知柏地黄丸联合大补阴丸治疗女童特发性性早熟80例

目的 观察知柏地黄丸联合大补阴丸治疗不同Tanner分期特发性性早熟女童的疗效.方法 性早熟女童80例按照Tanner分期分为2组:A组45例,为TannerⅡ期,平均年龄7.5岁;B组35例,为TannerⅢ、Ⅳ期,平均年龄7.7岁.2组均口服知柏地黄丸8～10粒/次,2次/d;大补阴丸40～50粒/次,2次/d,治疗3～6个月,每月随访1次,6个月后观察其第二性征、子宫、卵巢、骨龄变化,依据其变化判断疗效.结果 80例患儿经治疗后有效55例,无效25例,有效率68.75%.其中A组有效40例,无效5例,有效率88.9%;B组有效15例,无效20例,有效率42.8%.A组有效率明显高于B组(鳘2=19.417 P<0.001).结论 知柏地黄丸联合大补阴丸治疗女童特发性性早熟有效,治疗TannerⅡ期的患儿较Ⅲ、Ⅳ期效果更好.     

不同剂量促性腺激素释放激素类似物治疗女童特发性中枢性性早熟的临床观察

目的　观察不同剂量促性腺激素释放激素类似物 (GnRHa)缓释剂治疗特发性中枢性性早熟女童(ICPP)的疗效。方法　以达菲林针 1 0 0 μg/ (2 8d·kg)肌注两次 ,随机改用 40～ 50 μg/ (2 8d·kg) ;与随机改用 80～ 1 0 0 μg/ (2 8d·kg)治疗ICPP女童各 1 0例 ,每例共 1 2次 ,疗程 1年。起始年龄 (8.3± 0 .9)岁 ;观察治疗前后两组性征、身高、骨龄及预测成年身高及性激素、生长激素 (GH)变化。结果　两组治疗后性征均减退 ,性激素水平下降 ,骨龄增长受抑 ;两组预测成年身高改善相似。结论　小剂量达菲林治疗ICPP能有效抑制中枢性性早熟 ,改善患儿成年身高 ,较大剂量具有更实用、经济的优点     

性早熟女童血清骨钙素检测的临床意义

目的探讨特发性中枢性性早熟(ICPP)女童和单纯性乳房早发育(PT)女童血清骨钙素(OST)水平的变化及其意义。方法用放射免疫法检测25例ICPP女童和15例PT女童血清OST水平。以年龄相匹配的未发育健康女童25例作为健康对照。ICPP患儿11例接受促性腺激素释放激素拟似剂(GnRHa)治疗6个月,再次采血检测血清OST水平,与治疗前进行对比。结果ICPP组血清OST水平高于PT组和健康对照组,差异均有显著统计学意义(Pa<0.01)。PT与健康对照组血清OST水平无显著差异(P>0.05)。ICPP患儿11例GnRHa治疗后血清OST水平较治疗前显著降低(P<0.01)。结论血清OST有助于ICPP与PT的鉴别诊断,并可作为判断GnRHa延缓ICPP患儿骨骼成熟的疗效指标之一,同时也有助于监测其对骨密度的影响。     

学龄期肥胖儿童心脏自主神经功能分析

目的对学龄期肥胖儿童的心率减速力(DC)、心率加速力(AC)及心率变异性(HRV)进行分析,并观察肥胖儿童体重指数(BMI)与DC、AC及HRV的相关性。方法选取学龄期肥胖儿童108例,其中血脂正常组75例,血脂异常组33例,另选取103例学龄期健康儿童为对照组。对所有受试者行24 h动态心电图检查,分别将肥胖组与对照组、肥胖儿童中血脂异常组与血脂正常组的DC、AC及HRV进行比较,并将肥胖儿童的BMI与DC、AC及HRV进行相关性分析。结果肥胖组的DC、RR间期总体标准差(SDNN)、RR间期平均值的标准差(SDANN)、相邻RR间期差值的均方根(RMSSD)、低频功率(LF)、高频功率(HF)均低于对照组,AC高于对照组(P0.05)。肥胖儿童中血脂异常组的DC、SDNN、SDANN、RMSSD、LF、HF均低于血脂正常组,AC、BMI高于血脂正常组(P0.01)。肥胖儿童BMI与DC、SDNN、SDANN、RMSSD、HF呈明显负相关(P0.05);与AC呈明显正相关(P0.05)。结论学龄期肥胖儿童的自主神经功能受损,表现为迷走神经张力减低,其中合并血脂异常者以上表现尤为突出。肥胖程度越高,迷走神经张力越低,发生心血管疾病的危险性可能越大。     

