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??Using developmental assessmentscalesis one of theessentialwaysfor assessing children’s development in cognition.In this paper??we examined the key features and the clinical use ofthe commonly used developmental screening and diagnostic scales for childrenunder6 years old in China. Currently??there are not manydevelopmental assessmentscales in cognition that are available for clinical usein China. Most of the developmentalassessmentscales are used in the areaof child health??and mainlyfor research or atsmall-scale use stage.Developmentalassessmentfor young children under-6 years old isa criticaland promising area in child health and disease surveillance. It isurgentto promote the awareness of the importance of early detection and early intervention of cognitive developmental disability.     

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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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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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(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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目的分析毛细血管内增生性肾炎致肾病综合征患儿的临床表现,病理改变和治疗。方法2002-06—2004-08,对苏州大学附属儿童医院肾内科收治的毛细血管内增生性肾炎致肾病综合征6例进行回顾分析。结果(1)6例患儿的临床表现均为肾炎型肾病综合征;(2)其病理特点为:在毛细血管内增生的基础上有细胞新月体形成,可见IgG、C3的颗粒状沉积物沿毛细血管袢连续排列,且发现“不典型驼峰”和部分足突融合;(3)治疗:6例均予激素治疗。其中2例治疗4周内完全缓解;4例未能在4周内达到完全缓解者,2例加用环磷酰胺,2例予雷公藤,均缓解。结论(1)病理改变与临床表现关系密切,伴新月体者肉眼血尿时间较长,伴足突融合者蛋白尿程度重,持续时间长;(2)给予足量激素,或联合应用免疫抑制剂,可取得较好疗效,但长期预后尚待进一步观察。     

蚕豆病6例

本文报告6例蚕豆病的临床表现和治疗,临床表现有贫血,疲乏,发热,黄疸,纳差,尿色改变,恶心呕吐,抽搐,口竭,腹痛,腹泻,肝肿大、昏迷、脾肿大。实验室检查RBC均<300×10~(12)/L,Hb均小于50g/L,WBC均大于10×10~9/L,血小板均大于100×10~9/L,网织细胞计数0.02～0.14例,尿胆元及血红蛋白尿潜血试验均阳性,肝功能正常,血清胆红素均大于17μmol/L。高铁血红蛋白还原率小于70％4例。     

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目的总结6例儿童先天性Ⅲ度房室传导阻滞长期随访结果并探讨是否必需安装起搏器治疗。方法 1984年1月至2007年6月,南京军区福州总医院收治的6例先天性Ⅲ度房室传导阻滞患儿,回顾性分析患儿的临床资料及随访结果。结果 6例患儿中,男3例,女3例。年龄3~11岁,平均(7.8±2.9)岁。主要临床特征为心动过缓,均为体检时发现,均未安装起搏器,随访5~27年,未发生猝死,1例随访27年发现已有心脏扩大。结论儿童先天性Ⅲ度房室传导阻滞无症状时不需药物治疗,但何时安装起搏器尚需探讨,安装起搏器与否和是否会发展成扩张型心肌病也需探讨。     

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目的观察全身应用卡铂、替尼泊苷和长春新碱(CTV)方案化疗加鞘内注射治疗脑脊液细胞学检查肿瘤细胞阳性的视网膜母细胞瘤(RB)患儿的疗效。方法6例患儿资料来自首都医科大学附属北京同仁医院2006年11月至2008年7月临床确诊为RB且治疗前行脑脊液细胞学检查发现肿瘤细胞者,对其进行6～9次CTV方案化疗加8～10次鞘内注射治疗,同时结合局部治疗,随访12～20个月,观察疗效。结果6例患儿均完成治疗,病情稳定,复查脑脊液细胞学检查均未发现肿瘤细胞,无复发及死亡。结论全身化疗加鞘内注射是对RB颅内转移有效的治疗方法,由于观察时间较短,长期疗效尚须行观察。     

