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中华实用儿科临床杂志

中华实用儿科临床杂志

北大核心

CSCD

CSTPCD

Journal of Applied Clinical Pediatrics
本刊创刊20年来，严格遵守国家的出版法规及条例，执行出版编辑规范，始终坚持突出实用为主，理论联系实践，注重基础与临床相结合，报道儿科领域新的科研成果、新理论、新技术、新进展，为促进我国儿科医学领域的学术交流服务为办刊宗旨。2005年本刊的总被引频次1417，在18种妇、儿科医学类期刊中列第4位，在1606种中国科技论文统计源期刊中列第79位；影响因子0.659，在18种妇、儿科医学类期刊中列第6位，在1606种中国科技论文统计源期刊中列第221位；即年指标0.155,在8种儿科学类期刊中仅次于《中华儿科杂志》，位居第2位，其他学术期刊指标均较去年明显提高。目前已连续四次进入北京大学图书馆主编的《中文核心期刊要目总览》、列入中国科技论文统计源期刊（中国科技核心期刊），2003年被俄罗斯《文摘杂志》收录,2005年又被美国《化学文摘》收录。2004年被评为河南省优秀期刊。我刊已被中国生物医学文献数据库（CBMdisc）、Quick全文资料管理系统（FTME）、中文科技期刊数据库、中国学术期刊（光盘版）、万方数据库和美国《化学文摘》、俄罗斯《文摘杂志》、《中国医学文摘：儿科学分册》等多种权威性数据库与文摘期刊作为固定收录对象。
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  • 发文量：4931
  • 被引量：6088
  • H指数：3
  • 开始收录时间：1989,4(1)
  • CNKI综合因子：1.153
  • 维普期刊影响因子： 1.525
  • 万方期刊影响因子：1.6
  • 创刊时间：1986
  • 国内刊号/CN：10-1070/R
    国际刊号/ISSN：2095-428X
  • 发行周期：半月刊
    邮发代号：36-102
  • 主管单位：中国科学技术协会
    主办单位：中华医学会
中华实用儿科临床杂志-联系信息
  • 主编：-
  • 电话：0373-3029144 3831456
  • 邮箱：cjacp@cmaph.org；zhsyeklczz@163.com
  • 地址：河南省新乡市金穗大道601号新乡医学院
  • 邮编：453003
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  • 摘要：1．图和表图和表分别按其在文中出现的先后顺序连续编码。每幅图和每个表均应有图题和表题，说明性资料应置于图和表下方注释中，并在注释中标明图和表中使用的全部非公知公认的缩写。以计算机制图者应提供激光打印图样。照片图要求有良好的清晰度和对比度。图片不可折损。图中需标注的符号（包括箭头）应标在图的复印件上，不要直接标注在照片上，每幅图的背面应贴上标签，注明图号、作者姓名及图的上下方向。大体照片图应有尺度标记，显微镜照片图应标明放大倍数和染色方法。本刊采用三横线表，如有合计行或统计学处理行，则在此行上面加一条分界横线。表内数据要求同一指标的数字、小数点、字母和符号必须一致。“空白”表示无此项或未测，“一”代表阴性未发现。图与表随正文，先文字后图和表，并确保图、表均在正文中标明。
  • 摘要：正文包括4部分内容，即引言、材料（资料）与方法、结果、讨论。正文标题分层，序号用1、1．1、1．1．1等表示。1．引言引言一般不超过300字，主要阐明研究背景、目的，该研究或观察的理论依据、国内外研究现状，该研究思路、研究方法、预期结果及意义等。若称“首次发现”、“首次报道”类论文，须附有专业情报检索机构出具的查新报告。病例报告也应有不超过100字的引言。2．材料（资料）与方法应描述课题的基本设计，使用方法是双盲、单盲还是开放性，数据的精确程度等。研究对象是否遵循随机化、齐同化、对照原则；研究对象为人时，须注明病例和对照者的来源，选择时间、选择方法和标准（诊断标准须注明文献来源）及一般情况，必要时还应说明剔除标准；研究对象为实验动物时，须注明动物的名称、种系、等级、数量、来源、性别、年龄、体质量、饲养条件及健康状况等。所用药品或化学试剂名称、剂型、剂量、生产厂家所在地及名称和生产批号，药品和化学试剂必须使用通用名称，确实需要使用商品名称时，如新药的临床试验研究，应在其通用名称后的括号内注明商品名。
  • 摘要：目的分析儿童烟雾病临床及影像学特点。方法对2002年1月至2011年6月在首都医科大学附属北京天坛医院经数字减影血管造影（DSA）或磁共振血管成像确诊的141例烟雾病患儿病历资料进行回顾性分析。结果141例患儿中，男77例，女64例。发病年龄2—18岁[（9．50±4．06）岁]，5—10岁为发病高峰，共71例（50．4％）。以缺血为首发症状者136例（96．45％），出血为首发症状者5例（3．55％）。常见的首发症状包括肢体无力[80例（56．7％）]、头痛[41例（29．1％）]、失语或构音障碍E24例（17．0％）]，其他表现包括抽搐发作、面瘫、眩晕、眼部症状、感觉症状等。113例行DSA检查的患儿中，44例为颈内动脉末端病变，69例为颈内动脉末端合并大脑中动脉或大脑前动脉或大脑后动脉病变。56例（52．8％）患儿行间接血运重建术，39例（36．8％）行直接血运重建术，7例（6．6％）行直接＋间接血运重建术，4例（3．7％）行血管栓塞术或病变血管切除术。未经手术治疗的患儿予保守治疗。结论儿童烟雾病多以缺血性卒中为首发症状，颈内动脉末端、大脑中动脉或大脑前动脉起始部最常累及，DSA是确诊的首选检查。
  • 摘要：目的探讨Wnt／β-catenin信号传导在高压氧（HBO）治疗新生大鼠缺氧缺血性脑损伤（HIBD）中的作用。方法将新生SD大鼠神经干细胞（NSCs）随机分为对照组和8个处理组，处理组分别与正常脑组织匀浆及HIBD大鼠脑组织匀浆共培养，以模拟正常及HIBD时脑内微环境，通过电穿孔方法将β-cateninsiRNA和阴性对照质粒转染预先处理过的NSCs，再给予HBO处理。运用免疫细胞组织化学方法观察HBO对NSCs分化的影响；Westernblot、RT—PCR法观察β-catenin下游基因Ngn1和骨形成蛋白（BMP4）的表达。结果HBO可促进转染阴性对照质粒的NSCs（ncNSCs）分化为神经元和少突胶质细胞，抑制ncNSCs向星形胶质细胞分化；HIBD及正常大鼠脑组织匀浆均可促进ncNSCs分化为神经元及少突胶质细胞，抑制其向星形胶质细胞分化，HIBD大鼠脑组织匀浆的作用强于正常脑组织匀浆，且HBO可加强这一效果；转染β-cateninsiRNA后，NSCs向神经元分化减少，向星形胶质细胞分化增加，这一作用可被HBO减弱，且不可逆转；HBO促进NSCs分化为神经元的同时，Ngnl蛋白和mRNA的表达增加，β-cateninsiRNA促进NSCs分化为星形胶质细胞的同时，BMP4蛋白和mRNA的表达增加。结论HBO可促进离体HIBD大鼠NSCs分化为神经元和少突胶质细胞而抑制其向星形胶质细胞分化；HBO促进离体大鼠NSCs分化为神经元与β-catenin的激活有关；HBO是通过激活β-catenin上调Ngnl的表达促进NSCs分化为神经元，通过下调BMP4的表达减少NSCs分化为星形胶质细胞；BMP4和Ngnl在大鼠NSCs的增殖分化中起重要的协调作用，Ngnl可能是一种潜在的神经元促成剂。
  • 摘要：1．本刊自2013年起采用网上投稿，作者在线投稿后，须将从网站上下载的单位推荐信盖章并寄至我刊编辑部，内容应包括稿件内容属实及未一稿两投、重复发表和作者署名无争议，不涉及保密。研究生论文须有导师亲笔签字。作者可登陆中华医学会杂志社远程稿件处理系统查询处理情况，
