早期筛查诊断治疗的45例苯丙酮尿症患儿智力发育分析

目的了解经新生儿疾病筛查确诊治疗的苯丙酮尿症（PKU）患儿的智力发育情况与其临床治疗依从性关系。方法自1999年-2009年经新生儿疾病筛查确诊的苯丙酮尿症患儿45名,选用0-6岁儿童神经心理发育量表评估患儿智力发育情况。结果 45例PKU患儿,除2例放弃治疗、1例死亡之外,治疗的42例患儿,有73.8%患儿智能发育达正常水平。生后40 d内即开始治疗的11例PKU患儿,和家长完全配合治疗的31例患儿,全部智能发育正常。完全配合治疗组、不配合治疗组患儿智力发育程度存在显著性差异。结论新生儿疾病筛查是早期发现PKU患儿的有效方法 ,患儿开始治疗时间的早晚及家长配合治疗程度直接影响患儿治疗效果。若患儿能在生后1个月内治疗,同时家长给予配合,可以避免智力低下的发生。     

2000-2008年河北省新生儿苯丙酮尿症筛查结果分析

目的 分析河北省新生儿苯丙酮尿症(PKU)筛查状况、PKU发病率及治疗情况,总结经验,促进新生儿筛查工作.方法 以2000年1月-2008年9月在河北省11所市新生儿疾病筛查中心(新筛中心)进行PKU筛查的218万例新生儿作为分析对象,在出生后72 h采足跟血,分别采用细菌抑制法(BIA)、荧光法、定量酶法测定血苯丙氨酸(phe)水平.结果 218万例新生儿中,确诊PKU 249例,发病率为1:8 757,其中男婴145例,女婴104例,男女之比约为1.39:1,各年度的发病率比较差异无统计学意义(P＞0.05),各市的发病率比较差异有统计学意义(P＜0.05).确诊的138例PKU患儿接受正规治疗,治疗率为55.4%.结论 河北省是PKU高发区,新生儿疾病筛查是早发现、早诊断、早治疗PKU的重要措施,在今后的工作中要大力提高治疗率,使PKU患儿的智力和体格发育达到正常标准,减少残疾的发生,提高人口素质.     

综合干预措施在纠正阳春地区先天性甲状腺功能减退症儿童中的应用效果研究

目的观察干预对先天性甲状腺功能减退症(先天性甲低)儿童治疗效果。方法选取阳春地区2017年6～8月新生儿疾病筛查发现先天性甲低患儿62例作为研究对象,随机分为观察组与对照组,每组各31例,对照组患儿给予常规药物治疗及饮食指导,观察组在对照组基础上实施干预,持续干预1年后比较两组患儿体格发育情况、智力发育情况和心理运动发育情况。结果两组患儿的身高、体重比较差异无统计学意义(P 0.05),头围比较差异有统计学意义(P 0.05);两组患儿的大运动、精细运动、适应能力、语言、社交行为比较差异有统计学意义(P 0.05);观察组患儿第6、12个月末的运动发育评分均高于对照组,两组比较差异有统计学意义(P 0.05)。结论实施干预能够促进先天性甲低儿童的头围生长,提高其运动、语言能力,改善心理运动发育状况。     

早期中西医结合干预对改善早产儿运动发育迟缓的临床研究

目的:探讨早期中西医结合干预对运动发育迟缓早产儿的运动发育水平的影响,并对产生的机制进行讨论.方法:将2010年9月至2012年7月在我院进行康复评估、并确诊为运动发育迟缓的早产儿116例,分为治疗组66例及对照组50例,其中治疗组根据发育迟缓的表现制定干预治疗方案,对照组因不接受治疗而不予以干预,在治疗前、治疗后3月、6月、9月对2组早产儿均采用Peabody运动发育量表进行评估,观察粗大运动发育商(GMQ)、精细运动发育商(FMQ)、总体发育商(TMQ)的变化情况.结果:在初次评估后6月内,对照组的运动发育情况未显示出明显的统计学差异,在9月后,对比初次评估差异有统计意义(P＜0.05);治疗组在中西医结合干预3月后,即显示差异有统计意义(P＜0.05),且随着治疗时间的延长,统计学差异愈加显著.最终在治疗9月后,治疗组早产儿在GMQ,FMQ,TMQ上均明显优于对照组,差异有统计意义(P＜0.05).结论:早期中西医结合干预治疗能够有效提高早产儿运动发育水平,改善患儿的生存质量及生活质量,减轻社会、家庭及患儿个人的沉重负担.     