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新生儿先天性鼻后孔闭锁是新生儿期较少见的严重鼻部畸形,属家族性遗传疾病。可引起新生儿缺氧、窒息、甚至死亡。易与其它呼吸系统疾病混淆。现将我院6例先天性后鼻孔闭锁的小儿误诊情况分析如下。一般资料:我科1999—2005共收治先天性鼻后孔闭锁小儿6例。男4例,女2例;胎龄30~38周、体重1500~3600g;足月儿3例、早产儿3例;3例早产儿中有2例分别为胎龄30周、体重1500g和胎龄32周、体重2040g。其中1例为足月小于胎龄儿,1例合并先天性心脏病,2例直系亲属中有睡眠打鼾病史。临床表现:2例早产儿于生后表现呼吸浅促、呻吟、吸气凹陷明显;口周及眼…     

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目的加强对Crouzon综合征的认识。方法对2007年1月至2010年10月在中国人民解放军总院儿科和首都医科大学附属北京儿童医院临床诊断Crouzon综合征的6例患儿临床资料进行总结并回顾相关文献。结果 Crouzon综合征有典型的颅面特征,主要表现为短头、眼眶浅、眼球突出、鹰钩鼻、上颌骨发育不良和下颌相对前突等颅面畸形。手足外观正常。常伴视力障碍、听力异常、鼻塞及上下呼吸道阻塞等。结论 Crouzon综合征是一种常染色体显性遗传病,发病机制不明,目前主要倾向FGFR突变介导的颅缝早闭,致使面中部发育不良,尚无特殊治疗,部分可对症进行手术修复,对其病理机制应进一步探讨,早期进行产前诊断。     

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小儿心肌病较为常见,近年来尤其在遗传学方面研究很多,进展很快,但有些问题争议很多。笔者把这些问题做一剖析,使读者有更深入了解。由于这些问题有不同意见,作者的意见为一家之言,有些学者肯定有不同意见,欢迎提出讨论。既可提高对原发性心肌病的认识,又活跃了学术气氛。1发病率的变化近年来小儿原发性心肌病发病率显著升高。肥厚性心     

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目的提高对小儿急性淋巴细胞白血病前期(Pre-ALL)的认识。方法对西安市儿童医院1997—2004收治的6例Pre-ALL患儿的临床经过及实验室的特点进行分析,并复习相关文献。结果男4例,女2例,年龄2岁3个月至8岁7个月,6例均有发热、贫血、出血。初诊为再生障碍性贫血(AA)5例、骨髓异常增生综合征(MDS)1例。4例出现肝、脾、淋巴结肿大(非同时),三系细胞减少,骨髓穿刺涂片示:增生减低4例,重度减低2例;骨髓病理活检:4例均示脂肪组织增多,造血组织减少;2例出现干抽的患儿伴有纤维组织增生,2例出现病态造血。经或不经糖皮质激素治疗,在1~9个月内均转变为急性淋巴细胞白血病(ALL)。3例接受VDLP方案治疗获完全缓解。结论6例转变为ALL,对化疗敏感,部分Pre-ALL有形态学异常。     

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目的研究脐血红系祖细胞的HOXB6基因的表达情况以及人类巨细胞病毒(HCMV)感染对红系祖细胞的HOXB6基因表达的影响,以探讨HCMV导致脐血红系祖细胞损伤的机制。方法在泸州医学院附属医院儿科以实时荧光定量PCR技术(FQ-RT-PCR)检测人类巨细胞病毒感染和(或)用全反式维甲酸(ATRA)处理后HOXB6基因的表达水平。结果人脐血红系祖细胞可表达HOXB6基因;HCMV感染后,红系祖细胞HOXB6基因的表达明显下调;ATRA能显著上调红系祖细胞HOXB6基因的表达水平,且能够在一定时间内上调HCMV感染后的红系祖细胞HOXB6基因的表达。结论HCMV感染能诱导脐血红系祖细胞HOXB6的表达下调,HC-MV有可能通过调控HOXB6基因表达异常而引起红系祖细胞的增殖障碍。     

