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Cited by 158Sexual Differentiation and DisordersAdrenal Hormones and DisordersOvarian function and disorders

Premature adrenarche: novel lessons from early onset androgen excess

Idkowiak, Jan, Lavery, Gareth G, Dhir, Vivek, Barrett, Timothy G, Stewart, Paul M, Krone, Nils, Arlt, Wiebke·2011·European Journal of Endocrinology
DOI 10.1530/eje-11-0223
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Abstract

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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Registry

Crossref · 10.1530/eje-11-0223

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167 of 167 references verified0 citation errors · 0 not checkable

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  • Adrenal C19 steroid production is initiated during adrenarche, a pre-pubertal event approximately age 8 in girls and 9 in boys, unique to humans and higher primates.

    “It should be noted that adrenal C19 steroid production is initiated during adrenarche, a pre-pubertal event (approximately age 8 in girls and 9 in boys (Idkowiak et al., 2011)) unique to humans and higher primates (Abbott and Bird, 2009, Arlt et al., 2002, Auchus and Rainey, 2004, Miller, 2008).”

    From Intracrine androgen biosynthesis, metabolism and action revisited (2018) · cites this paper as Idkowiak et al., 2011

  • Affected patients may present with premature adrenarche in childhood including pubarche, or as adolescents and adults with androgen excess and a PCOS-like phenotype in females.

    “Affected patients may present with premature adrenarche in childhood including pubarche, or as adolescents and adults with androgen excess and a PCOS-like phenotype in females (4, 5, 10) .”

    From Novel H6PDH mutations in two girls with premature adrenarche: ‘apparent’ and ‘true’ CRD can be differentiated by urinary steroid profiling (2013) · cites this paper as [10]

  • CRD and ACRD present with a similar phenotype, with affected individuals developing androgen excess in childhood, coming to clinical attention with premature adrenarche in children of both sexes or in adolescent and young adult women with a PCOS-like phenotype.

    “Clinically, CRD and ACRD present with a similar phenotype, with affected individuals developing androgen excess in childhood, coming to clinical attention with premature adrenarche in children of both sexes or in adolescent and young adult women with a PCOS-like phenotype (90, 92–94).”

    From Steroid Metabolome Analysis in Disorders of Adrenal Steroid Biosynthesis and Metabolism (2019) · cites this paper as [93]

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Authors
Idkowiak, J., Lavery, G. G., Dhir, V., Barrett, T. G., Stewart, P. M., Krone, N., & Arlt, W.
Affiliations
University of Birmingham (GB)
Corresponding author
Arlt, Wiebke
ORCID
Venue
European Journal of Endocrinology
Publisher
Oxford University Press (OUP)
Published
2011-08
Volume
165
Issue
2
Pages
189-207
ISSN
  • 0804-4643 (ISSN-L)
  • 1479-683X
DOI
10.1530/eje-11-0223
OpenAlex
W2117532854
Semantic Scholar
fc0c3bb5a7c0fe1e0841440071cf156f0aa351d7
Cited by
158
References
167
Citations by year
2026: 6 · 2025: 27 · 2024: 3 · 2023: 7 · 2022: 5 · 2021: 6 · 2020: 13 · 2019: 10 · 2018: 9 · 2017: 8 · 2016: 9 · 2015: 11 · 2014: 14 · 2013: 11 · 2012: 15
FWCI
1.19
Type
journal article
Language
en
Open access
closed
Locations
  • European Journal of Endocrinology (publishedVersion)
  • PubMed (publishedVersion)
  • University of Birmingham Research Portal (University of Birmingham) (submittedVersion)
Funders
  • Wellcome Trust (066357)
  • National Institute for Health and Care Research (NF-SI-0508-10356)
  • Biotechnology and Biological Sciences Research Council (BB/G023468/1)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sexual Differentiation and Disorders
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
  • Health Sciences › Medicine › Reproductive Medicine › Ovarian function and disorders
Keywords
premature adrenarche, dehydroepiandrosterone, dehydroepiandrosterone sulfate, adrenal zona reticularis, precocious puberty, polycystic ovary syndrome, metabolic syndrome, insulin resistance, low birth weight, cardiovascular risk
MeSH terms
Adult, Androgens, Animals, Female, Humans, Male, Models, Biological, Osmolar Concentration, Puberty, Precocious, Time Factors, Age of Onset, Adrenarche

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