С�����鲡��״�������˵�Σ������

目的探讨川崎病(KD)患儿合并冠状动脉损伤的危险因素。方法按照KD诊断标准,对北京儿童医院2000-01-01—2004-12-31收治的644例KD患儿临床资料、治疗方法以及实验室资料进行分析。结果Pear-son卡方检验显示,患儿性别、发热持续时间、丙种球蛋白使用时间、丙种球蛋白使用剂量、血沉及C反应蛋白与KD合并冠状动脉损伤有关(P<0·05);进一步多因素Logistic回归分析显示,性别、发热持续时间、丙种球蛋白使用剂量与冠状动脉扩张显著独立相关(P<0·05)。结论对男性、发热持续时间长的KD患儿应予足够重视,早期足量使用丙种球蛋白以减少或减轻冠状动脉并发症。     

������Σ�ػ���ת�����缰������

??Establishing a regional pediatric transport network is crucial to treatment of critically ill children and implementation of hierarchical medical system. The transport mainly include s communication before transport??assessing and stabilizing patients in local hospital??life support during transport and high quality handoff communication between physicians in receiving hospitals. The transport team members should master the pediatric emergency skills??and the transport equipment and supplies must be in good condition. The transfer center should carry out quality control to improve the quality of transport??and train medical staff in the region and carry out scientific research to improve treatment for critically ill children.     

�ͷ��Ӹ��ظ���ŧ��֢��Ѫ�����ٴ���Ч�۲�

??Abstract??Objective To evaluate the efficacy of low molecular weight heparin in patients with sepsis . Methods??A total of 24 patients with sepsis were randomly divided into LMWH treatment group and control group. Both groups were examined TAT??AT-??PC??PS??PLT??PT??APTT??Fbg??D-d??FDP and severity score. Results??PC and PS in these two groups were increased after treatments??and those of LMWH treatment group were increased more significantly than those of control group?? there was a significant difference between these two groups ??P < 0.001??. TAT in these two groups fell after treatments??and that of LMWH treatment group fell more significantly than that of control group?? but there was no statistic difference between these two groups ??P > 0.05??. Severity score in these two groups was increased after 2 days′treatment?? severity score in LMWH treatment group was increased more significantly than that of control?? there was a significant difference between these two groups ??P < 0.05??. Conclusion??Low dosage of low molecular weight heparin treatment in patients with sepsis is effective on improving function of hemorrhagic-coagulation and the clinical condition without complications of bleeding.     

����ѪҺ���������ϲ�ŧ�����ݿ�����Σ�����ط���

??Objective??To study clinical characteristics and risk factors for mortality of septic shock patients with hematological malignancies in pediatric intensive care unit??PICU??. Methods??A retrospective analysis of records of pediatric patients with hematological malignancy and septic shock was performed. In our study??43 cases admitted to PICU in Sun Yat-sen Memorial Hospital between March 2012 and September 2015 were included. Results??Among the 43 cases??16 died??while the other 27 survived. The percentage of mortality was 37.2%. Pulmonary infection ??74.4%?? and gastrointestinal tract infection??39.5%?? were the common causes of septic shock in these patients. Etiological examination revealed 43 kinds of pathogenic bacteria in 26 cases. The percentage of gram-positive bacteria??gram-negative bacteria and fungus was 11.6%??72.1% and 16.3%??respectively. Escherichia coli was found to be the main pathogen??6/43??14.0%??. Between the survival group and the mortality group??there were significant differences in the parameters of age??chemotherapy courses??time from infection to shock??time from neutropenia to shock??multiple organs dysfunction??pulmonary infection??hypocalcemia??oxygenation index and serum lactic acid concentration after 6 hours of fluid resuscitation??requirement of dopamine and mechanical ventilation ??P??0.05??. By logistic regression analysis??mortality was associated with the variables of serum lactic acid concentration after 6 hours of fluid resuscitation and requirement of mechanical ventilation. Conclusion??The risk factors for mortality of septic shock in pediatric patients with hematological malignancies are hyperlactacidemia after 6 hours of fluid resuscitation??hypocalcemia and requirement of mechanical ventilation.     

