静脉注射丙种球蛋白治疗早产儿感染的研究

为了探讨静脉注射丙种球蛋白（ＩＶＩＧ）预防及治疗早产儿感染的价值，用单向免疫琼脂扩散法检测５１例出生１～３天（胎龄２８～３６周）的早产儿血清ＩｇＧ、ＩｇＡ、ＩｇＭ。结果显示ＩｇＧ均值随胎龄增加而升高（ｒ＝０．９９，Ｐ＜０．０１），２８～３６周者＜８．０ｇ／Ｌ（２８周仅４．５ｇ／Ｌ），＞３６周者＞８．０ｇ／Ｌ；而ＩｇＡ、ＩｇＭ差异无显著意义（２８～３６周者ＩｇＡ均值为６１５ｍｇ／Ｌ，＞３６周者为６２１ｍｇ／Ｌ；２８～３６周者ＩｇＭ均值为４２３ｍｇ／Ｌ，＞３６周者为４１８ｍｇ／Ｌ），，故胎龄越小越有应用静脉丙种球蛋白（ＩＶＩＧ）的指征。３０例重症感染的早产儿参考相似的孕周、体重及日龄，随机分为ＩＶＩＧ组及对照组各１５例，均选用同类抗生素，且不用其它免疫制剂及血浆治疗，ＩＶＩＧ组按每天０．５～１ｇ／ｋｇ稀释成５０ｍｌ在２～３小时内静脉滴注，连用２天，ＩＶＩＧ组治疗１周后均值从８．２ｇ／Ｌ升到１３．５ｇ／Ｌ（Ｐ＜０．０１）；而对照组治疗后ＩｇＧ均值仅６．７ｇ／Ｌ。提示早产儿感染用ＩＶＩＧ疗效十分满意。     

肺炎和腹泻患儿亚临床维生素A缺乏的研究

为探讨肺炎和腹泻患儿体内维生素Ａ（ＶＡ）状况及其与疾病的关系，对１２８例肺炎、１１６例腹泻患儿和１００名健康儿童测定血清ＶＡ，其均值分别为１．１±０．５μｍｏｌ／Ｌ，０．８±０．４μｍｏｌ／Ｌ和１．９±０．６μｍｏｌ／Ｌ（Ｆ＝１４６．６７０，Ｐ＜０．０１）。对其中６０例肺炎、８５例腹泻患儿进行ＶＡ的相对剂量反应（ＲＤＲ）测定，其ＲＤＲ均值分别为２２％±１９％，２３％±１０％，亚临床ＶＡ缺乏（ＰＶＡＤ）检出率分别为４３．３％，６１．２％。发病１，２～４，５～７天即入院的肺炎、腹泻患儿在入院次日所测的ＲＤＲ均值和ＰＶＡＤ检出率均相近（肺炎患儿的Ｈｃ＝２．５６０，χ２＝０．２６２，Ｐ均＞０．０５；腹泻患儿的Ｈｃ＝０．２２９，χ２＝０．５１０，Ｐ均＞０．０５）。在诊断肺炎、腹泻患儿ＰＶＡＤ时，均以ＲＤＲ法检出率为高。提示：肺炎、腹泻患儿血清ＶＡ水平低于健康儿童，约半数的患儿体内处于ＰＶＡＤ状态。在发病７天内体内ＶＡ水平不受病程影响。评价体内ＶＡ状况以ＲＤＲ法更为敏感可靠。     

嘉兴市20436例新生儿先天性心脏病流行病学调查

为取得嘉兴地区代表性的新生儿先天性心脏病（简称先心病）发病情况，对１９９２年１月１日～１９９５年１２月３１日嘉兴市市区产科出生的全部活产婴儿２０４３６例进行前瞻性调查。检出先心病２９４例，其中彩色多普勒超声心动图诊断２８４例，尸检确诊１０例。患病率１４．３９‰。以单纯性房间隔缺损（ＡＳＤ）２９．６％和单纯性室间隔缺损（ＶＳＤ）１９．４％居多，其中又以Ⅱ孔型ＡＳＤ和膜部ＶＳＤ最多。复合畸形占３７．１％，以动脉导管未闭（ＰＤＡ）＋ＡＳＤ（１１．９％）居多。出生～７天发生心力衰竭３６例，新生儿期死亡２４例，死亡率１．１７‰。以青紫型复杂畸形为主。提示：先心病的发生与母妊娠早期呼吸道感染有关（χ２＝２１．８４，Ｐ＜０．０１），ＰＤＡ与围产期窒息密切相关（χ２＝２８．００，Ｐ＜０．０１）。     

