血清促性腺激素基础值在性早熟女童诊断中的价值

目的：探讨血清促性腺激素基础值在性早熟女童诊断中的价值。方法：以促性腺激素释放激素（GnRH）激发试验结果作为性早熟诊断的金标准,将77例性早熟女童分为中枢性性早熟（CPP,n=45）和单纯性乳房早发育（IPT,n=32）两组,分别比较两组黄体生成素（LH）、卵泡刺激素（FSH）基础值及LH/FSH比值的差异;并采用受试者工作特征（ROC）曲线分析LH、FSH基础值及LH/FSH比值诊断性早熟的准确性。结果：CPP组患儿血清基础LH、FSH水平及LH/FSH比值均高于IPT组（P<0.01）;两组患儿LH基础值与GnRH激发试验中LH峰值存在正相关;LH、FSH和LH/FSH比值诊断CPP的曲线下面积（AUC）进行比较,AUCLH大于AUCFSH和AUCLH/FSH（均P＜0.05）,而AUCFSH和AUCLH/FSH之间比较差异无统计学意义。当血清LH基础值为0.62 IU/L时,敏感度为0.778,特异度为0.906,Youden指数最大（0.684）;当切割值为1.5 IU/L时,诊断敏感度下降为0.311,但特异度为1.0。结论：血清LH基础值诊断CPP的价值优于LH/FSH比值及FSH基础值,可用于性早熟女童门诊的初步诊断,但存在一定的误诊和漏诊率;对于LH基础值大于1.5 IU/L的患儿,结合临床表现可明确诊断,无需另行GnRH激发试验。     

性早熟伴肿瘤24例临床分析

目的探讨性早熟伴肿瘤患儿的临床特征。方法对住院的24例性早熟伴肿瘤患儿的临床资料进行统计分析。结果男女伴肿瘤性早熟占同性别性早熟的比例分别为12.93%及0.50%;以周围性性早熟（PPP）为表现的肿瘤患儿术后转变为中枢性性早熟（CPP）后其骨龄（BA）显著提前,黄体生成素（LH）、卵泡刺激素（FSH）基础值及GnRH激发试验峰值均明显升高。结论性早熟患儿中男性性早熟伴肿瘤的发生率高于女性,以周围性性早熟为表现的肿瘤患儿术后可转变为中枢性性早熟。     

4岁内儿童性早熟57例

目的探讨4岁内儿童性早熟的病因、诊断要点,研究简易的促性腺激素释放激素(GnRH)激发试验的可行性。方法对57例<4岁性早熟患儿的临床资料进行回顾性分析。57例均行GnRH激发试验,对中枢性与部分中枢性组患儿的LH值进行秩和检验。结果本组男3例,女54例。外周性性早熟36例(63.1%);中枢性性早熟(CPP)4例;部分性CPP17例。CPP促黄体生成素(LH)升高为甚,50%峰值落在60~90min,部分性CPP促卵泡生成素(FSH)升高为甚,84.2%峰值落在90~120 min;CPP与部分性CPP 30、60、90、120 min LH比较有显著差异(P均<0.01)。结论<4岁儿童性早熟以女性发病为主,多为外周性性早熟。GnRH激发试验对病因分类很必需,应在0、60、120 min测LH、FSH,以明确CPP和部分性CPP。     

中枢性性早熟的诊断和治疗

性早熟（sexual precocity）是指女童在8岁前，男童在9岁前呈现第二性征的病变。根据下丘脑-垂体-性腺（HPG）的提前激活情况，性早熟分为中枢性[CPP，或促性腺激素释放激素（GnRH）依赖性、真性、完全性]，外周性（PPP，或非GnRH依赖性、假性）。不完全性（部分性）性早熟为CPP的变异，包括单纯性乳房早发育（PT）、单纯性阴毛早发育（PP）和单纯性早初潮（PM）。在一般人群性早熟发病率为0．6％，以女孩多见，女童和男童之比为3：1～23：1。     

