High prevalence of abnormal adrenal response in girls with central precocious puberty at early pubertal stages

1995 ◽  
Vol 133 (4) ◽  
pp. 407-411 ◽  
Author(s):  
Liora Lazar ◽  
Rivka Kauli ◽  
Celia Bruchis ◽  
Jardena Nordenberg ◽  
Avinoam Galatzer ◽  
...  
Keyword(s):  
Precocious Puberty ◽  
Dehydrogenase Deficiency ◽  
Central Precocious Puberty ◽  
Hydroxysteroid Dehydrogenase ◽  
Gnrh Analog ◽  
Hpg Axis ◽  
Early Stages ◽  
High Prevalence ◽  
Pubertal Stages ◽  
Adrenal Response

Lazar L, Kauli R, Bruchis C, Nordenberg J. Galatzer A, Pertzelan A. High prevalence of abnormal adrenal response in girls with central precocious puberty at early pubertal stages. Eur J Endocrinol 1995;133:407–11. ISSN 0804–4643 Abnormal adrenal response is often observed in girls with precocious adrenarche (1). We studied the adrenal response in 112 girls with idiopathic true central precocious puberty (CPP) at early stages of puberty compared to that in 21 girls with normal puberty (controls). The aims of this study were to determine the prevalence of abnormal adrenal response at early stages of puberty, the possible correlation of abnormal adrenal response with pubertal signs at onset of puberty and with plasma androgen levels, and a possible association with the activity of the hypothalamic-pituitary-gonadal (HPG) axis. All participants underwent a combined iv adrenocorticotropic hormone (ACTH)gonadrotropin-releasing hormone (GnRH) test at Tanner stage 2-3: 62 of the CPP girls before and 50 during treatment with GnRH analog. The stimulated levels of 17-hydroxypregnenolone (17OHPreg) and the stimulated 17OHPreg/17-hydroxyprogesterone ratio were analyzed and compared to previously reported norms. The result revealed three patterns of adrenal response: normal (17OHPreg ⩽24 nmol/l and 17OHPreg/17OHP ratio ⩽7) in 50/112 (44.6%) CPP patients and 17/21 (80.9%) controls; exaggerated (17OHPreg> 24 nmol/l, 17OHPreg/17OHP ratio ⩽ 7) in 50/112 (44.6%) CPP patients and 3/21 (14.3%) controls; and non-classical 3β-hydroxysteroid dehydrogenase deficiency (17OHPreg> 24 nmol/l and 17OHPreg/17OHP ratio > 7) in 12/112 (10.8%) CPP patients and 1/21 (4.8%) controls. The clinical features at onset of puberty were comparable in all girls with the CPP in spite of the different adrenal response patterns. The levels of androstenedione and testosterone were within normal range in all cases. Dehydroepiandrosterone sulfate was significantly elevated only in 3β-hydroxysteroid dehydrogenase deficiency, 4.8 ± 2.7 (p < 0.03) as expected. The adrenal response was not affected by GnRH analog therapy. In conclusion, an abnormal adrenal response was found in 55.4% of girls with CPP in early puberty. This response did not cause clinical signs and laboratory findings of a hyperandrogenic state at early stages of puberty and was not affected by the activity of the HPG axis. Its role in evoking precocious puberty remains to be explored further. A Pertzelan, Institute of Adolescent Endocrinology, Children's Medical Center of Israel, Beilinson Medical Campus, Petah Tiqva 49202, Israel

High prevalence of syndromic disorders in patients with non-isolated central precocious puberty

2019 ◽  
Author(s):  
Wannes S ◽  
Elmaleh-Berges M ◽  
Simon D ◽  
Zenaty D ◽  
Martinerie L ◽  
...  
Keyword(s):  
Precocious Puberty ◽  
Central Precocious Puberty ◽  
High Prevalence

The utility of serum inhibin B, anti-Müllerian hormone and insulin growth factor-1 in predicting a positive response to GnRH analogs for diagnosing central precocious puberty in girls

