal telopeptide of type 1 collagen??β-CTx????. The bone metabolic markers were used to calculate percentile values for the 5th?? 10th?? 25th?? 50th?? 75th?? 90th?? and 95th percentiles in the 3 age groups respectively. Results??The variation trends of bone formation markers??P1NP??OC?? and bone resorption marker ??β-CTx?? in two gender groups were similar??being relatively low in preschool age and school age period??gradually increasing during preadolescence??reaching peak in adolescence and declining rapidly after adolescence. There were no significant differences in bone formation markers P1NP??P??0.404?? and OC ??P??0.766?? between male and female groups??but there was significant difference in bone resorption marker β-CTx??P??0.01??. Conclusion??There are no statistically significant differences in the serum levels of P1NP or OC between male and female groups in the bone formation markers of healthy children aged 5-14 years. There is significant difference in bone resorption marker β-CTx between the two groups??the level of the boys being higher than the girls. According to the age distribution of the reference values?? the increasing trend of bone metabolic parameters of the female group will appear 1 or 2 years earlier??the peak value being lower.     

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??Objective??To explore the correlation between FeNO levels and airway reversibility and its clinical significance in assessment of children with asthma. Methods??A total of 161 children at 5 to14 years old with asthma admitted to pediatric respiratory outpatient of Shengjing Hospital Affiliated to China Medical University from November 2014 to November 2015 were divided into allergic group and non-allergic group according to the allergic condition. FeNO and bronchial dilatation tests were made in the two groups. The correlation between FeNO levels and improvement rate after bronchodilator in two groups was analyzed. Results????1??FeNO level in allergy group was obviously higher than that in non-allergic group??P??0.002??. ??2?? FeNO level of children in allergic group was positively correlated with improvent of bronchial improvement??P??0.05????and negatively correlated with FEV1%?? FEV1/FVC%?? FEF50%?? FEF25% and FEF75/25% of basic lung function??P??0.05????but was irrelevant to FVC%?? PEF% and FEF75% of basic lung function??P??0.05??.??3??FeNO level of children in non-allergic group was irrelevant to improvement rates of bronchial dilation and basic lung function??P??0.05??. Conclusion??For asthmatic children with allergic constitution??FeNO level is positively correlated with airway reversibility. It may be a good noninvasive predictor for evaluating asthma and airway reversibility in children with asthma. While for children without allergic constitution??FeNO level cannot indicate the airway reversibility effectively.     

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目的 了解2004年北京市0～6岁残疾抽样调查诊断的270例智力低下患儿的智力发育特点及转归.方法 2007年6～11月北京市残疾人康复服务指导中心和北京妇幼保健院采用Gesell发育诊断法、韦氏儿童智力量表、婴儿初中生活能力量表、线索调查通过现场或入户调查,获得237例儿童的资料.采用SPSS 10.0统计软件分析数据.结果 124例(52.32%)诊断正常;113例(47.68%)诊断智力低下,其中轻度46例(40.71%)、中度32例(28.32%)、重度16例(14.16%)、极重度19例(16.81%).轻度以转归正常为多,中度转为轻度及正常者多于加重者,重度以程度加重为主.儿童好转影响因素有:2004年测查个人社会能区发育商(DQ)高、适应性能区D9高、接受过教育、具有早产因素的儿童、父母认为儿童将来生存状况较好、认为孩子发育属于正常.结论 "发育迟缓"诊断较"智力低下"更符合发育量表诊断的儿童;对北京市0～6岁轻、中度智力低下儿童发育转归研究宜采用定期监测方式.     