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目的探讨性早熟对2~10岁女童骨密度的影响。方法选择2003-01—2006-01在湖南省儿童医院内分泌专科就诊的2~10岁性早熟(明确诊断、并排除影响骨代谢性疾病)女童237例,根据真、假性性早熟(CPP、PPP)分为2组,各组再按年龄组分层,采用单光子骨矿物质密度测定仪测量左手桡骨中远1/3处桡、尺骨密度(BMD),并与同龄健康女童进行对比和分析。结果CPP、PPP和健康组BMD均随年龄增长而增加,3组各年龄桡骨BMD均高于尺骨;CPP桡、尺骨BMD均相对较高,8~10岁组中CPP较对照组约高6.4%~8.6%;3组桡、尺骨BMD均在8~10岁增长加速,特别是尺骨(P<0.05),分别较6~7岁组增长20.4%、17.8%和14.3%;以CPP组增幅最大,明显高于健康组,与健康组(6~7岁)增长比较差异有显著性(桡骨P<0.05、尺骨P<0.001)。PPP组则与健康女童差异不显著。结论健康女童骨矿化自9岁起开始青春期加速,CPP女童青春期尺骨生长加速的年龄提早,BMD相应增加,而PPP不像CPP那样明显影响女童的正常骨骼发育。     

性早熟女童性激素、瘦素水平及其影响因素

目的了解中枢性性早熟女童基础性激素、瘦素水平及相关影响因素。方法以78例中枢性早熟女童为研究对象,同时选择78例非性早熟女童为对照组,精确测量两组女童的身高、体质量,按G-P图谱测评骨龄,并取晨起空腹血清检测性激素及瘦素水平。采用t检验、秩和检验和Spearman相关分析进行统计学分析。结果性早熟女童的身高、体质量、骨龄、体质指数(BMI)、瘦素、促性腺激素(LH和FSH)、雌二醇(E2)水平均高于对照组女童;性早熟女童瘦素水平与LH、FSH、骨龄呈正相关,对照组瘦素与性激素水平无相关性;与骨龄相比,瘦素与LH、FSH的相关性较强。结论中枢性性早熟女童有明显的体格发育与基础性激素水平改变,瘦素对性早熟的发生起重要作用。     

简易化黄体生成素释放激素激发试验诊断性早熟

目的通过对性早熟患儿黄体生成素释放激素(LHRH)激发试验中各时间段黄体生成素(LH)、卵泡刺激素(FSH)、雌二醇(E2)、睾酮(T)水平分析,探求激发试验的简易化。方法运用全自动化学发光免疫分析系统(ACS:180)检测60例性早熟患儿基础血清E2、T、FSH、LH水平及LHRH激发试验后血清FSH、LH水平,并进行比较。结果中枢性性早熟(CPP)组39例LHRH注射30 min后FSH和LH明显升高(Pa<0.01),LH/FSH>1,LH峰值>12 IU/L。周围性性早熟(PPP)组21例注射LHRH前及注射后30、60、90 min后,血LH、FSH无显著变化(Pa>0.05)。E2和T水平于注射前和注射后60 min无差异显著性(P>0.05)。二组LH、FSH高峰均集中在30、60 min。结论LHRH激发试验主要依据LH峰值和LH/FSH值来判断;激发试验可简化为LHRH激发前和激发后30、60 min分别抽血检测LH、FSH,即能为临床提供诊断依据。     

骨骼及肌肉系统疾病

0721002~10岁性早熟女童骨密度检测分析/郑素平…∥中国实用儿科杂志.-2007,22(3).-199~201选择2~10岁性早熟(明确诊断、并排除影响骨代谢性疾病)女童237例,根据真、假性性早熟(CPP、PPP)分为2组,各组再按年龄组分层,采用单光子骨矿物质密度测定仪测量左手桡骨中远1/3处桡、尺骨密度(BMD),并与同龄健康女童进行对比和分析。结果:CPP、PPP和健康组BMD均随年龄增长而增加,3组各年龄桡骨BMD均高于尺骨;CPP桡、尺骨BMD均相对较高,8~10岁组中CPP较对照组约高6.4%~8.6%;3组桡、尺骨BMD均在8~10岁增长加速,特别是尺骨(P<0.05),分别较…     

女童性早熟与血清瘦素、类胰岛素样生长因子-1水平及脑电图关系的研究

目的 探讨特发性中枢性性早熟(ICPP)与单纯性乳房发育(SPT)女童血清瘦素、类胰岛素样生长因子-1(IGF-1)水平、脑电图异常率及这些指标对真性性早熟的诊断价值。方法 乳房早发育为主患儿中特发性中枢性早熟18例,单纯性乳房发育12例,用放射免疫法和酶标免疫法测定两组血清瘦素、IGF-1及行脑电图检查。结果 ICPP组血清瘦素、IGF-1水平明显高于SPT组(P均<0.001)。其脑电图异常率明显高于SPT组(P<0.01)。血清瘦素与IGF-1之间呈明显正相关(r=0.668 P<0.001)。血清瘦素与黄体生成素(LH)或LH/卵泡刺激素(FSH)间无相关性。结论 血清瘦素、IGF-1、脑电图可作为真性性早熟的早期诊断指标之一。     