  • 摘要：Objective To explore an endogenous self-repair potentiality for injured cerebral white matter from both of subependymal ventricular zone and white matter cell cultures in neonatal rats with oxygen glucose deprivation (OGD) in vitro.Methods The white matter and subependymal ventricular zone tissues from the neonatal rats within 5 days old were separately used to prepare primary glia-derived cell cultures,and these cell cultures were randomly divided into the control group and the OGD group.The double-label fluorescent immunoanalysis was used to observe the proliferation and differentiation of the glia-derived cells came from both of subependymal ventricular zone and white matter activated by OGD.The Hoechst33342/propidium iodide (PI) staining and the flow cytometry technology were used to assess the apoptotic rates of the newborn cells.Results More apoptotic and necrotic cells appeared in the OGD group than those in the control group both in subependymal ventricular zone and white matter cell cultures in the flow cytometry test and Hoechst33342/PI staining at 24 h,48 h,72 h,7 d and 14 d after OGD (all P ＜ 0.01).Furthermore,fluorescence microscope showed that the number of the NG2 + progenitor cells,the O4 + oligodendrocyte precursor cells in the OGD group were all significantly more than those in the control group during 72 h after OGD (all P ＜ 0.05,0.01),while the number of the immature and mature oligodendrocytes in the OGD group decreased significantly compared with those in the control group on 7 d and 14 d after OGD (all P ＜ 0.05,0.01).Conclusions OGD may activate 2 endogenous self-repair pathways from subependymal ventricular zone and white matter in vitro.The activated subependymal ventricular zone and white matter-glial progenitor cells appear to proliferate markedly,and differentiate along an oligodendroglial pathway.However,only a few newly generated precursor cells can be differentiated into the immature or mature oligodendrocytes and OGD may induce the newborn cells to appear apoptotic and necrotic.%目的 体外探讨缺血启动新生大鼠未成熟室管膜下区(SVZ)和脑白质内源性修复机制.方法 体外培养5日龄内SD新生大鼠的SVZ和脑室周围白质细胞,各分为正常对照组和氧气-葡萄糖剥夺(OGD)组,利用免疫荧光双标技术检测新生祖细胞的增殖分化情况,利用Hoechst33342/碘化丙啶双染技术和流式细胞术检测新生细胞成活情况.结果 OGD组SVZ和脑白质在OGD后24 h、48 h、72 h、7d及14 d均出现明显的凋亡和坏死细胞,各时段新生细胞的凋亡率均显著高于同时段对照组(P均＜0.01).荧光显微镜观察显示,与正常对照组比较,OGD组SVZ和脑白质新生NG2+祖细胞和O4+少突胶质细胞(OL)前体的细胞数量在OGD后72 h内均明显增加(P均＜0.05,0.01),CNPase+未成熟OL和MBP+成熟OL在OGD后7d和14 d则较同时段正常对照组明显减少(P均＜0.05,0.01).结论 缺血可诱导SVZ和脑白质神经祖细胞激活和明显增殖,并沿OL系分化,但最终仅有极少量新生OL前体能分化为未成熟和成熟OL;同时,缺血导致OGD组新生细胞出现明显凋亡和坏死.
  • 摘要：目的 探讨Wnt/β-catenin信号传导在高压氧(HBO)治疗新生大鼠缺氧缺血性脑损伤(HIBD)中的作用.方法 将新生SD大鼠神经干细胞(NSCs)随机分为对照组和8个处理组,处理组分别与正常脑组织匀浆及HIBD大鼠脑组织匀浆共培养,以模拟正常及HIBD时脑内微环境,通过电穿孔方法将β-catenin siRNA和阴性对照质粒转染预先处理过的NSCs,再给予HBO处理.运用免疫细胞组织化学方法观察HBO对NSCs分化的影响；Western blot、RT-PCR法观察β-catenin下游基因Ngn1和骨形成蛋白(BMP4)的表达.结果 HBO可促进转染阴性对照质粒的NSCs (ncNSCs)分化为神经元和少突胶质细胞,抑制ncNSCs向星形胶质细胞分化；HIBD及正常大鼠脑组织匀浆均可促进ncNSCs分化为神经元及少突胶质细胞,抑制其向星形胶质细胞分化,HIBD大鼠脑组织匀浆的作用强于正常脑组织匀浆,且HBO可加强这一效果；转染β-catenin siRNA后,NSCs向神经元分化减少,向星形胶质细胞分化增加,这一作用可被HBO减弱,且不可逆转；HBO促进NSCs分化为神经元的同时,Ngn1蛋白和mRNA的表达增加,β-catenin siRNA促进NSCs分化为星形胶质细胞的同时,BMP4蛋白和mRNA的表达增加.结论 HBO可促进离体HIBD大鼠NSCs分化为神经元和少突胶质细胞而抑制其向星形胶质细胞分化；HBO促进离体大鼠NSCs分化为神经元与β-catenin的激活有关；HBO是通过激活β-catenin上调Ngn1的表达促进NSCs分化为神经元,通过下调BMP4的表达减少NSCs分化为星形胶质细胞；BMP4和Ngn1在大鼠NSCs的增殖分化中起重要的协调作用,Ngn1可能是一种潜在的神经元促成剂.%Objective To investigate the role of Wnt/β-catenin signaling pathway in hyperbaric oxygen (HBO) therapy for hypoxic-ischemic brain damage(HIBD).Methods The neural stem cells (NSCs) of neonatal rats were randomly divided into control group and 8 treatment groups,then the treatment groups were cultured in the supernatant of brain homogenate to simulate micro-environment of HIBD and normal brain respectively.Through electroporation β-catenin siRNA and negative control plasmid was transfected into NSCs respectively,then HBO therapy was performed.Immunocytochemical staining was performed simultaneously in the 9 groups of NSCs on precoated chamber slides to detect the differentiation of NSCs.Quantitative reverse transcriptase(RT)-PCR was used to detect the relative content of Ngn1 mRNA and bone morphogenetic protein(BMP4) mRNA in the of NSCs.Western blot was used to detect the relative content of Ngn1 protein and BMP4 protein in the NSCs.Results In vitro,HBO alone promoted NSCs infected with negative control siRNA (ncNSCs) to differentiate into neurons and oligodendrocytes and depressed astrogliosis ; HIBD or normal brain tissue extract cultures promoted ncNSCs to differentiate into neurons and oligodendrocytes but depressed astrogliosis,and the effect of HIBD brain extract cultures was superior to the latter and could be further increased by HBO; β-catenin siRNA decreased the NSE-positive neurons and increased glial fibrillary acidic protein-positive astrocytes in the siNSCs (NSCs infected with β-catenin siRNA) in vitro,the effect could not be inversed by HBO,but could be alleviated; HBO increased the level of Ngn1 mRNA and Ngn1 protein,decreased BMP4 mRNA and BMP4 protein of ncNSCs,transfection of β-catenin siRNA could down-regulate the expression of Ngn1 mRNA and upregulate BMP4 mRNA of NSCs in vitro respectively.Conclusions HBO can promote NSCs cultured with HIBD brain extract cultures to differentiate into neuronal or Oligodendrocyte,and inhibit them to differentiate into astrocytes.HBO therapy promotes the proliferation of NSCs in vitro,an effect which is correlated with β-catenin protein.HBO therapy can promote neurogenesis by β-catenin-induced activated Ngn1,and repress astrocytogenesis by β-catenin-induced down-regulated BMP4.There are potential cooperative actions of BMP4 and Ngn1 on differentiating rat NSCs in cerebral ischemic brain.The ability of Ngn1 to promote neurogenesis may allow Ngn1 to act as a potent neuronal commitment factor.
  • 摘要：Objective To study the effect of progesterone on tumor necrosis factor (TNF-α),interleukin-1β (IL-1β),nerve growth factor(NGF) and brain derived neurotrophic factor(BDNF) expression in the cortex and hippocampus tissue in newborn rats with hypoxic-ischemic brain damage (HIBD),and to discuss the protective molecular mechanism of progesterone on HIBD in neonatal rats.Methods Forty-eight 7-day-old neonatal rats were randomly divided into 3 groups:sham-operated group,hypoxic-ischemic group and pretreatment group.Rats in hypoxic-ischemic group and pretreatment group were subjected to left common carotid artery ligation,then they were exposed to 80 mL/L oxygen and 920 mL/L nitrogen gas in the closed container at 37 ℃ for up to 2.5 h to establish HIBD models.Progesterone was injected intraperitoneally into the rats in the pretreatment group 30 min before hypoxia,and solution was injected into the first 2 groups.All the rats were killed at the 24 h after operation.The levels of TNF-α,IL-1β,NGF,BDNF were measured by enzyme linked immunosorbent assay and the expressions of TNF-α,IL-13,NGF,BDNF mRNA were analyzed by reverse transcription-polymerase chain reaction.Results The contents of TNF-α,IL-1β,NGF,BDNF and their mRNA expressions in hypoxic-ischemic group were significantly higher than those in the sham-operated group.In pretreatment groups,the levels of TNF-α,IL-1β and their mRNA expressions were significantly lower than those in hypoxic-ischemic group.The levels of NGF,BDNF and their mRNA expressions in pretreatment group were significantly higher than those in hypoxic-ischemic group (all P ＜ 0.05).Conclusions Progesterone exerts neuroprotective effect on hypoxic-ischemic encephalopathy-induced brain damage,and the action mechanism is related to down-regulate the expression of damage factor and up-regulate the expression of anti damage factor.%目的 观察孕酮对缺氧缺血性脑损伤(HIBD)新生大鼠皮层和海马组织中损伤因子TNF-α、IL-1β和抗损伤因子神经生长因子(NGF)、脑源性神经营养因子(BDNF)表达的影响,探讨孕酮神经保护作用的分子机制.方法 将48只7日龄新生Wistar大鼠随机分成3组:假手术组、缺氧缺血组、药物预防组.缺氧缺血组和药物预防组新生大鼠先行左侧颈总动脉结扎术,然后置于37℃恒温的密闭容器中,以1.5 L/min的速度吸入80 mL/L氧气和920 mL/L氮气混合气体2.5h建立HIBD动物模型.假手术组仅分离左侧颈总动脉,不结扎,亦不做缺氧处理.药物预防组新生大鼠于建立模型前30 min按8 mg/kg腹腔注射0.5 g/L孕酮溶液,假手术组和缺氧缺血组注射等量的9 g/L盐水溶液,24h后处死全部新生大鼠,取左侧脑组织,采用ELISA法检测脑组织TNF-α、IL-1β、NGF和BDNF水平,反转录聚合酶链式反应(RT-PCR)检测TNF-α、IL-1β、NGF和BDNF mRNA的表达.结果 缺氧缺血组新生大鼠皮层和海马组织中TNF-α、IL-1β、NGF、BDNF水平及其mRNA的表达明显高于假手术组；药物预防组TNF-α、IL-1β水平及其mRNA的表达明显低于缺氧缺血组,而NGF、BDNF水平和mRNA的表达则明显高于缺氧缺血组(P均＜0.05).结论 孕酮可以保护新生大鼠缺氧缺血后引起的皮层和海马损伤,其作用机制与抑制损伤因子的表达和促进抗损伤因子的表达有关.
