支原体肺炎伴喘息患儿外周血IgE水平分析

【目的】　探讨支原体肺炎伴喘息患儿外周血IgE水平变化及意义。【方法】　测定100 例支原体肺炎患儿外周血IgE水平,其中伴喘息组36例,不伴喘息组64例,对照组50 例为非支原体感染、不伴喘息的支气管肺炎患儿组,比较支原体肺炎组和对照组、支原体肺炎伴喘息组和非喘息组的IgE水平并进行分析。　【结果】　支原体肺炎组患儿IgE水平显著高于对照组,支原体肺炎伴喘息组显著高于不伴喘息组(P<0.001)。　【结论】　肺炎支原体感染IgE抗体明显升高,支原体肺炎伴喘息患儿外周血具有高水平的IgE。     

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目的分析男性儿童同性性早熟的病因及临床特点。方法回顾性分析1988年1月至2009年4月中山大学附属第一医院收治的明确病因诊断的78例男性同性性早熟病例的病因及临床特点。结果中枢性性早熟(CPP)55例(70.51%),按构成比前三位病因为特发性性早熟、下丘脑错构瘤、先天性肾上腺皮质增生症(CAH)继发,其中下丘脑错构瘤患儿就诊年龄小、GnRHa激发试验后LH浓度最高,CAH患儿骨龄提前最多、HtSDSba负值最大;外周性性早熟(PPP)23例(29.49%),分泌HCG生殖细胞瘤和CAH为主要病因,CAH患儿由PPP转变为CPP的比例较大(5/9),尤其是初治年龄较大者更易发生。分泌HCG的生殖细胞瘤血和(或)脑脊液的β-HCG水平均升高。结论男性儿童性早熟以器质性病变引起多见,在诊治过程中应积极寻找病因。     

小于胎龄儿青春前期线性生长方式与血清胰岛素水平关系的研究

Objective To evaluate the association between two different linear growth patterns with the levels of serum insulin in children bem small for gestational age(SGA).Methods Serum fasting glucose,fasting insulin,and insulin-like growth factor-I(IGF-I)concentrations were determined in 30 catch-up growth(CUG)children bern SGA [CUG-SGA,16 females,14males,(6.62±0.66)year],37 non-catch-up growth(NCUG)children born SGA[NCUG-SGA,15 females,22 males,(5.97±0.56)year],and42 appropriate for gestational age(AGA)children with normal height[AGA,16females,26males,(7.05±0.39)year].Results (1) Basal fasting insulin and homeostasis model assessment for insulin resistance(HOMA-IR)were significantly higher in CUG-SGA group than in NCUG-SGA and AGA group(P<0.01 or P<0.05).But there was no difference in fasting insulin between NCUG-SGA group and AGA group.IGF-I levels in CUG-SGA were significantly higher than in NCUG-SGA group[(212.61±17.81 vs 137.40±14.66)ng/ml,P=0.001],but showed no difference from AGA group(P=0.095).(2)In the SGA group,HOMA-IR showed positive correlation with age,△height SDS,and current body mass index.Fasting insulin showed positive correlation with △height SDS(r=0.500,P=0.002)in≤6 year group as well as with △weight SDS(r=0.496,P=0.030)in>6 year group.Conclusions Insulin as a growth factor may participate in postnatal catch-up growth accompanied with increased insulin resistance in SGA children.     