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目的探讨干/髓系表面抗原标记对儿童T-急性淋巴细胞白血病的预后影响。方法回顾性分析2002-01-01至2008-12-31上海交通大学医学院附属儿童医学中心收诊的57例T细胞型急性淋巴细胞白血病(ALL)和伴有骨髓转移的T细胞性淋巴母细胞型非霍奇金淋巴瘤(NHL)患儿干/髓系表面抗原表达情况,将其分为T-ALL伴有髓系表达组(T/My+ALL)和无髓系表达组(T/My-ALL),分析其与预后的相关性。结果T/My+ALL组31例(54.6%)和T/My-ALL组26例(45.4%),两组细胞形态学、遗传学及临床特征差异均无统计学意义(P>0.05);近期疗效中,泼尼松诱导实验差异无统计学意义(P>0.05),19d骨髓象及第1疗程完全缓解率T/My+ALL组均较T/My-ALL组低,差异有统计学意义(P值分别为0.002和0.006);T/My+ALL组和T/My-ALL组的初治完全缓解率分别为90.3%和96.2%,5年无事件生存率分别为35.4%和65.7%。虽结果提示T/My+ALL组预后较T/My-ALL组差,但差异无统计学意义(P=0.209)。结论T-ALL/NHL伴有干/髓系表面抗原表达时预后...     

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??Abstracts?? Objective To explore the prevalence and risk factors of asthma in children aged 0??14 years old in Changle rural area?? providing data for prevention and treatment of childhood asthma in rural area. Methods Multi-stage?? stratified and random cluster sampling was used to recruit children. The same screening questionnaires for the national epidemiological survey of children’s asthma were distributed among parents of children aged 0??14 years old at schools??kindergartens and communities. Asthmatic children were picked among the screening-positive children based on on-the-spot inquiries??physical examinations??medical records and supporting test results??Further survey of asthmatics was carried out to investigate the diagnosis and treatment status of childhood asthma and other associated allergic diseases. Results Among a total of 6000 questionnaires?? 5860 were completed with a response rate of 96.67%; and 135 ??2.3%??children were recognized as asthma??typical asthma??n=119??2.03%???? cough variant asthma??n=13??0.22%?? and suspected asthma??n=3??0.05%????. The prevalence of asthma was higher in boys??n=81??2.51%?? than in girls??n=38?? 1.44%?? ??χ2=8.27??P??0.05??. Conclusions The prevalence of asthma is 2.03% in children under 14 years old in Changle rural area and it varies in children with different genders and ages??Taking the age of onset into account?? the asthma prevalence was lowest in the children aged 0??1 years old??0.37%??2/533???? and highest??5.32%??20/376????in those aged 3??4 years old??The main cause of asthma in children is upper respiratory tract infection?? and the important risks include individual allergic history?? family history and genders.     

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??Abstract??Objective??To determine the appropriate perioperative management of children with obstructive sleep apnea-hypopnea syndrome accompanied by pulmonary hypertension. Methods??Two children with obstructive sleep apnea-hypopnea syndrome accompanied by pulmonary hypertension were admitted to our department from April 2006 to May 2009?? their cases were analyzed and relevant literature was reviewed. Conventional adenotonsillectomy was performed for one child before controlling pulmonary hypertension. The other child received preoperative continuous positive airway pressure ventilation?? then coblation-assisted adenotonsillectomy was performed. Results??The child who had received preoperative continuous positive airway pressure ventilation underwent an uneventful operation?? no intra-or postoperative complications were reported. In contrast?? the child who did not receive preoperative pulmonary hypertension control experienced severe hypoxemia recurrently after surgery?? leading to difficulty of extubation. The child remained in the intensive care unit for two days before being transferred to the general ward. The two children were followed up for more than three months and both showed improved sleeping with less snoring?? open mouth breathing?? and dyspnea. Preoperative apnea-hypopnea indices were 90.0 and 29.2??and postoperative apnea-hypopnea indices were 3.5 and 4.6 for the children. Lowest nocturnal oxygen saturation also improved after surgery for both children ??0.43 and 0.63 vs 0.95 and 0.92??. Conclusion??Pulmonary hypertension is a rare but severe complication of obstructive sleep apnea-hypopnea syndrome in children. It can result in severe consequences or even death if not appropriately managed. Surgery is the first-line treatment for children with this syndrome who have enlarged tonsils and adenoids?? however?? the risk is considerable when accompanied by pulmonary hypertension?? and incidence of complications is high. Thus?? perioperative management with continuous positive airway pressure ventilation to control pulmonary hypertension is critical.     

