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??Objective??To analyze the clinical characteristics??pathological features and treatment responses of 6 pediatric patients with C3 glomerulonephritis??C3GN????in order to improve the understanding and treatment of this disease for pediatricians. Methods??Analyze the clinical manifestations??pathological features??therapies??prognosis of patients who were diagnosed with C3 glomerulonephritis from September??2010 to June??2016 retrospectively. Results??Clinical characteristics and laboratory examination??2 patients’ first symptom was hematuria??4 patients’ first symptom was hematuria and proteinuria??3 patients presented as acute nephritic syndrome??one presented as nephrotic syndrome. All the patients showed that the level of serum complement C3 was reduced??while sernm complement C4 was normal. Pathological character??6 patients showed strong positive complement C3 deposition under immunofluorescence. Lightmicroscopy showed mesangial proliferative glomerulonephritis in 5 cases??1 case was diagnosed as endocapillary proliferative glomerulonephritisin??and 3 patients presented electrondense depositionin under electron microscope. Treatment and prognosis??after conventional treatment??2 patients who were with crescent were treated with glucocorticoid .After a follow-up from 6 months to 42 months ??the prognosis was pretty good. Conclusion??Children with C3GN are usually presented with hematuria and ??or??proteinuria??characterized by strong positive C3 deposition. Lightmicroscopy always shows mesangial proliferative glomerulonephritis. Electron microscope show electron dense deposition??and short-term prognosis is pretty good.     

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目的了解北京市0~6岁儿童视力、听力、肢体、智力、精神残疾的现患率及其分布特征,重点考察智力低下儿童(残疾)的现患及分布特点。方法2003-07—2004-11北京市残疾人联合会、北京市卫生局联合组织采用概率比例、二阶段分层整群抽样的方法进行现况调查,使用丹佛发育筛查测验及Gesell发育诊断量表进行智力筛查和诊断,采用SPSS10·0软件对数据进行统计分析。结果北京市0~6岁儿童智力低下现患率为9·31‰,城市为7·60‰,农村10·37‰,男童(11·24‰)高于女童(7·25‰)(χ2=12·42,P<0·01),现患率有随年龄增长而增高的趋势(趋势χ2=23·58,df=6,P<0·01),智力低下严重程度构成分别为轻度65·54%、中度20·97%、重度5·99%、极重度7·49%。结论北京市儿童智力低下的防治应以轻度类型为主,重点在农村地区;北京市需结合世界发达国家的经验,建立自己的智力低下监测、防治体系。     

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总结本院1999-01—2004-12住院诊治的3例Tolo-sa-Hunt(THS)患儿病例资料,并结合文献进行分析,现报道如下。1临床资料3例THS中女1例,男2例。年龄分别为4岁、6岁和12岁,1例2年前被诊断为眼肌麻痹性偏头痛。3例既往均无心、肺、肝、肾疾病史。急性起病2例,亚急性起病1例。1例病前有“上感”、发热、伴眼睑肿胀、眼部红肿、流泪不适等症状,1例起病前1周患鼻炎,另外1例无前驱症状。首发症状为一侧头痛或(和)眼眶痛,一侧头痛者2例,一侧眼眶痛者1例。疼痛呈持续性,阵发性加剧,性质为“钻痛”、“咬痛”或撕裂样疼痛。伴有恶心、呕吐1例。头痛(…     

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目的评价极低出生体重6胞胎中存活的4例0~6岁以来的体格生长情况。方法监测1998-12-23在中国医科大学盛京医院出生的6胞胎中存活的4例小儿的体格生长情况并给予早期干预。结果6胞胎出生时身长、体重均低于同胎龄儿标准,为小于胎龄儿。存活的4例小儿体重于生后1.5岁、身高于生后3岁达正常同龄儿的均值。结论4例6胞胎极低出生体重儿在系统保健监测情况下,生后2~3年内可表现出良好的追赶生长趋势,体格生长各指标均在正常范围内。     

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患儿男,3岁,主因“左耳后突起伴发热2月余”于2004年7月30日入院。患儿于入院前2个月无明显诱因出现左耳后突起,外耳道流脓,伴发热,体温波动于38～39℃,故以“化脓性中耳炎伴骨膜下脓肿”于外院住院治疗。于2004年6月5日至7月12日期间共行4次耳部手术,包括:左乳突凿开术,左乳突凿开引流术,左乳突根治术及左颈内静脉结扎术,乙状窦探查术。期间于第2次手术后即6月24日头颅MRI提示脑内多发异常信号灶,考虑合并脑脓肿。住院期间先后给予青霉素、罗氏芬、马斯平等抗生素控制感染,体温始终未降,最高达40.5℃。患儿于外院作血培养及外耳分泌物培…     