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Liu Ziqin ◽  
Song Qinwei ◽  
Chen Xiaobo ◽  
Li Xiaohui
Keyword(s):  
Growth Factor ◽  
Precocious Puberty ◽  
Operating Characteristic ◽  
Positive Response ◽  
Roc Curves ◽  
Central Precocious Puberty ◽  
Inhibin B ◽  
Gnrh Analog ◽  
Insulin Growth Factor ◽  
Chinese Girls

Abstract Objectives The use of inhibin B (INHB), anti-Müllerian hormone (AMH) and insulin-like growth factor-1 (IGF-1) in differentiating central precocious puberty (CPP) from non-CPP was evaluated. Methods In total, 115 Chinese girls were recruited (CPP: 44, non-CPP: 71). The diagnostic performance of INHB, AMH and IGF-1 in differentiating CPP from non-CPP was analyzed using receiver operating characteristic (ROC) curves. Results INHB levels were higher in the CPP group than in the non-CPP group (55.56 ± 22.42 vs. 32.97 ± 15.59 pg/mL; p<0.001). AMH levels were similar in the CPP and non-CPP groups (6.63 ± 3.74 vs. 5.70 ± 3.15 pg/mL; p=0.158), and IGF-1 levels were much higher in the CPP group than in the non-CPP group (290.75 ± 79.78 vs. 200.10 ± 54.01 pg/mL; p<0.001). The area under the ROC curve (AUC) was greatest for INHB (0.819, standard error (SE) 0.041), followed by IGF-1 (0.809, SE 0.047) and AMH (0.567, SE 0.057). Among the ROC curves including combinations of these parameters, the AUC for INHB + IGF-1 was 0.849 and that for INHB + AMH was 0.768. Conclusions Serum INHB and IGF-1 measurements could predict positive responses to gonadotropin-releasing hormone (GnRH) analog stimulation in girls with precocious puberty.

scholarly journals Hypothalamic hamartoma presenting as central precocious puberty: a rare case report

2020 ◽  
Vol 7 (7) ◽  
pp. 1634
Author(s):  
Priyanka Sharma ◽  
Nishant Acharya ◽  
Trilok C. Guleria
Keyword(s):  
Case Report ◽  
Precocious Puberty ◽  
Rare Case ◽  
Treatment Options ◽  
Central Precocious Puberty ◽  
Breast Size ◽  
Hypothalamic Hamartoma ◽  
Gnrh Analog ◽  
Gnrh Analogs ◽  
Rare Case Report

Precocious puberty is defined as children attaining puberty more than 2.5 to 3 standard deviations (SD) earlier than the median age, or before the age of eight years in girls and nine years in boys. Hypothalamic hamartoma (HH) are rare, non progressive tumor like malformation. Precocious puberty due to HH occurs particularly at early ages, even 2 or 3 years. Treatment options for isolated CPP due to HH include GnRH analogs agonists continuously stimulates pituitary gonadotrophs, which further help in decreasing and desensitizing the release of LH, and to a lesser extent, FSH till the time puberty naturally set in. We present a case of precocious puberty due to hypothalamic hamartoma in 3 years old girl. Treated with GnRH analog lupirode and responded well to treatment with cessation of menstruation and reduction in breast size.

scholarly journals The Influence of GnRH Analog Therapy on Growth in Central Precocious Puberty

2016 ◽  
Vol 25 (1) ◽  
pp. 27-32 ◽  
Author(s):  
Ewa Głąb ◽  
Beata Wikiera ◽  
Jolanta Bieniasz ◽  
Ewa Barg
Keyword(s):  
Precocious Puberty ◽  
Central Precocious Puberty ◽  
Gnrh Analog

Early polycystic ovary-like syndrome in girls with central precocious puberty and exaggerated adrenal response

