268例儿童矮小原因分析及其预防措施

目的：探讨儿童矮小症的致病因素,为临床预防和治疗提供参考。方法：对2006年7月～2009年10月湘潭县妇幼保健院儿科保健门诊及住院部临床诊断为矮小症的患儿病因进行回顾性分析。结果：268例矮小症患儿中引起儿童矮小症的前10位疾病分别是生长激素神经分泌障碍（GHND）、体质性青春发育延迟、宫内发育迟缓、特发性矮小（ISS）、性早熟、营养缺乏性生长迟缓、Turner综合征（TS）、生长激素缺乏症（包括部分生长激素缺乏症）、原发性甲状腺功能减低症、21-三体综合征等。其中,〈6岁的病因主要为营养缺乏性生长迟缓、宫内发育迟缓、21-三体综合征;7～12岁的病因主要为生长激素神经分泌障碍（GHND）、特发性矮小（ISS）、宫内发育迟缓、Turner综合征（TS）、生长激素缺乏症（GHD）、原发性甲状腺功能减低症;13～18岁的病因主要为体质性青春发育延迟、性早熟及生长激素神经分泌障碍GHND。结论：引起儿童矮小症的病因多种多样,临床上要综合分析,给予针对性的预防和治疗措施。     

生长激素-胰岛素样生长因子轴功能检测在矮小症儿童病因诊断中的意义

目的 通过检测矮小症患儿生长激素(GH)-胰岛素样生长因子(IGF)轴功能,探讨其在矮小症病因诊断中的意义.方法 50例矮小症患儿采用精氨酸激发试验和可乐定激发试验检测血清生长激素(GH)水平,同时检测其血清胰岛素样生长因子-1(IGF-1)和胰岛素样生长因子结合蛋白-3(IGFBP-3)水平,根据GH和IGF-1检测结果,对矮小症儿童进行病因分类:(1)GH、IGF-1皆缺乏,为生长激素缺乏症(GHD);(2)GH正常但IGF-1缺乏,为GH不敏感综合征(GHIS);(3)GH缺乏但IGF-1正常,为可疑GHD;(4)GH和IGF-1皆正常,为特发性矮小(ISS).结果 50例矮小症患儿中,GHD 8例(占16％),怀疑GHIS 1例(占2％),可疑GHD 7例(占14％),ISS 34例(占68％).结论 通过检测GH-IGF轴功能,可对矮小症进行病因诊断.矮小症中最多是ISS,其次是GH-IGF轴功能缺陷.     

儿童矮小症病因及临床诊治研究分析

目的 儿童矮小症病因及临床诊治研究分析。方法 纳入我院2016年2月1日至2022年9月1日收治的110例矮小症儿童的临床资料进行回顾性分析。结果 入组矮小症儿童男性55.45%,女性44.55%,平均年龄（7.91±2.69）岁。完全性生长激素缺乏症（GHD）40.00%,部分性GHD43.64%,特发性身材矮小（ISS）和家族性矮小症(FSS)13.64%,体质性生长和青春期延迟0.91%,Turner综合征1.82%。GHD组和ISS+FSS组对比年龄均值、性别构成、BMI均值无明显差异（P> 0.05）。GHD组和ISS+FSS组对比骨龄落后（实际年龄-骨龄）平均值更大（P <0.05）。BMI与骨龄落后值相关系数为-0.356(P <0.01)。激发试验峰值出现时间空腹（1.8%）、30 min(10.0%)、60 min(49.1%)、90 min(29.1%)、120 min(10.0%)。结论 GHD是矮小症的主要病因。GHD常见骨龄落后,但骨龄正常或者超前也可能存在GHD。BMI与骨龄有相关性,BMI越大可能存在骨龄正常或超前。胰岛素和可乐定激发试验...     