SUBJECTINDEX TO VOLUME 6

AAcousticstimulation　 ( 6 ) :4 79Administration ,inhalation　 ( 1) :19Adolescence　 ( 3) :176Albumins　 ( 2 ) :139All transretinoicacid/therapeuticuse　 ( 2 ) :89Ambroxol/threapeuticuse　 ( 5) :4 2 5,( 3) :2 2 7,( 5) :4 2 5,( 6 ) :536Amyloidbeta proteinp     

毒鼠强中毒6例

目的 探讨小儿毒鼠强中毒的救治体会。方法 对6例毒鼠强中毒患儿在常规治疗基础上采用血液灌流,早期应用神经康复治疗。结果 治愈1例,好转4例,死亡1例。结论 毒鼠强中毒发病凶险,病死率高,缺乏特效解毒剂,早期使用血液灌流清除毒素、应用神经康复等综合治疗可取得较好效果。     

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目的探讨6月龄内健康男婴睾酮、黄体生成素(LH)与促卵泡生成素(FSH)的血清浓度变化及意义。方法收集2010年4月至2011年10月重庆医科大学附属儿童医院6月龄内健康足月分娩男婴336名的血清标本,采用免疫化学发光法测定其睾酮、LH及FSH的浓度。结果睾酮于生后<1d达第一个高峰[(28.45±11.92)nmol/L];于10～<50d达第二个高峰[(8.41±3.83)nmol/L],峰值较第一个低,随后下降,4～6个月降至(0.48±0.45)nmol/L。LH、FSH于生后数月内均有短暂升高,以LH升高为主。LH、FSH在生后数天内保持在一个低浓度,随后开始升高,于10～<50d达高峰,之后下降,于4～6个月LH浓度降至(0.43±0.47)U/L,FSH浓度降至(0.48±0.51)U/L。结论男婴生后6个月内血清睾酮及促性腺激素浓度均有短暂升高,对男婴性腺发育有重要作用。     

儿童Gitelman综合征6例

目的探讨儿童Gitelman综合征的临床特点及其与Bartter综合征的鉴别。方法总结本院住院的6例儿童Gitelman综合征的临床表现、实验室检查、治疗方法及效果,回顾分析Gitelman综合征及Bartter综合征相关文献。分析二者发病机制、临床表现治疗等不同点。结果6例均起病早,婴幼儿期起病,以生长迟缓、无力及抽搐为主要表现,血压正常。实验室检查主要表现低血钾、低血镁,代谢性碱中毒,血浆肾素、血管紧张素明显升高,醛固酮升高不明显或正常。治疗需补钾、补镁症状才能改善。结论Gitelman综合征与Bartter综合征临床表现及发病机制均有不同,相应治疗也不同。     

