SEARCH · Search PubMed

Results for “PUBERTY”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

SourceResearch connectionFrom yearThrough year

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 1,027 records · Page 57Linked to original sources

PubMedDate not supplied

Central precocious puberty in 48,XXYY Klinefelter syndrome variant.

We report the first case of central precocious puberty in a patient with 48,XXYY Klinefelter syndrome variant. We also report clinical characteristics, growth pattern, endocrine data and pathological testicular findings. The patient did not receive medical care for his precocious pubertal development, because of adequate height prognosis, and reached normal height for both his target height and Klinefelter patients. Since precocious puberty seems to occur in Klinefelter syndrome and its variants, we advise karyotype analysis in boys with mental retardation, gynecomastia, small testes and precocious onset of puberty.

Abnormalities, Multiple↗

PubMedDate not supplied

Precocious puberty in a patient with indicanuria.

A 7-5/12 year-old girl, who was followed-up after diagnosis of indicanuria, presented with symptoms of bilateral breast enlargement. Her breast development was at Tanner stage II. No pubic or axillary hair was observed. Pelvic ultrasonography revealed multiple follicles on both ovaries. Basic endocrinological evaluation and cranial magnetic resonance imaging (MRI) were normal. The diagnosis of precocious puberty was established with respect to the pubertal response to GnRH stimulation test. Although precocious puberty has been reported associated with some metabolic diseases, this is the first description in a patient with indicanuria. The question of whether precocious puberty in our patient with indicanuria is a coincidence or whether it is related to metabolic changes activating the hypothalamo-pituitary-gonadal axis remains open.

Amino Acid Metabolism, Inborn Errors↗

PubMed2000

Central precocious puberty: clinical and imaging aspects.

We review briefly the definition of central precocious puberty (CPP), and discuss early puberty and very early puberty. The association of hypothalamic hamartoma and empty sella with CPP is described. The contribution of new imaging techniques - CT, MRI and ultrasound in the differential diagnosis of CPP is discussed.

Brain Diseases↗

PubMed2000

Treatment of central precocious puberty: lessons from a 15 years prospective trial. German Decapeptyl Study Group.

There is still controversy about the auxological outcome of GnRH agonist treatment in patients with CPP and about the favorable age and auxological characteristics at start of treatment for achieving a normal final height (FH) or for preserving height potential. We analyzed the FH data of 52 young women from a prospective multicentric trial which was started in 1985. The aim of this analysis was to determine factors that may predict a favorable FH or a good height gain. Chronological age (CA) was 5.2 +/- 2.1 yr (+/- SD) at start of puberty, 6.2 +/- 2.0 yr at start of triptorelin depot treatment, 11.1 +/- 1.1 yr at end of treatment, and 16.7 +/- 2.6 yr at FH evaluation. After 4.8 +/- 2.2 yr (1.1-9.9 yr) of treatment duration, FH was 160.6 +/- 8.0 cm (vs 154.9 +/- 9.6 cm of initial height prediction [PAH], p<0.05). A FH within TH range or in excess of mean TH was achieved by 78% or 41% of patients. FH was above the 3rd percentile of the normal German population in 29% of patients (63% had an initial PAH < 156 cm). The group of patients with start of puberty at age < or = 6 yr (Group 1) showed a significantly higher height gain (FH - initial PAH) and lower height deficit compared to TH than older patients (Group 2). Furthermore, the percentage of patients from Group 1 reaching TH range or mean TH showed a significant increase with GnRH agonist treatment whereas this was not the case in Group 2. Stepwise regression analysis showed that height SDS at end of treatment, age at menarche, bone age (BA) at start of treatment, and BA advancement at end of treatment were determinants of FH (r2=0.923). Initial BA advancement and treatment duration were the factors that explained 68% of the variability of height gain. Although BA advancement at initiation of treatment was negatively associated with FH it was a positive predictor of height gain. In addition, height gain correlated significantly with CA and BA at start of treatment (r= -0.430, p=0.004 and r=0.359, p=0.018). Growth after interruption of treatment had no significant predictive effect on FH. It is concluded that a higher percentage of patients below 6 yr of age at start of puberty do profit from GnRH agonist treatment with respect to achieving a normal FH. BA, BA advancement, and height SDS at treatment start are important factors for determining outcome.