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目的 探讨HO-CO系统与小于胎龄儿(small for gestational age,SGA)发病的关系及相关机制,为SGA的病因学防治开辟新思路.方法 选择2004年11月至2005年11月间正常分娩的非匀称型SGA50例(其中母亲无妊高征为SGA1组30例,母亲患中重度妊高征为SGA2组20例),并以健康足月适于胎龄儿(appropriate for gestational age,AGA组)20例为对照.于新生儿娩出后立即采集脐动脉血标本并留取近胎盘处脐带标本,分别用双波长分光光度法测定新生儿脐血中血红素氧合酶-1(heme oxygenase-1,HO-1)的活性,用Chalmers血红蛋白结合及连二亚硫酸钠还原法测定脐血中碳氧血红蛋白(HbCO)百分含量,并通过免疫组化法观察新生儿脐血管上HO-1的表达.结果 (1)SGA1组及SGA2组脐血HO-1活性、HbCO百分含量均显著低于AGA组(P＜0.01).而SGA1组与SGA2组脐血HO-1活性、HbCO百分含量比较差异无统计学意义(P＞0.05).(2)AGA组、SGA1组及SGA2组脐血HO-1活性与新生儿出生体重均呈正相关(P＜0.05).(3)脐血管上HO-1的表达SGA1组和SGA2组与AGA组比较HO-1表达强度高,差异有统计学意义(P＜0.01);SGA1组与SGA2组之间比较差异无统计学意义(P＞0.05).结论 脐血HO-1活性与胎儿宫内发育及新生儿营养状况密切相关     

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??Abstract?? Objective??To explore the normal value of serum lipids and the correlation between atherogenic index and serum lipid in healthy children aged 2 to 14. Methods??The serum TG?? TC?? HDL-C and LDL-C were detected by Siemens 2400 automatic biochemical analyzer in 988 healthy children. The atherogenic index LDL-C/ HDL-C and ??TC??HDL-C??/HDL-C were calculated. Results????1??The diagnostic criteria of lipid disorder were as follows?? 2?? < 4-year-old?? TC > 4.02 mmol / L?? TG > 1.25 mmol / L?? HDL-C < 0.83 mmol / L?? LDL-C > 2.26 mmol / L?? 4?? < 7-year-old?? TC > 4.47mmol / L?? TG > 1.35mmol / L?? HDL-C < 0.90 mmol / L?? LDL-C > 2.55 mmol / L?? 7?? < 10-year-old?? TC > 4.78 mmol / L?? TG > 1.42 mmol / L?? HDL -C < 0.99 mmol / L?? LDL-C > 2.49 mmol / L?? 10?? < 13 -year-old?? TC > 4.71 mmol / L?? TG > 1.49 mmol/L?? HDL-C < 0.89 mmol / L?? LDL-C > 2.44 mmol / L?? 13??14 -year-old?? TC > 5.00 mmol / L?? TG > 1.48 mmol / L?? HDL-C < 0.95 mmol / L?? LDL-C > 2.96 mmol/L. ??2??The criteria of atherosclerosis index in children aged 2 ????4?? ?? 7 ?? ?? 10 ?? and 13 ?? 14-year-old were?? LDL-C/HDL-C > 2.38?? > 1.98?? > 1.95?? > 2.35 and > 2.28?? ??TC??HDL-C??/ HDL-C > 2.59?? > 2.55?? > 2.11?? > 2.46 and > 2.50?? respectively. ??3??The levels of serum TC?? TG?? HDL-C and LDL-C increased with age. ??4??The atherosclerosis index had significant positive correlation with TG and LDL-C. Conclusion??Criteria of serum lipids for children aged 2 to 14 years in Chongqing are established. The level of serum lipids is a dynamic process for children. The predictive value of atherosclerosis index should be noted in children with lipid disorder.