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Premature pubarche: etiological heterogeneity.

Abstract
Premature pubarche is characterized by pubic hair, adult type body odor, acne, and axillary hair before 8 yr of age in girls and 9.5 yr of age in boys. Causes of this premature virilization include premature adrenarche, mild errors of steroidogenesis, precocious puberty, and adrenal and gonadal tumors. To determine whether any clinical parameters are helpful in distinguishing which children should undergo further evaluation for mild congenital adrenal hyperplasia, we performed ACTH stimulation tests in 69 children with premature pubarche and 8 pubertal controls. Patients were categorized as having typical (pubic hair with or without axillary hair and body odor) or atypical (pubic hair and genital enlargement) premature pubarche. Blood samples, before and 30 min after iv bolus administration of synthetic ACTH, were obtained for progesterone, 17-hydroxypregnenolone, 17-hydroxyprogesterone, dehydroepiandrosterone, androstenedione, 11-deoxycortisol, and cortisol measurements. The patients were divided into 4 groups based on their individual responses to ACTH stimulation: premature adrenarche (no apparent defect in steroidogenesis), possible decreased 21-hydroxylase activity, possible decreased 3 beta-hydroxysteroid dehydrogenase activity, and indeterminate responses. Five of 11 (45%) children with atypical premature pubarche and 7 of 58 (12%) children with typical premature pubarche were found to have evidence of mild defects in steroidogenesis. Similar to previous reports in postpubertal women, only responses to ACTH stimulation allowed accurate classification of these patients.
AuthorsS F Siegel, D N Finegold, M D Urban, R McVie, P A Lee
JournalThe Journal of clinical endocrinology and metabolism (J Clin Endocrinol Metab) Vol. 74 Issue 2 Pg. 239-47 (Feb 1992) ISSN: 0021-972X [Print] United States
PMID1309831 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Hydroxyprogesterones
  • 17-alpha-Hydroxypregnenolone
  • Androstenedione
  • Dehydroepiandrosterone
  • Progesterone
  • 17-alpha-Hydroxyprogesterone
  • Adrenocorticotropic Hormone
  • Cortodoxone
  • Hydrocortisone
Topics
  • 17-alpha-Hydroxypregnenolone (blood)
  • 17-alpha-Hydroxyprogesterone
  • Adrenal Cortex Diseases (classification, etiology, physiopathology)
  • Adrenocorticotropic Hormone
  • Androstenedione (blood, metabolism)
  • Child
  • Cortodoxone (blood)
  • Dehydroepiandrosterone (blood, metabolism)
  • Female
  • Genitalia, Female (growth & development)
  • Genitalia, Male (growth & development)
  • Hirsutism (classification, etiology, physiopathology)
  • Humans
  • Hydrocortisone (blood, metabolism)
  • Hydroxyprogesterones (blood)
  • Male
  • Progesterone (blood, metabolism)
  • Reference Values

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