Aging↗

PubMed2001

New thoughts on female precocious puberty.

Much effort has been devoted in recent years to unravel the neuroendocrine mechanisms responsible for the initiation of mammalian puberty. The concept that has emerged is that puberty results from the unfolding of a centrally originated process involving the concerted influence of neuronal systems that utilize excitatory and inhibitory amino acids as transmitters and astroglial networks that produce growth factors able to affect LHRH secretion. We discuss the idea that an isolated alteration of each of these components may result in the precocious activation of pulsatile LHRH release, and thus lead to idiopathic sexual precocity. According to this notion, such a premature activation of LHRH neuronal function would be neither associated with structural damage of the neuroendocrine brain system, nor related to a generalized activation of the neuronal-glial mechanisms underlying the onset of puberty. On the contrary, localized activation of discrete cellular subsets functionally connected to LHRH neurons would suffice to promote an increase in LHRH release of sufficient magnitude and duration to initiate the pubertal process.

Child↗

PubMed2001

Role of inhibins in childhood and puberty.

Inhibins, produced mainly in the gonads, suppress FSH synthesis. The bioactive dimeric forms of inhibin (A and B) have been proposed as peripheral markers of Sertoli and granulosa cell function. The determination of serum dimeric inhibins from birth through adulthood reflects a distinct pattern of both inhibins in males and females. Concomitantly with the gonadotrophin surge, an important production of inhibin B is observed during the first months of life. In males, inhibin B levels are higher than in females and persist elevated up to childhood, whereas in females they decrease up to prepubertal levels by 6 months of age. In girls, high serum levels of inhibin A are observed during the first two months of life; thereafter, they are undetectable until puberty. An active secretion of inhibin B persists in both males and females in the period of maximal LHRH pulse generator restraint; however, the possible gonadotrophin dependence of this production remains controversial. At puberty, a progressive rise in serum inhibin B occurs concomitantly with the increased production of sex steroids in both males and females. A similar secretion pattern of inhibin A is observed in girls. This increment is mainly exerted by gonadotrophins and modulated by multiple paracrine/autocrine mechanisms within the ovary and the testis that regulate the dimerization of the inhibin subunits throughout pubertal maturation. The differences observed in males and females between circulating dimeric inhibins in relation to gonadotrophins and sex steroid concentrations from birth through puberty has opened a new perspective for research in human reproduction. These new markers may contribute to a better knowledge of the regulation of the hypothalamic-pituitary-gonadal axis function and the physiopathology of the mechanisms involved in sexual differentiation and/or fertility disorders.

Adolescent↗

PubMedDate not supplied

Central precocious puberty in a girl with triple X syndrome and neonatal diabetes mellitus associated with paternal isodisomy of chromosome 6.

We describe a girl with triple X syndrome and paternal isodisomy of chromosome 6 (UPD6), who developed neonatal diabetes mellitus (NDM) and precocious puberty. At birth she presented growth retardation and congenital anomalies (ventricular septal defect, macroglossia, umbilical hernia). Diabetes mellitus (DM) was diagnosed at 31 days of life and treated with insulin for 13 months. DM recurred at 4 years of age and since that time it required insulin, in spite of preserved beta-cell function. Tall stature was present from early childhood. At 7 years of age the girl presented central precocious puberty, height velocity further increased, but her near-final height was normal. This patient is unique in that precocious puberty has never been described in triple X females. Moreover it is a further example of paternal UPD6 causing NDM with a predisposition to type 2 DM in later life.

DNA↗

PubMed2004

Priming with testosterone enhances stimulated growth hormone secretion in boys with delayed puberty.

BACKGROUND AND OBJECTIVE: Tests for growth hormone (GH) deficiency are not always helpful in the differential diagnosis of physiological delay of growth and puberty and GH deficiency. PATIENTS AND METHODS: To enhance diagnostic specificity, we used a single dose testosterone priming before repeating the arginine stimulation test in 26 boys with short stature and only early signs of puberty who failed to show an adequate response of serum GH in the first test. RESULTS: 77% (20/26 patients) increased their serum GH peak to more than 10 ng/ml, whereas six patients were still below this concentration. CONCLUSION: We propose that testosterone priming is a useful tool to distinguish between physiological delay of growth and puberty and GH deficiency and should be included in the diagnostic procedure.