  • 摘要：Objective To study the effect of Phenobarbital (PB) on experimental rats,observe the histological changes of immature brain and the expressions of apoptosis-related proteins Bcl-2 and Bax in neurons detected by immunohistochemistry,and to explore the influence and mechanism of PB on brain damage at therapeutic levels to immature brain maturation of rat in order to provide the theoretical and experimental base for clinic.Methods A total of 40 healthy 18-day-old Sprague-Dawley(SD) rats (male or female) were randomly assigned into 2 groups:normal saline (NS) treated as control group(20 cases),PB group(20 cases).Each group was further randomly divided into longterm(4 weeks) treated group and short-term(2 weeks) treated group(10 rats in each group).The rats in PB group received intragastricadministration with PB (30 mg/kg).The rats in control group were handled by injection of NS into stomach and abdomen according to 4 mL/kg.All performances were undertaken for twice every day.At the end of the therapeutic period,body and brain weight were measured when the rats were sacrificed.Histological studies on the tissues of frontal lobes and hippocampus were performed by Hematoxylin-Eosin(HE) staining and Nissl staining.Expressions of apoptosis-related proteins Bcl-2 and Bax in neurons were detected by immunohistochemistry.Results There were no significant differences in body and brain weights or histological studies index among control group as well as PB group before and after treatment for short term(P ＞0.05).Remarkable reduction of brain weight was only observed in immature rats exposed to PB compared to control group for long period,and significant neurodegeneration,neuronal necrosis were observed in immature rats exposed to PB compared to control group(all P ＜ 0.01).The expression of Bax protein in the frontal lobe increased significantly in immature rats receiving PB for long period comparing with control (P＜0.01).In contrast,expression of Bcl-2 protein did not change at therapeutic level.The ratio of Bax/Bcl-2 was obviously increased.Conclusions Chronic treatment with PB will result in significant neuronal apoptosis and necrosis and persistent cognitive impairment and brain damage to immature rates.Brain damage of PB at therapeutic level to immature brain may be irreversible.The significant expression of Bax protein in the frontal lobe and the high rate of Bax/Bcl-2 are probably the main reasons which cause brain weight decreasing and brain damage by PB in immature brain tissue.%目的 通过对苯巴比妥(PB)进行模拟临床用药的动物实验研究,观察不同给药时程幼鼠脑组织的形态结构变化,探讨治疗血浓度下抗癫(癎)药物对幼鼠脑发育的影响及其对认知损害的可能机制,为临床儿童癫(癎)的合理治疗提供科学依据和理论基础.方法 将40只(雌、雄不拘)日龄18 d的健康SD大鼠随机分为2组(每组20只):9 g/L盐水对照组(对照组)、PB组.根据给药时程又将每个给药组分为2周短程及4周长程给药组,共4组,每组10只.PB组予PB 30 mg/kg灌胃；对照组予等体积9 g/L盐水灌胃.以上操作均为2次/d.分别于停药当天将各组幼鼠称体质量后处死,称脑质量,脑组织石蜡包埋,切片HE染色及尼氏染色光镜下观察脑组织形态变化.用免疫组织化学方法检测各组大鼠脑切片海马及额叶皮层凋亡相关蛋白Bcl-2和Bax的表达水平.结果 短程给药后,PB组与对照组比较,其体质量、脑质量及脑组织结构变化差异均无统计学意义(P均＞0.05).而长程给药后,PB组幼鼠脑质量较对照组明显减轻,差异有统计学意义(P＜0.01)；额叶皮层神经细胞减少,且神经元结构明显异常.长程给药后,光镜下观察,PB组幼鼠脑组织额叶皮层Bax蛋白表达高于对照组(P＜0.01),Bcl-2蛋白的表达则无明显改变,Bax/Bcl-2比值明显升高.结论 长程服用PB可引起发育中脑组织明显的组织学损伤及神经元坏死与过度凋亡,而且这种损伤可能是不易恢复的.长程服用PB后幼鼠脑组织额叶皮质Bax蛋白表达显著增强和Bax/Bcl-2比值增高可能是未成熟鼠脑质量减轻和引起脑功能障碍重要原因.
  • 摘要：Objective To investigate the change of expression pattern of microRNA in the lung with acute lung injury (ALI)/acute respiratory distress syndrome(ARDS) induced by lipopolysaccharide (LPS),and to provide an evidence that microRNAs are involved in the pathogenesis of ALI/ARDS.Methods Twenty-four C57BL mice were randomly divided into control group and LPS treated group,12 mice in each group.The rats in LPS treated group were treated with intratracheal injection of LPS at a dose of 10 mg/kg body weight into the lung.The rats in control group were treated with the same dose of saline instead.All mice were sacrificed 24 hours after operation,the left lung was excised to measure the wet-to-dry weight (W/D) ratio,and the right upper lobe was stained with hematoxylin and eosin (HE).A microRNA microarray chip was used to profile miRNA expressions in the lung of rats in both LPS treated group and control group.Online software packages were used to predict the gene targeted by microRNAs.Results Compared with the control group,the LPS treated mice had obvious respiratory symptoms,the W/D ratio was significantly increased (P ＜ 0.01),and the pathology was characterized with ALI/ARDS.The microarray chip results demonstrated that the expressions of 48 microRNAs were significantly changed in the ALI/ARDS mice.Among these miRNA,27 cases were up-regulated,21 cases were down-regulated.The target genes of these microRNAs might be involved in regulating the signal pathway of inflammation.Conclusions Some miRNAs express differently in the model of ALI/ARDS,and they may play an important role in pathophysiological process of ALI/ARDS.%目的 研究脂多糖(LPS)诱导的小鼠急性肺损伤(ALI)/急性呼吸窘迫综合征(ARDS)肺组织中microRNA表达谱的改变,探讨microRNA在ALI/ARDS发生机制中的作用.方法 C57BL小鼠24只,随机分为实验组和对照组,每组12只.实验组小鼠通过气管切开经呼吸道向肺内注入LPS(6 mg/kg),构建小鼠ALI/ARDS模型.对照组用同样的方法注入相应剂量的9 g/L盐水.所有小鼠均于手术后24h处死,取左肺组织用于测量干湿质量比,右中肺叶行HE染色观察病理变化.应用microRNA芯片技术检测对照组及实验组小鼠肺组织microRNA表达谱的差异,用生物信息学方法进行靶基因预测及信号通路分析.结果 与对照组比较,实验组小鼠出现明显的呼吸系统症状,肺组织干湿质量比明显升高(P＜0.01),病理结果符合ALI/ARDS特征.芯片结果显示ALI/ARDS小鼠肺组织中明显差异表达的microRNA有48个,其中上调的有27个,下调的有21个,这些microRNA的靶基因可能参与调节炎症相关的信号通路.结论 在ALI/ARDS小鼠模型中,部分microRNA的表达发生明显的变化,microRNA在ALI/ARDS病理生理过程中起重要作用.