实验诊断生长激素缺乏症最佳截断值的探讨

目的运用受试者工作特性（ROC）曲线分析法,分析矮小儿童不同的生长激素（GH）激发试验峰值、胰岛素样生长因子1（IGF-1）及胰岛素样生长因子结合蛋白3（IGFBP-3）值对生长激素缺乏（GHD）症诊断的临床价值;并确定诊断GHD的最佳截断值。方法选择141例矮小患儿,其中临床诊断为可疑GHD矮小患儿71例,作为观察组,非GHD矮小患儿70例,作为对照组;用化学发光法测定患儿的血清GH、IGF-1、IGFBP-3值。采用中文ROC曲线软件进行ROC曲线分析。结果GH峰值应用ROC曲线分析,曲线下面积（91．6％）最大,当GH峰值为7．93μg／L时,兼顾了较好的敏感性（0．83）及特异性（0．86）。IGF-1、IGFBP-3值应用ROC曲线分析,两者的曲线下面积（69．5％、62．4％）均小;两者均表现出较低的敏感性（0．54、0．31）,即有较高的漏诊率（46％、69％）。结论GH激发试验峰值最具诊断价值;IGF一1、IGFBP-3值用于诊断GHD的价值不大。GH峰值为7．93μg/L时,是诊断GHD的最佳截断值。     

儿童矮小262例病因分析

目的探讨本地区儿童矮小的病因及干预。方法回顾性分析262例身高儿童矮小的身高、骨龄、生长激素水平、甲状腺功能及微量元素等指标。结果262例儿童矮小中，内分泌异常所致的矮小占首位（38，55％，101／262），其中生长激素缺乏性矮小（GHD）38例，性早熟22例；体质延迟性矮小67例，男女发病比例为2.75；遗传性矮小35例；特发性矮小33例。67例体质延迟性矮小患儿的病因：食欲不振41例，睡眠不足29例，活动过多23例，微量元素缺乏36例。结论内分泌异常仍然是矮小的主要原因。     

重组人生长激素治疗ISS及GHD患儿的疗效及安全性分析

目的探讨重组人生长激素治疗特发性矮小症(ISS)及生长激素缺乏症(GHD)的临床疗效及安全性。方法选择2011年9月至2012年12月在我院采用重组人生长激素治疗的28例ISS及30例GHD患儿为研究对象,记录治疗前后患儿生长速率(GV)、体重、身高、骨龄、身高标准差计数(HtSDS)、胰岛素样生长因子-1(IGF-1)水平及不良反应发生率。结果治疗后患儿GV、HtSDS与治疗前比较差异有统计学意义(P<0.05);治疗后IGF-1水平显著升高(P<0.05);两组治疗前后体重、身高、骨龄均未见显著改变(P>0.05);ISS患儿GV与IGF-1水平未见相关性(r=0.235,P>0.05);GHD患儿GV与IGF-1水平显著相关(r=0.638,P<0.05);ISS及GHD患儿不良反应发生率12.1%,经处置后患儿均顺利完成治疗。结论重组人生长激素可促进ISS及GHD患儿生长,不会使骨龄明显提前,安全性好。     

血清胰岛素样生长因子-1生成试验在矮小症儿童诊断中的价值初探

目的:初步探讨胰岛素样生长因子-l(IGF-1)生成试验在矮小症儿童诊断中的价值。方法:对35例青春发育期前矮小症儿童进行左旋多巴和精氨酸激发试验,根据GH峰值诊断为生长激素缺乏(GHD组,14例)、特发性矮小(ISS组,21例),对所有矮小症儿童予国产重组人生长激素(rhGH)0.3 IU/(kg.d)睡前皮下注射,连续4 d,在试验前及试验结束后次日晨留取标本,采用CLIA法测定血清IGF-1水平。结果:GHD组与ISS组临床资料在身高、骨龄、身高落后方面比较差异无统计学意义,GHD组与ISS组血清IGF-1基础水平分别为(172.61±65.38)ng/mL和(241.90±56.33)ng/mL,GHD组IGF-1基础水平低于ISS组,两组比较差异有统计学意义(P<0.05)。生成试验后GHD组与ISS组IGF-1反应值(△IGF-1)、反应值百分数(△%IGF-1)分别为(195.8±157.90)ng/mL、(133.8±135.13)%和(115.57±74.40)ng/mL、(52.74±40.02)%,GHD组△IGF-1、△%IGF-1高于ISS组,差异均有统计学意义(P<0.05)。结论:IGF-1生成试验可为GHD诊断提供进一步参考,ISS儿童存在GH-IGF-1轴功能异常,IGF-1生成试验在预测rhGH促生长疗效中可能具有一定的价值。     