Adolescent↗

PubMed2004

Adopted girls with idiopathic central precocious puberty: observations about character.

The aim of this study was to investigate the psychological traits of adopted girls affected by idiopathic central precocious puberty (ICPP). Twelve adopted girls were evaluated before, during GnRH agonist (GnRHa) treatment, and after the treatment was stopped. Before, during and after treatment, adopted girls with ICPP showed a normal degree of anxiety, an adequate level of maturity, and a correct sexual identification. None of them had symbiotic character traits as previously reported in non-adopted girls with ICPP. The perception of their body was poor before treatment, but improved during GnRHa treatment and psychological support. The changes in the life of adopted girls are so numerous that precocious puberty may represent an additional risk for psychological discomfort. Therefore to treat precocious puberty it may not only be necessary to prevent premature changes in their body but also to give them enough time to face up to their emotional and psychological problems and to become competent adolescents.

Adolescent↗

PubMed2005

Gonadotropin releasing hormone analogue therapy in children with isolated growth hormone deficiency: final height benefit from postponing puberty?

Although growth hormone (GH) treatment has improved final height prognosis in children with GH deficiency (GHD), adult heights are still disappointing. Final height could be improved by increasing the duration of puberty and in this way increasing total pubertal height gain. Many studies have been published on the effect of gonadal suppression, mostly by gonadotropin releasing hormone (GnRH) analogues, on final height in children with GHD. Because of the different methodologies used in these studies, results are difficult to compare. Both positive and marginal effects on final height have been reported; however, patient numbers are limited. Children with GHD who start puberty at a relatively young age and who have a poor predicted adult height, can benefit from the addition of GnRH analogues. From previous studies, we might conclude that when there is a positive effect, height benefit is marginal. However, additional prospective, randomized controlled trials are needed to further elucidate whether delaying puberty is indicated in children with GHD to improve final height.

Adolescent↗

PubMed2005

Management of boys with short stature and delayed puberty.

OBJECTIVE: To review the management of boys with short stature and delayed puberty and the testosterone priming protocol. METHODS: In 148 boys aged > 14 years seen for height < -2 SDS and constitutional delayed puberty we evaluated growth hormone (GH) secretion and final height (80 boys). RESULTS: The GH peak was < 10 microg/l after arginine-insulin tests performed with testosterone heptylate priming in 8/32 (25%) and without in 62/153 (41%), including first and second evaluations. It was low in 7/11 boys given 2 x 100 mg testosterone (14.7 +/- 1.7 microg/l) and in 1/21 given 4 x 100 mg (21.3 +/- 2.0 microg/l, p = 0.04). It was low during sleep in 4/29 (14%) boys, all having basal plasma testosterone below 3.5 nmol/l. The basal insulin-like growth factor (IGF)-I concentration was below -2 SDS in 22% of the boys evaluated. Final height was -0.8 +/- 0.1 SDS. It was similar in those with low (n = 9) and normal (n = 71) GH peak, and in those treated (n = 22) or untreated (n = 58) with testosterone. It was over 1 SDS lower than the target height in 20% and than the predicted height at the initial evaluation in 14% of the boys. Pubertal growth was not correlated with the GH peak or plasma IGF-I. CONCLUSIONS: The GH peak during the sleep is more frequently normal than the peak after stimulation. The number of testosterone doses influences the quality of priming. The medical problems involved in treating boys with delayed puberty are excluding disease and deciding on testosterone treatment.

Adolescent↗

PubMed2005

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.

Brain Neoplasms↗

PubMed2005

Constitutional delayed puberty in males and hypogonadotropic hypogonadism: a reliable and cost-effective approach to differential diagnosis.

