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Results for “Puberty, Precocious”

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PubMed2003

Lack of efficacy of fadrozole in treating precocious puberty in girls with the McCune-Albright syndrome.

We administered the aromatase inhibitor fadrozole to 16 girls with gonadotropin-independent precocious puberty due to the McCune-Albright syndrome. The girls' ages ranged from 3.2-9.7 yr, and their bone ages ranged from 5.75-14.25 yr. After baseline evaluations, fadrozole was started at a dose of 240 microg/kg.d (equivalent to the dose recommended for therapy of estrogen-dependent breast cancer) for 12-21 months and increased to 480 microg/kg.d for an additional 12 months in 10 girls. During treatment, seven girls had evidence of central precocious puberty; hence, the GnRH agonist deslorelin (4 microg/kg.d sc) was added to their regimen. One girl was on a long-acting GnRH agonist from the start of treatment. Patients were evaluated at 2-6-month intervals throughout treatment. After the first 6-12 months of treatment, fadrozole showed some benefits in 10 girls, including decrease in frequency of menses and/or rates of linear growth and bone maturation; however, fadrozole had no significant benefit in the group as a whole. The seven girls with evidence of central precocious puberty had no slowing in the progression of their puberty during the combined fadrozole and GnRH analog treatment. Adverse effects of fadrozole included inhibition of cortisol and aldosterone biosynthesis at the dose of 480 microg/kg.d, without clinical evidence of adrenal insufficiency. In addition, three patients complained of nonspecific abdominal pain during fadrozole treatment. In one patient, this resolved with a reduction in dose from 480 to 240 microg/kg.d; in two patients, it resolved spontaneously. One girl had muscle weakness and constipation on the 480 microg/kg.d. This resolved after discontinuation of the drug. We conclude that fadrozole is not sufficiently potent to block estrogen synthesis in most girls with gonadotropin-independent precocious puberty due to the McCune-Albright syndrome and may impair the adrenocortical stress response.

Age Determination by Skeleton↗

PubMed1977

The LH and FSH responses to LH-releasing hormone (LH-RH) in girls with true precocious puberty treated with cyproterone acetate.

Ten girls with precocious puberty ranging in age from 7 to 10 7/12 years who were treated with oral cyproterone acetate on a long term basis, were subjected to LH-RH tests, prior to and 3 to 16 months after the institution of therapy. Cyproterone acetate was given in doses from 60 to 153 mg/m2, which proved to be clinically effective, as evidenced by the slowing down of sexual maturation. The basal levels of LH were found to be unaffected by therapy and corresponded to the pubertal stages of the individual girls. The peak increment of LH after LH-RH stimulation was markedly suppressed by the therapy. FSH secretion and its responsiveness to LH-RH was not affected by cyprotereone acetate. The basal levels of FSH were higher during therapy than before, but the peak FSH increment remained the same. An escape phenomenon in the LH peak response was evident in 2 patients upon retesting after prolonged therapy. It is possible that the antigonadotrophic action of cyproterone acetate is due to its progestational nature.

Child↗

PubMed1987

Intracranial embryonal carcinoma and precocious puberty.

A boy aged 7.5 years presented with precocious onset of secondary sexual characteristics and symptoms and signs of neurological dysfunction. A CT brain scan demonstrated a suprasellar mass lesion. The elevated beta-hCG and alpha-fetoprotein concentrations in cerebrospinal fluid and in serum were consistent with a diagnosis of intracranial embryonal carcinoma, although histological diagnosis was not available. The tumour markers beta-hCG and alpha-FP should be looked for in all children with precocious puberty presenting with midline brain tumours.

Brain↗

PubMed1997

Case of the month. July 1996--precocious puberty.

A 4 year old girl presented with precocious puberty. She had pubic hair, breast buds and showed a height and weight well above the 95th percentile. An MRI scan of the head revealed a discrete mass in the tuber cinereum which was surgically removed. Microscopic examination showed a hypothalamic hamartoma. The clinical, radiologic and microscopic findings in this case are "classic" for hypothalamic hamartoma Other clinical presentations are discussed. The patient's symptoms have regressed following surgery and there has been no regrowth in six months.

Child, Preschool↗

PubMed1978

Epileptic laughter with precocious puberty.

A child with associated epileptic laughter and precocious puberty is reported. The significance of epileptic laughter as a possible marker of hypothalamic disease is noted.

Brain Diseases↗

PubMed1984

Production of human chorionic gonadotrophin by a hepatoblastoma resulting in precocious puberty.

A two year old boy presented with precocious puberty associated with hepatoblastoma. Serum concentrations of beta human chorionic gonadotrophin and alpha-fetoprotein were raised. An aggressive chemotherapeutic regimen resulted in useful palliation and interesting changes in the beta human chorionic gonadotrophin and alpha-fetoprotein concentrations. In contrast to a previous report, the ultrastructure of the tumour showed frequent Golgi apparatus but no other electron dense membrane bound vesicles.