新生儿窒息多器官损害的临床研究

为了解窒息后多器官损害的发生率、临床特点及围产因素与多器官损害间的关系，对本院１４７例窒息新生儿进行了分析。结果：（１）窒息后器官损害的发生率为７４．８％，脑损害的发生率为６５．３％，其中轻中度８１例，重度１５例，肾脏损害４２．９％，肺脏损害３７．４％，心脏损害３３．３％，胃肠损害５．４％；（２）轻度窒息的多器官损害为２８．１％，明显低于重症窒息的６９．６％（Ｘ２＝２５．２５，Ｐ＜０．０１）；（３）器官损害的发生率及严重程度与孕周、重度窒息、５分钟Ａｐｇａｒ评分＜５分及机械通气相关，而与羊水胎粪污染程度及宫内窘迫关系不大。提示：应加强围产期保健，预防早产，防止窒息，推广新法复苏及复苏后监护。     

应用血管活性药物辅佐抢救小儿MODS疗效分析

目的 探讨血管活性药物对小儿ＭＯＤＳ的治疗价值。方法 通过临床随机对照试验的方法,分出治疗组及对照组。治疗组在综合治疗的基础上应用血管活性药物,并在治疗过程中动态的对比两组患儿病情变化。结果 治疗１、３、７ｄ后,两组患儿危重评分有显著变化（Ｐ〈０．０１及〈０．０５）,治疗组病死率３２．１４％,治愈率６０．７１％。对照组病死率５７．１４％,治愈率２８．５７％。两组病死率比较有显著差异（χ＾２＝３．９     

新生儿窒息与多脏器功能障碍综合征的临床关系分析

目的 探讨新生儿窒息后多脏器功能障碍综合征（ＭＯＤＳ）的发生率、病死率及其与窒息程度、胎龄、出生体重的关系。方法 对１００例新生儿窒息进行前瞻性研究，观察轻、重度窒息组ＭＯＤＳ及单器官损害的发生率和病死率，以及围产因素与ＭＯＤＳ的关系。结果 重度窒息组ＭＯＤＳ的发生率为７１．４％，高于轻度窒息组（３０．８％）（χ２＝１５．２０，Ｐ＜ ０．０１）；重度窒息组脑、肺、心血管、肾、胃肠损害发生率分别为９１．４％，７７．１％，５４．３％，３４．３％，２２．９％，轻度窒息组分别为３８．５％，３３．８％，２０．０％，１２．３％，７．７％，差异均有显著性（Ｐ＜００１或 ０．０５），重度窒息组病死率（２２．９％）高于轻度窒息组（６．２％）（χ２＝６．０１，Ｐ＜０．０５）。窒息患儿中早产儿、低出生体重儿ＭＯＤＳ的发生率及病死率较足月儿、正常体重儿高，差异有显著性（Ｐ＜０．０５）。结论 重视围产期保健，降低早产儿、低出生体重儿的发生率，防止新生儿窒息，是减少ＭＯＤＳ的关键。     

幽门螺杆菌相关性胃炎患儿的食管胃pH值监测及部分胃肠激素研究

目的探讨幽门螺杆菌（Ｈｐ）相关性胃炎患儿的胃肠动力变化特点。方法应用放射免疫分析和食管胃ｐＨ监测技术对５８例Ｈｐ阳性和４７例Ｈｐ阴性慢性胃炎患儿的血清胃泌素、血浆胃动素、十二指肠胃食管返流等进行了对比研究。结果Ｈｐ阳性组血清胃泌素水平高于Ｈｐ阴性组和正常对照组（ｔ值分别为３．３２６、３．８３７，Ｐ均＜０．０１）；Ｈｐ阳性组血浆胃动素水平低于Ｈｐ阴性组和正常对照组（ｔ值分别为２．５９４、４．４３２，Ｐ＜０．０５和０．０１）。Ｈｐ阳性组胃食管返流发生率为２１％，低于Ｈｐ阴性组中的４８％（χ２＝３．８１２，Ｐ＞０．０５）；Ｈｐ阳性组十二指肠胃返流发生率为５０％，高于Ｈｐ阴性组中的１３％（χ２＝７．３８３，Ｐ＜０．０１）。结论Ｈｐ感染可以导致慢性胃炎患儿血中胃泌素水平升高和血中胃动素水平降低；Ｈｐ相关性胃炎患儿胃食管返流的发生有减少的可能，十二指肠胃返流的发生显著增多     