Forty-six male teenagers 13-19 years old with delayed puberty (DP) underwent gonadotropin releasing hormone (GnRH) and human chorionic gonadotropin (HCG) stimulation as part of their work-up. All were followed to age 18 and beyond. Thirty-seven had constitutional delayed puberty (CDP). Nine had hypogonadotropic hypogonadism (HH). At referral 34 youngsters with CDP were properly diagnosed when the lower limit for the luteinizing hormone (LH) response to GnRH (Factrel 0.1 mg i.v.) was set at 12 IU/l. Three boys with CDP failed to reach that level and were not assigned appropriately. All nine patients with HH had basal serum testosterone (T) < 50 ng/dl when first seen and LH responses to GnRH stimulation < 8.0 IU/l. In the late 1970s, five subjects with DP were given HCG 3,000 IU (two patients daily for 5 days; three on 3 alternate days). Serum T was measured before the first, and 48 hours after the last injection (day 7). With recognition of the long biological half-life of injected HCG and receptor downregulation by daily doses, the protocol was changed. In the early 1980s, the dose of HCG was randomized to either 500 IU or 1,000 IU given on 3 alternate days. T was measured before the first injection (basal), 48 hours later (day 3) and 48 hours after the third injection (day 7). At referral 35 patients with CDP, including one GnRH failure, met the criterion for a positive response to HCG stimulation based on their own reactions (T > 170 ng/dl on day 3; > 200 on day 7). Eleven patients with DP failed the test. Nine had HH and two had CDP. The nine patients with HH included the two given daily injections and the three given HCG 3,000 IU on 3 alternate days. Of the two with CDP, one, an obese boy with a normal GnRH test, only received 500 IU HCG (5.6 IU/kg), which may have been inadequate. The other failed both tests. Of the 35 responders, 17 (group 1) were given HCG 500 IU and 18 (group 2) were given 1,000 IU i.m. on 3 alternate days. Seven boys in group 1 and 12 in group 2 had serum T determined on day 3, and all 35 had T measured on day 7. There was no significant difference between the basal T levels in the two groups or in their responses to HCG, and the results were pooled. The combined data in the patients with CDP were then compared with those of the nine patients with HH, recognizing that the result on day 7 in the two patients given daily injections may reflect receptor down-regulation. Setting the limit for a normal T response at > 170 ng/dl 48 hours after a single injection of HCG and > 200 ng/dl after the third injection assigned all the patients with CDP appropriately. The data on the patients with HH are less complete, with only three T values 48 hours after the first injection However, even after three injections of HCG on alternate days, only one of nine patients with HH approached the lowest level achieved by the patients with CDP after a single injection. The data confirm that a basal serum T 50 ng/dl is evidence of the onset of puberty. In those with serum T < 50 ng/ dl our data suggest that a single injection of HCG 15 IU/kg, with serum T determined 48 hours later, is more discriminatory and offers the most reliable, easy to perform, least painful, and by far the most cost effective test to differentiate CDP from HH.

Adolescent↗

PubMed2002

Therapeutic potential of GnRH antagonists in the treatment of precocious puberty.

Pituitary-gonadal axis activation depends upon pulsatile hypothalamic gonadotropin-releasing hormone (GnRH) secretion. This phenomenon has led to clinical use of GnRH agonists in the treatment of central precocious puberty. GnRH analogues contain substitutions of the native decapeptide. Depending upon the substitutions, the analogues have GnRH agonistic or antagonistic properties. The pharmacokinetics of GnRH agonists, the established treatment of precocious puberty, includes an initial 'flare-up' of the pituitary-gonadal axis, followed by a reduced luteinising hormone secretion by desensitisation of pituitary GnRH receptors. Antagonistic GnRH analogues act by competitive binding to the pituitary GnRH receptors, thereby preventing the action of endogenous GnRH - theoretically offering a more direct and dose-dependent treatment alternative. The antagonist available today in Germany is a concomitant in assisted reproduction with only 1 - 3 days duration. However, long-acting depot preparations of other GnRH antagonists are in primate-testing phase. Our animal tests indicate strong potential for the development and testing of long-acting depot preparations of GnRH antagonists in treating precocious puberty.

Animals↗

PubMed2006

Treatment of central precocious puberty.