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?? AbstractObjectiveThe present study was designed to understand the prevalence of dyslipidemia in children aged 6 to 18 years in Beijing area. MethodsCluster random sampling methods were used in the investigation.During April to October in 2004,our epidemiological survey group developed field investigations to 19,593 children and adolescents aged 6 to 18 years in Beijing area.Fasting capillary blood total cholesterol (TC) and triglyceride (TG) were detected. ResultsTotal prevalence rate of hyperlipidemia in Beijing children was 9.61 percent.About 1.21 percent children had higher TC than normal value??8.79 percent had high TG level.Only 0.39 percent people had both high TC and TG level.The prevalence rate of urban children was 10.55 percent,10.16 percent in boys and 10.94 percent in girls.The prevalence rate of dyslipidemia in countryside was 8.62 percent,6.11 percent in boys and 11.18 percent in girls. ConclusionThe prevalence rate of children hyperlipidemia in Beijing significantly increases compared to previous studies.This came with epidemiological characteristics of urban??countryside,gender or age differences. KeywordsLipid;Children and adolescents;Prevalence rate ???? ek2007-02-0101-02 ????????6~18????????????????? ???1?????2???????1 ??????????????п????????(H030930030530??H030930030031) 1.?????????????????????100034??2.????????о?????????100020 E??mail:junbaodu@ht.rol.cn.net ???????????? ???????????????в?????飬?????????????????????????????????????????????????????????????????????????????? ????????????????????????2004??04??2004??10??????в???????飬???????7????????????19593??6~18???????????????????飬????????????????(TC)?????????(TG)???м??????????????6~18???????????????????????к???????в???о??? ???????????????????????????9.61%(1874/19501)??????TC??????????????1.21%(236/19501)??TG???????8.79%(1714/19501)??TC??TG?????????0.39%(76/19501)????????????????????10.55%(1053/9978)????????????10.16%(503/4950)???????????10.94%(550/5028)??????????ò???????8.62%(821/9523)????????????6.11%(294/4811)???????????11.18%(527/4712)?? ????????ж???????????????????????????????????????????????в???????? ??????????????????? A survey on dyslipidemia of 6~18??year old children in Beijing area. LIU Ying,MI Jie,DU Jun??bao.Department of Pediatrics,Peking University First Hospital,Beijing 100034,China AbstractObjectiveThe present study was designed to understand the prevalence of dyslipidemia in children aged 6 to 18 years in Beijing area. MethodsCluster random sampling methods were used in the investigation.During April to October in 2004,our epidemiological survey group developed field investigations to 19,593 children and adolescents aged 6 to 18 years in Beijing area.Fasting capillary blood total cholesterol (TC) and triglyceride (TG) were detected. ResultsTotal prevalence rate of hyperlipidemia in Beijing children was 9.61 percent.About 1.21 percent children had higher TC than normal value??8.79 percent had high TG level.Only 0.39 percent people had both high TC and TG level.The prevalence rate of urban children was 10.55 percent,10.16 percent in boys and 10.94 percent in girls.The prevalence rate of dyslipidemia in countryside was 8.62 percent,6.11 percent in boys and 11.18 percent in girls. ConclusionThe prevalence rate of children hyperlipidemia in Beijing significantly increases compared to previous studies.This came with epidemiological characteristics of urban??countryside,gender or age differences.     

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??Objective??To establish diagnostic criteria for asthma in children aged??6 years in China. Methods??From October 2016 to July 2017??a total of 785 wheezing children aged ??6 years were enrolled from a asthma clinic for children in Xinhua hospital affiliated to Shanghai Jiao Tong University School of Medicine. The questionnaire was used to collect data for a database of childhood asthma. The method of literature search and expert consultation was used to select parameters for the diagnosis of asthma. Univariate analysis was first applied to study the significance of these parameters??and multivariate logistic regression analysis was then performed to establish diagnostic model for asthma in children. According to the β value of the regression model??the scoring system was established. Area under receiver operating characteristic curve was used to test the differential diagnostic efficacy of the scoring system. Results??The diagnostic criteria for asthma in children aged??6 years was developed based on the logistic regression model??and the scoring system was established according to the β value of the model. The main contents included accumulated times of wheezing attacks ≥ 4??3 points????the existence of reversible airflow limitation??3 points????the existence of allergic rhinitis and/or atopic dermatitis??1 point????the existence of allergic history among first-degree relatives??1 point????positive allergen test in vivo or in vitro??1 point??. The area under ROC curve of the scoring system was 0.908??indicating that the diagnostic model and scoring system was of great value in diagnosis of asthma in children aged??6 years. Based on the ROC curve??the optimal cut-off value for asthma diagnosis was four points??with a sensitivity of 95.8% and a specificity of 85.9%. Conclusion??The diagnostic criteria for asthma in children aged??6 years has been established preliminarily??which is simplified and practical and need a large-scale prospective study for further validation and optimization before clinical application.