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PubMedDate not supplied

[Value of the assay of urinary gonadotropins in pediatric endocrinology].

An assay for urinary gonadotropins (UG) performed after acetone extraction is presented. This dosage was performed either on a sample of the 24 assay urine, or on the fractionated 12 hr/12 hr urines (night/day) in normal children whose ages ranged from 2 to 20 years and in children presenting with various endocrine diseases (on about 2,000 urine samples). Normal values were established according to sex and stage of puberty. In boys, the lack of overlap between values of LH (UI/24 hr) observed in stage I (prepubescent, 9-13 yrs) and those observed in stage II represents an obvious biological marker of the onset of puberty. The night/day ratio of LH also increases close to puberty, reflecting the onset of the well-known night secretion of LH, at the time of the first stages of puberty. In girls, the preferential increase in FSH is the best criterion for the onset of puberty. In children with endocrine diseases, assay for UG/24 hr is a valuable parameter of the gonadotropic function allowing 1. to separate delayed puberty from hypogonadotropic hypogonadism; 2. to confirm a diagnosis of precocious puberty and 3. to control a treatment with LHRH analogous.

Adolescent↗
PubMed1998

Cystic fibrosis through a female perspective: psychosocial issues and information concerning puberty and motherhood.

The purpose of the study was to investigate psychosocial issues concerning puberty and motherhood among CF adult females, to see how they had obtained and conceived information on these matters and how they would like information to be given. Fourteen adult CF females were interviewed. The majority of the women felt socially accepted and did not remember being ashamed over their delayed puberty. Thirteen of the women had been or were living in stable sexual relationships. However, the study revealed problems with destructive behaviour during puberty due to thoughts about premature death, secret worries over delayed puberty, poorly received information about puberty and fertility, avoidance of close relationships with the opposite sex during adolescence and concerns about being a mother with a chronic illness. Information about puberty and fertility should be given individually and in small discussion groups with teenage girls combined with thorough medical and psychological guidance concerning motherhood.

Adolescent↗
PubMedDate not supplied

[Endocrine biochemistry of puberty].

Puberty corresponds to the development of gonads and secondary sexual characteristics, and on a biological point of view, to the functional maturation of the gonadal axis. Puberty begins at the age of 11.5 to 12 years in males and 10.5 to 11 years in females. Depending on secondary sexual characteristics, particularly pubic pilosity, puberty is classified in five stages (Tanner's stages). During puberty growth velocity increases in response to gonadal steroid secretion. From a biochemical point of view, three steps are involved in the development of the hypothalamo-hypophysogonadal axis: 1. nocturnal hypothalamic GnRH secretion increases and becomes pulsatile (a peak every 60 to 90 min); 2. the pituitary gonadotrophins FSH and LH follow the same pattern of secretion as GnRH; increase of GnRH and FSH/LH secretion is due to a decrease in hypothalamo-hypophysal sensitivity to the negative feed back exerted by circulating gonadal steroids; 3. secretion of estradiol in females and testosterone in males increases, as a consequence of pituitary stimulation. Hormonal exploration of puberty is mainly based on the measurement of FSH-LH and testosterone or estradiol. SDHA is also measured to investigate adrenal androgen secretion, which increases three or four years before puberty; this is related to the maturation of adrenal androgenic function (adrenarche). Dynamic tests are used to evaluate the biological stage of puberty (LH-RH test) and to measure the functional capacity of the testes. Pubertal abnormalities can theoretically be divided into precocious and delayed puberty. In the former, clinical and biological characteristics are used to define: dissociated puberties, central precocious puberty and peripheral precocious puberty. In the latter, hypogonadism has either a central origin (hypogonadotropic hypogonadism) or peripheral origin (hypergonadotropic hypogonadism).

Child↗
PubMed1979

[Retardation of puberty and growth].

The disturbed puberty is most frequently manifested by a retardation of sexual maturation and growth. For a correct classification of a developmental disturbance the knowledge of the endocrine processes, of the stages of puberty, tables of height and methods of the assessment of the skeletal maturity is necessary, the use of which for the population of the GDR is discussed. The large differential diagnosis of the delayed puberty and of the short stature must be above all concentrated to the causal-therapeutically influencible endocrinopathies, even though the proportion of the constitutional delayed puberty as an extreme variant of the norm with a good spontaneous prognosis prevails. In order to prevent serious psychosocial conflicts, after transgression of a critical age limit of about 16 years in sexual immaturity an adequate hormone treatment should be introduced even then, when the etiopathogenesis of the disturbance could not be completely clarified.