������θ������80���ٴ��ص㼰Ԥ�����

??Objective To compare the etiologic factors??clinical features and prognosis in newborns with gastrointestinal perforation. Methods A retrospective study of the clinical data from 80 cases with diagnosis of gastrointestinal perforation collected from January 2004 to December 2015 was performed. Based on whether they were full-term??the cases were divided into two groups??then the clinical features??etiologic factors and prognosis were compared. Results Among the 80 neonates with gastrointestinal perforation??there were 62 preterm infants and 18 full-term infants. The main causes of gastrointestinal perforation for the two groups was necrotizing enterocolitis??with the most frequent clinical symptom of abdominal distension.cases. Compared with full-term infants??the incidence of preterm shock??dyspnea??DIC??and poor reaction was significantly higher in preterm infants??P??0.05??.The mean time period of perforation in preterm infants group was 9??1.75??20?? d??while it was 4.5??1??7.75?? d in full-term infants group.There were 62 cases that received surgical treatment??8 cases of which were gastric perforation and 54 cases were intestinal perforation. The exact site of perforation of the 18 cases who refused surgical treatment was not clear. Of all the cases??32 infants died with the overall mortality rate of 40%. For the preterm infants??the mortality was 41.9% ??26 cases????while it was 33.3% ??6 cases?? in the full-term infants group. Conclusion Neonatal gastrointestinal perforation is one of the serious diseases in neonatal period??which has a very high mortality rate. Early diagnosis??active treatment and timely surgical intervention can significantly improve survival rates and improve the prognosis.     

��ͯ��Ѫϸ���ܰ���֯ϸ������֢ ��ҪΣ��������Ԥ���ϵ�о���չ

1概述噬血细胞淋巴组织细胞增生症(hemophagocytic lymphohistiocytosis,HLH)是一组单核-巨噬细胞系统反应性增生而发生过度的炎症反应,造成多组织器官损害的综合征。目前,其病因及发病机制未完全阐明。促炎症反应的细胞因子不断生成,巨噬细胞和细胞毒性T淋巴细胞浸润多组织是其最显著特点[1]。1.1发病率及发病机制HLH可分为家族性HLH     

��������֢һ�ڸ���������������Σ�����ط���

目的研究与法洛四联症(TOF)一期根治手术早期死亡相关的危险因素。方法回顾1995年4月至2004年3月在复旦大学附属儿科医院心血管中心行一期根治术的TOF病例152例,术后早期死亡17例,对其术前、术中27个可能的危险因素指标与术后早期死亡的关系进行单因素和多因素分析。结果单因素分析结果显示与术后早期死亡有关的指标包括手术时体重、发生青紫年龄、动脉血氧饱和度、升主动脉与肺动脉干直径比AO/MPA、McGoon比值、Nakata指数、肺体循环血流量之比Qp/Qs、右向左分流量占体循环血流量比例(QseQp)/Qs、体肺侧支循环和跨瓣补片。多因素分析结果显示AO/MPA和McGoon比值与手术早期死亡有关。结论肺动脉的发育情况是TOF一期根治术后能否生存的关键。手术病例的选择需要综合考虑多种危险因素。     