促性腺激素释放激素类似物对特发性中枢性性早熟女童体重指数影响的长期观察

目的：动态研究促性腺激素释放激素类似物（GnRHa）治疗对特发性中枢性性早熟（ICPP）女童体重指数（BMI）的影响。方法：对2003年1月至2006年1月确诊为ICPP 的134例女童进行随访研究,其中57例给予GnRHa治疗（疗程1.69±0.43年）,测量治疗前、治疗结束时及近成年身高时的身高、体重、骨龄及BMI等,并与未治疗组（77例）比较。结果：（1）GnRHa治疗组在治疗结束时的预测终身高标准差分值（SDS）较治疗前明显升高（P0.05）,且近成年身高SDS明显高于靶身高SDS（P0.05）;近成年身高时的BMI SDS比治疗前和未治疗组明显降低（P<0.01）,但治疗前、治疗结束时及近成年身高时BMI平均值都在±1SD内,处于正常范围。结论：GnRHa对改善ICPP女童终身高有显著疗效;GnRHa治疗后的BMI改变处于正常范围。     

双酚A与女童性早熟的关系

目的探讨双酚A(BPA)与女童性早熟的关系。方法选择2012年8月至12月就诊的103例6~8岁性早熟女童,根据性早熟分类标准分为特发性中枢性性早熟(ICPP)组(n=47)及单纯乳房发育(PT)组(n=56),选取同期同年龄乳房未发育女童53例为正常对照组。高效液相色谱串联质谱法(HPLC-MS-MS)测定血清BPA,酶联免疫法测定血清Kisspeptin,化学发光法测定血清性激素,比较和分析三组间的差异。结果 ICPP组和PT组的血清BPA检出率高于对照组,ICPP组血清BPA水平高于PT组及对照组,差异有统计学意义(P均0.05);ICPP组血清Kisspeptin水平也高于PT组及对照组,差异有统计学意义(P均0.05);ICPP组血清黄体生成素(LH)峰值及LH/卵泡刺激素(FSH)比值高于PT组,ICPP组及PT组血清雌二醇(E2)水平高于对照组,差异有统计学意义(P均0.05)。三组的血清BPA水平与E2、LH峰值及LH/FSH比值有相关性(P0.05),与血清Kisspeptin水平及FSH峰值无相关性(P0.05)。结论 BPA与女童ICPP发生有一定的相关性。     

特发性中枢性性早熟女童血清Kisspeptins水平变化及意义

目的 通过检测Kisspeptins在特发性中枢性性早熟(ICPP)女童血清中的水平,探讨其在ICPP发生发展过程中的作用.方法 以20名6～8岁Tanner Ⅰ期健康女童作为健康对照组,经生长发育评价结合骨龄、盆腔B超及促性腺激素释放激素激发试验结果选取20名6～8岁处于TannerⅡ期的ICPP女童作为实验1组,20名6～8岁处于TannerⅢ期的ICPP女童作为实验2组;用放射免疫法测定血清中黄体生成素(LH)水平;Western blot法检测3组女童血清中Kisspeptins水平.采用SPSS 12.0软件进行统计学分析.结果 实验1组血清Kisspeptins水平较健康对照组增加了0.86倍(P＜0.01),实验2组血清Kisspeptins水平较健康对照组增加了 1.68倍(P＜0.01);实验1组血清Kisspeptins水平与LH峰值、骨龄均呈正相关(r=0.42、0.38,Pa＜0.05);实验2组血清Kisspeptins水平与LH峰值、骨龄也均呈正相关(r=0.48、0.40,Pa＜0.05).结论Kisspeptins/G蛋白偶联受体54信号在女童ICPP的启动和发展过程中起重要作用.     

