scholarly journals Update on the etiology, diagnosis and therapeutic management of sexual precocity

2008 ◽  
Vol 52 (1) ◽  
pp. 18-31 ◽  
Author(s):  
Vinicius Nahime Brito ◽  
Ana Claudia Latronico ◽  
Ivo J. P. Arnhold ◽  
Berenice Bilharinho Mendonça
Keyword(s):  
Precocious Puberty ◽  
Sex Steroids ◽  
Therapeutic Option ◽  
Receptor Gene ◽  
Pubertal Development ◽  
Protein A ◽  
Activating Mutations ◽  
Sexual Precocity ◽  
Gonadotropic Axis ◽  
Hormonal Evaluation

Precocious puberty is defined as the development of secondary sexual characteristics before the age of 8 years in girls and 9 years in boys. Gonadotropin-dependent precocious puberty (GDPP) results from the premature activation of the hypothalamic-pituitary-gonadal axis and mimics the physiological pubertal development, although at an inadequate chronological age. Hormonal evaluation, mainly through basal and GnRH-stimulated LH levels shows activation of the gonadotropic axis. Gonadotropin-independent precocious puberty (GIPP) is the result of the secretion of sex steroids, independently from the activation of the gonadotropic axis. Several genetic causes, including constitutive activating mutations in the human LH-receptor gene and activating mutations in the Gs protein a-subunit gene are described as the etiology of testotoxicosis and McCune-Albright syndrome, respectively. The differential diagnosis between GDPP and GIPP has direct implications on the therapeutic option. Long-acting gonadotropin-releasing hormone (GnRH) analogs are the treatment of choice in GDPP. The treatment monitoring is carried out by clinical examination, hormonal evaluation measurements and image studies. For treatment of GIPP, drugs that act by blocking the action of sex steroids on their specific receptors (cyproterone, tamoxifen) or through their synthesis (ketoconazole, medroxyprogesterone, aromatase inhibitors) are used. In addition, variants of the normal pubertal development include isolated forms of precocious thelarche, precocious pubarche and precocious menarche. Here, we provide an update on the etiology, diagnosis and management of sexual precocity.

scholarly journals A Limited Repertoire of Mutations of the Luteinizing Hormone (LH) Receptor Gene in Familial and Sporadic Patients with Male LH-Independent Precocious Puberty1

1999 ◽  
Vol 84 (3) ◽  
pp. 1136-1140
Author(s):  
H. Kremer ◽  
J. W. M. Martens ◽  
M. van Reen ◽  
M. Verhoef-Post ◽  
J. M. Wit ◽  
...  
Keyword(s):  
Mutation Analysis ◽  
Precocious Puberty ◽  
Receptor Gene ◽  
Receptor Protein ◽  
Negative Family History ◽  
Lh Receptor ◽  
Activating Mutations ◽  
Sensitive Tool ◽  
The U.S

Herein, we report mutation analysis of the LH receptor gene in 17 males with LH-independent precocious puberty, of which 8 were familial and 9 had a negative family history. A total of 7 different mutations (all previously reported) were detected in 12 patients. Among 10 European familial male-limited precocious puberty (FMPP) patients who had a LH receptor gene mutation, none had the Asp578Gly mutation, which is responsible for the vast majority of cases in the U.S. The restricted number of activating mutations of the LH receptor observed in this and other studies of FMPP strongly suggests that an activating phenotype is associated with very specific sites in the receptor protein. Clinical follow-up of the 5 patients who did not have LH receptor mutations shows that such cases most likely do not have true FMPP. LH receptor mutation analysis provides a sensitive tool for distinguishing true FMPP from other causes of early-onset LH-independent puberty in males.

scholarly journals Three-month sustained-release triptorelin (11.25 mg) in the treatment of central precocious puberty