������Ѫ֬������̥�����������ಡ������ϵ̽��

??Objective To investigate the relationship between the presence of dyslipidemia in pregnant females and the risk of congenital heart defects??CHD??. Methods A total of 54 pregnant females at 24 to 28 weeks??w?? of gestation admitted between March 2013 and June 2014 in Department of Pediatric Cardiology??First Affiliated Hospital of SUN Yat-sen University were enrolled in the study . The case group included 18 females who had a fetus with cardiac defects??and each case was matched with 2 controls with no pregnancy complications. The mean ages were 29.06??SD=3.11?? years and 29.03??SD=3.9?? years in the case and control groups??respectively. The main outcome measurements were total cholesterol??TC????triglycerides??TG????low-density lipoprotein cholesterol??LDL-c????high-density lipoprotein cholesterol??HDL-c????apolipoprotein and homocysteine. Results Patients in the case group had a significantly higher mean LDL-c??4.15 mmol/L vs. 3.45 mmol/L?? and apolipoprotein B ??2.23 g/L vs. 1.78 g/L????all P??0.05?? than those in the control group. Correlation analysis indicated that LDL-C??r=0.32??P=0.017????apolipoprotein B??r=0.33??P=0.016?? and homocysteine??r=0.34??P=0.011?? were associated with fetal congenital heart disease.Conclusion Serum LDL-cholesterol and apolipoprotein B levels in the case group are significantly higher than those in the control group. Pregnancy dyslipidemia may be associated with a higher incidence rate of CHD.     

С����ŧ�˾���Ѫ֢����Σ�����ط���

目的探讨影响小儿绿脓杆菌败血症病死率的因素。方法回顾性分析1991～2003年间中山医科大学附属第一医院收治的16例14岁以下绿脓杆菌败血症患儿的临床资料。结果发热和腹泻为最常见的初始症状。病死率与性别、年龄、感染途径、是否化疗和使用激素、白细胞数无关(P>0.05),而与基础疾病的严重程度、有无严重并发症、有无低钠血症、高血糖症、血小板减少及有无敏感抗生素联合用药有关(P<0.05)。结论绿脓杆菌败血症是免疫功能低下患儿的常见危重并发症之一,病死率极高,早期经验性联合应用抗绿脓杆菌抗生素可能在一定程度上降低病死率。     

婴儿痉挛的视频脑电图特征分析

目的　分析婴儿痉挛的临床发作特征 ,探讨视频脑电图 (Video EEG)检查在诊断中的作用。方法　对 1999年 1月至 2 0 0 3年 10月中国医科大学深圳儿童医院收治的 6 1例婴儿痉挛患儿采用Video EEG监测 ,观察婴儿痉挛发作的临床表现和发作期以及发作间期的脑电图特征。结果　共监测到 87次痉挛发作 ,可见 4种表现类型 ,主要为屈肌型痉挛。发作时脑电图可见 7种形式 ,主要为快波节律或高波幅单个尖慢复合波 (或仅慢波 ) ,后随电压抑制。 13次发作仅出现 1种形式脑电图改变。 74次出现 2种或 2种以上形式的脑电图改变 ,最多可在一次发作中见到 4种形式脑电图改变。发作间期的脑电图背景活动均失去正常结构 ,以典型的高峰节律紊乱和变异型的高峰节律紊乱为主。在同一个患儿的监测中 ,可同时见到多种形式脑电图改变。结论　婴儿痉挛独特的轴性痉挛可表现为多种形式和合并其它形式发作。EEG在发作期和发作间期有多种形式 ,高峰节律紊乱是大多数婴儿痉挛的特征性脑电图形。Video EEG监测可为诊断和鉴别诊断婴儿痉挛提供依据     

Infantile spasms: Etiological factors,clinical aspects,and long term prognosis in 200 cases