一氧化氮合酶mRNA在缺氧性肺动脉高压大鼠肺动脉的表达

目的探讨一氧化氮体系在缺氧性肺动脉高压形成机制中的作用。方法采用地高辛精标记的一氧化氮合酶（ＮＯＳ）ｃＲＮＡ探针对缺氧组大鼠（６只）及对照组大鼠（７只）进行原位杂交。结果缺氧２周后的大鼠肺动脉收缩压（３．８±０．７ｋＰａ）（２８±５ｍｍＨｇ，１ｋＰａ＝７．５ｍｍＨｇ）、肺动脉平均压（２．８±０．６ｋＰａ）及肺动脉舒张压（１．４±０．４ｋＰａ）与对照组（２．９±０．５ｋＰａ，１．９±０．５ｋＰａ及０．９±０．５ｋＰａ）相比均显著升高。缺氧组大鼠肺动脉内皮细胞中ＮＯＳｍＲＮＡ表达信号为弱阳性（３只）及阴性（３只），平滑肌细胞中表达信号均为阴性；对照组大鼠肺动脉内皮细胞中ＮＯＳｍＲＮＡ表达信号为阳性（７只），平滑肌细胞中表达信号均为阴性。ＮＯＳｍＲＮＡ的表达强度与大鼠肺动脉收缩压、肺动脉平均压及肺动脉舒张压分别呈负相关（ｒｓ＝－０．６７３、－０．５９６及－０．６２１，Ｐ均＜０．０５）。结论缺氧时肺动脉内皮细胞ＮＯＳｍＲＮＡ表达的改变可能参与慢性缺氧性肺动脉高压的形成。     

十二指肠胃上皮化生及其临床意义的研究

为探讨小儿十二指肠胃上皮化生的产生机理及其临床意义，对３１５例疑诊为上消化道疾病患儿的十二指肠球部活检粘膜标本做胃上皮化生（ＧＭ）及幽门螺杆菌（Ｈｐ）的组织学检测，并应用免疫组化法测定ＧＭ后胃窦部促胃酸激素产生细胞（Ｇ细胞）密度。结果显示，小儿十二指肠球部ＧＭ率为１２．１％（３８／３１５），活动性十二指肠炎组ＧＭ率和十二指肠球部Ｈｐ感染率为５０．０％（１５／３０）及１６．７％（５／３０），十二指肠球部溃疡组ＧＭ率和十二指肠球部Ｈｐ感染率为５２．４％（１１／２１）及２３．８％（５／２１），而慢性十二指肠炎和正常十二指肠球部粘膜组ＧＭ率仅占７．３％（１１／１５０）及０．９％（１／１１４）；且十二指肠球部未见Ｈｐ感染。胃窦部Ｈｐ阳性组十二指肠球部中重度ＧＭ率显著高于胃窦部Ｈｐ阴性组（χ２＝２７．３７，Ｐ＜０．０１），中重度ＧＭ其胃窦Ｇ细胞密度显著高于正常对照组（ｔ＝３．２０，Ｐ＜０．０１）。提示ＧＭ、Ｈｐ感染与小儿活动性十二指肠炎及十二指肠溃疡形成有较密切关系。但ＧＭ率及十二指肠球部Ｈｐ感染率均较成人低。中重度ＧＭ的发生与胃窦部Ｈｐ感染及Ｇ细胞密度增高有关。     