2006 ◽  
Vol 154 (1) ◽  
pp. 119-124 ◽  
Author(s):  
Jean-Claude Carel ◽  
Joëlle Blumberg ◽  
Christine Seymour ◽  
Catherine Adamsbaum ◽  
Najiba Lahlou ◽  
...  
Keyword(s):  
Precocious Puberty ◽  
Sex Steroids ◽  
Pubertal Development ◽  
Central Precocious Puberty ◽  
Bone Age ◽  
Injection Pain ◽  
Open Label ◽  
Clinical Onset ◽  
Serum Lh ◽  
Fold Reduction

Objective: Depot GnRH agonists are commonly used in the treatment of central precocious puberty (CPP). The triptorelin 11.25 mg 3-month depot, currently used in adult indications, had not previously been evaluated in CPP. Design: This was a multicenter, open-label, 12 month trial conducted in 64 CPP children (54 girls and 10 boys), treated quarterly. Methods: Children with a clinical onset of pubertal development before the age of 8 years (girls) or 9 years (boys), pubertal response of LH to GnRH ≥7 IU/l, advanced bone age >1 year, enlarged uterus (≥36 mm) and testosterone level ≥0.5 ng/ml (boys), were included. Suppression of gonadotropic activation, as determined from serum LH, FSH, estradiol or testosterone, and pubertal signs were assessed at Months 3, 6 and 12. Results: GnRH-stimulated peak LH ≤3 IU/l, the main efficacy criterion, was met in 53 out of 62 (85%), 60 out of 62 (97%) and 56 out of 59 (95%) of the children at Months 3, 6 and 12 respectively. Serum FSH and sex steroids were also significantly reduced, while pubertal development regressed in most patients. Mean residual triptorelin levels were stable from Month 3 through to Month 12. The triptorelin 3-month depot was well tolerated. Severe injection pain was experienced in only one instance. Five girls experienced mild-to-moderate or severe (one girl) withdrawal bleeding. Conclusions: The triptorelin 3-month depot efficiently suppresses the pituitary–gonadal axis and pubertal development in children with CPP. This formulation allows a 3-fold reduction, over the once-a-month depot, in the number of i.m. injections required each year.

scholarly journals Juvenile granulosa cell tumor

2016 ◽  
Author(s):  
Geetanjali Tuteja ◽  
S. Unmesh ◽  
S. Shree ◽  
S. Rudra ◽  
Keyword(s):  
Granulosa Cell ◽  
Precocious Puberty ◽  
Early Stage ◽  
Pubertal Development ◽  
Granulosa Cell Tumor ◽  
Cell Tumor ◽  
Cell Tumour ◽  
Tumour Resection ◽  
Granulosa Cell Tumour ◽  
Juvenile Granulosa Cell Tumor

The differential diagnosis for precocious puberty in a young female includes peripheral causes. This case report documents a rare cause of isosexual precocious puberty, a juvenile granulosa cell tumour of the ovary–and a brief literature review. A one year-old baby girl presented with mass abdomen, vaginal discharge and rapid onset of pubertal development. She underwent an exploratory laparotomy for tumour resection. Pathology reported a juvenile granulosa cell tumour of the ovary. Early stage granulosa cell tumor surgically treated has good prognosis. Adjuvant chemotherapy is not indicated in this setting.

MiR-664-2 impacts pubertal development in a precocious-puberty rat model through targeting the NMDA receptor-1†

2019 ◽  
Vol 100 (6) ◽  
pp. 1536-1548 ◽  
Author(s):  
Minda Ju ◽  
Liu Yang ◽  
Jing Zhu ◽  
Zhejun Chen ◽  
Mizhen Zhang ◽  
...  
Keyword(s):  
Precocious Puberty ◽  
Bioinformatics Analysis ◽  
Pubertal Development ◽  
Follicle Stimulating Hormone ◽  
Gonadotropin Releasing Hormone ◽  
Untranslated Regions ◽  
Vaginal Opening ◽  
Protein Levels ◽  
Pulsatile Gnrh ◽  
Puberty Onset

