scholarly journals Premature Adrenarche—Normal Variant or Forerunner of Adult Disease?*

2000 ◽  
Vol 21 (6) ◽  
pp. 671-696 ◽  
Author(s):  
Lourdes Ibáñez ◽  
Joan DiMartino-Nardi ◽  
Neus Potau ◽  
Paul Saenger
Keyword(s):  
Final Height ◽  
Later Life ◽  
Pubic Hair ◽  
Adrenal Androgen ◽  
Positive Role ◽  
Adult Disease ◽  
Premature Adrenarche ◽  
Functional Ovarian Hyperandrogenism ◽  
Ovarian Hyperandrogenism ◽  
Ovarian Syndrome

Abstract Adrenarche is the puberty of the adrenal gland. The descriptive term pubarche indicates the appearance of pubic hair, which may be accompanied by axillary hair. This process is considered premature if it occurs before age 8 yr in girls and 9 yr in boys. The chief hormonal product of adrenarche is dehydroepiandrosterone (DHEA) and its sulfated product DHEA-S. The well documented evolution of adrenarche in primates and man is incompatible with either a neutral or harmful role for DHEA and implies most likely a positive role for some aspect of young adult pubertal maturation and developmental maturation. Premature adrenarche has no adverse effects on the onset and progression of gonadarche in final height. Both extra- and intraadrenal factors regulate adrenal androgen secretion. Recent studies have shown that premature adrenarche in childhood may have consequences such as functional ovarian hyperandrogenism, polycystic ovarian syndrome, and insulin resistance in later life, sometimes already recognizable in childhood or adolescence. Premature adrenarche may thus be a forerunner of syndrome X in some children. The association of these endocrine-metabolic abnormalities with reduced fetal growth and their genetic basis remain to be elucidated.

scholarly journals Normal and Premature Adrenarche

2021 ◽  
Author(s):  
Robert L Rosenfield
Keyword(s):  
Polycystic Ovary ◽  
Molecular Genetic ◽  
Bone Age ◽  
Dehydroepiandrosterone Sulfate ◽  
Hydroxysteroid Dehydrogenase ◽  
Pubic Hair ◽  
Growth Potential ◽  
Adrenal Androgen ◽  
Premature Adrenarche ◽  
Premature Pubarche

Abstract Adrenarche is the maturational increase in adrenal androgen production that normally begins in early childhood. It results from changes in the secretory response to ACTH that are best indexed by dehydroepiandrosterone sulfate (DHEAS) rise. These changes are related to the development of the zona reticularis (ZR) and its unique gene/enzyme expression pattern of low 3ß-hydroxysteroid dehydrogenase type 2 with high cytochrome b5A, sulfotransferase 2A1, and 17ß-hydroxysteroid dehydrogenase type 5. Recently 11-ketotestosterone was identified as an important bioactive adrenarchal androgen. Birth weight, body growth, obesity, and prolactin are related to ZR development. Adrenarchal androgens normally contribute to the onset of sexual pubic hair (pubarche) and sebaceous and apocrine gland development. Premature adrenarche causes ≥90% of premature pubarche. Its cause is unknown. Affected children have a significantly increased growth rate with proportionate bone age advancement that typically does not compromise growth potential. Serum DHEAS and testosterone levels increase to levels normal for early female puberty. It is associated with mildly increased risks for obesity, insulin resistance, and possibly mood disorder and polycystic ovary syndrome. Five-10% of premature pubarche is due to virilizing disorders, which are usually characterized by more rapid advancement of pubarche and compromise of adult height potential than premature adrenarche. Most cases are due to nonclassic congenital adrenal hyperplasia. Algorithms are presented for the differential diagnosis of premature pubarche.This review highlights recent advances in molecular genetic and developmental biologic understanding of ZR development and insights into adrenarche emanating from mass spectrometric steroid assays.

