II. Pathophysiology
- Benign self-limited cause of Precocious Puberty
- Results from adrenal androgen release as opposed to the pubertal Hypothalamus-Pituitary-Gonadal activation
III. Epidemiology
- Onset before age 6 years
IV. Signs
- Early secondary sexual characteristics in children
- Early pubic hair and axillary hair development
- Increased sebaceous activity
- Adult-type body odor
- Mild acne
- No Sexual Development
- Normal linear growth pattern
V. Imaging
- Bone Age consistent Chronological age
- Other imaging studies normal
VI. Labs (May delay evaluation)
- Slight increase in Serum Dehydroepiandrosterone (DHEA) level
- Other adrenal steroid Hormones normal (e.g. Cortisol, Aldosterone)
- Sex Hormone levels normal (Estradiol, Testosterone)
- Normal Luteinizing Hormone (LH) and Follicle Stimulating Hormone (FSH)
- ACTH Stimulation Test normal
-
GnRH Stimulation Test normal
- Consistent with prepubertal pattern
- 17-Hydroxyprogesterone
- Elevated in <5% of cases (due to mild congeital adrenal hyperplasia)
VII. Management
- Reassurance
VIII. References
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Related Studies
| Definition (NCI) | Premature onset of adrenal androgen-mediated secondary sexual characteristics.(NICHD) |
| Concepts | Disease or Syndrome (T047) |
| ICD10 | E27.0 |
| SnomedCT | 190521009, 190516006, 103021001 |
| Dutch | vroegtijdige adrenarche, adrenarche; prematuur, prematuur; adrenarche |
| French | Adrénarche précoce |
| German | vorzeitige Adrenarche |
| Italian | Adrenarca precoce |
| Portuguese | Adrenarca prematura |
| Spanish | Adrenarquia prematura, adrenarca prematura (hallazgo), adrenarca prematura |
| Japanese | 早発性副腎皮質機能障害, ｿｳﾊﾂｾｲﾌｸｼﾞﾝﾋｼﾂｷﾉｳｼｮｳｶﾞｲ |
| English | precocious adrenarche (diagnosis), precocious adrenarche, premature adrenarche, Premature adrenarche (disorder), Premature Adrenarche, Premature adrenarche, Premature adrenarche (finding), adrenarche; premature, premature; adrenarche |
| Czech | Předčasná adrenarché |
| Hungarian | Korai adrenarche |
