‘Exaggerated adrenarche’ in children presenting with premature adrenarche

Likitmaskul, Supawadee; Cowell, CT; Donaghue, KC; Kreutzmann, D; Howard, N. J.; Blades, Barbara; Silink, Martin

doi:10.1111/j.1365-2265.1995.tb01874.x

Cited by 47 publications

(20 citation statements)

References 25 publications

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“…Thus, none of our unselected prepubertal children with SAA exhibited even a mild steroidogenic enzyme defect. This finding disagrees with some previous reports (16,23,25), but is in line with the previously reported extremely low prevalence of NC 21-OHD in our population (19).…”

Section: Discussioncontrasting

confidence: 57%

“…This indicates that PA rarely manifests 'exaggerated' (defined as DHEAS exceeding the pubertal range O6.0 mmol/l) (16) at prepubertal age in our population. Our findings differ from those of an Australian study reporting 9.1% prevalence of 'exaggerated adrenarche' among PA children (16). Ibanez and co-workers have shown that girls with a history of PP and low BW have often adrenal hyperandrogenism in adolescence (40).…”

Section: Discussioncontrasting

confidence: 57%

“…None of these nine SAA children had either D4A or DHEA above the 75th percentile of the controls. Only one SAA subject exhibited 'exaggerated adrenarche' with DHEAS exceeding 6 mmol/l (16). Notably, all the SAA children in whom axillary odor was the only androgenic sign had a DHEAS concentration of at least 1 mmol/l.…”

Section: Serum Hormone Concentrationsmentioning

confidence: 99%

“…In addition to the circulating AA concentrations and their peripheral conversion, the clinical signs of PA depend also on individual peripheral sensitivity to androgens determined by transactivational activity of the androgen receptor; some PP subjects actually show normal circulating prepubertal AA concentrations (8,15). Few studies have reported highly elevated androgen concentrations in PP girls, referred as 'exaggerated adrenarche' or 'exaggerated adrenarcheal response' (16,17), while in other reports serum androgen concentrations in PA children have usually been as expected for the pubic hair stage (10,11). Before the clinical diagnosis of PA can be accepted on the basis of PP or other androgenic signs, central puberty, congenital adrenal hyperplasia (CAH), and virilizing tumors have to be excluded.…”

Section: Introductionmentioning

confidence: 99%

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Continuum of phenotypes and sympathoadrenal function in premature adrenarche

Utriainen¹,

Voutilainen²,

Jääskeläinen³

2009

European Journal of Endocrinology

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Objectives: Premature 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 methods: We 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 hairZPAH; 38 withoutZnonPAH), and 98 age-and sex-matched controls. Circulating adrenal steroid and catecholamine concentrations were measured and correlated with clinical parameters. Results: None 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, PZ0.03). Conclusions: PA 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.

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Section: Discussioncontrasting

confidence: 57%

Section: Discussioncontrasting

confidence: 57%

Section: Serum Hormone Concentrationsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Continuum of phenotypes and sympathoadrenal function in premature adrenarche

Utriainen¹,

Voutilainen²,

Jääskeläinen³

2009

European Journal of Endocrinology

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“…Conversely, some investigators have defined PA very strictly as the biochemical detection of a premature increase in circulating adrenal androgens irrespective of the clinical presentation (52). The additional use of the term 'exaggerated adrenarche' has created further confusion, as it has been used to describe adrenal androgen levels above the pubertal range in children of pre-pubertal age (53,54) but also as a definition for the presence of exaggerated clinical features of androgen action associated with physiological, normal-timed adrenarche (55).…”

Section: Pa: Definitions and Differential Diagnosismentioning

confidence: 99%

Premature adrenarche: novel lessons from early onset androgen excess

Idkowiak

Lavery

Dhir

et al. 2011

European Journal of Endocrinology

110

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Adrenarche reflects the maturation of the adrenal zona reticularis resulting in increased secretion of the adrenal androgen precursor DHEA and its sulphate ester DHEAS. Premature adrenarche (PA) is defined by increased levels of DHEA and DHEAS before the age of 8 years in girls and 9 years in boys and the concurrent presence of signs of androgen action including adult-type body odour, oily skin and hair and pubic hair growth. PA is distinct from precocious puberty, which manifests with the development of secondary sexual characteristics including testicular growth and breast development. Idiopathic PA (IPA) has long been considered an extreme of normal variation, but emerging evidence links IPA to an increased risk of developing the metabolic syndrome (MS) and thus ultimately cardiovascular morbidity. Areas of controversy include the question whether IPA in girls is associated with a higher rate of progression to the polycystic ovary syndrome (PCOS) and whether low birth weight increases the risk of developing IPA. The recent discoveries of two novel monogenic causes of early onset androgen excess, apparent cortisone reductase deficiency and apparent DHEA sulphotransferase deficiency, support the notion that PA may represent a forerunner condition for PCOS. Future research including carefully designed longitudinal studies is required to address the apparent link between early onset androgen excess and the development of insulin resistance and the MS.

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Adrenal Androgens and Androgen Precursors—Definition, Synthesis, Regulation and Physiologic Actions

Turcu

Smith

Auchus

et al. 2014

Comprehensive Physiology

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The human adrenal produces more 19 carbon (C19) steroids, by mass, than either glucocorticoids or mineralocorticoids. However, the mechanisms regulating adrenal C19 steroid biosynthesis continue to represent one of the most intriguing mysteries of endocrine physiology. This review will discuss the C19 steroids produced in the human adrenal and the features within the adrenal that allow production of these steroids. Finally, we consider the effects of these steroids in normal physiology and disorders of adrenal C19 steroid excess.

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‘Exaggerated adrenarche’ in children presenting with premature adrenarche

Cited by 47 publications

References 25 publications

Continuum of phenotypes and sympathoadrenal function in premature adrenarche

Continuum of phenotypes and sympathoadrenal function in premature adrenarche

Premature adrenarche: novel lessons from early onset androgen excess

Adrenal Androgens and Androgen Precursors—Definition, Synthesis, Regulation and Physiologic Actions

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