Adolescent↗
PubMed1986

2-bromo-alpha-ergocryptine mesylate (CB-154) inhibits prolactin and luteinizing hormone secretion in the prepubertal female rat.

Treatment of immature female rats with 100 micrograms 2-bromo-alpha-ergocryptine mesylate (CB-154) per ml drinking water beginning on Day 30 of age until vaginal opening delayed puberty by 6 days. Rats treated with CB-154 exhibited vaginal opening at 43.3 +/- 0.6 days whereas controls exhibited vaginal opening at 37.9 +/- 0.8 days. Most interestingly, serum levels of luteinizing hormone (LH) and prolactin (PRL) on Days 31-35, determined by a homologous radioimmunoassay were significantly lower in treated rats than in controls. The ovarian concentrations of progesterone (P) and androstenedione (A) were lower in rats treated with CB-154 than in controls; ovarian estradiol (E2) concentrations were low in both groups. Serum levels of P (but not A and E2) were reduced on Days 31-35 of the treatment period. Cessation of the CB-154 treatment on the morning of Day 35 returned the onset of puberty to normal values; steroid and gonadotropin levels also returned to normal values within 2 days after removal of the CB-154 from the drinking water. Near the time of onset of puberty, serum levels of LH in rats treated with CB-154 returned to control values. These data indicate that in the female rat the delay in puberty induced by CB-154 might be due to a reduction in the secretion of LH, especially since the onset of delayed puberty in rats treated with CB-154 correlates with an increase in the serum level of LH. Further studies are needed to elucidate the specific effects of hypoprolactinemia on ovarian function and the onset of puberty in the rat.

Androstenedione↗
PubMed2006

[Influence of different parameters on final height of boys with constitutional delay of growth and puberty].

BACKGROUND: Constitutional delay of growth and puberty (CDGP) is a variant of normal pattern of growth and development with strong familial impact. THE AIM of the study was to assess the influence of birth weight and length, parents' height, boys height and bone age at the age of 14-16 years and mutation age on their final height. MATERIAL AND METHODS: In 69 boys in the mean age of 14.9+/-1.2 years, whose height fell below the 3rd percentile, CDGP was diagnosed. After completion of growth (at the age of 22.6+/-3.5 years) correlations of different parameters with final height were estimated. RESULTS: Statistically significant correlation between birth weight, parents' height, mutation age and final height was observed, but no association between birth length, height and bone age in the age group of 14-16 years and the final height was found. CONCLUSIONS: Adult height of boys with CDGP is directly proportional to birth weight, mutation age and parents' height.

Adolescent↗
PubMed1997

Ovarian granulosa and theca interstitial cells: a morphological and physiological analysis in guanethidine denervated rats at pre-puberty.

Since ovary denervation causes delayed puberty, we investigated the relative importance of ovary innervation on the morphology and physiology of theca interstitial cells (TIC) and granulosa cells (GC) in female rats at pre-puberty. Elimination of the sympathetic innervation was performed by long term post natal treatment with guanethidine (GD), an adrenergic blocking agent. The sympathectomized rats exhibited: reductions in follicular volume (40%), granulosa cells area (43%) and theca interstitial cell volume (50%). Ovarian concentrations of pregnenolone (P5) and progesterone (P4) were decreased whereas no differences were observed in androstenedione (A) and estradiol (E2). The intensity of the immunocytochemical reaction for 3 beta hydroxysteroid dehydrogenase (3 beta-HSD) detected only in interstitial cells, did not show any difference. These in vivo results include the TIC in the bulk of ovarian structures affected by GD denervation at pre-puberty as it was already observed for GC. The reduced area/volume occupied by these cells in the GD treated ovary is associated to a blockade of the initial steps of the steroidogenic pathway, probably at the level of the cholesterol side chain cleavage enzyme (P450 s.c.c.), previously to P5 synthesis, since P5 is reduced. Similar intra ovarian concentrations of androgens are discussed in terms of possible pineal deafferentation promoted by GD at high doses.

Adrenergic Antagonists↗
PubMed2006

Prolongation of growth by treatment of 11-hydroxylase deficiency with depot-leuprolide, growth hormone, and hydrocortisone.