新生儿甲状腺功能低下症筛查及早期左甲状腺素治疗效果评价

目的探讨新生儿甲状腺功能低下症筛查及早期左甲状腺素的治疗效果。方法分析20992例新生儿甲状腺功能低下症的筛查结果。采用左甲状腺素钠片治疗确诊的患儿,对比治疗前后患儿的三碘甲状腺原氨酸(T3)、四碘甲状腺原氨酸(T4)和促甲状腺激素(TSH)水平的变化情况。同时观察患儿的体格和智力发育情况。结果本研究新生儿甲状腺功能低下筛查阳性率为0.27%,确诊24例(0.11%)。经过治疗后的T3、T4均显著高于治疗前,而TSH显著低于治疗前,差异具有统计学意义(P<0.05)。治疗后的身高、体重均可达到所对应的年龄标准,平均智商水平(106.64±12.91)分。结论早期筛查有助于新生儿甲状腺功能低下症的早期诊断,而左甲状腺素钠片的早期治疗可促进患儿体格、智力的正常发育。     

悬吊疗法对运动发育迟缓儿童粗大运动的影响

目的 探讨悬吊疗法对运动发育迟缓儿童粗大运动的影响.方法 选取2019年1月至2020年9月萍乡市妇幼保健院儿童康复科收治的运动发育迟缓患儿60例作为研究对象,采用随机数字表法分为对照组和观察组,每组各30例.对照组采用常规综合早期干预训练,观察组在对照组的基础上联合悬吊疗法.比较两组的粗大运动功能量表(GMFM-88)评分和Peabody运动发育量表(PDMS-2)评分.结果 治疗前,两组的GMFM-88区域A～E评分比较,差异无统计学意义(P＞0.05);治疗后,两组GMFM-88区域A～E评分高于本组治疗前,差异有统计学意义(P＜0.05);治疗后,观察组GMFM-88区域A～E评分高于同期对照组,差异有统计学意义(P＜0.05).治疗前,两组PDMS-2各项目评分比较,差异无统计学意义(P＞0.05);治疗后,两组PDMS-2各项目评分高于本组治疗前,差异有统计学意义(P＜0.05);治疗后,观察组PDMS-2各项目评分高于同期对照组,差异有统计学意义(P＜0.05).结论 悬吊疗法能够改善患儿的运动和神经发育,同时增强患儿依从性,运动发育迟缓患儿通过综合干预治疗后可减少日后神经系统异常的发生率,改善运动姿势控制能力,提高核心肌群力量,促进患儿早期康复.     

早期干预对重度缺血缺氧性患儿的疗效和预后影响

目的：探讨对重度缺血缺氧性患儿进行临床干预的疗效。方法：选取本院2006年7月～2008年8月收治的120例缺血缺氧性脑病患儿的临床资料,随机分为治疗组（60例）和对照组（60例）,两组患儿均按照常规治疗,治疗组患者进行早期干预,随访2～4年,比较两组患儿的体格发育情况、智力发育、听力、视力。结果：治疗组患者的平均智力发育指数和运动发育指数高于对照组患者,两组患者的疗效比较,差异有统计学意义（P〈0.05）。治疗组患儿听力视力明显优于对照组患儿,两组患儿听力视力比较,差异有统计学意义（P〈0.05）。结论：对重症缺血缺氧性脑病患儿进行早期干预,能明显促进患儿智力和体格发育,疗效满意,值得在临床推广。     

针灸配合小儿智力糖浆治疗精神发育迟滞疗效观察

目的探讨针灸配合小儿智力糖浆治疗精神发育迟滞患儿的临床疗效及安全性。方法 82例精神发育迟滞患儿,随机分为观察组(40例)和对照组(42例)。对照组给予精神发育迟滞康复治疗,观察组在此基础上实施针灸干预,应用儿童心理发育量表评价临床疗效。结果观察组总有效率为87.5%,显著高于对照组的78.6%,差异有统计学意义(P<0.05)。两组治疗后发育商(DQ值)均较治疗前出现上升,差异有统计学意义(P<0.05),且观察组上升幅度显著大于对照组,差异有统计学意义(t=4.252,P<0.05)。结论针灸配合口服小儿智力糖浆能促进患儿脑发育,改善发育迟滞状况,具有推广应用价值。     

高危儿的早期干预体会

目的分析研究高危儿的早期干预体会。方法取自我院收治的240例高危因素的新生儿为研究对象,均分为两组,对照组患儿不采用干预治疗,观察组患儿采用药物以及康复训练等综合治疗措施,定期对患儿进行体检、神经运动检查以及智力水平测试,评估患儿的身体情况、运动状况以及智力水平。结果研究表明,观察组患者的大运动、精细动作、语言、个人社会、适应行为评分情况明显优于对照组,组间有差异有统计学意义(P<0.05),观察组患者的运动发育落后情况少于对照组,脑瘫发生率少于对照组,P<0.05比较有差异有统计学意义。结论给予高危儿实施早期有效的护理干预,有利于促进高危儿健康成长发育,提高患儿生命质量。     