GnRHa治疗对特发性中枢性性早熟女童肾上腺功能初现的影响

目的 通过随访特发性中枢性性早熟(ICPP)女童经促性腺激素释放激素类似物(GnRHa)治疗前、后阴毛发育情况和血硫酸脱氧表雄酮(DHEAS)水平,探讨肾上腺功能初现与性腺轴发育的关系.方法 对确诊为ICPP且按年龄判断血DHEAS的z分值超过正常上限(+2 s)的49例女童在GnRHa治疗前后每3-6个月行性征检查,治疗后每年检测血DHEAS.平均随访(4.08+0.83)年,其中16例随访至停药后1年.结果 治疗前阴毛发育2期(PH2)和阴毛发育3期(PH3)的达到年龄均小于正常[(8.07对11.16)岁,(8.82对12.40)岁],ICPP女童GnRHa受治期间阴毛发育进程显著慢于正常对照[PH2-PH3:(1.69对0.83)年;PH3-阴毛发育4期(PH4):(1.64对0.60)年];同时也长于治疗前的自然进程[PH2-PH3:(1.69对0.88)年].乳房开始发育至阴毛萌出的间期亦然,ICPP治疗前后和正常对照分别为1.13、3.62和0.76年.在GnRHa治疗期间ICPP女童每年DHEAS Z分值呈逐年递减,停药后又显增.结论 大于6岁的性腺轴初现提前可诱致肾上腺功能初现提前,而GnRHa治疗在抑制性腺轴的同时也能延缓肾上腺功能发育的进程.

Abstract：

Objective To explore the relationship between adrenarche and gonadarche.Methods Total 49 idiopathic central precocious puberty(ICPP)girls,whose serum dehydroepiandrosterone sulfate(DHEAS)Z scores for chronological age were higher than+2 s at diagnosis.were enrolled.Physical examinations during pubertal stage were repeated at 3-6 months intervals,and serum DHEAS levels were monitored yearly within an average period of 4.08 years.Of them,16 girls were followed up until more than one year after discontinuation of gonadotropin-releasing hormone analogue(GnRHa)treatment.Results Before GnRHa treatment,these49 girls presented a younger average age at attainment of pubic hair stage2(PH2)and pubic hair stage3(PH3)than normal(8.07 years vs 11.16 years,8.82 years vs 12.40 years respectively).During GnRHa treatment,the intervals between PH2 and PH3,PH3 and pubic hair stage4(PH4),breast stage 2(B2),and PH2 were longer than normal(1.69 years vs 0.83 years,1.64 years vs 0.60 years,and3.62 years vs 0.76 years respectively).The intervals between PH2 and PH3,as well as B2 and PH2 during GnRHa treatment were also longer than that before GnRHa treatment(1.69 years/35 0.88 years,3.62 years vs 1.13 years respectively).The serum DHEAS Z scores decreased during GnRHa treatment,and increased significantly after GnRHa cessation.Conclusion Gonadarche after age of 6-year-old may lead to earlier adrenarehe.GnRHa treatment might slow down the progression of adrenarche and suppress the hypothalamuspituitary-gonadal axis.     

生长激素对于GnRHa治疗中生长过度减速的特发性中枢性性早熟女孩的远期疗效观察

目的 观察重组人生长激素(rhGH)对于促性腺激素释放激素类似物(GnRHa)治疗中生长过度减速的特发性中枢性性早熟(ICPP)女孩的最终成年身高(FAH)的影响.方法 49例ICPP女孩接受GnRHa治疗,当身高增长速度减慢至4 cm/年以下时,其中26例联用rhGH治疗为联合治疗组,23例拒绝加用rhGH但继续使用GnRHa为单用组.比较2组治疗前后的预测成年身高(PAH)和FAH.结果 联合治疗组联用rhGH前半年,身高增长速度均小于4 cm/年[(3.2±1.0)cm/年],PAH无明显改善[联用rhGH前半年和rhGH开始时PAH分别为(152.5±4.0)cm和(152.6±3.7)cm];联用rhGH(11.4±5.4)个月后,身高增长速度增加至(6.7±2.0)cm/年,PAH增加至治疗结束时的(157.1±4.7)cm(均P<0.01);FAH[(157.5±4.5)cm]显著高于GnRHa开始时的PAH[(148.1±4.6)cm]和遗传靶身高[(154.4±4.6)cm,均P<0.01].单用组治疗结束时的PAH[(153.9±6.3)cm]较拒绝联用rhGH时的PAH[(153.1±6.2)cm]无差异;FAH[(154.7±5.5)cm]高于治疗开始时的PAH[(150.3±6.0)cm,P<0.01],但与遗传靶身高[(155.6±4.3)cm]无差异.结论 联用rhGH能够显著加快GnRHa治疗中生长过度减速的ICPP女孩的身高增长速度,进一步改善PAH和FAH.