Carcinoma, Hepatocellular↗

PubMed2002

Sertoli-Leydig cell tumor of the ovary, a rare cause of precocious puberty in a 12-month-old infant.

We report a 12-month-old infant who presented with a 4-month history of isosexual precocious puberty secondary to an estrogenizing Sertoli-Leydig cell tumor of the ovary. Total serum immunoreactive inhibin and subunits A and B were markedly elevated before surgical resection and subsequently decreased 7 wk later into the normal prepubertal range. Twenty weeks following surgical removal, the patient presented again with central precocious puberty; inhibin B levels were raised on this occasion, a luteinizing releasing hormone stimulation test confirmed central precocious puberty. This is the youngest reported occurrence of this rare sex cord stromal neoplasm. The prognosis of this extremely rare tumor presenting at this early juvenile stage is uncertain. This report illustrates the usefulness of serum inhibin as a tumor marker during therapeutic suppression with leuprorelin acetate for central precocious puberty. Analysis of genomic and tumor DNA revealed a normal nucleotide sequence for the LH receptor and the Galpha(s) gene. To understand the molecular pathogenesis of this tumor we analyzed mRNA levels for the inhibin A and B subunits, FSH receptor, LH receptor aromatase, steroidogenic factor-1 and the ER beta genes. Molecular characterization reveals the presence of genes specific for granulosa and Leydig cells; the relative expression of these genes, in addition to its histologic characteristics, suggests that this tumor may result from a dysdifferentiation of a primordial follicle.

Androstenedione↗

PubMed2006

[Familial precocious puberty -- a variant of norm or pathology?].

Since 1937, when familial precocious puberty (FPP) was described for the first time, only few reports on FPP have been published. The majority of them is concerned with the most investigated form of FPP, occurring only in male --testotoxicosis (male-limited precocious puberty -- MLPP). Recently another form of FPP -- familial hyperestrogenism (aromatase excess syndrome -- AES) has been described. The authors aimed to review the literature data concerning different forms of FPP emphasizing the diagnostic criteria, etiology, mode of inheritance and treatment.

Adolescent↗

PubMed1979

Fibrous dysplasia and precocious puberty (McCune-Albright syndrome).

A patient with fibrous dysplasia and precocious puberty, with pneumogynographic demonstration of an enlarged uterus and ovaries at age 16 months has been followed up to age 14 years. The pneumogynographic findings and the chemistries implicate central nervous system stimulation as the cause of the precocious puberty.

Air↗

PubMed1977

[A case of HCG-producing ectopic pinealoma in a girl with precocious puberty (author's transl)].

HCG-producing ectopic pinealoma of two cell pattern type was demonstrated in a 5-year old girl who presented precocious puberty and bilateral choked discs. The tumor was localized at the anterior third ventricle and suprasellar region. Endocrinological findings are as following: Plasma basal LH was markedly elevated to 306 mIU/ml and HCG was elevated to 1,192 ng/ml. Provocative test of hypophyseal function revealed low response. Plasma estrogen was not detectable. HCG content of resected tumor tissue was elevated to 400 ng/mg. FSH, however, was not detectable. Histological findings of this tumor showed atypical teratoma, so-called two cell pattern pinealoma. Electron microscopic findings revealed two types of cells, dark and clear cells. Many secreting granules were found in the dark cells. In this case, HCG in plasma, CSF and tumor tissue was remarkably elevated. In addition, plasma FSH was also elevated to 8.9 mIU/ml. Precocious puberty associated with tumor in the pineal-suprasellar region has been seen only in boys. There has been no case report of precocious puberty in girls. This case is the first female case is which HCG-producing ectopic penealoma is caused in precocious puberty.

Brain Neoplasms↗

PubMed1989

Luteinizing-hormone-mediated precocious puberty induced in female rats by a prepuberal pituitary graft.

The present study analyzes the mechanism of precocious puberty induced in female rats after a 'young' pituitary graft (obtained from 21-day-old animals). For this purpose, the following experiments have been performed: (1) female rats were grafted or sham-operated on day 21 with a littermate's pituitary the follicle-stimulating hormone (FSH), luteinizing hormone (LH) and estradiol plasma levels as well as the ovarian, uterine and adrenal weights were determined at different times after the graft; (2) female rats grafted or sham-operated on day 21 were treated with 0.2 ml of LH antiserum (LHAS) or the same volume of a normal horse serum (NHS); (3) female rats were injected on day 1 of life with 0.1 mg of estradiol benzoate or olive oil. Groups of these animals were decapitated daily between days 6 and 21 in order to measure gonadotropins and prolactin (PRL) pituitary content. Since on day 21 estrogenized females showed decreased gonadotropin content and normal PRL content, females in experiment 4 were grafted on day 21 with pituitaries obtained from control or neonatally estrogenized female rats. The results obtained showed that FSH, LH and estradiol plasma levels as well as ovarian and uterine weights increased after pituitary grafts. LHAS blocked the precocious puberty induced by the pituitary graft, and pituitaries obtained from neonatally estrogenized female rats were unable to modify the occurrence of puberty when grafted. In conclusion, this work evidences that precocious puberty induced by 'young' pituitary grafts was mediated by the increase in LH secretion from the graft.