A 4-10/12 year-old boy presented with tall stature and advanced secondary sexual characteristics. His bone age was 13 years giving him a height prediction of 147 cm. An initial 11-deoxycortisol level of 13,770 ng/dl and associated hypertension suggested the diagnosis of 11-hydroxylase deficiency, which was confirmed by dexamethasone suppression and genotyping. Treatment strategy was based on the premise that known hypothalamic priming resulting in early pubertal development could be averted by delaying puberty with leuprolide; also that effects of hydrocortisone and leuprolide on attenuating growth could be counteracted by growth hormone. The combined treatment resulted in a final height at age 12 years which was 25.4 cm greater than predicted, and bone density above average. We conclude that delaying puberty until an appropriate age, offsetting growth suppression, and improving bone mineralization can be effectively achieved using glucocorticoids, leuprolide and growth hormone in patients with 11-hydroxylase deficiency.

Adrenal Hyperplasia, Congenital↗
PubMed1981

Acceleration and delay of puberty in female housemice: methods of delivery of the urinary stimulus.

A series of 7 experiments tested various methods for delivery of male and female urinary chemosignals which affect age of 1st estrus in young female mice. An initial experiment demonstrated that in and of themselves none of the delivery techniques used alters puberty without the accompanying urine stimulus. A series of 4 experiments with male urine revealed that acceleration of puberty occurred only when the females could achieve direct contact with the urine. Possible reception via the vomeronasal organ is suggested as an explanation for these data. In contrast, the urinary chemosignal from grouped females which delays 1st estrus produces its effect regardless of the mode of delivery. Reception of this chemosignal apparently may occur at the olfactory epithelium.

Age Factors↗
PubMed2005

Assessment of pubertal development in Egyptian girls.

Puberty is a significant event of human growth and maturation associated with marked physiological and psychological changes. The aim of this study was to assess normal pubertal development in Egyptian girls to define normal, precocious and delayed puberty. The present study included a cross-sectional sample of 1,550 normal Egyptian girls of high and middle socioeconomic class living in Cairo. Their ages ranged from 6.5 to 18.5 years. Pubertal assessment was made according to Tanner staging. The mean menarcheal age (MMA) was estimated using probit analysis. Weight and height were measured and body mass index (BMI) was calculated. The mean age at breast bud stage (B2) was 10.71+/-1.6, pubic hair stage (PH2) was 10.46+/-1.36, while axillary hair stage (A2) was 11.65+/-1.62 and MMA was 12.44 years. The mean age at attainment of puberty was compared with those of other Egyptian studies and other populations. Girls of the present study started pubertal development and achieved menarche earlier than those of previous Egyptian studies confirming a secular trend. Differences between the present study and other worldwide studies can be attributed to various genetic, racial, geographical, nutritional, and secular trend factors.

Adolescent↗
PubMed1994

[Endocrine diagnosis in puberty--pathophysiologic bases].

Puberty is characterized by activation of the maturing gonads and by the thus started increased secretion of sexual steroids. Consequences are the appearance of secondary signs of puberty sensu strictori, i. e. the development of breasts in girls, the increase of testicle volume in boys, often followed by growing pubic hair, axillary hair, menarche or laryngeal growth (puberty vocal change) respectively. The most important accompanying symptom is the spurt of growth starting around 12 to 18 months after the onset of the development of the secondary pubertal signs. From the time sequence of the development and the possible delays, valuable diagnostic hints can be gained, giving rise to a more precise analysis of the hormonal phenomena of adolescence. In cases of pubertas tarda a primary malfunction must be differentiated from secondary hypogonadotropic functional defect. The syndromes should be classified correctly according to their etiology. The most frequent diagnosis is that of a simply delayed puberty. Acne, hypertrichosis, hirsutism are concomitant phenomena of puberty development which can indicate a hormonal imbalance (differential diagnosis AGS, ovarian hyperandrogeny). The swelling of breasts in boys (gynecomastia) is a common transitory phenomenon in male adolescence (DD, tumor of the gonads or Klinefelter syndrome). Interesting considerations of differential diagnosis apply also to the assessment of the enlargement of the thyroid gland in puberty, which affects more often girls than boys.

Adolescent↗
PubMed1992

Aberrant puberty.