  • 摘要：患儿为16岁青春期女孩,主因“间断抽搐12 h”就诊,入院前40 d有坠楼史,之后出现精神行为异常,左足切口感染.入院后出现发热,白细胞、CRP升高,查体发现患儿血压明显升高,最高达25.3/16.7kPa.入院时结合患儿足部切口化脓性感染、发热及CRP升高曾考虑脑脓肿,血压高为颅压高所导致,但入院后发现患儿血压升高不能用颅压高解释,且无其他颅内压增高的表现,因而考虑患儿血压升高并非高颅压所致,而是高血压脑病直接导致惊厥发作.头颅MRI弥散加权像显示后头部为主的枕叶、额叶、顶叶高信号,符合大脑后部可逆性脑病综合征的影像诊断,亦支持高血压脑病的临床诊断.检查发现患儿左侧肾小且为无功能肾,左肾动脉发育畸形,考虑高血压产生的原因与左肾血管先天发育异常有关.经过降血压及对症治疗,患儿临床症状明显好转,血压稳定(收缩压波动在11.3 ～15.9 kPa,舒张压波动在8.7 ～11.3 kPa),复查头颅MRI病灶明显缩小.总结:重视临床体格检查,尤其是血压等临床基础项目；重视多学科、多专业的交叉合作.%There was a 16-year-old adolescent girl complaining of intermittent convulsions in 12 hours.Forty days before her admission,she fell from a building and had mental and behavioral disorders since then;she wound her left foot and got infected.After her admission,she had a fever,hypertension (blood pressure up to 25.3/16.7 kPa),and elevation of C-reactive protein (CRP).At the beginning of admission,there was a suspect that she had brain abscess because of her fever,elevated CRP and an infectious wound in left foot,and the high blood pressure might be attributed to the intracranial hypertension.But soon,it was found that the hypertension could not be explained by intracranial hypertension because she had no other signs of high intracranial pressure.Therefore,the hypertensive encephalopathy might be resulted in convulsions.The head magnetic resonance imaging diffusion weighted imaging displayed high signal mainly in the head including occipital,frontal and parietal lobes.These images were in accordance with posterior reversible encephalopathy and supported the diagnosis of hypertensive encephalopathy.Further examination for the cause of hypertension showed that her left kidney was small and functioned poorly,and the left renal artery was malformed.After antihypertensive and symptomatic treatment,the girl's condition was significantly improved.Her blood pressure became stable (11.3-15.9 kPa / 8.7-11.3 kPa).The lesions on head magnetic resonance imaging were significantly reduced.Conclusions:More attention should be paid on basal physical examination (such as measurement of blood pressure),and multidisciplinary collaboration should be emphasized and developed.
  • 摘要：伴皮质下囊肿的巨脑性白质脑病（megalencephalic leukoen-cephalopathy wit hsubcortical cysts，MLC）是一种常染色体隐性遗传疾病。1993年由荷兰儿科医师vanderKnaap和Scheper最先报道此病，故又称为VailderKnaap病。近10年来，国外报道逐渐增多，而国内鲜有介绍，现报告1例。
  • 摘要：目的了解郑州市区3—5岁学龄前儿童乳牙猛性龋罹患状况，为群体防治猛性龋提供依据。方法采用整群、分层随机多阶段抽样方案抽取郑州市区幼儿园适龄儿童1866例对猛性龋罹患情况进行调查，并对数据进行统计分析。结果所检儿童整体患龋率为51．28％，猛性龋患病率为20．20％。随年龄增长，猛性龋患病率、龋均及龋面均逐渐增加，各年龄组之间的差异有统计学意义（患龋率：F=5．463，P〈0．05；龋均：F=3．693，P〈0．05；龋面均：F=4．432，P〈0．05）。龋齿充填构成比随年龄增长逐渐增加，差异有统计学意义（F=10．16，P〈0．05）。结论郑州市区学龄前儿童总体患龋率及猛性龋发病率较高，龋齿充填率较低。应尽早加强对学龄前儿童龋病的群体防治。
  • 摘要：生酮饮食（KD）是治疗儿童难治性癫痫的重要方法之一，其安全性受到人们的高度重视。实施KD前，应排除某些遗传代谢性缺陷病，如脂肪酸线粒体转运障碍疾病、β-氧化作用缺陷症等；并对患儿的全身状况进行细致的检查和评估，知情告知等。KD的常见早期并发症为脱水，其次是胃肠功能紊乱，包括恶心、呕吐、腹泻或便秘等；少见不良反应有低血糖、酸中毒、血脂异常、暂时性高尿酸血症、低蛋白血症、低镁血症、反复低钠血症、高密度脂蛋白水平降低等；急性胰腺炎及持续性代谢性酸中毒等罕见。后期并发症主要有骨质疏松、肾结石、心脏病变等，对生长发育的影响尚不明确。大部分早期和晚期并发症都为暂时性，经过严密观察、随访和预防处理及对症治疗，一般不影响KD的实施，且愈后良好。
  • 摘要：目的分析常染色体显性遗传夜间发作性癫癎（ADNFLE）患者CHRNA4基因序列的变异性，探讨其与ADNFLE发病的关系。方法收集临床诊断为ADNFLE的主要来源于广东和广西的6个家系，其总成员66例，发病24例，同时收集健康志愿者200例作为健康对照。提取二基因组中DNA并特异性扩增CHRNA4的1～6号外显子片段，对其进行纯化与测序。对测序结果进行序列对比分析。采用SPSS13．0软件进行统计学分析。结果在CHRNA4基因第5号外显子中发现4个碱基替换．c.909T〉G、c．1440G〉T、c．1458T〉C及c．942C〉T，均为同义替换。前3种替换属纯合变异，在24例ADNFLE患者和200例健康对照者中均存在，发生率为100％，而942C〉T替换仅出现在1例患者中，属杂合变异。结论ADNFLE患者CHRNA4基因1—6号外显子片段中均未发现已报道的热点突变。CHRNA4基因是否参与ADNFLE的发病，需进一步扩大样本量和进行功能学的研究。
  • 摘要：目的探讨自闭症患儿的生存质量状况及其影响因素，为临床干预提供理论依据。方法采用儿童生存质量自评式测定量表（PedsQL4．0），分别对200例自闭症患儿（自闭症组）及120例健康儿童（健康对照组）进行生存质量评定，应用Gesell发展诊断量表（1981年）进行智能发育测验，应用克氏自闭症行为量表、自闭症儿童行为检查量表、儿童自闭症评定量表评定自闭症病情程度。结果自闭症组儿童的生存质量明显低于健康对照组儿童，自闭症组儿童的生理功能、情感功能、社会功能、心理领域及PedsQL生存质量总分均低于健康对照组儿童，差异均有统计学意义（P均〈0．01）；高功能自闭症患儿各方面及总体生存质量均高于低功能自闭症患儿，差异均有统计学意义（P均〈0．01）。不同的智能类型因素对患儿的生存质量有影响，差异有统计学意义（P〈0．01）。自闭症患儿病情程度越重、智力水平越低，生存质量的损害越严重，差异有统计学意义（P〈0．05）。结论自闭症儿童生存质量存在明显的损害，需要社会高度关注这类疾病患儿的家庭。
  • 摘要：目的探讨儿童大脑后部可逆性脑病综合征（PRES）的临床及头颅影像学特点。方法回顾性分析7例PRES患儿的临床和头颅影像学资料。结果患儿的原发病分别为自身免疫性疾病4例、肾脏疾病2例和Blau综合征1例；6例应用过细胞毒性药物；3例有继发性高血压。临床表现：血压增高7例，惊厥发作7例，头痛、呕吐4例，意识障碍4例，视觉异常3例，偏瘫1例。5例短期内好转，1例遗留认知功能损害，1例死亡。2例复发。7例头颅MRI均示顶、枕叶皮质及皮质下白质受累，同时累及额叶3例，岛叶、颞叶、海马、丘脑、胼胝体各2例，基底核1例；6例信号特点为长T1加权成像（T1W）、长T2加权成像（T2W）、T2自由水抑制（T2FLAIR）高信号、弥散加权成像（DWI）等信号，1例为等T1W、长T2W、T2FLAIR高信号、DWI高信号。4例8d内复查MRI，其中3例病灶基本消失，1例无明显变化，3个月随访时MRI示病灶范围稍缩小，且有脑萎缩。结论儿童PRES异质性强，并非均可逆，具有多样的临床表现和头颅影像学特点；强调早期诊断、积极有效的治疗，以防止发生不可逆的神经系统损害。