The etiological factors, clinical aspects and long term prognosis were studied in 200 patients with infantile spasms. Forty-eight (24.0%) died and the rest were aged 6 years or more at the time of final follow-up. In 73 (36.5%) the etiology was prenatal, in 44 (22.0%) perinatal, and in 17 (8.5%) postnatal: 18 cases (9.0%) were cryptogenic. The remaining 48 (24.0%) patients were doubtful cases. The mortality of the pre-and perinatal cases at 35.6% and 34.1% respectively was significantly higher than that in the other etiologic groups (P<0.001).With regard to the onset of spasms, these were very carly in the pre-and perinatal groups, whereas in the postnatal group the onset was late. The onset in the doubtful and cryptogenic groups was in between. Some 44.4% of the cryptogenic cases showed normal mental and physical development after the age of 6 years, whereas less than 10% of the prenatal and perinatal group did so. Almost all of the doubtful cases became mentally subnormal.The incidence of a family history of epilepsy or other convulsive disorders in the first, second and third degree relatives was highest in the cryptogenic cases (40.0%), and lowest in the perinatal cases (9.3%) (P<0.01). The incidence of laughing attacks was highest in the postnatal cases (42.9%), against no such attacks in cryptogenic cases (P<0.01). The attacks seemed to be linked with organic brain lesions. Among various factors related to the etiology of infantile spasms, genetic propensity seemed especially important in cryptogenic cases.     

��Դ��Ӥ�����λ����ԴŹ�����̬ѧ��������

目的通过定量分析隐源性婴儿痉挛患儿脑灰质结构,寻找其发病原因和探讨发病机制。方法使用无创性的MR形态学定量对比分析技术,对2003-10—2004-10北京儿童医院23例隐源性婴儿痉挛(观察组)和27例同龄健康儿童(对照组)脑部高分辨解剖成像,进行灰质浓度的测定,比较两组之间的差异。结果两组灰质浓度比较,观察组在右额上回(10区)和中回(9区)、左额下回(47区)和上回(9、10区)、左额内侧(10区)、右颞上回(22、38区)、左颞中回(21区)以及右侧海马(36区)等灰质浓度减低,差异有显著性意义,P<0·001(未校正);双侧颞下回(20区)、左侧中央前回(6区)和左海马(36区)研究组灰质浓度增高,差异有显著性意义,P<0·001(未校正)。结论隐源性婴儿痉挛患儿额叶和颞叶灰质浓度明显减低,以额叶和颞叶为主的皮层发育不良可能是患儿致病的主要原因;在发病机制中皮层异常是发病的起源。     

促肾上腺皮质激素治疗36例婴儿痉挛症的疗效分析

目的分析促肾上腺皮质激素（adrenocorticotrop hichormone,ACTH）治疗婴儿痉挛症的疗效。方法收集我院2009年1月至2012年10月36例婴儿痉挛症患儿的临床资料,分析其ACTH治疗前后发作缓解率、脑电图改变及智力发育的改善情况。结果（1）治疗结束后5d、3个月、6个月的临床完全缓解率分别为86．1％（31／36）、77．8％（28／36）、50．0％（18／36）;有效率分别为11．1％（4／36）、13．9％（5／36）、36．1％（13／36）;无效率分别为2．8％（1／36）、8．3％（3／36）、13．9％（5／36）。脑电图完全缓解率分别为75．0％（27／36）、58．3％（21／36）、44．4％（16／36）。（2）治疗后3个月、6个月分别与治疗前比较,各能区功能、智龄及发育商均有不同程度的提高（P〈0．001）;治疗后6个月与治疗后3个月比较各能区功能、智龄及发育商均有提高（P〈0．001）。尤其在大动作及适应行为、社交行为方面进步显著。结论确诊婴儿痉挛症后及时应用ACTH治疗,对控制痉挛发作、短期内提高智力发育及改善脑电图具有积极的作用。     