光疗对新生儿姊妹染色体单体交换及微核频率的研究

为探讨蓝光治疗对新生儿姊妹染色体单体交换（ＳＣＥ）及微核频率的影响，将蓝光治疗新生儿高胆红素血症患儿分为光疗＞７２小时的甲组（１６例），光疗≤７２小时的乙组（１８例）。另设对照组２０例，分别在光疗前、光疗后、恢复期检测了ＳＣＥ及微核水平。结果：（１）光疗前除甲组ＳＣＥ低于对照组外，余差异无显著意义（Ｐ均＞０．０５）；（２）甲组ＳＣＥ水平光疗后、恢复期均高于光疗前，且恢复期又高于光疗后（Ｐ均＜０．０１）；乙组光疗后、恢复期ＳＣＥ水平均有所下降；（３）光疗后、恢复期微核阳性率差异有显著意义（Ｐ＜０．０５，＜０．０１），且甲组高于乙组及对照组。提示光疗时间以７２小时内为宜，以避免光疗对染色体的损伤。     

关节内侧间隙测量对髋关节脱位术后稳定性的预测

目的探讨关节内侧间隙测量对髋关节脱位术后稳定性的预测意义。方法分析2004～2007年本院10例髋关节再脱位患儿以及随机抽取的50例术后未发生再脱位患儿的影像学资料,对其术后1d、1周、1．5个月、3个月、4个月、5个月、6个月骨盆平片进行患侧髋关节内侧间隙值OA以及泪滴至髋臼外缘的距离OA的测量,并采用D值（D=OA／OA）进行标准化处理。结果无再脱位组D值基本小于0．8。其中30例采用髋关节外展支具,未出现再脱位,D值位于0．66～0．8之间;脱位组在石膏同定期间,D值基本小于0．7,此时股骨头位于髋臼内,未出现脱位;术后6周至3个月拆除石膏后,D值为0．66．0．8,未采取措施,相继出现脱位,此时D值基本大于0．8。结论测量关节内侧间隙对于评价髋关节脱位术后关节的稳定性以及预测早期再脱位有重要意义。D值小于0．66,关节稳定,不会出现再脱位;D值为0．66～0．8,关节稳定性受到影响,可能出现再脱位,需尽早采取干预措施;D值大于0．8,出现再脱位,简单保守治疗措旆失去作用．需再次手术蚕新复位。     

Tacrolimus: the good, the bad, and the ugly

The aim of this study was to evaluate the efficacy and side-effects of tacrolimus in pediatric transplant patients previously receiving cyclosporin A (CsA). This study was a retrospective chart review strengthened by a concomitant patient interview. Eleven pediatric cardiac or renal transplant patients, who had been converted from CsA to tacrolimus from October 1995 to January 1999 at The Cleveland Clinic Foundation, were included; there were six renal and five cardiac transplant patients. Each chart was reviewed to assess transplanted organ function pre- and post-conversion. For the six renal transplant patients, creatinine levels and biopsy findings were evaluated. For the five cardiac transplant patients, cardiac catheterization and routine biopsy data were analyzed likewise. Epstein Barr virus (EBV) status was also evaluated in each patient. In addition, each parent or patient was interviewed to ascertain dates of transplant, current medications, and side-effects. The patients' ages ranged from 6 to 20 yr (mean age 14.6 yr). All patients had been converted to tacrolimus. Eight patients were converted for treatment of refractory rejection, two were converted because of CsA-associated side-effects, and one patient was converted empirically for a history of multiple previous transplant rejections. Seven out of eight patients who received tacrolimus for rejection therapy improved. One patient had complete resolution of gingival hyperplasia. Another patient who previously developed hemolytic uremic syndrome on CsA had no further evidence of hemolysis. Four patients were weaned off steroid therapy. Despite conversion, two renal transplant patients progressed to chronic rejection. Five patients exhibited no side-effects. Side-effects experienced included transient hyperglycemia in conjunction with steroid use, headaches, and tremors that subsided rapidly. Four of 11 patients developed post-transplant lymphoproliferative disease (PTLD). Fortunately, reducing the dose of tacrolimus and/or surgical resection of the mass (if present), eradicated the disease. In conclusion, conversion therapy successfully provides an alternate treatment for acute rejection. It also enabled some patients to discontinue steroid therapy, maximizing growth potential. PTLD is a severe, potentially life-threatening complication that needs to be recognized and monitored closely. In conclusion, tacrolimus has been shown to be a very effective agent for the treatment of refractory organ rejection, but must be used cautiously.     