Lack of an ovarian function influence on the increased adrenal androgen secretion present in women with functional ovarian hyperandrogenism

1997 ◽  
Vol 67 (4) ◽  
pp. 654-662 ◽  
Author(s):  
Héctor F. Escobar-Morreale ◽  
Joaquin Serrano-Gotarredona ◽  
Rafael Garcia-Robles ◽  
JoséM. Sancho ◽  
César Varela
Keyword(s):  
Ovarian Function ◽  
Adrenal Androgen ◽  
Androgen Secretion ◽  
Functional Ovarian Hyperandrogenism ◽  
Ovarian Hyperandrogenism

scholarly journals Continuum of phenotypes and sympathoadrenal function in premature adrenarche

2009 ◽  
Vol 160 (4) ◽  
pp. 657-665 ◽  
Author(s):  
Pauliina Utriainen ◽  
Raimo Voutilainen ◽  
Jarmo Jääskeläinen
Keyword(s):  
Clinical Signs ◽  
Dehydroepiandrosterone Sulfate ◽  
Pubic Hair ◽  
Adrenal Androgen ◽  
Pronounced Increase ◽  
Adrenal Steroid ◽  
Premature Adrenarche ◽  
Adrenal Hyperandrogenism ◽  
Early Rise ◽  
Design And Methods

ObjectivesPremature adrenarche (PA), the early rise in adrenal androgen (AA) production, can manifest with different clinical signs of androgen effect. Premature pubarche defined as appearance of pubic hair before the age of 8/9 years in girls/boys, is the most prominent clinical sign of PA and often erroneously described as a synonym of PA. Our aim was to determine the association of circulating AA concentrations with different prepubertal signs of androgen action (SAA). Secondly, we tested whether adrenomedullary function is altered in children with SAA, as it is in congenital adrenal hyperplasia (CAH) also causing adrenal hyperandrogenism.Design and methodsWe examined 73 Finnish prepubertal children with any hyperandrogenic sign(s) having appeared before the age of 8/9 years (girls/boys) (35 with pubic and/or axillary hair=PAH; 38 without=nonPAH), and 98 age- and sex-matched controls. Circulating adrenal steroid and catecholamine concentrations were measured and correlated with clinical parameters.ResultsNone of the children with SAA had CAH or virilizing tumor. Serum dehydroepiandrosterone, dehydroepiandrosterone sulfate, and androstenedione concentrations overlapped between the SAA and control children, and they were lower in the nonPAH than PAH group (P<0.01). SAA children had similar plasma epinephrine but higher norepinephrine (NE) concentrations than their controls (mean (95% confidence interval) 1.61 (1.44, 1.77) versus 1.39 (1.30, 1.49) nmol/l, P=0.03).ConclusionsPA forms a continuum with more pronounced increase in circulating androgens in children with PAH than in those without. Some children show SAA with fairly low androgen concentrations. The clinical significance of elevated NE concentrations associated with SAA needs to be confirmed in further studies.

Premature Adrenarche and its Association with Cardiovascular Risk in Females

2020 ◽  
Vol 26 (43) ◽  
pp. 5609-5616
Author(s):  
Sarantis Livadas ◽  
Christina Bothou ◽  
Djuro Macut
Keyword(s):  
Polycystic Ovary ◽  
Dehydroepiandrosterone Sulfate ◽  
Adrenal Androgen ◽  
Premature Adrenarche ◽  
Adrenal Hyperandrogenism ◽  
Long Term Follow Up ◽  
History Of ◽  
Hormonal Milieu ◽  
The Impact