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??Abstract??Objective??To analyze the relationship between blood pressure and BMI??waist circumference??WC?? and fat mass percentile??FMP?? among children and adolescents??7??17 years?? in Beijing. Methods??As part of the Beijing Children and Adolescent Metabolic Syndrome Study??BCAMS????data of weight?? height?? FMP??WC?? and blood pressure were collected from 19 488 subjects aged 7??17 years from April to October in 2004. Overweight and obesity were defined according to BMI??WC and FMP criteria. Analyze the relationship between blood pressure and BMI??WC and FMP??as well as the change and trend of hypertension. Results??The levels of SBP and DBP increased in the order of normal weight to overweight and obesity. With age and gender controlled??BMI??WC and FMP were independently positively correlated with SBP and DBP ??P < 0.001??. The incidence of hypertension was significantly higher in the BMI??WC and FMP overweight and obesity groups than in the normal group??and the difference had statistical significance. The relative risk of hypertension in the obesity group is 3 to 7 times that of the normal group??and the risk in BMI obesity/FMP obesity group and the BMI obesity/abdominal obesity group was 7.3 and 6.8 times that of the normal group respectively.Conclusion??BMI??WC and FMP are closely related to SBP and DBP in children and adolescents. BMI is a better index for hypertension??the heavier the childrn??the higher the risk of hypertension.     

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第9届亚洲小儿肾脏病会于2005年10月28日至30日在北京举行,会议由北京大学第一医院、中华医学会儿科分会肾脏病学组、中华医学会共同承办。会议期间还有国际小儿肾脏病学会(IPNA)理事会、中华医学会儿科分会儿肾学组第12届学术会议同时进行。故除亚洲各国儿肾工作者外,还有国际儿肾界的专家及我国儿肾界代表参加,计32个国家、地区代表共380人参加,我国就有26个省市代表参加。充分展现了世界—亚洲—中国3个范畴和层面的有关儿科肾脏病界的成就和经验。会议主题口号为“关怀今日亚洲的肾脏病儿”(caring forchildrenwithrenaldiseasesintod…     

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患儿,女,3·5h,以“发现手脚畸形3·5h”为主诉2005年8月6日入院。孕1产1,孕39+4周剖宫产,出生体重1800g,羊水量多,脐带绕颈3周,生后无窒息。父亲44岁,轧钢工人;母亲41岁,橡胶工人,身体均健康。入院查体:神清,反应可,发育差,身长40cm,头围30cm,胸围26cm,哭声弱,前囟平软,胸廓对称,呼吸平稳,无三凹征,双肺呼吸音清,心音有力,律齐,心率120/min,第一心音亢进,未闻及杂音,腹平坦,肝脾未及,肌张力增加,握持反射(+),余原始反射未引出,肛门正常,耳廓薄,左耳位较右耳位略低,下颌略内收,四肢前四指(趾)相连,小指(趾)可分开。心脏彩超:左心发育不良综…     

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目的研究葡萄糖6磷酸脱氢酶(G6PD)缺乏的新生儿高胆红素血症发生率及发病特点。方法对近5年来中山市陈星海医院产科分娩的足月健康新生儿脐带血进行G6PD定量测定,对G6PD活性缺乏的患儿,按性别和酶活性缺乏的程度分组调查其高胆红素血症的发生率及发病时间。结果(1)418例G6PD活性缺乏的患儿共发生高胆红素血症82例,占19.62%。(2)在G6PD活性缺乏的患儿中,男性酶的活性极显著低于女性(P<0.01);高胆红素血症的发生率男性极显著高于女性(P<0.01);酶活性缺乏程度不同的3组患儿高胆红素血症发生率有显著差别(P<0.05);G6PD活性缺乏的患儿发生高胆红素血症的时间主要在出生后的1周内。结论在新生儿期,G6PD活性缺乏的患儿高胆红素血症的发生率较高,发病具有男性多于女性、酶活性缺乏程度越重高胆红素血症的发生率越高的特点,患儿发生高胆红素血症的高峰时间在出生后的2～4d。     