  • 摘要：目的研究脂多糖（LPS）诱导的小鼠急性肺损伤（ALI）／急性呼吸窘迫综合征（ARDS）肺组织中microRNA表达谱的改变，探讨microRNA在ALI／ARDS发生机制中的作用。方法C57BL小鼠24只，随机分为实验组和对照组，每组12只。实验组小鼠通过气管切开经呼吸道向肺内注入LPS（6mg／kg），构建小鼠ALI/ARDS模型。对照组用同样的方法注入相应剂量的9g／L盐水。所有小鼠均于手术后24h处死，取左肺组织用于测量干湿质量比，右中肺叶行HE染色观察病理变化。应用microRNA芯片技术检测对照组及实验组小鼠肺组织microRNA表达谱的差异，用生物信息学方法进行靶基因预测及信号通路分析。结果与对照组比较，实验组小鼠出现明显的呼吸系统症状，肺组织干湿质量比明显升高（P〈0．01），病理结果符合ALI／ARDS特征。芯片结果显示ALI／ARDS小鼠肺组织中明显差异表达的microRNA有48个，其中上调的有27个，下调的有21个，这些microRNA的靶基因可能参与调节炎症相关的信号通路。结论在ALI／ARDS小鼠模型中，部分microRNA的表达发生明显的变化，microRNA在ALI／ARDS病理生理过程中起重要作用。
  • 摘要：目的探讨夜间较大剂量丙戊酸钠治疗夜间和清晨癫癎发作的疗效及安全性。方法选择69例新诊断的完全在夜间或清晨发作的癫痫患儿，随机分为试验组（35例）和对照组（34例）。均予丙戊酸钠片治疗，从小剂量开始，逐渐增加至有效维持量，分2次给药。试验组夜间剂量为清晨剂量的2倍；对照组夜间剂量与清晨剂量相同。随访6个月后评价疗效和不良反应。结果69例患儿全部完成观察和随访。试验组28例完全控制发作（80．0％），总有效率为85．7％；对照组20例完全控制发作（58．8％），总有效率为64．7％。2组总有效率比较差异有统计学意义（P〈0．05）。2组均未见严重不良反应，试验组日间嗜睡发生率（0／35例）低于对照组（2／34例）。结论夜间较大剂量丙戊酸钠能更好地控制儿童夜间和清晨癫癎发作，同时稳定夜间睡眠，减少13间嗜睡，不良反应短暂轻微。
  • 摘要：Objective To investigate the efficacy of continuous blood purification (CBP) in treatment of acute respiratory syndrome (ARDS) in children,and the methods as well as the key points in nursing care.Methods There were 97 cases of ARDS,and 18 cases were treated with CBP.The model for CBP was continuous veno-venous hemofiltration dialysis (CVVHDF) or high volume hemofiltration (HVHF).The observational index included arterial partial pressure of oxygen [pa(O2)],arterial partial pressure of carbon dioxide [Pa(CO2)],mechanical ventilation parameters,complications and side reaction during CBP.Results The pediatric critical illness score in CBP treatment group and non-CBP treatment group were (62.5 ± 25.2) and (78.1 ± 16.3) respectively,and the difference between the 2 groups was significant (P ＜ 0.05) ; the median of pediatric risk of score mortality Ⅲ score in CBP treatment group and non-CBP treatment group were 16(10-20) and 12(7-14),and the difference was significant between the 2 groups (all P ＜0.05).The average duration of CBP in 18 cases was 58 hours(12-232 hours),death occurred in 5 cases,and 2 cases of them were due to giving up treatment,the mortality was 27.8％ ;while in non-CBP treatment group,21 cases occurred death and the mortality was 26.6％.The difference of mortality between the 2 groups was not significant (P ＞ 0.05).Pa (O2)/fraction of inspired oxygen (FiO2) and dynamic compliance (Cdyn) were improved after 2-6 hours CBP,and the pulmonary exudation alleviated after 2 hours treatment.Mechanical ventilation parameters including FiO2,peak inspiratory pressure and positive end expiratory pressure were reduced.The complications related with CBP treatment included aggravating anemia,thrombopenia,lower body temperature,hemorrhage,oliguria,thick sputum,pain and dysphoria.No serious lethal complication was observed.Conclusions CBP treatment is a safe and effective rescue method for ARDS in children,it can reduce pulmonary edema,improve pa (O2)/FiO2 and Cdyn,and improve mechanical ventilation parameters.During CBP therapy,the key points include monitoring vital sign and the changes of hemodynamic,keeping body position nursing care well,tranquilizing,as well as maintaining the pipe unobstructed.%目的 探讨连续性血液净化(CBP)治疗儿童急性呼吸窘迫综合征(ARDS)的作用,管理与方法.方法 ARDS患儿97例,对其中18例在常规治疗基础上进行CBP治疗,模式为连续性静-静脉血液滤过透析(CVVHDF)或高容量血液滤过(HVHF).观察CBP治疗期间动脉氧分压[pa(O2)]、动脉二氧化碳分压[pa(CO2)]等机械通气参数,及CBP治疗相关并发症与不良反应.结果 CBP患儿危重病例评分(PICS)为(62.5±25.2)分,中位儿童死亡危险因素评估(PRISMⅢ)值为16(10 ～20)；非CBP治疗组PICS评分和中位PRISMⅢ值分别是(78.1±16.3)分和12(7 ～14),2组比较差异均有统计学意义(P均＜0.05).18例患儿CBP治疗平均持续时间为58 h(12～232 h),死亡5例,其中2例为放弃抢救后死亡,病死率为27.8％；同期79例未进行CBP患儿中,死亡21例,病死率为26.6％.CBP治疗组和非CBP治疗组病死率比较差异无统计学意义(P＞0.05).CBP治疗2～6h后,患儿pa(O2)/吸入氧体积分数(FiO2)和肺动态顺应性(Cdyn)较治疗前明显改善,6h后X线见肺部渗出减轻,呼吸机主要参数包括FiO2、吸气峰压和呼气末正压开始下调.与CBP治疗有关的并发症:贫血加重、血小板减少、低体温、出血、少尿、痰液黏稠、疼痛与躁动等,未发生致死性严重并发症.结论 采用CBP治疗ARDS是安全有效的抢救方法,可以减轻肺水肿,提高pa(O2)/FiO2和Cdyn,及时降低机械通气参数.CBP治疗过程中,严密观察生命体征和血流动力学变化,做好体位护理,采用合适的镇静措施,维护管路通畅是保证CBP治疗顺利运行的关键.