肥胖对中枢性性早熟女童GnRH激发试验促黄体生成素峰值及相关激素的影响

目的探讨肥胖对中枢性性早熟女童GnRH激发试验促黄体生成素(LH)峰值及相关激素的影响。方法选取我院2012~2014年完成GnRH激发试验的中枢性性早熟女童333例,根据体重指数(BMI)分为正常体重组(n=123)、超重组(n=108)和肥胖组(n=102),并对3组性发育指标进行比较分析。再分别从各组中随机抽取20例,检测血清瘦素(leptin)、性激素结合蛋白(SHBG)、神经激肽B(neurokini B)和吻素(kisspeptin)水平,Pearson相关分析分析BMI与各激素水平的相关性。结果各组平均诊断年龄差异无统计学意义,但超重及肥胖组的骨龄显著大于正常体重组(P0.05)。正常体重组血清LH激发峰值、SHBG水平显著高于超重及肥胖组,leptin及neurokini B水平显著低于超重及肥胖组(P0.05)。BMI与GnRH激发试验LH峰值、SHBG水平呈负相关(P0.05),与leptin和neurokini B水平呈正相关(P0.05)。结论在分析性早熟女童GnRH激发试验结果及相关激素水平时需考虑BMI对结果的影响。     

A study of anthropomorphic and biochemical characteristics in girls with central precocious puberty and thelarche variant

BACKGROUND: Premature thelarche in later childhood may progress to central precocious puberty (CPP), which does not spontaneously resolve. Thelarche variant (TV) is a slowly progressive variant of precocious puberty. AIM: To determine and compare levels of insulin-like growth factor-I (IGF-I), IGF binding protein-3 (IGFBP-3) and anthropomorphic measures in girls with TV and CPP. SUBJECTS: Prepubertal controls and girls with TV and CPP. METHODS: Chronological and bone age, weight, height, BMI, height velocity (HV), and serum IGF-I, IGFBP-3, leptin, follicle-stimulating hormone (FSH) and lutenizing hormone (LH) levels were assessed. RESULTS: Serum IGF-I levels, HV and IGF-I/ IGFBP-3 ratio were significantly higher in girls with CPP compared to both controls and girls with TV. IGFBP-3 values for bone age (IGFBP-3BA) were significantly higher in the TV group compared to both controls and girls with CPP. FSH and LH concentrations were significantly higher in the CPP group compared to TV. CONCLUSION: HV, IGF-I, LH and FSH levels and IGF-I/IGFBP-3 ratio are elevated in girls with CPP compared to those with TV.     

Changes in body composition and bone density after discontinuation of growth hormone therapy in adolescence: an interim report

Growth hormone deficiency (GHD) in adults and children is associated with decreased lean tissue mass (LTM), increased fat mass and reduced bone mineral density (BMD). The changes in BMD and body composition, 6 and 12 months after ceasing GH treatment, were assessed using dual-energy X-ray absorptiometry in eight patients with GHD (age range, 13.8–17.5 years). Seven age-matched normal subjects who had completed growth were assessed at 0 and 12 months. Total body BMD was low at baseline ( p < 0.05) in patients with GHD compared with the predicted values based on sex-specific regression equations, with height, weight and age taken into account. Total body, lumbar spine and femoral neck BMD increased in the patients and controls at 12 months. LTM decreased significantly by a mean of 1.37 kg in the patients with GHD at 12 months whereas there was a non-significant increase in LTM in the control group. The percentage of body fat increased in all patients with GHD at 6 and 12 months, from 27.2 ± 11% (mean ± SD) at baseline to 32 ± 9.9% at 12 months ( p = 0.009). There was no significant increase in mean percentage body fat in the control group. The ratio of android (trunk):gynoid (legs) fat was calculated using default settings of dual-energy X-ray absorptiometry. The mean android:gynoid fat ratio increased, though non-significantly, in patients with GHD at 12 months, with 6 of 7 showing an increase; no change was observed in the control group. These results indicate that BMD continues to increase 12 months after ceasing GH therapy in adolescents with GHD, but that unfavourable alterations in body composition occur.     