With the emergence of an understanding of the processes at the cellular level that control the differentiated functions of tissues and eventually manifest as the developmental sequence in the whole animal, it is possible to describe pubertal aberrations in terms that are at once grounded in fundamental concepts of cellular biology and of practical value to the clinician. Delayed puberty and its treatment represent a relatively straightforward problem, submitting to division into two groups of patients: those whose gonadotropin levels are elevated (hypergonadotropic hypogonadism), implying primary gonadal failure; and those whose gonadotropin levels are low (hypogonadotropic hypogonadism), implying either a failure of the central control axis or an adaptive response to a disruptive stress. Precocious puberty is also usefully separable into two distinct groups of patients: those in whom the normal central control axis is activated and those in whom it is not. The workup beyond the basic general stage and the approach to therapy are both determined by into the group into which the patient can be assigned. Advances in imaging technology have enhanced the diagnostic process, and chemical remodeling of the hypothalamic peptide, which mediates control of the pituitary's contribution, has revolutionized the treatment of CMPP, making it imperative that the clinician be able to distinguish those conditions that merit treatment from those that may be observed safely.

Adolescent↗
PubMed1992

The natural course of multiple endocrine neoplasia type IIb. A study of 18 cases.

BACKGROUND: Multiple endocrine neoplasia (MEN) type IIb is an autosomal dominantly inherited disorder associated with medullary thyroid cancer, pheochromocytoma, and a characteristic phenotype. The present study was performed to investigate the natural course of the syndrome and to describe its expression. METHODS: The medical records of 18 patients with MEN IIb, seven male and 11 female, were reviewed. RESULTS: The mean age at diagnosis of MEN IIb was 18 years (range, 8 to 41 years). All 18 patients had medullary thyroid cancer. In three patients, medullary thyroid cancer was diagnosed via screening. In two of these patients, the calcitonin value normalized after thyroidectomy. One patient died of metastases from medullary thyroid cancer at the age of 20 years (median duration of follow-up, 10 years). Eight of the 18 patients had pheochromocytomas. All of our patients had neuromas and bumpy lips, and all but one had a marfanoid habitus. A large proportion of the patients had intestinal abnormalities (75%), thickened corneal nerves (69%), skeletal abnormalities (87%), and delayed puberty (43%). CONCLUSIONS: The course of medullary thyroid cancer in MEN IIb is not always as aggressive as is generally thought. Periodic examination of relatives who are at risk may lead to early diagnosis and curative treatment. Intestinal abnormalities, skeletal abnormalities, and delayed puberty are commonly found in association with MEN IIb.

Adolescent↗
PubMedDate not supplied

Nephropathy in arteriohepatic dysplasia (Alagille's syndrome).

A 15-year-old girl evaluated for short stature and delayed puberty was found to have features of syndromatic arteriohepatic dysplasia or Alagille's syndrome. The characteristic triangular face, stubby nose, peripheral pulmonic stenosis, a history of prolonged neonatal jaundice and evidence of hepatic parenchymal disease were present as well as bilateral small kidneys and delayed puberty. The recognition of kidney involvement in this syndrome and the characterization of the frequency and nature of the renal disorder are of great prognostic significance and could influence the morbidity and mortality of these patients.

Abnormalities, Multiple↗
PubMed1993

Association between puberty and delayed phase preference.

Many teenagers go to bed and wake up significantly later than younger children, a developmental progression thought to reflect adolescent psychosocial processes. To determine whether biological processes may underlie a delay of phase preference in adolescents, 183 sixth-grade boys and 275 sixth-grade girls completed questionnaires for morningness/eveningness (M/E) and pubertal status. School environment and birth order were also evaluated. A significant relationship of pubertal status to M/E was found in girls, with a similar though nonsignificant trend in boys. No relationship between M/E and psychosocial factors was found. These data support involvement of a biological factor in the adolescent phase preference delay and indicate that our current understanding of adolescent sleep patterns may need revision.

