骨龄指数在儿童性早熟诊断中的临床意义

目的:探讨性早熟患儿骨龄指数(BAI)与促性腺激素释放激素(GnRH)激发试验各指标之间的相关性.方法:对临床诊断为性早熟的38例女童均做GnRH激发试验,进行左手腕正位X线摄影并用中国人手腕骨骨龄评价方法与标准(CHN法)测骨龄,骨龄除以实际年龄得BAI,根据临床表现和实验室结果将患儿分为特发性中枢性性早熟(ICPP)组和外周性性早熟(PPP)组,并分别对ICPP组和PPP组女童的BAI与GnRH激发试验各指标做相关性分析.结果:(1)GnRH激发试验前后2组促黄体生成素(LH)和卵泡刺激素(FSH)基础值间差异无统计学意义(P＞0.05),LH和FSH峰值均较基础值明显升高,且2组间LH峰、LH/FSH差异均有统计学意义(P＜0.01).(2)ICPP组骨龄较实际年龄明显提前,差异有统计学意义(t=2.756,P＜0.05),PPP组骨龄虽较实际年龄有所提前,但差异无统计学意义(t=0.308,P＞ 0.05).(3)ICPP组BAI与LH峰、FSH峰、LH/FSH峰之间呈正相关(r分别为0.684,0.544和0.753,P＜ 0.05或P＜0.01).结论:BAI越大中枢性性早熟可能性越大,越需要做GnRH激发试验.     

青春期糖尿病鼠睾丸支持细胞功能研究

４０日龄雄性Ｗｉｓｔａｒ大鼠４０只，分为对照（Ｃ）组：腹腔注射赋形剂；糖尿病（Ｄ）组：腹腔注射ＳＴＺ，５５ｍｇ／ｋｇ体重；糖尿病胰岛素及时治疗组（Ｃｏｎｔ．Ｄ）：给ＳＴＺ３天后胰岛素治疗；糖尿病胰岛素延迟治疗（Ｄｅｌ．Ｄ）组：注射ＳＴＺ３０天后胰岛素治疗，睾丸ＡＢＰ和培养支持细胞功能被研究，结果是：Ｃ，Ｃｏｎｔ．Ｄ，Ｄｅｌ．Ｄ和Ｄ组的睾丸ＡＢＰ平衡解离常数（Ｋｄ）分别是５．９１±１．７４，２．２１±０．４５，２．１３±０．７９和５．５４±１．１０ｎｍｏｌ／Ｌ，Ｃｏｎｔ．Ｄ和Ｄｅｌ．Ｄ组显著低于Ｃ和Ｄ组（Ｐ＜０．０５，ｎ＝３）；ＡＢＰ的最大结合容量（Ｂｍａｘ）分别是３１．７８±５．４７，２９．８８±３．７４，２６．７４±３．６９和２８．６９±４．５４ｆｍｏｌ／ｍｇ蛋白，组间比较差异不显著（Ｐ＞０．０５，ｎ＝３）；培养支持细胞的ＡＢＰ２４ｈ分泌量各组分别是２３．５４±５．５７，２３．６１±８．６０，１８．０６±７．５３和１４．９２±７．８９ｆｍｏｌ／１０￣６细胞，Ｄ组显著低于Ｃ和Ｃｏｎｔ．Ｄ组（Ｐ＜０．０５）。     

Testosterone, antisocial behavior, and social dominance in boys: pubertal development and biosocial interaction.

BACKGROUND: Studies linking testosterone and antisocial behavior in humans have produced mixed results. Adolescence offers a promising period to study this relationship; circulating testosterone increases dramatically in boys during puberty, and antisocial behavior increases during the same period. METHODS: Our analyses were based on boys aged 9-15 years who were interviewed during the first three waves of the Great Smoky Mountains Study. Measures included interview assessment of DSM-IV conduct disorder (CD) symptoms and diagnosis, blood spot measurement of testosterone, Tanner staging of pubertal development, and assessment of leadership behaviors and peer deviance. RESULTS: The adolescent rise in CD was primarily attributable to an increase in nonphysically aggressive behaviors. Increasing levels of circulating testosterone and association with deviant peers contributed to these age trends. There was no evidence that physical aggression was related to high testosterone. Evidence of biosocial interactions was identified; testosterone was related to nonaggressive CD symptoms in boys with deviant peers and to leadership in boys with nondeviant peers. CONCLUSIONS: The results are consistent with the hypothesis that testosterone relates to social dominance, with the assumption that behaviors associated with dominance differ according to social context.     

Pineal ganglioglioma with premature thelarche

We report a case of a 6-year-old girl with a ganglioglioma in the pineal region presenting with a rare clinical picture of premature thelarche. Intracranial gangliogliomas are rare, especially in the pineal region. Their character, origin, treatment, and prognosis remain controversial. The pathophysiology of precocious puberty associated with pineal ganglioglioma is discussed.     