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??Abstract??Objective??To observe the correlation between the expression of Toll-like receptor 4??TLR4??in monocytes and the concentration of TNF-αin children with dilated cardiomyopathy ??DCM ????in order to explore the relationship between TLR4 and the occurrence and development of children with DCM. Methods??In 18 children with DCM?? TLR4 level in monocytes was measured by flow cytometry?? the concentration of TNF-αin serum was determined by ELISA??left ventricular ejection fraction??LVEF????left ventricular end-diastolic volume??LVEDV???? left ventricular end-systolic volume??LVESV?? and left ventricular mass??LVmass?? were detected by echocardiography. Seventeen healthy children served as controls. Results??The mean fluorescence intensity?? MFI ??of TLR4 in monocytes was higher in children with DCM at preliminary diagnosis?? compared to that of treatment of one year . TNF-αhad a similar result??P < 0.05??.Moreover??they were both higher in chidren with DCM compared with controls. The improvement of LVEF?? LVEDV?? LVESV and LV mass in children with DCM correlated with the decrease of TLR4MFI and with the concentration of TNF-α. Conclusion??Upregulation of TLR4 in monocytes of children with DCM may play a critical role in the mechanism of cardiac damage through the secretion of inflammatory factors??which mediates the development and progression of children with DCM.     

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??Objective To investigate the serological patterns of hepatitis B virus and the distribution characteristics among 0-7-year-old children. Methods The detection results of hepatitis B virus serological markers ??HBVM?? in 0-7-year-old children and the family history of ‘HBsAg??+??’ children from Children's Hospital of Chongqing Medical University in 2013 were analyzed retrospectively. Results Seventeen serological patterns of hepatitis B virus were found??and the detection rate of each pattern in different age groups was different. The positive rate of HBsAg was 0.32%??and there were no significant differences in HBsAg positive rate in different age groups. The detection rates of ‘anti-HBs??+?? anti-HBc??+??’??‘anti-HBs??+?? HBeAg??+?? anti-HBc??+??’??‘anti-HBs??+?? anti-HBe??+?? anti-HBc??+??’??‘anti-HBc??+??’ and ‘anti-HBe??+?? anti-HBc??+??’ in 0 to 28 day age group and 1 month to 1-year age group were significantly higher than other age groups ??P??0.05??. PreS1-Ag was detected in five serological patterns??the highest frequency pattern was ‘HBsAg??+?? HBeAg??+??’ ??100%???? followed by ‘HBsAg??+?? HBeAg??+?? anti-HBc??+??’. The detection rate of PreS1-Ag in the HBeAg ??+?? group was 80.77%??which was significantly higher than the HBeAg ??-?? group ??χ2??14.083??P??0.000??. Conclusion There is no significant change in positive rate of HBsAg with the increase of age among 0-7-year-old children in Chongqing??but the distribution of serological patterns is associated with age. There is correlation between PreS1-Ag and HBeAg??and the combined test of the two serological markers can better reflect the HBV replication??which has important clinical value.     

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新生儿脑梗死(neonatalcerebralinfarction,NCI)临床上并不罕见,其发生率大约占活产足月新生儿的1/4000[1],它不仅可以危害新生儿的生命,而且存活者可遗留有永久性神经系统后遗症。现将我院2004年诊治的3例NCI报告如下,供同行斟酌。1临床资料3例均为男性足月儿,剖宫产1例,顺产1例,土法接生1例。其中,顺产者有轻度窒息史,土法接生者可疑重度窒息并有胎膜早破48h。就诊时间17～31h,1例以新生儿肺炎收住入院,之后出现神经系统症状,1例以呼吸暂停为首要表现。临床症状:3例均为难治性惊厥,常规止惊药物效果差,1例为一侧肢体抽搐,2例伴有频繁呼…     

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阵发性运动诱发性运动障碍(paroxysmal kinesigenic dyskinesia,PKD)是一种运动诱发的以肢体和躯干阵发性舞蹈、手足徐动症或肌张力不全等运动障碍为特征的一种不常见散发性、家族性或继发性疾病。家族性大部分呈常染色体显性遗传,少数也可隐性遗传。其基因定位及发病     

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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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本文总结1980～1993年中国医科大学第一临床学院收治的3例特异性心肌病患儿临床资料,报道如下。例1.男,12岁。3岁会走,智力落后,学习成绩差。入院前7个月开始自觉心悸、气短、乏力、多汗,以后又出现面色青紫和水肿。当地医院曾按“风湿性心脏病”合并心力衰竭诊治。查体:头颅增大、面容特殊。口唇发绀。心界向两侧扩大,心音低钝,心率124/min,心尖区有Ⅱ级收缩期杂音。两肺无干湿啰音?未笥依呦?cm。脊柱明显后突,双手指变形不能握拳,下肢指压痕(+)。X线检查:心影明显增大呈普大型,肺血增多。肋骨呈飘带样,T11、T12和L1椎骨变形后凸。心电图…