Adolescent↗
PubMed2000

Growth hormone deficiency as the only identifiable cause for primary amenorrhea

Background: There is much evidence that growth hormone plays an important role in the development and function of the reproductive system of both males and females. Growth hormone exerts its effects on the ovarian follicular cycle directly or by local production of insulin-like growth factor 1 (IGF-1). It is known that growth hormone deficiency during childhood may delay pubertal development, but there is limited data about primary amenorrhea in GH-deficient girls with sufficient stimulated gonadotropin levels.Methods: Case series.Results: In the evaluation of primary amenorrhea and delayed puberty, 3 cases of adolescent females aged 17-19 years were identified as isolated GH-deficiency. Among the 3 patients, 2 had history of intracranial surgery due to hydrocephalus (shunt operation) or prolactin-secreting pituitary macro-adenoma (transphenoidal surgery, one year before). 17-year-old patient with shunted hydrocephalus and 19-year-old patient with primary amenorrhea showed short statue (< 5%) and delayed bone maturation. The patient undertaken transphenoidal surgery for prolactinoma showed normal height and bone maturation. There was no familial history of delayed puberty. On physical examination, 3 patients showed variable degree of breast development from Tanner stage II to IV without sex-steroid replacement. In sella MRI, small pituitary gland were identified in 2 patients with short statue and delayed bone maturation. All of the 3 patients underwent combined pituitary function test. After insulin-induced hypoglycemia, peak growth hormone levels of the 3 patients were 0.08, 1.4 and 1.4 ng/ml and were compatible with growth hormone deficiency. Peak LH after intravenous gonadrelin (FACTREL) were 19.0 to 56.1 mIU/ml and LH % responses were 217 to 1100% and were hence defined as not being gonadotropin deficiency. Other anterior pituitary functions were normal in all of the 3 patients.Conclusions: We found isolated growth hormone deficiency as the only identifiable cause for primary amenorrhea in three patients with sufficient gonadotropins secretion. These findings suggest a complementary role of GH to gonadotropins in the occurrence of menarche.

Journal Article↗
PubMed1999

Hypoleptinemia in female and male elite gymnasts.

BACKGROUND: Elite gymnasts favour low body fat mass as the current aesthetic ideal required for complex movements in this sports discipline. Pubertal development and growth are retarded in juvenile gymnasts. Leptin, the protein product of the ob-gene, is secreted by fat cells. Besides its role in regulation of body weight, leptin also stimulates the reproductive axis. We investigated various serum hormones including leptin, body composition and nutrition in cohorts of female and male elite gymnasts to elucidate if there is a relationship between leptin levels and delayed puberty in elite gymnasts. MATERIALS AND METHODS: Twenty-two female and 18 male elite gymnasts were enrolled in this study. Pubertal stage, various hormonal levels and body composition were determined and nutritional intake was assessed. Leptin was analysed using a specific RIA. RESULTS: Pubertal development and growth were delayed in the study group, especially in girls. The percentage of body fat was reduced as compared to a normal age-matched population: 14.4% versus 21.9% in girls and 10.4% versus 15.1% in boys. Serum leptin levels were decreased, especially in pubertal girls, and did not show the normal developmental pattern with a steady increase in girls and a peak in boys of pubertal stage 2. In all gymnasts leptin levels correlated with the amount of fat mass (r = 0.6, P = 0.005 in girls; r = 0.44, P = 0.038 in boys). When leptin levels were transformed into standard deviation scores (SDS) it became obvious that the gymnasts, especially pubertal females, had significantly lower values than normal controls of the same sex, pubertal stage and body mass index (BMI): leptin SDS (BMI) = -1.21 and -3.99 in prepubertal and pubertal girls, - 0.94 and -0.91 in prepubertal and pubertal boys, respectively. When leptin SDS were based on % body fat instead of BMI, mean values were still significantly decreased compared to normal controls: -1.05 in girls (P < 0.001) and -0.60 in boys (P = 0. 025). CONCLUSIONS: Adjustment of serum leptin levels in elite gymnasts for gender, pubertal stage and BMI or % body fat reveals inappropriately low values. The reason for this hypoleptinemia is most probably insufficient caloric intake. The data suggest that hypoleptinemia in turn causes delayed puberty and growth in this particular group of athletes.

Adipose Tissue↗
PubMed1999

Disorders of puberty.

Normal puberty begins between eight and 14 years of age in girls and between nine and 14 years of age in boys. Pubic hair distribution is used to stage puberty, along with breast size and contour in girls and testicular volume in boys. Some children experience constitutional sexual precocity, but precocity is likely to be pathologic if it occurs in very young children, if there is contrasexual development or if the sequence of normal pubertal milestones is disrupted. Delayed puberty may be constitutional, but pathologic causes should be considered. The etiology of a pubertal disorder can often be determined with the use of a focused medical history, a directed physical examination and appropriate diagnostic tests. Treatment for disorders of puberty is determined by the underlying cause.

Adolescent↗