袋鼠式护理在极低出生体重儿家庭延续护理中的应用

目的 研究袋鼠式护理在极低出生体重儿家庭延续护理中的应用价值。方法 选取我院2017年1月～2018年8月收治的130例极低出生体重儿作为研究对象。按照随机数字表法将其分为观察组和对照组,其中观察组65例在家庭延续护理中采用袋鼠式护理模式,对照组采用常规护理方式。均随访12个月,每1个月入院随访,测量患儿身高、体重、头围;家属记录患儿每日睡眠时间,分别于3、6、9、12个月时采用儿童智力发育筛查(developmental screening test,DST)检测分析患儿智力情况;比较两组患儿家属出院12个月护理满意度。结果 观察组平均每日身高、头围生长速度与对照组比较,差异无统计学意义(P 0.05),观察组平均每日体重增长速度、睡眠时间明显高于对照组(P 0.05)。两组第3、6、9、12个月发育商(developmental quotient,DQ)正常占比均呈增长趋势,观察组各个时间点的DQ明显高于对照组,差异均有统计学意义(P 0.05)。观察组护理总满意度为98.46%,高于对照组(84.62%),差异有统计学意义(χ2=8.044,P 0.05)。结论 在家庭延续性护理中采用袋鼠式护理,可以有效给予患儿温暖,稳定患儿体温水平,有助于患儿身高体重的增加,促进神经智力行为发育,值得推广应用。     

Efficacy of gut lavage,hemodialysis, and hemoperfusion in the therapy of paraquat or diquat intoxication

Clinical and in vitro investigations were carried out to test the efficacy of gut lavage, hemodialysis, and hemoperfusion in the treatment of poisoning with paraquat or diquat. In a patient suffering from diquat intoxication 130 times more diquat was removed by gut lavage 30 h after ingestion than was removed by complete aspiration of the gastric contents.Determination of in vitro clearances for paraquat and diquat by hemodialysis showed that, at serum concentrations of 1–2 ppm, such as are frequently encountered in poisoning in man, toxicologically relevant quantities of herbicide cannot be removed from the body. At a concentration of 20 ppm, on the other hand, hemodialysis proved to be effective, the clearance being 70 ml/min at a blood flow rate of 100 ml/min. The efficacy of hemoperfusion with coated activated charcoal was on the whole better. Especially at concentrations around 1–2 ppm, the clearance values for hemoperfusion were some 5–7 times higher than those for hemodialysis.In a patient suffering from paraquat poisoning, both hemodialysis as well as hemoperfusion were carried out. The in vitro results could be confirmed: At serum concentrations of paraquat less than 1 ppm no clearance could be obtained by hemodialysis while by hemoperfusion with activated charcoal quite high clearance values were measured and the serum level dropped down to zero.

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Zusammenfassung Klinische Untersuchungen und Laboratoriumsversuche wurden durchgeführt, um die Wirksamkeit von Darmspülung, Hämodialyse und Hämoperfusion bei Paraquat- und Deiquat-Vergiftungen zu prüfen.Bei einem Patienten wurde 30 Std nach Deiquat-Aufnahme durch Darmspülung 130mal mehr Deiquat entfernt als durch vollständige Aspiration des Mageninhaltes. In vitro-Versuche ergaben, daß bei Blutserumkonzentrationen von 1–2 ppm, die bei Vergiftungen oft gemessen werden, durch Hämodialyse keine toxikologisch relevanten Paraquat- oder Deiquat-Mengen entfernt werden können. Dagegen erwies sich die Hämodialyse bei 20 ppm und einer Blutumlaufgeschwindigkeit von 100 ml/min mit einer Clearance von 70 ml/min als wirksam. Die Hämoperfusion mit beschicheter Aktivkohle war in diesen Versuchen aber eindeutig überlegen, denn insbesondere bei Konzentrationen um 1–2 ppm waren die Clearance-Werte 5–7mal höher als bei der Hämodialyse.Die in vitro-Ergebnisse wurden bei einem Patienten mit einer Paraquat-Vergiftung bestätigt: Bei Konzentrationen unter 1 ppm war die Hämodialyse wirkungslos, während durch Hämoperfusion relativ hohe Clearance-Werte erreicht wurden, so daß der Serumspiegel rasch unter die Nachweisgrenze abfiel.