Adrenal Glands↗

PubMed2004

A single luteinizing hormone determination 2 hours after depot leuprolide is useful for therapy monitoring of gonadotropin-dependent precocious puberty in girls.

Long-acting GnRH analogs represent the standard treatment for gonadotropin-dependent precocious puberty. The aim of this study was to determine the hormonal parameters for monitoring the adequacy of depot leuprolide acetate treatment in girls with clinical and hormonal diagnosis of gonadotropin-dependent precocious puberty. Eighteen girls were treated monthly with 3.75 mg depot leuprolide acetate. Adequate hypothalamic-pituitary-gonadal axis suppression during treatment was achieved in 16 of the 18 girls according to the clinical parameters and prepubertal LH levels. In these 16 well-controlled girls, the LH peak after a classical GnRH test was compared with a single LH measurement obtained 2 h after depot leuprolide acetate administration before and during GnRH analog treatment. Before therapy, the mean +/- sd LH peak after a classical GnRH test was 18.4 +/- 11.2 IU/liter (ranging from 7-41.5 IU/liter), and it was 22.6 +/- 8.3 IU/liter 2 h after the first depot leuprolide dose (ranging from 10-35.3 IU/liter). During therapy, the mean +/- sd of LH peak after classical GnRH test was 1.4 +/- 0.6 IU/liter (ranging from <0.6 to 2.3 IU/liter), and it was 2.7 +/- 1.9 IU/liter (ranging from 0.7-6.6 IU/liter) 2 h after depot leuprolide. The LH peak after a classical GnRH test and that 2 h after depot leuprolide administration correlate significantly before and during treatment. In conclusion, we established the LH cut-off values for an adequate depot leuprolide therapy as an LH peak below 2.3 IU/liter after a classical GnRH test or below 6.6 IU/liter 2 h after depot leuprolide. The latter measurement may replace the classical GnRH test as a reliable and convenient tool for monitoring therapy in female gonadotropin-dependent precocious puberty.

Child↗

PubMed2000

Experience with cyproterone acetate in the treatment of precocious puberty.

The authors review their experience (1967-present) in the use of cyproterone acetate (CPA) in precocious puberty. CPA was found effective in persistently suppressing pituitary gonadotropic secretion when administered orally at a dose of 50 mg b.i.d. (70-100 mg/d). After the introduction of gonadotropic analogues (GnRHa) for treatment of central precocious puberty, short term use of CPA was found useful to counteract the initial stimulatory effect of the GnRHa as well as an adjunct drug in case of very active adrenarche causing advanced bone age during GnRHa treatment. The final heights of girls treated with CPA and girls treated with D-Trp6-LHRH were found comparable: 157.8+/-5.1 cm vs 159.6+/-6.3 cm, respectively. The main adverse effects were occasional fatigue due to partial adrenal insufficiency with CPA and gynecomastia in a few boys. Liver function tests were normal in all patients with the exception of one boy with severe hypothalamic disease, including precocious puberty, who developed liver cirrhosis 3 years after stopping CPA following 5 years treatment. Other indications for CPA treatment during childhood and adolescence, such as fast puberty, congenital adrenal hyperplasia and acne, are also mentioned.

Body Height↗

PubMed2001

[Precocious puberty in girls depending on choriogonadotropin - case reports and literature review].

A 5-year-old girl presented typical features of isosexual precocity with breast and pubic hair development (Tanner stage 3) and menarche, following a few months history of hirsutism of the back and thighs. Stimulation testing revealed GnRH-independent precocious puberty, secondary hypothyroidism, hyperprolactinemia and mild testosteronemia. The ovaries in ultrasound examination were prepubertal. Tumor markers beta-HCG and AFP were markedly elevated and a suprasellar germ cell tumor 25x15 mm in size was visualized by MRI. Combined chemotherapy followed by radiotherapy resulted in normalization of pubertal features along with estrogen and marker levels. These data indicate a possible development of precocious puberty in girls induced by choriogonadotropin (hCG) released by a supracellar germ cell tumor. Based on our observations and the literature on the subject the authors believe that the autonomous presence of aromatase inside the tumor may be responsible for precocious puberty in girls with hCG-releasing germ cell tumors, regardless of their location. In such cases the rare character of isosexual precocious puberty in girls may result from a unique combination of high hCG-releasing activity and high aromatase activity. In girls with precocious puberty, when discrete androgenization signs develop, the authors postulate to extend the diagnostic management so that hCG-dependent precocious puberty could be ruled out.

