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Related Experiment Videos

Premature pubarche: etiological heterogeneity.

S F Siegel1, D N Finegold, M D Urban

  • 1Children's Hospital of Pittsburg, University of Pittsburgh, Pennsylvania 15213.

The Journal of Clinical Endocrinology and Metabolism
|February 1, 1992
PubMed
Summary

Premature pubarche, early pubic hair development, can indicate underlying steroidogenesis defects. ACTH stimulation tests accurately identified mild defects in 45% of atypical and 12% of typical cases.

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Area of Science:

  • Pediatric Endocrinology
  • Steroidogenesis
  • Adrenal Function

Background:

  • Premature pubarche (PP) involves early development of secondary sexual characteristics.
  • Causes include premature adrenarche, steroidogenesis errors, precocious puberty, and tumors.
  • Distinguishing PP causes requires further evaluation, particularly for mild congenital adrenal hyperplasia (CAH).

Purpose of the Study:

  • To assess clinical parameters for identifying children needing evaluation for mild CAH.
  • To differentiate causes of premature pubarche using ACTH stimulation tests.

Main Methods:

  • ACTH stimulation tests were performed on 69 children with PP and 8 controls.
  • Patients were classified by PP type: typical (pubic hair, odor) and atypical (genital enlargement).

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  • Steroid hormone levels (progesterone, 17-hydroxypregnenolone, etc.) were measured pre- and post-ACTH stimulation.

    • Main Results:

    • Patients were grouped by ACTH response: premature adrenarche, possible 21-hydroxylase deficiency, possible 3β-hydroxysteroid dehydrogenase deficiency, or indeterminate.
    • Mild steroidogenesis defects were found in 45% of atypical PP and 12% of typical PP cases.
    • ACTH stimulation tests proved essential for accurate patient classification.

    Conclusions:

    • ACTH stimulation testing is crucial for diagnosing mild steroidogenesis defects in children with premature pubarche.
    • Atypical premature pubarche is more frequently associated with these underlying defects.
    • This approach aids in distinguishing causes of premature virilization and guiding further management.