  • 摘要：儿童多发性硬化近几年来受到越来越多的关注,现对其流行病学、病因及发病机制、临床特点、影像特征、诊断标准、治疗及预后等方面进行简要综述.%The awareness of pediatric multiple sclerosis had increased during the past 10 years.Now,the current state of knowledge on pediatric multiple sclerosis,including pathogenesis,clinical manifestations,MRI features,diagnosis,treatment and prognosis were summarized.
  • 摘要：Objective To study the surgical treatment and outcome of anomalous origin of one pulmonary artery branch(AOPA).Methods Twenty-two patients (13 boys and 9 girls)with AOPA underwent surgical treatment,the age ranged from 1.5 months to 19.0 years old.Fifteen patients had anomalous origin of right pulmonary artery (AORPA),7 patients had anomalous origin of left pulmonary artery(AOLPA).The aorta-pulmonary window or interrupt aorta arch was found in the patients with AORPA,and the tetralogy of Fallot was only found in the patients with AOLPA.Eighteen procedures were performed by median sternotomy and cardiopulmonary bypass(CPB),4 cases were performed by off-CPB.The anomalous pulmonary artery was cut from the aorta at its origin and anastomosed to the lateral aspect of the main pulmonary artery,which sometimes artificial grafts or autologous pericardial were employed.Results One patient died during the operation because of crisis of pulmonary hypertense,and another patient died postoperatively of serious sepsis,the others were discharged healed.The mortality rate was 9％.The follow-up periods ranged from 6 to 149 months.All the patients were excellent but 1 patient was found a mild stenosis in the originate of right pulmonary artery.Conclusions It is important to correctly diagnosis and surgically treat for the patients with AOPA in time.A suitable surgical option by the pathological variation can obtain excellent result.%目的 研究外科治疗一侧肺动脉起源于主动脉(AOPA)的手术方法及疗效.方法 AOPA患儿22例接受外科矫治手术.其中男13例,女9例；年龄1.5个月～19.0岁.其中右肺动脉异常(AORPA) 15例,左肺动脉异常(AOLPA)7例.合并畸形中,主肺动脉窗和主动脉弓中断只见于AORPA组,而法洛四联症只见于AOLPA组.18例经正中开胸,体外循环下矫治,4例在非体外循环下矫治.切下的异常肺动脉通过直接吻合或人工血管及自体心包连接到主肺动脉.同期矫治心内其他畸形.结果 术中死亡1例,死因为肺动脉高压危象.1例术后死于肺部感染,其余均治愈出院,病死率为9％.随访6 ～ 149个月,仅1例右肺动脉起始部有轻度狭窄,其余未见异常.结论 对一侧肺动脉起源异常进行正确诊断,尽早进行外科干预.根据不同病理类型,选择合适的手术方式,可获得满意的治疗效果.
  • 摘要：生酮饮食(KD)疗法治疗癫(癎)的适应证包括葡萄糖载体蛋白1缺陷症,丙酮酸脱氢酶缺陷症,肌阵挛-失张力癫(癎),结节性硬化症,Rett综合征,严重的婴儿肌阵挛型癫(癎),婴儿痉挛症,Landau-Kleffner综合征,Lafora病,其他类型难治性癫(癎),难治性癫(癎)持续状态,部分线粒体病,Ⅴ型糖原病,亚急性硬化性全脑炎等疾病.脂肪代谢途径先天缺陷的患者不能使用KD疗法.近来,KD正在不同年龄段和多种新的适应证如肿瘤、老年痴呆等进行研究.KD疗法的并发症少,多数通过保守治疗是可逆的.从普通饮食过渡到KD需要1～2周,2∶1～4∶1的饮食可以产生有临床治疗作用的酮症状态；推荐至少尝试3个月,无效才放弃；有效的遗传性疾病往往需长期使用KD疗法.从KD恢复到普通饮食通常需2～3个月.
  • 摘要：目的 对3例分别因高肾素血症并发高血压、急性淋巴细胞白血病(ALL)诱导缓解化疗阶段引发的儿童大脑后部可逆性脑病综合征(PRES)患儿的临床表现、影像特征、脑电图特点及治疗结果进行分析,结合文献复习,了解儿童病例的特点.方法 回顾性分析3例PRES患儿的相关病因、临床表现、头颅MRI、脑电图及预后.结果 3例患儿的临床表现包括感觉异常、头痛、(癎)性发作、昏迷、视觉障碍、高血压或血压高于基准25％以上；3例患儿头颅MRI均显示顶枕叶对称性受累,其中例2患儿的MRI示双侧额颞部亦广泛受累；例2及例3患儿的脑电图示(癎)性波,例3患儿出现癫(癎)持续状态(SE),脑电图显示非惊厥性SE(NCSE).例1经降血压处理临床症状消失,复查MRI未见异常；2例ALL患儿均停用化疗药物,例3在急性期使用地塞米松并 加用多种抗癫(癎)药物治疗；例2脑病症状短期内缓解,随访3年无神经系统异常表现；例3在随访1.5年后仍有频繁癫(癎)发作,MRI示左侧枕颞区为主的后头部皮质萎缩海马硬化.结论 血压增高和ALL诱导化疗药物是本组PRES病例重要因素；儿童病例惊厥发生率高；PRES并不完全可逆,SE和NCSE可遗留后遗症如难治性癫(癎)；大剂量糖皮质激素可能诱发PRES,治疗PRES时需慎用.%Objective Three patients with posterior reversible encephalopathy syndrome (PRES) in childhood were reviewed retrospectively in this study.There were 1 case (case 1) with hypertension due to hyperreninism,and 2 patients (case 2 and case 3)with induction chemotherapy for childhood acute lymphoblastic leukemia (ALL).Clinical radiological features,electroencephalogram (EEG) data and neurological outcomes of the cases were analyzed with related literature reviews to highlight the clinical features of PRES in childhood.Methods Retrospective analysis was made in terms of etiology,clinical manifestations,magnetic resonance imaging (MRI) scanning of the brain,EEG and neurological outcomes of 3 cases of PRES.Results Present symptoms included seizures,altered mental function,headache and visual disturbance,with hypertension or the blood pressure 25％ higher than the baseline.Typical PRES findings were seen in 2 patients,consisting of bilateral symmetrical in parietal and occipital lobes,and 1 case with extensive involvement in bilateral frontotemporal lobe in adition.Case 2 and case 3 had epileptiform discharges on their EEG,while continuous EEG monitoring found non-convulsive status epilepticus (NCSE) in case 3 who had experienced status epilepticus (SE).Case 1 showed complete recovery after antihypertensive treatment during the acute stage; Associated offending chemotherapeutic agents were withdrawn in both case 2 and case 3,while dexamethasone plus variety of anti-epileptic agents were provided in case 3,and case 2 remained neurologically normal during the following 3 years.Case 3 subsequently developed refractory epilepsy and was found with hippocampal sclerosis in MRI after 1.5 years.Conclusions Hypertension and ALL induction chemotherapy regimens comprised the main predisposing factors for PRES in these cases.There is a high rate of convulsions incidence in children.PRES is not fully reversible,SE and NCSE during acute stage will cause sequelae and refractory epilepsy.High-dose steroids may trigger PRES,which should be used with caution in treatment of PRES.