Abstract Precocious puberty (PP) commonly results from premature activation of the hypothalamic–pituitary–gonadal axis (HPGA). Gonadotropin-releasing hormone (GnRH) is the initial trigger for HPGA activation and plays an important role in puberty onset. N-methyl-D-aspartate (NMDA) can promote pulsatile GnRH secretion and accelerates puberty onset. However, the mechanism of N-methyl-D-aspartate receptors (NMDARs) in PP pathogenesis remains obscure. We found that serum GnRH, luteinizing hormone (LH), follicle-stimulating hormone (FSH), estrogen (E2) levels, hypothalamic NMDAR1, and GnRH mRNA expression peaked at the vaginal opening (VO) day. Next, the hypothalamic NMDAR1 mRNA and protein levels in rats treated with danazol, a chemical commonly effecting on the reproductive system, were significantly increased at the VO day (postnatal day 24) compared to controls, accompanied by enhanced serum GnRH, LH, FSH, and E2 levels. Further, microRNA-664-2 (miR-664-2) was selected after bioinformatics analysis and approved in primary hypothalamic neurons, which binds to the 3′-untranslated regions of NMDAR1. Consistently, the miR-664-2 expression in hypothalamus of the Danazol group was decreased compared to Vehicle. Our results suggested that attenuated miR-664-2 might participate in PP pathogenesis through enhancing the NMDAR1 signaling.

scholarly journals Unusual Presentation of Precocious Puberty Associated with Primary Hypothyroidism in a 8.5 Years Old Girl

2018 ◽  
Vol 6 (2) ◽  
pp. 102-105
Author(s):  
Shamsun Naher Rikta ◽  
Fatema Ashraf ◽  
Samira Areen
Keyword(s):  
Precocious Puberty ◽  
Rare Complication ◽  
Pubertal Development ◽  
Thyroid Function Test ◽  
Ovarian Tumour ◽  
Primary Hypothyroidism ◽  
Monthly Interval ◽  
Close Monitoring ◽  
College Hospital ◽  
Ovarian Volume

Precocious puberty is one of the gynaecological problems in childhood. Children with hypothyroidism generally have delayed pubertal development. But primary hypothyroidism is one of the rare causes of precocious puberty especially in long standing untreated patients. Bilateral ovarian enlargement due to multiple cystic ovaries is a rare complication of primary hypothyroidism. For ovarian enlargement no treatment is required at all. This is a case report of an 8.5 years old girl diagnosed as a case of precocious puberty with benign ovarian tumour at a peripheral hospital and was being prepared to undergo laparotomy. When patient was admitted at Shaheed Suhrawardy Medical College Hospital (ShSMCH), Dhaka, Bangladesh, after thorough investigations primary hypothyroidism was diagnosed. She was given thyroxin replacement and close monitoring was done by doing USG of uterus and adnexae, thyroid function test at monthly interval. USG after 2 months revealed significant reduction of ovarian volume and at 6 month ovarian volume reduced to normal. It was also noticed that the rate of reduction of ovarian volume corresponds to the rate of reduction of serum TSH level measured serially at every 2 months interval.Delta Med Col J. Jan 2018 6(2): 102-105

scholarly journals Gonadotropin-Releasing Hormone Receptor Gene Expression During Pubertal Development of Female Rats1

2004 ◽  
Vol 70 (2) ◽  
pp. 348-355 ◽  
Author(s):  
Helena Zapatero-Caballero ◽  
Franco Sanchez-Franco ◽  
Carolina Fernandez-Mendez ◽  
Miriam García-San Frutos ◽  
Luisa M. Botella-Cubells ◽  
...  
Keyword(s):  
Gene Expression ◽  
Hormone Receptor ◽  
Receptor Gene ◽  
Pubertal Development ◽  
Gonadotropin Releasing Hormone ◽  
Releasing Hormone ◽  
Gonadotropin Releasing Hormone Receptor ◽  
Receptor Gene Expression ◽  
Hormone Receptor Gene
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