The problems of central precocious puberty (CPP) are serious enough to the patient to deserve treatment. There is a general consensus among paediatric endocrinologists that the treatment of true CPP (i.e., in children young enough to have a formal diagnosis) is indicated in many cases. In children with modestly early puberty who are not fulfilling the diagnostic criteria, this is not the case. The treatment of choice is a gonadotropin-releasing hormone (GnRH) analogue. Prolonged analogues are more effective than short-acting ones and, most importantly, independent of patient compliance. Data on agonists have accumulated over two decades and evidence of effects is rich in girls but sparse in boys. GnRH agonists are generally effective and safe drugs; the suppression of puberty is reversible and there is much information on GnRH agonists for the treatment of CPP showing very few adverse effects and the effects on final height are well documented in girls < 6 years of age. There is some (but not highly convincing) evidence for their effect on final height for those of 6 - 8 years of age and there is no evidence for an increase in final height after the age of 8 years in girls. If a decision to have treatment is taken, treatment should start immediately as a possible benefit is less probable if the start of treatment is delayed. When treatment should be stopped is a matter of controversy. Combination with growth hormone increases final height, but the clinical relevance can be discussed as well as the health economy aspects. The limits of indications are still to be defined.

Gonadotropin-Releasing Hormone↗

PubMed1984

Serum sex hormone-binding globulin during puberty in girls and in different types of adolescent menstrual cycles.

Serum sex hormone binding globulin (SHBG) concentrations were measured by an immunoradiometric assay, as part of a longitudinal study of puberty in girls, and were related to age, pubertal stage, age at menarche, weight, nature of the menstrual cycle and serum concentrations of sex steroids. A slow but very significant decrease was seen in SHBG from 77 nmol/l at 8-10 years of age to about 50 nmol/l after 15 years of age. Serum SHBG concentrations showed weak negative correlations with those of androstenedione and testosterone during puberty. The closest associations found between SHBG and the parameters measured were negative correlations with weight and body fat percentage in both pre-menarcheal and post-menarcheal girls, even after the effect of age was accounted for by calculating partial correlation coefficients. Girls who experienced early menarche (before 13.0 years) had lower SHBG but higher oestradiol serum concentrations at 10.0-15.9 years of age compared to girls with later menarche. In ovulatory menstrual cycles, a significant increase in SHBG was found from the early to the late part of the cycle, whereas no changes took place in anovulatory cycles. Serum concentrations of SHBG showed positive correlations with those of oestradiol and progesterone in specimens taken in the late part of the cycle. In view of the weak relationships between serum SHBG and sex steroid concentrations, and the strong relationships between SHBG, weight and body fat percentage, factors other than steroids have to be considered in the regulation of SHBG levels during puberty.

Adolescent↗

PubMed1985

Testolactone treatment of precocious puberty in McCune-Albright syndrome.

Current medical and surgical therapies of precocious puberty in McCune-Albright syndrome are often unsatisfactory. We used an aromatase inhibitor, testolactone, to treat precocious puberty in a girl with McCune-Albright syndrome. This child was unresponsive to 28 weeks of treatment with the long-acting agonist of LRH, D-trp6-pro9-NEt-LRH. During testolactone therapy, menses ceased, bone age advancement and height velocity diminished, and plasma oestradiol levels were suppressed. Serum gonadotrophin levels remained in the prepubertal range. Testolactone may be an effective therapy of precocious puberty in girls with McCune-Albright syndrome.

Androstenedione↗

PubMed1987

Sex-steroid-binding plasma protein (SBP), testosterone, oestradiol and dehydroepiandrosterone (DHEA) in prepuberty and puberty.

Measurement of sex-steroid-binding plasma protein in serum of healthy individuals in prepuberty and puberty (74 males and 94 females) was performed using a radioimmunoassay procedure. An age-related decrease of serum SBP was demonstrated during these ages in both sexes. In parallel studies, the serum level of testosterone, oestradiol and dehydroepiandrosterone was evaluated in subjects under 20 years of age. A rise of serum dehydroepiandrosterone levels in both sexes was observed to occur at approximately 8 years of age, being a little bit earlier than the ages for testosterone to rise in males and for oestradiol to rise in females, both of them being at about 10 years of age, respectively. When the testosterone/sex-steroid-binding plasma protein ratio was evaluated as an index of free testosterone concentration in serum, a sharp increase of the ratio was found to occur at 10 years of age and to continue during puberty in both sexes with more marked increment in males than in females. It was suggested that the decrease of SBP in puberty might be largely influenced by alteration in the hormonal balance of testosterone, oestradiol and dehydroepiandrosterone.

Adolescent↗