女童性早熟的临床诊断程序

女童性早熟是指女童在8岁以前出现第二性征.对性早熟患儿恰当的临床评估基于对正常青春期发育过程及引起性早熟多病因的了解.临床诊断始于详细的病史采集及全面的体格检查,在此基础上选择适当的实验室及影像学检查,根据下丘脑-垂体-性腺轴是否提前激活(促性腺激素释放激素刺激试验)来区分中枢性性早熟及外周性性早熟,对于进一步的病因鉴别诊断及治疗策略的选择均具有重要意义.     

特发性中枢性性早熟的发病机制及其诊断

数10年来，儿童性早熟渐趋增多，绝大多数为特发性中枢性性早熟，表现为患儿骨成熟加速，长骨骨骺提前愈合，其成年后身高较正常人矮，性发育提前，月经早潮，给家庭和患儿带来一定的社会心理压力，因而正确认识此病，及时明确诊断，有利于采取正确而有效的治疗措施。本文从青春期的发动年龄、机制、性早熟的诊断及其评估等几个方面作一综述。     

女童全身体脂比率与性早熟的关系

目的 既往研究提示体重指数（BMI）与女童发育年龄有关,但是否与女童全身脂肪比率相关尚不清楚。该研究旨在分析全身脂肪比率与性早熟的关联性。方法 依据中枢性性早熟诊断与治疗共识将2017年7~8月收治的128例性早熟患儿分为中枢性性早熟组（CPP组,87例）和外周性性早熟组（PPP组,41例）,同时纳入51例未发育女童作为对照组。利用双能X线吸收测量法检测上肢组织、腿部组织、躯干组织、男性区、女性区和全身组织的脂肪比率,结合研究对象的年龄、BMI、BMI-Z值、骨龄、卵巢体积、激素水平等实验室检查结果,综合分析脂肪比率和性早熟的相关性。结果 与对照组比较,CPP组和PPP组患儿上肢、腿部、躯干、男性区、女性区和全身组织的体脂率参数以及腿部/全身脂肪比和（上肢+腿部）/躯干脂肪比均显著升高（均P < 0.05）,而上述所有体脂率和脂肪分布指标在CPP组和PPP组间比较差异均无统计学意义（均P > 0.05）。在性早熟女童中,高体脂率组的黄体生成素（LH）基础值及黄体生成素释放激素（LHRH）激发试验的LH峰值、LH/卵泡刺激素峰值均显著高于低体脂率组,差异均有统计学意义（均P < 0.05）。同时高体脂率组和低体脂率组LH基础值相比于对照组均显著升高（均P < 0.05）。结论 体脂含量的增加可能是诱发女童性早熟的因素,但具体机制尚有待进一步研究。     

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。     

The long-term effect of cyproterone acetate on growth in girls with idiopathic precocious puberty