The gonadotrophins response to GnRH test is not a predictor of neurological lesion in girls with central precocious puberty

OBJECTIVES: To assess the value of gonadotrophin releasing hormone (GnRH) stimulation test in identifying intracranial abnormality in girls with central precocious puberty (CPP). PATIENTS AND METHODS: A study of 67 girls diagnosed with CPP who underwent cranial MRI scans. Patients were not receiving any therapy and there were no neurological signs or symptoms at presentation. Patients underwent evaluation of GnRH stimulation test and plasma oestradiol levels at presentation. RESULTS: Mean age at onset of puberty was 6.2 years (range 2.0 to 8.0 years). Intracranial abnormalities were present in 10 (15%) patients, while 57 girls (85%) had no abnormalities. No significant difference was shown between girls with intracranial abnormality and girls without intracranial abnormality in basal LH or FSH values, peak LH or FSH values, LH/FSH peak ratios, peak LH/basal LH ratios, peak FSH/ basal FSH ratios at presentation. CONCLUSION: GnRH stimulation test does not identify those with underlying intracranial abnormality at presentation. MRI imaging remains necessary in all cases of central precocious puberty in girls.     

基础血清黄体生成素水平对女孩中枢性性早熟诊断价值的探讨

目的评价基础血清黄体生成素(LH)对女性中枢性性早熟(CPP)的诊断价值。方法以279例女性性早熟患儿为研究对象,其中CPP 175例、单纯乳房早发育(PT)104例,均行体格生长评价、骨龄测定以及基础LH、卵泡刺激素(FSH)检测和性激素激发试验等。采用受试者工作曲线(ROC)分析基础LH、FSH及其峰值对诊断CPP的意义,并分析基础LH与LH峰值的相关性。结果 CPP组的骨龄,基础LH、FSH,以及LH和FSH峰值,LH/FSH峰值之比均较PT组升高(P0.01)。以基础LH和LH峰值诊断效果较好。基础LH对骨龄7.0~9.0岁,9.0~11.0岁,11.0岁3个CPP亚组的诊断价值以11.0岁组的曲线下面积(AUC)最大。基础LH在0.45 IU/L时Youden指数最大,灵敏度为0.667、特异度为0.8;LH峰值在9.935 IU/L时Youden指数最大,灵敏度为0.748、特异度为1。基础LH与LH峰值成正相关(r=0.440、P0.01)。结论基础LH可作为诊断CPP的初筛指标,在不同骨龄阶段均有一定的诊断价值,并可作为疗效监测指标。     

下丘脑-垂体-性腺轴抑制程度对中枢性性早熟女童成年预测身高的影响

目的 研究促性腺激素释放激素类似物(GnRHa)治疗过程中下丘脑-垂体-性腺轴(HPGA)抑制程度与中枢性性早熟(CPP)女童成年预测身高(PAH)的关系,以指导临床个体化调节GnRHa 治疗剂量。方法 收集75 例CPP 女童的临床资料,记录GnRHa 治疗的不同时间点身高、骨龄(BA)、子宫卵巢容积及LH、FSH 峰值、E2 水平,计算各时间点PAH,分析PAH 改善(ΔPAH=PAH-靶身高)的情况及其与HPGA 抑制的关系,并采用阈值效应分析寻找ΔPAH 的最佳HPGA 抑制范围。结果 GnRHa 治疗后PAH 较治疗初期有明显改善。ΔPAH 与ΔBA 呈负相关;治疗24 月时ΔPAH 与LH 呈负相关。将子宫容积控制在2.3~3.0 mL 之间,LH 控制在0.8 IU/L 以下,FSH 控制在2.4 IU/L 以下对延缓BA 的增长及改善PAH 有利。结论 GnRHa 治疗能改善CPP 女童的PAH。选择合适的GnRHa 治疗剂量,将子宫容积、LH、FSH 控制在一定范围内,有利于延缓BA 及改善PAH。     

Precocious puberty

Puberty occurring before the age of 8 years in girls and 9 years in boys is considered precocious. The numerous causes of precocity can be classified as central or peripheral. Central or true precocious puberty (CPP) is due to premature activation of the hypothalamopituitary-gonadal axis and is isosexual. Peripheral or pseudoprecocious puberty (PPP) results from the production of sex steroids independent of the H-P-G axis and may be isosexual or heterosexual. CPP is the most common form of precocity involving more than 50% of children and is much more common in girls than boys. CPP is more common between 4 and 8 years. A peak serum LH levels >10 iu/1 following GnRH stimulation is the absolute evidence of CPP. Serum IGF-I levels are predictive of the outcome. Availability of CT and MRI has helped to determine the cause of CPP in most cases. Hypothalamic hamartoma is the most common tumour causing CPP especially in boys. Adrenal causes, particularly CAH, are the commonest cause of PPP in boys whereas ovarian causes are more likely in girls. Long acting GnRH analogues provide a safe and effective form of treatment of CPP.