Abstract：

Objective To evaluate the long-term final adult height outcome of combined treatment with gonadotropin-releasing hormone analogue(GnRHa)and recombinant human growth hormone(rhGH)in girls with idiopathic central precocious puberty(ICPP).Methods Out of 49 sirls with ICPP[treated with GnRHa at a dose of 60-80 μg/kg every 4 weeks for at least 6 months,whose height velocity fell below 4 cm/year and showed no improvement of predicted adult height(PAH)in 6 months],26 received(rhGH-combined group),in addition to chronological age,and duration of GnRHa treatment,who showed the same growth pattern but refused rhGH treatment,served to evaluate the efficacy of rhGH in addition.At the conclusion of the smdy,all the girls had been followed up for(3.3±1.9)years,and(3.2±0.9)years in rhGH-combined group and control group,respectively;and had achieved adult heisht.To compare the PAH with the final adult height(FAH)before and after treatment in the two groups.Results During rhGH treatment, height velocity of the rhGH-combined girls increased significantly[(6.7±2.0 vs <4)cm/year baseline],RhGH-combined gids showed an adult height far higher than pretreatment PAH [(157.5±4.5 vs 148.1±4.6)cm,P<0.01],and target height[(154.4±4.6)cm] was,significantly excceded.The control group reached an adult heisht also significandy higher than pretreatment PAH[(154.7±5.5vs 150.3±6.0)cm,P<0.01],while target height[(155.6±4.3)cm]was just reached but not significantlyexcceded.The gain in height obtained,calculated between pretreatment PAH and final heisat,(9.4±4.9)cm in rhGH-combined group was much more than that(4.3±4.2)cm in the control group(P<0.01).Conclusion RhGH may accelerate the linear growth and improve adult height of GnRHa-treated ICPP girls.     

促性腺激素释放激素类似物简易激发试验对性早熟诊断的评价

目的　评价促性腺激素释放激素类似物 (GnRHa)简易激发试验对性早熟的诊断价值。方法　乳房发育提前的 74例女孩 (就诊时行 0、30和 6 0min简易GnRHa激发试验 )被分为 4组 :真性性早熟 (CPP)B2 组 19例 ;CPPB3 组 2 3例 ;单纯性乳房早发育 (PT)组 2 2例 ;外周性性早熟 (PPP)组 10例。对各组激发试验进行比较和分析。结果　PPP组 3个时相血清FSH及LH浓度差异无显著性。PT、CPPB2 和CPPB3 组 3个时相的血清FSH及LH浓度差异均有显著性 (P <0 .0 1)。PT组ΔFSH(8.0± 3.2 )IU/L显著高于ΔLH〔(1.9± 1.4 )IU/L ,P <0 .0 1〕。CPPB2 组ΔFSH(7.0± 2 .9)IU/L与ΔLH(8.2± 6 .7)IU/L差异无显著性。CPPB3 组ΔLH(2 3.0± 2 2 .0 )IU/L显著高于ΔFSH〔(9.0± 4 .3)IU/L ,P <0 .0 5〕。PT、CPPB2 和CPPB3 组的ΔLH及峰LH/FSH〔(0 .4± 0 .2 )、 (1.1± 0 .8)和 (1.9± 1.7)〕差异有显著性 (P <0 .0 1)。以LH峰值≥ 4 .4IU/L和峰LH/FSH≥ 0 .4 6为前提 ,诊断CPP的灵敏度和特异度分别为 90 .5 %和 90 .9%。结论　PPP组垂体对GnRHa无应答。PT组反应以FSH升高为主 ,CPPB2 和CPPB3 组以LH升高为主。此激发试验对性早熟性质具早期鉴别诊断价值     

促性腺激素释放激素和人绒毛膜促性腺激素激发试验在鉴别低促性腺激素性性腺功能减退和体质性生长和青春发育延迟的作用

青春发育延迟是男孩来小儿内分泌科就诊的最常见原因之一.其发生率在14岁时约为5%,3年后仍有0.1%未进入青春期.其鉴别诊断为区分常见的体质性生长和青春期延迟(CDGP)和罕见的(发生率0.025%)低促性腺激素性性腺功能减退症(HH).     