儿童矮小症112例病因分析

目的探讨儿童身材矮小的病因。方法对112例矮小症儿童进行全面的病史采集和体格检查及相关实验室检查。结果生长激素缺乏症（GHO）25例,占22．3％;家族性矮小20例,占17．9％;体质性青春发育延迟15例,占13．4％;特发性矮小14例,占12．5％;余为性早熟、甲状腺功能减低症、宫内发育迟缓、Turner综合征等引起的矮小症。结论内分泌异常仍然是儿童矮小的主要病因。     

青春期前特发性矮小症患儿临床特征与维生素D受体BmsI基因多态性的关系

目的：探讨青春期前特发性矮小症（ISS）患儿临床特征与维生素D受体（VDR）BmsI基因多态性的关系，分析影响重组生长激素（rhGH）疗效的相关因素。方法：选取2016年3月至2018年6月我院收治的72例ISS患儿为ISS组，另选取同期参加体检的60例健康儿童为健康对照组。记录所有儿童临床资料，采用聚合酶链反应（PCR）检测两组儿童BsmI基因多态性，采用Hardy-Weinberg遗传平衡吻合度检验法计算各基因型理论值。ISS组患儿给予rhGH治疗1年以上，并根据不同基因型分为BB、Bb及bb 3种基因型，观察治疗3、6、12个月后不同基因型患儿生长速率和身长标准差积分（Ht-SDS）变化情况。采用Pearson分析影响rhGH疗效的相关因素。结果：治疗前，健康对照组与ISS组年龄比较差异无统计学意义（P>0.05）；健康对照组儿童身长、体质量、生长速率、胰岛素样生长因子（IGF-1）和生长因子结合蛋白-3（IGFBP-3）水平明显高于ISS组（P<0.05）。健康对照组与ISS组BB、Bb及bb基因型均符合Hardy-Weinberg遗传平衡定律，两组儿童3种基因型频率及B、b等位基因频率比较差异均有统计学意义（P<0.05）。治疗前，3组基因型ISS患儿出生体质量、出生身高、身高、体质量、父亲身高、母亲身高、实际年龄、骨龄比较差异无统计学意义（P>0.05）。治疗前及治疗3个月后，BB、Bb及bb基因型组患儿生长速率和Ht-SDS比较差异无统计学意义（P>0.05）；治疗6、12个月后，3组患儿生长速率和Ht-SDS均明显升高（P<0.05），且bb组患儿变化更明显（P<0.05）。相关性分析显示，初始治疗年龄与△Ht-SDS（即Ht-SDS变化）呈负相关，初始IGF-1和IGFBP-3水平、Bsm I基因多态性、初始治疗年龄、初始生长速率、治疗时间与△Ht-SDS呈正相关。出生身高和体质量、治疗前身高和体质量、遗传靶身高骨龄与△Ht-SDS无明显相关性。结论：BsmI基因多态性可能与ISS发病有关，并且可能是影响rhGH疗效的因素。此外。rhGH疗效亦受IGF-1、IGFBP-3影响，越早治疗，治疗前生长速率越大，疗程越长则治疗效果越好。     

生长激素缺乏症临床分析

生长激素缺乏症（growth hormone defeiciency，GHD）是导致儿童身材矮小的常见原因之一，2006至2008年我科矮小门诊在以身材矮小为主诉就诊的儿童中查出GHD患者27例，报告如下。     

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.     

Pharmacological treatment of COVID-19: an opinion paper

The precocity and efficacy of the vaccines developed so far against COVID-19 has been the most significant and saving advance against the pandemic. The development of vaccines has not prevented, during the whole period of the pandemic, the constant search for therapeutic medicines, both among existing drugs with different indications and in the development of new drugs. The Scientific Committee of the COVID-19 of the Illustrious College of Physicians of Madrid wanted to offer an early, simplified and critical approach to these new drugs, to new developments in immunotherapy and to what has been learned from the immune response modulators already known and which have proven effective against the virus, in order to help understand the current situation.     

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.     

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.