To define the effect of cyproterone acetate (CPA) on statural growth, 25 girls with idiopathic precocious puberty who had been treated with CPA were studied retrospectively. The final height SDS was –1.12±1.16 (mean±SD). The daily CPA dose was negatively related to the final height SDS. We divided our subjects into two groups according to the daily CPA dose [low dose, 84.9±15.4 mg/m² (n=19) vs high dose, 135.8±17.1 mg/m² (n=6)]. In the low dose group, the difference of the final height SDS minus height SDS for bone age at the initiation of CPA treatment was 0.55±1.16 and final height SDS was –0.82±1.05. The final height was not significantly different from the target height in the low dose group subjects whose target heights were obtained. Since the increment of height age to the increment of bone age during the treatment was significantly less in the group needing and treated with high dose CPA, high doses of CPA may reduce growth velocity more than its suppressive effect on bone maturation. These results suggest that CPA has an effect on statural growth in girls whose clinical symptoms can be controlled with a low dose of CPA, although they have not been compared with final height in untreated Japanese girls.Androcur Study Group consists of the following personnel: K. Fujieda, Department of Paediatrics, Hokkaido University School of Medicine; A. Okuno, Department of Paediatrics, Asahikawa Medical College; K. Sanayama, Department of Paediatrics, Chiba University School of Medicine; K. Shimozawa, Department of Paediatrics, Tokyo Medical and Dental University School of Medicine; N. Matsuo, Department of Paediatrics, Keio Gijuku University School of Medicine; Y. Oyama, Department of Paediatrics, Kitasato University School of Medicine; Y. Igarashi, Department of Paediatrics, Hamamatsu Medical College; M. Ogawa, Department of Paediatrics, Nagoya University School of Medicine; S. Okada, Department of Paediatrics, Osaka University School of Medicine; T. Kondo, Third department of Internal Medicine, Osaka Children's Medical Centre; Y. Nishi, Department of Paediatrics, Hiroshima Red Cross Hospital     

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。     

女性真性性早熟药物治疗疗效分析

目的　评价亮丙瑞林、达那唑、酮康唑、醋酸甲羟孕酮治疗女性真性性早熟的疗效。方法　女性性早熟 6 1例分别用酮康唑 4～ 8mg/ (kg·d) ,达那唑 5～ 10mg/ (kg·d) ,亮丙瑞林 30～ 90 μg/kg ,醋酸甲羟孕酮 8～10mg/d治疗。观察治疗前后患儿第二性征、骨龄、血清性激素变化。 结果　亮丙瑞林组治疗后第二性征减退 ,血激发试验峰黄体生成素 (LH)、卵泡刺激素 (FSH)及基础FSH、血清胰岛素样生长因子 1(IGF 1)显著下降 (P均 <0 .0 5 ) ,骨龄增长减慢 ;达那唑组第二性征减退 ,但血指标改变不大 ,个别病例有丙氨酸氨基转移酶 (ALT)改变等副作用 ;酮康唑对第二性征和激素变化均不明显 ;醋酸甲羟孕酮组除乳房略回缩外其他性征及激素无变化 ,且阴道出血率高。结论　亮丙瑞林治疗女性真性性早熟除可使第二性征逆转外 ,还可使激素下降、骨龄生长延缓 ;而达那唑、酮康唑、醋酸甲羟孕酮仅能改变第二性征 ,不能改变其他指标 ,且副作用大 ,故认为亮丙瑞林可作为治疗女性真性性早熟的理想药物     

Early growth acceleration in girls with idiopathic precocious puberty

OBJECTIVE: To determine the growth pattern of girls with idiopathic precocious puberty (IPP) from birth until diagnosis. STUDY DESIGN: We studied 47 girls with IPP and 35 control girls. In each subject, height and weight were measured at diagnosis, whereas data on height from birth until diagnosis were taken from the personal health book of the patient. Height standard deviation score (HSDS) and body mass index SDS were calculated. RESULTS: Mean age (+/-SD) of the girls with IPP was 7.6 (1.1) years and of control girls was 7.5 (0.9) years. At birth, HSDS of the patients with IPP was -0.01 (0.8); at the age of 2 years, 0.42 (1.2); at the age of 4 years, 0.64 (1.1); and at diagnosis, 1.23 (1.7) (P < .001). HSDS of control girls was 0.02 (0.8) at birth, 0.25 (0.8) at 2 years, 0.12 (0.9) at 4 years, and 0.19 (1.1) at assessment (P > .05). There was no statistical difference between body mass index SDS of the patients 0.6 (1.1) versus that of control girls 0.5 (1.0). CONCLUSIONS: The early growth acceleration pattern may be used as an additional clue to the diagnosis of idiopathic precocious puberty.     