  • 摘要：目的 探讨血清超敏CRP(hs-CRP)、IL-6和粒细胞-巨噬细胞集落刺激因子(GM-CSF)水平在肺炎支原体肺炎(MPP)患儿病情及疗效评估中的作用.方法 收集MPP患儿60例为MPP组,选取同期体检的30例健康儿童为健康对照组.应用放射免疫法和免疫比浊法检测2组儿童血清hs-CRP、IL-6和GM-CSF水平.结果 与健康对照组比较,MPP组患儿血清hs-CRP、IL-6和GM-ESF水平均显著升高(P均＜0.05)；MPP组患儿治疗前临床肺部感染评分≤6分者与＞6分者比较,hs-CRP、IL-6和GM-ESF水平显著增高,差异均有统计学意义(P均＜0.05)；治疗前MPP患儿血清hs-CRP、IL-6和GM-ESF水平明显高于治疗后水平,差异均有统计学意义(P均＜0.05).MPP患儿血清hs-CRP水平与IL-6、GM-CSF水平呈正相关(r=0.6012、0.5784,P均＜0.01).结论 血清hs-CRP、IL-6和GM-CSF参与肺炎支原体感染的全过程,监测这些细胞因子的变化有助于诊断、治疗和预后.
  • 摘要：生酮饮食(KD)是治疗儿童难治性癫(癎)的重要方法之一,其安全性受到人们的高度重视.实施KD前,应排除某些遗传代谢性缺陷病,如脂肪酸线粒体转运障碍疾病、β-氧化作用缺陷症等；并对患儿的全身状况进行细致的检查和评估,知情告知等.KD的常见早期并发症为脱水,其次是胃肠功能紊乱,包括恶心、呕吐、腹泻或便秘等；少见不良反应有低血糖、酸中毒、血脂异常、暂时性高尿酸血症、低蛋白血症、低镁血症、反复低钠血症、高密度脂蛋白水平降低等；急性胰腺炎及持续性代谢性酸中毒等罕见.后期并发症主要有骨质疏松、肾结石、心脏病变等,对生长发育的影响尚不明确.大部分早期和晚期并发症都为暂时性,经过严密观察、随访和预防处理及对症治疗,一般不影响KD的实施,且愈后良好.
  • 摘要：目的研究外科治疗一侧肺动脉起源于主动脉（AOPA）的手术方法及疗效。方法AOPA患儿22例接受外科矫治手术。其中男13例，女9例；年龄1．5个月-19．0岁。其中右肺动脉异常（AORPA）15例，左肺动脉异常（AOLPA）7例。合并畸形中，主肺动脉窗和主动脉弓中断只见于AORPA组，而法洛四联症只见于AOLPA组。18例经正中开胸，体外循环下矫治，4例在非体外循环下矫治。切下的异常肺动脉通过直接吻合或人工血管及自体心包连接到主肺动脉。同期矫治心内其他畸形。结果术中死亡1例，死因为肺动脉高压危象。1例术后死于肺部感染，其余均治愈出院，病死率为9％。随访6～149个月，仅1例右肺动脉起始部有轻度狭窄，其余未见异常。结论对一侧肺动脉起源异常进行正确诊断，尽早进行外科干预。根据不同病理类型，选择合适的手术方式，可获得满意的治疗效果。
  • 摘要：生酮饮食（KD）是一个高脂、低碳水化合物和适当蛋白质的饮食，尽管其治疗难治性癫癎的疗效和安全性得到公认，但严格的饮食控制降低了其耐受性和可操作性。“改良的阿特金斯饮食”和“低血糖生成指数治疗（LGIT）”是近年在KD基础上发展起的2种新疗法。其本质上也是一种高脂、低碳水化合物的饮食，但脂肪比例较低，患者更易耐受，操作更简便，疗效接近KD，不良反应较KD小，可作为成人和儿童难治性癫癎患者的首选饮食疗法，也可以与KD互换，具有广泛的应用前景。作用机制与KD一样尚不明确，但LGIT的研究提示血糖可能在饮食治疗中起一定作用。

    中华实用儿科临床杂志的期刊信息

    • 曾用名：实用儿科临床杂志
    • 创刊时间：1986
    • 地区：CN
    • 语言：中文
    • 热门主题：中华医学会
    • 学科分类： 儿科学;

    中华实用儿科临床杂志的获奖情况

    • • 中国科技论文统计源期刊
    • • 临床医学类核心期刊
    • • 河南省优秀科技期刊二等奖

    中华实用儿科临床杂志的收录情况

    • • 北大核心期刊（2004版）
    • • 北大核心期刊（2008版）
    • • 北大核心期刊（2011版）
    • • 北大核心期刊（2014版）
    • • 北大核心期刊（2017版）
    • • 中国科学引文数据库（2013-2014）
    • • 中国科学引文数据库（2015-2016）
    • • 中国科学引文数据库（2017-2018）
    • • 中国科学引文数据库（2019-2020）
    • • 日本科学技术振兴机构数据库
    • • 中国科技核心期刊

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    • 期刊栏目：专家论坛；论著；小儿神经基础与临床；儿童保健；小儿外科；药物与临床；讲座与综述；临床应用研究
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