大剂量甲泼尼龙对婴儿痉挛幼鼠脑组织的保护作用

目的 婴儿痉挛症(infantile spasms,IS)是婴儿期最常见的难治性癫痫性脑病,但相关动物实验缺乏,该研究通过构建IS动物模型,探讨大剂量甲泼尼龙对IS幼鼠脑组织的影响.方法 将生后10 d(P10)SD大鼠随机分为对照组、模型Ⅰ组和模型Ⅱ组,每组30只.模型Ⅰ组和模型Ⅱ组分别在P10、P13腹腔注射N-甲基-D-天冬氨酸(N-methyl-D-aspartate,NMDA)诱发癫痫发作,模型Ⅱ组在P13应用NM-DA前连用3 d甲泼尼龙.观察幼鼠癫痫发作并评分,原位末端标记法(TUNEL)检测凋亡情况,免疫组织化学法测Bax、Bcl-2蛋白表达、实时荧光定量PCR检测海马组织Bcl-2、Bax和caspase-3 mRNA的表达.结果 (1)P13模型Ⅰ组癫痫发作率83.3%(25/30),模型Ⅱ组均未出现发作,模型Ⅱ组较模型Ⅰ组癫痫发作率明显降低;(2)模型Ⅱ组凋亡细胞数(14.37±2.02)少于模型Ⅰ组(25.67±1.52),多于对照组(9.00±2.50),差异均有统计学意义(均P<0.05);(3)模型Ⅱ组Bax蛋白及mRNA表达量(44.55±3.58,2.35±1.01)明显低于模型Ⅰ组(58.05±4.62,3.27±0.95),高于对照组(28.90±5.14,1.68±0.50),差异均有统计学意义(均P<0.05);(4)模型Ⅱ组caspase-3mRNA表达量(5.99±1.75)低于模型Ⅰ组(7.88±1.60),高于对照组(3.60±1.70),差异均有统计学意义(均P<0.05).结论 大剂量甲泼尼龙能减轻NMDA致IS幼鼠癫痫发作;大剂量甲泼尼龙对NMDA致IS幼鼠具有脑保护作用,这种保护作用可能与其减轻癫痫发作,减少细胞凋亡有关.     

不同剂量促肾上腺皮质激素对婴儿痉挛症疗效和安全性的Meta分析

目的:评价不同剂量促肾上腺皮质激素（ACTH）治疗婴儿痉挛症（IS）的疗效及安全性。方法:检索PubMed、EMBASE、Ovid、ScienceDirect、中国期刊全文数据库、万方数据库、CiNii（国立情報学研究所論文情報ナビゲータ）和Medical Online等数据库,并手工检索会议论文汇编和专题论文集等,收集关于不同剂量ACTH治疗IS的RCT文献。依据随机分配方法、分配隐藏、盲法、结果数据的完整性、选择性报告研究结果及其他偏倚来源进行文献偏倚评价。采用Review Manager 5.0软件进行Meta分析,无法进行合并分析的资料进行描述性分析。结果:共检索到836篇相关文献,符合纳入标准的4篇RCT文献进入Meta分析。文献偏倚评价结果显示,1篇文献存在中度偏倚风险,3篇文献存在高度偏倚风险。Meta分析结果显示,不同剂量ACTH组痉挛发作缓解率（OR=1.09,95%CI：0.54~2.17,P=0.82）、EEG高度失律消失率（OR=1.04,95%CI：0.43~2.51,P=0.94）及复发率（OR=1.27,95%CI：0.64~2.54,P=0.50）差异均无统计学意义。进一步按症状性和隐原性IS行亚组分析,结果显示两亚组不同剂量ACTH上述疗效指标差异均无统计学意义。大剂量ACTH组高血压和体重增加等不良反应的发生率高于小剂量ACTH组。结论:现有证据提示,不同剂量ACTH治疗IS在痉挛发作缓解率、EEG高度失律消失率及复发率方面疗效相近。小剂量 ACTH所致不良反应更少。鉴于纳入Meta分析的RCT文献数量较少且质量较低,明确结论仍需进一步的RCT研究。     