Neuroimaging in the developmental disorders: the state of the science

The developmental disorders of childhood autistic, developmental language, reading (dyslexia), and attention deficit-hyperactivity disorders-manifest with deficits in the traditional behavioral domains of cognition, language, visual-spatial function, attention, and socialization. However, none of these disorders has been associated with characteristic discrete focal lesions or recognized encephaloclastic processes. Developmental cognitive neuroscientists must therefore begin with the spectrum of sometimes divergent behaviors occurring within these disorders and work backward in an attempt to identify the responsible anomalous neural systems. Since the advent of "brain imaging" two decades ago, much effort has focused on identifying brain-behavior correlates in these disorders. The results of these neuropathologic, structural, and functional neuroimaging studies are presented and the reasons for the often divergent findings are discussed. As we approach the end of the Decade of the Brain, current neuroimaging techniques give us the technology for the first time to apply a fundamental cognitive approach to brain-behavior relationships in the developmental disorders, to eliminate the conglomeration of "apples and camels" phenomenon. Researchers are working together to create comparable protocols and to adhere to methods that can be replicated across sites. The future prospects for a greater understanding of the developmental disorders are now much brighter with neuroimaging technology.     

抗癫(癎)药物对骨代谢的影响和防治研究进展

癫痫是神经科常见病,目前控制癫痫发作的主要手段是药物治疗。抗癫痫药物（anti—epileptic drugs,AEDs）对骨骼代谢的影响在上个世纪60年代末就有报道,近年来广大学者对此进行了深入的研究。现就国外关于AEDs对骨代谢的影响、监测和防治的研究作综述如下。     

Flaws in the assessment of the best interests of the newborn

We examined the literature on ethical decisions regarding neonates, to assess whether personal beliefs and prejudices influence end-of-life decisions taken by caregivers. Studies show that religion and familiarity with disability influence caregivers' decisions, whereas the influx of already being a parent, age, sex and professional experience is controverse. Caregivers' attitudes towards end-of-life decisions are also affected by personal concerns about litigation, prejudices and their view of disability. The concept of 'poor quality of life' is widely used as a reference in end-of-life decisions, but this can be interpreted differently, leaving room for a wide range of personal viewpoints. In most cases, parents' opinions are considered important and are sometimes the main determinant in decision making. However, it is unclear whether parents' decisions are based on their own wishes or on the best interests of the newborn.
Conclusion: In neonatal end-of-life decisions, patients may not receive cures based only on their best interests.     

Effect of the adaptation of the food of the hospitalized child on the cover of the nutritional needs]

AIMS: Denutrition remains a major concern in hospitalized children. Daily experience suggests that the meals proposed by hospital dietetic service, although well-balanced and in accordance with the recommendations, may be poorly accepted and consumed by children. The aims of this study were to assess the effect of modification of foods offer on energy intakes as well as nutriments and minerals and trace elements in hospitalized children. PATIENTS AND METHODS: During a 1-month period, 25 consecutive children (range 4-17 years; 13 girls), hospitalized in our pediatric department were included in the study (reasons for hospitalisation comprised: medical reasons [n=7], orthopedic problem [n=16] or surgery [n=2]). They had no restricted diet and received the usual pediatric hospital feeding according to the French recommended dietary allowances (RDA) (D1). They were compared to 21 children--matched for age, sex, nutritional status and pathology, hospitalized during the following 1-month period--who received a modified diet (D2), elaborated by dieticians according to the child's preference and excluded or limited food usually nonconsumed by the children. Food consumption was prospectively measured for 24h by analysis of the nonconsumed foods, as well as browsing and extra food brought by the family. Analysis of energy, carbohydrate, lipid, protein, iron and calcium intake was made using Bilnut 3 software (Nutrisoft, France). RESULTS: D2 covered 119+/-37% of the median energy needs versus 89+/-37% for D1 (p<0.05). The median energy needs were more often reached with D2 as compared to D1 (62% versus 32%, p<0.05). Protein intake was high in both groups, more importantly with D2 (266+/-111% of RDA versus 193+/-77% with D1, p<0.05). We observed no difference between the 2 diets in regards of fat/carbohydrate balance and iron intake. Calcium intake was increased with the adapted diet: 68+/-26% of RDA with D2 versus 49+/-26% with D1 (p<0.01). CONCLUSION: Adapting food offers to preference influences food and caloric intakes in hospitalized children. This could be an efficient strategy to prevent acute undernutrition in hospital.