Early activation of the adrenal zona reticularis, leading to adrenal androgen secretion, mainly dehydroepiandrosterone sulfate (DHEAS), is called premature adrenarche (PA). The fact that adrenal hyperandrogenism in females has been linked to a cluster of cardiovascular (CV) risk factors, even in prepubertal children, warrants investigation. Controversial results have been obtained in this field, probably due to genetic, constitutional, and environmental factors or differences in the characteristics of participants. In an attempt to understand, in depth, the impact of PA as a potential activator of CV risk, we critically present available data stratified according to pubertal status. It seems that prepubertally, CV risk is increased in these girls, but is somewhat attenuated during their second decade of life. Furthermore, different entities associated with PA, such as polycystic ovary syndrome, non-classical congenital adrenal hyperplasia, heterozygosity of CYP21A2 mutations, and the impact of DHEAS on CV risk, are reviewed. At present, firm and definitive conclusions cannot be drawn. However, it may be speculated that girls with a history of PA display a hyperandrogenic hormonal milieu that may lead to increased CV risk. Accordingly, appropriate long-term follow-up and early intervention employing a patient-oriented approach are recommended.

REPLACEMENT ORAL ETHINYLOESTRADIOL THERAPY FOR GONADAL DYSGENESIS: GROWTH AND ADRENAL ANDROGEN STUDIES

1979 ◽  
Vol 91 (3) ◽  
pp. 519-528 ◽  
Author(s):  
Anne W. Lucky ◽  
Samuel P. Marynick ◽  
Robert W. Rebar ◽  
Gordon B. Cutler ◽  
Michael Glen ◽  
...  
Keyword(s):  
Gonadal Dysgenesis ◽  
Bone Age ◽  
Pubic Hair ◽  
Height Velocity ◽  
First Year ◽  
Adrenal Androgen ◽  
Secondary Sexual Characteristics ◽  
Sexual Characteristics ◽  
Second Year ◽  
Pre Treatment

ABSTRACT We have studied growth and adrenal dehydroepiandrosterone (DHA) responses to iv synthetic adrenocorticotrophic hormone (ACTH, Cortrosyn) in 6 girls with gonadal dysgenesis before and during treatment with lowdose ethinyloestradiol (EOe2). In all patients there was a statisfactory induction of secondary sexual characteristics including increase in breasts and pubic hair and onset of withdrawal bleeding within 6 months of therapy. Height velocity increased from 2.8 ± 0.9 cm/year pre-treatment to 5.3 ± 1.5 cm/year (P < 0.02) in the first year. There was deceleration to 1.9 ± 1.1 cm/year in the second year. There was no disproportionate advancement in bone age and thus, presumably, no loss of ultimate height. We could demonstrate no change in basal or ACTH-stimulated levels of DHA, a specific adrenal androgen, to account for the increased pubic hair and growth in these patients.

scholarly journals SUN-222 Pre- and Post-Pubertal Reference Ranges for Oxygenated Androgens in Saliva

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Joanne Adaway ◽  
Graeme Eisenhofer ◽  
Angela Huebner ◽  
Nils Krone ◽  
Malcolm McTaggart ◽  
...  
Keyword(s):  
Reference Interval ◽  
Reference Intervals ◽  
Range Data ◽  
Reference Ranges ◽  
Non Invasive ◽  
Premature Adrenarche ◽  
Upper Limits ◽  
Males And Females ◽  
Monitoring Treatment ◽  
Ovarian Syndrome

Abstract Oxygenated androgens such as 11 β-hydroxyandrostenedione (11OHA4) and 11-ketotestosterone (11KT) contribute significantly to the androgen pool in humans and their measurement has been shown to be useful in diagnosing disorders such as polycystic ovarian syndrome or premature adrenarche and also in monitoring treatment of congenital adrenal hyperplasia, alongside the classical androgens. Their measurement in saliva is particularly advantageous due to the non-invasive nature of sampling, meaning samples can easily be taken regularly to monitor treatment; however reference range data is not currently available for 11OHA4 and 11KT, limiting their clinical use. These analytes were measured in saliva samples from pre and post-pubertal males and females to inform reference ranges for these analytes. Samples collected into salivettes as part of the PRIMMS study (Technische Universität Dresden) were used for this work. A total of 130 samples (35 from pre-pubertal females, 43 from post-pubertal females, 42 from pre-pubertal males and 20 from post-pubertal males) were analysed for 11OHA4 and 11KT by LC-MS/MS. The ages of the participants ranged from 3.77 to 14.0 years in the pre-pubertal samples and 13.9-17.9 years in the post-pubertal samples. Pubertal status was determined clinically. The upper cut-off of the reference interval for 11OHA4 was 560 pmol/L in pre-pubertal females and 590 pmol/L in males, whilst 11KT had an upper limit of 216 pmol/L in females and 205 pmol/L in males. The upper limits of the ranges were higher in post-pubertal samples, with ranges of up to 1542 pmol/L in females and 1775 pmol/L in males for 11OHA4; the ranges for 11KT were up to 654 pmol/L for post pubertal females and 585 pmol/L for post-pubertal males. The data shows, as expected, a rise in the 11 oxygenated androgens post puberty. The upper limits of reference intervals for both analytes were very similar in males and females both pre- and post pubertally. These data can be used to inform clinical interpretation of the 11-oxygenated androgens; further work is required with larger cohorts of samples to develop more robust reference ranges.