有生长追赶SGA大鼠促生长素轴的改变

目的: 研究青春前期有生长追赶的小于胎龄(SGA)大鼠促生长素轴的改变,探讨SGA个体生长追赶可能的发生机制。方法: 应用孕鼠全程饮食限制法制备SGA大鼠模型,测定4周龄子鼠尿生长激素(GH)排泄率、血胰岛素样生长因子-1(IGF-1)浓度、肝脏组织IGF-1 mRNA表达和胫骨生长板IGF-1、胰岛素样生长因子-1受体(IGF-1R)表达。结果: 有生长追赶SGA(CUG-SGA)大鼠尿GH水平显著高于无生长追赶SGA(NCUG-SGA)和适于胎龄(AGA)大鼠(P<0.05,P<0.01);血IGF-1水平CUG-SGA显著高于NCUG-SGA组(P<0.01),与AGA组无显著差异。CUG-SGA组肝脏IGF-1 mRNA表达较NCUG-SGA显著增高(P<0.01),与AGA相比无显著差异。胫骨生长板形态学的改变:SGA鼠生长板增殖区每柱细胞数较少,各期软骨细胞排列不规则。 NCUG-SGA组生长板总厚度、各区厚度均较CUG-SGA和AGA组显著降低;CUG-SGA组生长板厚度与AGA组比较无显著差异,但肥大区厚度较NCUG-SGA和AGA组显著增高(P<0.01,P<0.05)。CUG-SGA 组胫骨生长板软骨细胞IGF-1表达较NCUG-SGA显著增多(P<0.01),与AGA组比较无显著差异;3组间IGF-1R表达无显著差异(P>0.05)。结论: SGA大鼠无论生后追赶与否均存在GH/IGF-1轴抵抗,其生后不同的线性生长追赶方式可部分归咎于其抵抗程度。     

无生长追赶SGA幼鼠生长激素和胰岛素受体后信号交联对话的研究

目的：在生长激素（GH）和胰岛素（INS）共享受体后PI3K通路基础上探讨无生长追赶的出生低体重（NCU-SGA）幼鼠GH和INS抵抗的受体后机制，以及2者受体后信号通路的交联对话（cross-talk）。方法:取4周龄NCU-SGA雄性大鼠，采用Western印记及免疫共沉淀技术分别测定NCU-SGA幼鼠在基础状态下、胰岛素激发以及先给予GH受体后信号通路JAK2阻滞剂AG490后再行胰岛素激发后（AG490+INS组）肝组织胰岛素受体底物-1（IRS-1）及其下游信号磷酸化Akt(p-Akt)的表达。结果:（1）IRS-1信号表达： SGA鼠基础状态、INS激发后和AG490+INS组，3组间的IRS-1总蛋白及IRS-1磷酸化水平与正常对照组（C组）无显著差异（P＞0.05）。（2）p-Akt信号表达： C组基础状态时无p-Akt信号表达，INS刺激后表达明显增强。SGA鼠基础状态时p-Akt已有显著表达（慢性激活），INS刺激后表达较基础状态增加，但增殖显著低于正常对照组（P＜0.01）；AG490+INS组的p-Akt较JAK2未被阻断时明显增强（P＜0.01），但仍显著低于正常对照组（P＜0.01)，提示GH的信号干扰了INS受体后IRS-1至Akt的信号转导。结论:NCU-SGA幼鼠INS抵抗的发生与IRS-1-Akt通路受损有关，GH抵抗经GH和INS 2者受体后信号通路间的交联对话（cross-talk）使IRS-1至Akt间的信号转导解偶联,诱导和加重了INS抵抗；而PI3K-Akt可能是发生该解偶联的主要交汇点。