1995 ◽  
Vol 133 (4) ◽  
pp. 403-406 ◽  
Author(s):  
Liora Lazar ◽  
Rivka Kauli ◽  
Celia Bruchis ◽  
Jardena Nordenberg ◽  
Avinoam Galatzer ◽  
...  
Keyword(s):  
Precocious Puberty ◽  
Polycystic Ovary ◽  
Medical Center ◽  
Hormone Secretion ◽  
Clinical Signs ◽  
Central Precocious Puberty ◽  
Early Puberty ◽  
History Of ◽  
Adrenal Response ◽  
Pco Syndrome

Lazar L, Kauli R, Bruchis C, Nordenberg J, Galatzer A, Pertzelan A. Early polycystic ovary-like syndrome in girls with central precocious puberty and exaggerated adrenal response. Eur J Endocrinol 1995;133:403–6. ISSN 0804–4643 Exaggerated adrenal response (ExAR), i.e. hypersecretion of both 17-hydroxypregnenolone (170HPreg) and 17-hydroxyprogesterone(17OHP) in response to adrenocorticotropic hormone (ACTH) stimulation, is frequently found in women with polycystic ovary (PCO) syndrome who had precocious adrenarche. In an earlier study we found an abnormal adrenal response in girls with idiopathic true central precocious puberty (CPP) at early stages of puberty. On follow-up it was noted that a significant number of girls with CPP develop PCO-like syndrome at a relatively young age. The aim of the present study was to determine if there is an association between ExAR and early PCO in girls with a history of CPP. Included were 49 girls with a history of CPP, 34 of whom were treated with gonadotropin-releasing hormone (GnRH) analog. All 49 were evaluated at full maturity, at ages 12.5–14 years, 0.5–4 years after menarche or resumption of menses. Of the 49 girls, 20 had at least 3/4 clinical signs of PCO (irregular menses, hirsutism, acne and obesity) and were defined as PCOlike+, whereas 29 did not fulfil the criteria and were considered PCO-like -. Girls with a definite enzyme deficiency were excluded from the study. All participants underwent a combined iv ACTHGnRH test at early follicular phase. The PCO-like + girls all revealed ExAR, i.e. an elevated stimulated 17OHPreg of 63.4 ± 9.6 nmol/l (normal 28.6 ± 9.2 nmol/l) and a normal stimulated 17OHPreg/ 17OHP ratio of 7.1 ± 1.8 (normal 6.2 ± 2.7), whereas all the PCO-like – had a normal adrenal response (30.0 ±8.7 and 5.3 ± 2.0 nmol/l, respectively). Compared to the PCO-like – girls, those with PCO-like± had significantly higher levels of testosterone (1.8 ± 0.7 vs 1.0 ± 0.5 nmol/l; p < 0.001), androstenedione (6.6 ±3.2 vs 4.7 ± 1.8 nmol/l; p < 0.02) and dehydroepiandrosterone sulfate (7.8 ± 4.7 vs 4.2 ± 2.5 μmol/l; p < 0.004), and a trend toward inappropriate luteinizing hormone secretion. The prevalence of ExAR (40.8%) in the mature CPP girls (confined to only PCO-like ±) was similar to that previously found by us in another group of girls with CPP at early puberty (44.6%). In conclusion, our findings indicate that the pattern of adrenal response remains unchanged from early puberty to adulthood and is probably inherent. As only the girls with CPP who developed early PCO syndrome showed ExAR, it is suggested that ExAR in early puberty may serve as a predictive marker for the eventual development of PCO. A Pertzelan, Institute of Pediatric and Adolescent Endocrinology, Children's Medical Center of Israel, Beilinson Medical Campus, Kaplan Street, Petah Tiqva 49202, Israel

scholarly journals CENTRAL PRECOCIOUS PUBERTY IN TWO BOYS WITH PRADER-WILLI SYNDROME ON GROWTH HORMONE TREATMENT

2019 ◽  
Vol 5 (6) ◽  
pp. e352-e356 ◽  
Author(s):  
Elena Monai ◽  
Anders Johansen ◽  
Erik Clasen-Linde ◽  
Ewa Rajpert-De Meyts ◽  
Niels Erik Skakkebæk ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Precocious Puberty ◽  
Growth Hormone Treatment ◽  
Pubertal Timing ◽  
Pubertal Development ◽  
Central Precocious Puberty ◽  
Hormone Treatment ◽  
Prader Willi Syndrome ◽  
Gnrh Analog ◽  
Premature Adrenarche