     

In vitro studies on sterically stabilized liposomes (SL) as enzyme carriers in organophosphorus (OP) antagonism

This study describes a new approach for organophosphorous (OP) antidotal treatment by encapsulating an OP hydrolyzing enzyme, OPA anhydrolase (OPAA), within sterically stabilized liposomes. The recombinant OPAA enzyme was derived from Alteromonas strain JD6. It has broad substrate specificity to a wide range of OP compounds: DFP and the nerve agents, soman and sarin. Liposomes encapsulating OPAA (SL)* were made by mechanical dispersion method. Hydrolysis of DFP by (SL)* was measured by following an increase of fluoride ion concentration using a fluoride ion selective electrode. OPAA entrapped in the carrier liposomes rapidly hydrolyze DFP, with the rate of DFP hydrolysis directly proportional to the amount of (SL)* added to the solution. Liposomal carriers containing no enzyme did not hydrolyze DFP. The reaction was linear and the rate of hydrolysis was first order in the substrate. This enzyme carrier system serves as a biodegradable protective environment for the recombinant OP-metabolizing enzyme, OPAA, resulting in prolongation of enzymatic concentration in the body. These studies suggest that the protection of OP intoxication can be strikingly enhanced by adding OPAA encapsulated within (SL)* to pralidoxime and atropine.     

Aquatic Insects and Trace Metals: Bioavailability,Bioaccumulation, and Toxicity

Abstract

The uptake of metals from food and water sources by insects is thought to be additive. For a given metal, the proportions taken up from water and food will depend both on the bioavailable concentration of the metal associated with each source and the mechanism and rate by which the metal enters the insect. Attempts to correlate insect trace metal concentrations with the trophic level of insects should be made with a knowledge of the feeding relationships of the individual taxa concerned. Pathways for the uptake of essential metals, such as copper and zinc, exist at the cellular level, and other nonessential metals, such as cadmium, also appear to enter via these routes. Within cells, trace metals can be bound to proteins or stored in granules. The internal distribution of metals among body tissues is very heterogeneous, and distribution patterns tend to be both metal and taxon specific. Trace metals associated with insects can be both bound on the surface of their chitinous exoskeleton and incorporated into body tissues. The quantities of trace meals accumulated by an individual reflect the net balance between the rate of metal influx from both dissolved and particulate sources and the rate of metal efflux from the organism. The toxicity of metals has been demonstrated at all levels of biological organization: cell, tissue, individual, population, and community. Much of the literature pertaining to the toxic effects of metals on aquatic insects is based on laboratory observations and, as such, it is difficult to extrapolate the data to insects in nature. The few experimental studies in nature suggest that trace metal contaminants can affect both the distribution and the abundance of aquatic insects. Insects have a largely unexploited potential as biomonitors of metal contamination in nature. A better understanding of the physico-chemical and biological mechanisms mediating trace metal bioavailability and exchange will facilitate the development of general predictive models relating trace metal concentrations in insects to those in their environment. Such models will facilitate the use of insects as contaminant biomonitors.     

Steroidal sapogenin contents in some domestic plants

In order to find out the values of the steroid resources for the future use. the compositions and contents of steroidal sapogenins from 13 domestic plants have been investigated. As a result,Dioscorea nipponica, D. quinqueloba andSmilax china were found to have large amount of diosgenin. And pennogenin inTrillium kamtschaticum andParis verticillata, yuccagenin inAllium fistulosum, hecogenin inAgave americana and neochlorogenin inSolanum nigum were appeared to be major steroidal sapogenins.     

Alzheimer’s disease – current trends in basic and clinical research. Highlights from the 16th ECNP

Advances in the molecular biological knowledge of neuronal nicotinic acetylcholine receptors (nAChRs) have led to a growing interest by the pharmaceutical industry in the development of novel compounds that selectively modulate nAChR function. The ability of (-)-nicotine, an activator of nAChRs, to enhance attentional aspects of cognition in animals and humans, to exert neuroprotective and anxiolytic-like effects, and presumably to mediate the negative correlation between smoking and Alzheimer's (and Parkinson's) Disease, has focused interest on the potential therapeutic utility of modulators of nAChR function for treatment of some of the deficits associated with these progressive, neurodegenerative conditions. Numerous compounds are known which activate nAChRs and which might serve as lead compounds toward the development of such agents. The pharmacologic diversity of neuronal nAChR subtypes suggests the possibility of developing selective compounds which would have more favourable side-effect profiles than existing agents. This broader class of agents, collectively called cholinergic channel modulators (ChCMs), is anticipated to encompass compounds which would have more favourable side-effect profiles than existing agents, which generally exhibit low selectivity. This selectivity may be achieved by preferentially activating some subtypes of nAChRs (i.e., Cholinergic Channel Activators, ChCAs) or inhibiting the function of other subtypes (Cholinergic Channel Inhibitors, ChCIs). An overview of the biology of nAChRs and the rationale for the use of ChCMs for the treatment of dementia related to neurodegenerative diseases are presented, followed by a discussion of lead compounds and compounds under consideration for clinical evaluation.