English Abstract↗

PubMed2005

[Treatment of precocious puberty by GnRH agonists].

The objective of GnRH agonist treatment in precocious puberty is to block pubertal development, and to reduce the action of sex steroids on bone maturation in order to restore normal long-term growth of the skeleton. Currently available studies show a positive benefit-risk ratio of GnRH analogs in the treatment of precocious puberty. Adult heights obtained are in average greater than those predicted in the absence of treatment and close to the normal target height. The side effects observed during treatment such as headaches, asthenia or hot flushes, are related to sex steroid deprivation and are observed in 20 to 30% of cases. Questions remain concerning the impact of these treatments on intellectual development and body composition. Finally, to assess the impact on fertility, IPSEN laboratories decided to organize a prospective study on patients treated in the 1980-1990s. In pediatrics, a reduction in the number of injections is an important objective to improve compliance. "Depot" formulations with prolonged release over three months, already used in adult indications, represent a useful progress in pediatrics and have recently been assessed. The efficacy and safety of triptorelin acetate (Decapeptyl) administered at a dose of 11.25 mg every trimester for one year were evaluated in a European multicentre open trial including 54 girls and 10 boys. The LH peak during the GnRH test at three months was used as primary end-point. This slow release triptorelin was shown to be effective, similarly to delayed-acting leuprorelin. The auxological effects, in particular on adult height, were not evaluated in this study.

Body Composition↗

PubMed2002

Pelvic sonography and uterine artery color Doppler analysis in the diagnosis of female precocious puberty.

OBJECTIVES: To evaluate the role of ultrasound and color Doppler analyses in the diagnosis of precocious puberty. METHODS: Gray-scale sonographic uterine and ovarian evaluation together with color Doppler analysis of the uterine artery were prospectively performed in 29 girls presenting with premature breast development and pubic hair growth. The values were compared with results obtained from the gonodotrophin releasing hormone stimulation test. Excluded from the study were patients with isolated thelarche or isolated pubarche and those patients with gonodotrophin releasing hormone-independent puberty and with polycystic ovaries. RESULTS: According to the Tanner scale, all the girls presented a breast stage of 2-3 and pubic hair stage 2-3. The uterine size was greater in those girls who presented a pubertal response to the gonodotrophin releasing hormone test (Group II; n = 20) (8.07 +/- 4.47 mL) than in those who did not (Group I; n = 9) (3.07 +/- 1.18 mL; P = 0.001). The ovarian volume and the number of small follicles was not significantly different between the groups. On Doppler analysis, more elevated impedances were observed in Group I (pulsatility index = 3.28 +/- 0.37) than in Group II (pulsatility index = 2.29 +/- 0.19; P = 0.001) girls. The presence of a low pulsatility index (< 2.5) at the level of the uterine arteries had a high diagnostic value for precocious puberty (sensitivity 86%, specificity 100%). CONCLUSIONS: Uterine artery Doppler analysis may assist the diagnosis of gonodotrophin releasing hormone-dependent precocious puberty, may be useful for the selection of those girls needing treatment, and may simplify the follow-up of girls treated for precocities.

Child↗

PubMed1996

Moyamoya disease with precocious puberty and pustular psoriasis.

An 8-year-old girl suffering from precocious puberty and pustular psoriasis with moyamoya disease is described. The possibilities of a causal relationship between precocious puberty and moyamoya disease are discussed. The relevance of the patient's earlier upper respiratory tract infections to the pustular psoriasis and moyamoya disease is also considered.

Child↗

PubMed1986

Studies of gonadotrophin pulsatility and pelvic ultrasound examinations distinguish between isolated premature thelarche and central precocious puberty.

We have studied the pulsatile secretion of gonadotrophins at night and made ovarian ultrasound examinations in three girls with central precocious puberty and three with isolated premature thelarche. The three girls with precocious puberty had well-defined pulsatile secretion of LH and FSH with LH predominating, as would be expected in normal puberty. Pulsatile secretion of gonadotrophins was also seen in girls with premature thelarche but the pattern was reversed. In girls with precocious puberty, large "multicystic" ovaries and large uteri were seen on ultrasound examination, whereas girls with isolated premature thelarche had small uteri and ovaries with less than four "cysts" up to 15 mm in diameter. These data provide the key to understanding the aetiology of isolated premature thelarche.

Breast↗