先天性肾上腺皮质增生症合并中枢性性早熟临床分析

目的分析先天性肾上腺皮质增生症(CAH)患儿合并中枢性性早熟的临床表现。方法通过回顾性分析和临床随访,在12例21羟化酶缺乏患儿中发现20例合并中枢性性早熟。根据治疗和非治疗情况分为A组(9例)和B组(11例),分析其发生的年龄、骨龄以及与激素替代治疗的关系。结果A组中发生中枢性性早熟的实际年龄平均为(5.6±2.1)岁,骨龄平均为(12.0±3.2)岁;B组中诊断中枢性性早熟平均年龄在(6.8±1.1)岁;骨龄平均值在(11.7±2.0)岁,两组在统计学上差异无显著性。B组应用氢化可的松治疗后平均2.3年出现中枢性性早熟。结论CAH患儿骨龄发育提前是发生性早熟的主要原因,早诊断和早治疗可改善预后。     

来曲唑治疗特发性中枢性性早熟男童的临床观察

目的 对骨龄大于13岁、身高偏矮小的中枢性性早熟（ICCP）男童应用来曲唑（letrozole）治疗,观察该治疗方案对延缓骨龄老化和改善成年预测身高的效果和不良反应。方法 将骨龄大于13岁的ICCP男童20例随机分为2组,每组10例,一组予来曲唑口服治疗6个月[2.5 mg/（m2·d）,Qd];另一组为对照组,定期观察,不予特殊治疗。观察两组男童骨龄、生长速度、身高标准差积分、预测成年身高标准差积分、性征发育情况及性激素水平的变化。并观察来曲唑治疗相关不良反应。结果 试验开始6个月后,来曲唑组和对照组男童骨龄均显著增加,但来曲唑组男童骨龄增长（13.82±0.23岁）较对照组男童（14.47±0.30岁）明显缓慢（PPP结论 对骨龄超过13岁、身高受损显著的ICCP男童应用来曲唑能够有效减缓骨龄老化,改善预测成年身高,且未见明显不良反应。     

Value of basal luteinizing hormone level combined with uterine volume measurement in the early diagnosis of central precocious puberty in girls with different Tanner stages北大核心CSCD

Objective To study the value of basal luteinizing hormone (LH) level combined with uterine volume measurement in the early diagnosis of central precocious puberty (CPP) in girls with different Tanner stages. Methods A retrospective analysis was performed on the girls who presented with breast development before the age of 8 years and attended the Third Affiliated Hospital of Zhengzhou University from January 2017 to September 2022. According to the results of gonadotropin-releasing hormone (GnRH) agonist test, the girls with peak LH ≥5.0 IU/L and peak LH/follicle stimulating hormone ≥0.6 were enrolled as the positive group, and the other girls were enrolled as the negative group. The two groups were compared in terms of the basal LH level and uterine volume. The receiver operating characteristic (ROC) curve was used to analyze their value in the early diagnosis of CPP. Results For the girls with Tanner B2 and B3 stages, the positive group had significantly higher basal LH level and uterine volume than the negative group (P<0.05). The basal LH level had an optimal cut-off value of 0.325 IU/L and 0.505 IU/L respectively in the diagnosis of Tanner stage B2/B3 CPP, while uterine volume had an optimal cut-off value of 1.639 mL and 2.158 mL respectively. Basal LH level combined with uterine volume measurement had a significantly larger area under the ROC curve than uterine volume measurement alone (P<0.001), but with no significant difference compared with that of basal LH level measurement alone (P>0.05). Conclusions Basal LH level combined with uterine volume measurement is valuable in the early diagnosis of CPP in girls with different Tanner stages, which provides a basis and guiding significance for clinical diagnosis of CPP. © 2023 Xiangya Hospital of CSU. All rights reserved.