促肾上腺皮质激素治疗婴儿痉挛55例临床研究

目的 总结首次应用促肾上腺皮质激素(ACTH)治疗婴儿痉挛的临床疗效、不良反应、用药前后的脑电图变化,为ACTH的普及应用提供临床借鉴.方法 对我中心既往3年间收治的首次应用ACTH治疗的婴儿痉挛患儿的临床资料进行回顾性分析.结果 (1)控制痉挛发作显效率为54.7%,好转率为15.1%,无效率为30.2%,且疗效与发病年龄、病程及病因有关;(2)不良反应有睡眠周期改变(85.5%)、皮肤颜色变深(81.8%)、心律不齐(65.5%)、血压升高(38.2%)、低钾血症(21.8%)、感染(20.0%)等;(3)用药前脑电图多以高峰节律紊乱为主要背景,用药两周后脑电图高峰节律消失41例(74.5%),好转14例(25.5%).结论 首次应用ACTH治疗婴儿痉挛疗效肯定,尤其可明显改善患儿高峰节律紊乱脑电背景,用药期间也存在一定的不良反应,应密切观察.     

视频脑电图评估促肾上腺皮质激素治疗婴儿痉挛症的疗效

目的 分析促肾上腺皮质激素(adrenocorticotrophic hormone,ACTH)治疗婴儿痉挛症(infantile spasms,IS)前后视频脑电图变化的特点.方法 收集我院2005年1月至2009年12月住院的65例IS患儿的临床资料,分析其用ACTH治疗前后视频脑电图变化的特点.结果 65例患儿用ACTH治疗前IS发作间期脑电图背景均出现高峰失律现象;丛集性痉挛发作频繁出现,并可见同期特征性发作图形.ACTH治疗后,40例(61.5%,40/65)完全控制发作的患儿中,27例(67.5%,27/40)脑电图高峰失律背景消失或明显好转;另25例(38.5%,25/65)仍有发作的患儿中,仅5例(20.0%,5/25)脑电图高峰失律背景消失或明显好转.完全控制发作和仍有发作患儿的脑电图高峰失律背景消失或好转率比较差异有统计学意义(x2=13.888,P＜0.000).结论 IS患儿发作前视频脑电图监测是正确诊断IS的重要依据,ACTH治疗后视频脑电图监测是判断短期疗效的重要指标.     

A proposed diagnostic approach for infantile spasms based on a spectrum of variable aetiology

AimTo identify the aetiology of patients with infantile spasms and propose practical guidelines for diagnostic strategies.MethodWe performed a retrospective study of children with West syndrome. Prenatal and birth medical history, characteristics of epilepsy, psychomotor development, biological and genetic screening, and aetiology were reported. Brain MRI was performed at least once and was repeated after two years of age if no aetiology was identified.ResultsEighty children were included. Aetiology was identified in 40 children: 17 with acquired cause (seven with stroke and six with hypoxic-ischaemic encephalopathy) and 23 with developmental pathology (seven with tuberous sclerosis, eight with cerebral malformations, and eight with various genetic abnormalities). The yield of brain imaging was high, providing a diagnosis for 32 patients. Two subtle brain lesions were detected only after two years of age, based on subsequent MRI. Genetic testing provided a diagnosis for the remaining eight patients.InterpretationAlthough this is a retrospective study, the results provide a basis to review the aetiology of infantile spasms and confirm the role of cerebral MRI in first-line diagnosis. Cases with a genetic aetiology have been diagnosed with increasing frequency due to better diagnostic capabilities. We propose guidelines for a practical diagnostic approach and discuss the relevant use of genetics in the future.     

Congenital microcephaly,infantile spasms,psychomotor retardation,and nephrotic syndrome in two sibs

Two boys are described with congenital microcephaly, infantile spasms, psychomotor retardation and an early-onset nephrotic syndrome. The autopsy findings of one patient are described in detail. Polymicrogyria was the most prominent feature and the kidneys showed focal segmental glomerulosclerosis. These findings have been described as a clinical entity, the leading symptoms being congenital microcephaly, early-onset nephrotic syndrome and mental retardation, accompanied by various other clinical symptoms. A review of the literature suggests an autosomal recessive mode of inheritance.