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Rosenfield RL. Normal and Premature Adrenarche. Endocr Rev. 2021;42(6):783-814doi: 10.1210/endrev/bnab009.
Rosenfield, R. L. (2021). Normal and Premature Adrenarche. Endocrine reviews, 42(6), 783-814. https://doi.org/10.1210/endrev/bnab009
Rosenfield, Robert L. "Normal and Premature Adrenarche." Endocrine reviews vol. 42,6 (2021): 783-814. doi: https://doi.org/10.1210/endrev/bnab009
Rosenfield RL. Normal and Premature Adrenarche. Endocr Rev. 2021 Nov 16;42(6):783-814. doi: 10.1210/endrev/bnab009. PMID: 33788946; PMCID: PMC8599200.
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Endocr Rev. 2021 Nov 16;42(6):783-814. doi: 10.1210/endrev/bnab009.

Normal and Premature Adrenarche.

Endocrine reviews

Robert L Rosenfield

Affiliations

  1. University of Chicago Pritzker School of Medicine, Section of Adult and Pediatric Endocrinology, Metabolism, and Diabetes, Chicago, IL, USA.
  2. Department of Pediatrics, University of California, San Francisco, CA, USA.

PMID: 33788946 PMCID: PMC8599200 DOI: 10.1210/endrev/bnab009

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 adrenocorticotropin (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 (PP). 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. Between 5% and 10% of PP 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 PP. 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.

© The Author(s) 2021. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: [email protected].

Keywords: adrenal androgens; adrenarche; polycystic ovary syndrome; pubarche; steroidogenic enzyme expression; zona reticularis

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