Brain and Pituitary Melatonin Receptors in Male Rat during Post-Natal and Pubertal Development and the Effect of Pinealectomy and Testosterone Manipulation

Using quantitative autoradiography, melatonin receptors have been studied during post-natal and pubertal development of the rat in 2 brain and 2 pituitary structures. In the pars distalis of anterior pituitary, melatonin receptors decrease gradually in density after birth and disappear in 30 day-old animals. In contrast melatonin binding is only expressed in the paraventricular nuclei of the thalamus at the age of 21–23 days and is always present in adult animals. In the suprachiasmatic nuclei and in the pars tuberalis of the pituitary, melatonin receptor density decreases after birth, remains stable for approximately 1 month and increases again at puberty to reach the birth values in the adult. This increase was absent in pinealectomized and in castrated animals but present in castrated animals receiving testosterone suggesting that it depends upon circulating testosterone and melatonin levels. These results show that melatonin receptors are differentially regulated during post-natal development in each of the 4 structures studied, and that melatonin and testosterone are 2 factors which could be involved in the regulation of melatonin receptor density in the suprachiasmatic nuclei and pars tuberalis.     

Application of the assay of urine FSH B-subunit in patients with pubertal disorders

<正>Objective:To evaluate the diagnostic predictive value in the identification of puberty disorders by means of ELISA of β-FSH subunit levels in successively collected urine samples compared to RIA of intact FSH in serum obtained from the normal control subjects and patients with puberty disorders, respectively. Subjects and Methods: Five male and four female volunteers were recruited as controls. Four patients with the hypogonadotropic hypogonadism, five patients with hypergonadotropic hypogo-nadism, four patients with the central precocious puberty and one patient with isosexual peripheral precocious puberty collected successively their early-morning urine samples for 30 to 32 days. The urine β-FSH subunit was assayed with the method of ELISA, then adjusted by creatinine (Cr) concentration. Results:Comparing with their cotemporary groups, patients with the hypogonadotropic hypogonadism had lower levels of urine β-FSH, and patients with idiopathic hypergonadism had higher levels with irregular fluctuation. Meanwhile, patients with the central precocious puberty had much higher levels of urine β-FSH with irregular peaks, and patients with isosexual peripheral precocious puberty had almost normal levels. The patterns were coincident with the clinical characteristics and serum FSH levels. Conclusion: The ELISA of urine β-FSH subunit possesses a number of advantages over the RIA of serum FSH level, such as low cost, simplicity and reliability in the clinical practice. It can be used for the diagnoses of puberty disorders. In addition, it is possible and much easier, comparing with blood samples, to collect successively urine samples for research of pathophysiologi-cal dynamics of FSH secretion in puberty disorders and other reproductive dysfunction.     

Age at Puberty and Risk of Testicular Germ Cell Cancer (Ontario, Canada)

Objectives: Incidence rates of testicular cancer are increasing among postpubescent men. This suggests that putative exposures may operate early in life and have changed over time. The age at which endocrine activity accelerates (age at puberty) may be such an exposure. This study was undertaken to investigate the relationship between age at puberty and testicular cancer risk.Methods: A population-based case-control study was conducted in the province of Ontario, Canada which included males, aged 16 to 59 years, diagnosed with testicular germ cell cancer between 1987 and 1989, and age-matched controls. Data were collected on 502 cases, 346 case mothers, 975 controls, and 522 control mothers. Surrogate measures for age at puberty included age at starting to shave, appearance of hair, growth spurt, and voice change.Results: A protective effect of later puberty was evident for all four measures of puberty as reported by both subjects and mothers, and greater protection was conferred when the greatest number of later puberty events were reported. Risk associated with earlier puberty was inconclusive.Conclusions: As age at puberty is decreasing in the population, the proportion of boys experiencing the protective effect of later puberty may be diminishing. This may help explain the increasing incidence of testicular cancer. Cancer Causes and Control 1998, 9, 253-258     

An age-related homeostasis mechanism is essential for spontaneous amelioration of hemophilia B Leyden

Regulation of age-related changes in gene expression underlies many diseases. We previously discovered the first puberty-onset gene switch, the age-related stability element (ASE)/age-related increase element (AIE)-mediated genetic mechanism for age-related gene regulation. Here, we report that this mechanism underlies the mysterious puberty-onset amelioration of abnormal bleeding seen in hemophilia B Leyden. Transgenic mice robustly mimicking the Leyden phenotype were constructed. Analysis of these animals indicated that ASE plays a central role in the puberty-onset amelioration of the disease. Human factor IX expression in these animals was reproducibly nullified by hypophysectomy, but nearly fully restored by administration of growth hormone, being consistent with the observed sex-independent recovery of factor IX expression. Ets1 was identified as the specific liver nuclear protein binding only to the functional ASE, G/CAGGAAG, and not to other Ets consensus elements. This study demonstrates the clinical relevance of the first discovered puberty-onset gene switch, the ASE/AIE-mediated regulatory mechanism.     

青少年钙负荷的降压效应研究

对９６名１４～１７岁学生进行了２个月的钙干预试验。在实验前后进行体格指标、血清及尿电解质、血浆维生素Ｄ３、血小板内Ｃａ（２＋）及Ｍｇ（２＋）的测定。结果表明：青少年血压与皮脂厚度、脉率、血小板内Ｃａ（２＋）及尿Ｃａ（２＋）呈显著正相关关系（ｒ＝０．２７～０．３３，Ｐ＜０．０５）；血压下降与血浆维生素Ｄ３、肾排Ｎａ＋能力呈负相关关系（ｒ＝－０．４２～－０．４４．Ｐ＜０．０５）；血小板内Ｃａ（２＋）与舒张压下降，尿Ｎａ＋与有家族史组血压下降关系密切。提示钙负荷对青少年有显著的降压作用，但降压作用具有选择性。