Objective: Prader-Willi syndrome (PWS) is a rare genetic neuroendocrine disorder characterized by hypotonia, obesity, short stature, and mental retardation. Incomplete or delayed pubertal development as well as premature adrenarche are usually found in PWS, whereas central precocious puberty is rarely seen. Methods: This study reports the clinical, biochemical, and histologic findings in 2 boys with PWS who developed central precocious puberty. Results: Both boys were started on growth hormone therapy during the first years of life according to the PWS indication. They had both bilateral cryptorchidism at birth and had orchidopexy in early childhood. Retrospective histologic analysis of testicular biopsies demonstrated largely normal tissue architecture and germ cell maturation, but severely decreased number of prespermatogonia in one of the patients. Both boys had premature adrenarche around the age of 6. Precocious puberty was diagnosed in both boys with enlargement of testicular volume (>3 mL), signs of virilization and a pubertal response to a gonadotropin-releasing hormone (GnRH) test and they were both treated with GnRH analog. Conclusion: The cases described here displayed typical characteristics for PWS, a considerable heterogeneity of the hypothalamic-pituitary function, as well as testicular histology. Central precocious puberty is extremely rare in PWS boys, but growth hormone treatment may play a role in the pubertal timing.

scholarly journals Circulating ghrelin levels in girls with central precocious puberty are reduced during treatment with LHRH analog

2007 ◽  
Vol 156 (1) ◽  
pp. 99-103 ◽  
Author(s):  
Claudio Maffeis ◽  
Roberto Franceschi ◽  
Paolo Moghetti ◽  
Marta Camilot ◽  
Silvana Lauriola ◽  
...  
Keyword(s):  
Precocious Puberty ◽  
Pubertal Development ◽  
Central Precocious Puberty ◽  
Bone Age ◽  
Serum Levels ◽  
Gnrh Analog ◽  
Therapy Discontinuation ◽  
17Β Estradiol ◽  
Therapy Withdrawal ◽  
Ghrelin Secretion

Decreased levels of ghrelin have been measured in growing children during puberty. No data are available for girls with central precocious puberty (CPP). Aims: To explore ghrelin changes before, during, and after GnRH analog treatment in girls with CPP. Subjects and methods: A sample of 20 Caucasian girls (8.08 ± 0.65 years of age) with CPP was recruited. Height and weight, bone age, LH, FSH, 17β estradiol (E2), and ghrelin were measured before starting treatment with GnRH analog, 18 months after therapy began and again 6 months after therapy discontinuation. Results: LH and E2 serum levels decreased significantly during treatment (2.45 ± 2.03 vs 0.67 ± 0.49 UI/l, P < 0.01 and 28.17 ± 9.7 vs 15 pmol/l, P < 0.01 respectively), returning to baseline levels after the discontinuation of therapy (4.75 ± 1.66 UI/l and 29.23 ± 6.99 pmol/l respectively). LH peaked following LHRH stimulation significantly (P < 0.01) decreased during treatment (24.45 ± 14.17 vs 1.3 ± 0.18 UI/l) and then increased after therapy discontinuation (12.58 ± 6.09, P < 0.01). Ghrelin decreased significantly (P < 0.05) during treatment (1849 ± 322 vs 1207 ± 637 pg/ml), and increased, though not significantly (P = 0.09) after therapy withdrawal (1567 ± 629 pg/ml). Conclusions: Contrary to what is expected in physiologic puberty, where ghrelin is progressively reduced, the prepubertal hormone milieau induced by GnRHa treatment in patients suffering from central precocious puberty (CPP) did not promote an increase in ghrelin circulating levels. Therefore, in CPP, ghrelin secretion seems to be independent from pubertal development per se. Concomitant estrogen suppression during treatment may play a potential role in the regulation of ghrelin secretion in these girls.

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