009. The effect of maternal asthma during pregnancy on placental function, fetal growth and childhood development

2005 ◽  
Vol 17 (9) ◽  
pp. 65 ◽  
Author(s):  
V. L. Clifton
Keyword(s):  
Fetal Growth ◽  
Later Life ◽  
Growth Patterns ◽  
Childhood Development ◽  
Antenatal Visit ◽  
Contributing Factors ◽  
Male Fetus ◽  
Female Fetus ◽  
Adult Disease ◽  
Increased Risk

Fetal growth and neonatal birth weight are significant contributing factors to the development of adult disease states in later life. In human pregnancy, we have identified sexually dimorphic differences in fetal growth with the female fetus reducing growth in response to maternal asthma and the male fetus continuing to grow at a normal rate but being at an increased risk of in utero death. The physiological mechanisms that confer sex-specific differences in the fetal response to maternal asthma are unknown. However our research has identified differences in mechanisms associated with fetal glucocorticoid regulation, which are also associated with changes in childhood growth patterns. Asthmatic and control pregnant women were recruited at their first antenatal visit and followed through to delivery. Subjects were assessed for severity of asthma and their use of medication, including glucocorticoid therapy, was recorded. In addition to routine antenatal care, fetal growth was determined using Doppler ultrasound. Following delivery placentas and cord blood were collected. The children of the women followed during the study were examined by a paediatrician at 6 months of age and every 12 months after that initial visit. Our data shows that in response to maternal asthma, the female fetus has an increase in cortisol, which downregulates placental GR expression, immune and hypothalamic-pituitary-adrenal function and is associated with decreased growth. The male fetus responds to increased cortisol with an increase in GR expression and no change in HPA or immune function or growth. These data indicate that the male and female fetus have different strategies to control growth and in their response to a maternal stress, such as asthma.

scholarly journals Approach to the Girl with Early Onset of Pubic Hair

2011 ◽  
Vol 96 (6) ◽  
pp. 1610-1622 ◽  
Author(s):  
Sharon E. Oberfield ◽  
Aviva B. Sopher ◽  
Adrienne T. Gerken
Keyword(s):  
Normal Development ◽  
Preventive Measures ◽  
Polycystic Ovary ◽  
Pubic Hair ◽  
Children At Risk ◽  
Ovary Syndrome ◽  
Zona Reticularis ◽  
Premature Adrenarche ◽  
Axillary Hair ◽  
Premature Pubarche

Premature pubarche, or the development of pubic hair before the age of 8 in girls or 9 in boys, is most commonly caused by premature adrenarche. Adrenarche is the maturation of the adrenal zona reticularis in both boys and girls, resulting in the development of pubic hair, axillary hair, and adult apocrine body odor. Although originally thought to be a benign variant of normal development, premature adrenarche has been associated with insulin resistance and the later development of metabolic syndrome and polycystic ovary syndrome. Although further studies are needed to confirm these relationships, the case presented herein argues for periodic assessment of children at risk. Indeed, recognition of these associations may allow for early preventive measures.

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