II. Pathophysiology

  1. Benign self-limited cause of Precocious Puberty
  2. Results from adrenal androgen release as opposed to the pubertal Hypothalamus-Pituitary-Gonadal activation

III. Epidemiology

  1. Onset before age 6 years

IV. Signs

  1. Early secondary sexual characteristics in children
    1. Early pubic hair and axillary hair development
    2. Increased sebaceous activity
    3. Adult-type body odor
    4. Mild acne
  2. No Sexual Development
    1. No penis or Testes enlargement
    2. No Breasts, clitoris or ovary enlargement
  3. Normal linear growth pattern

V. Imaging

  1. Bone Age consistent Chronological age
  2. Other imaging studies normal

VI. Labs (May delay evaluation)

  1. Slight increase in Serum Dehydroepiandrosterone (DHEA) level
  2. Other adrenal steroid hormones normal (e.g. cortisol, aldosterone)
  3. Sex hormone levels normal (estradiol, testosterone)
  4. Normal Luteinizing hormone (LH) and Follicle Stimulating Hormone (FSH)
  5. ACTH Stimulation Test normal
    1. No Congenital Adrenal Hyperplasia
  6. GnRH Stimulation Test normal
    1. Consistent with prepubertal pattern
  7. 17-Hydroxyprogesterone
    1. Elevated in <5% of cases (due to mild congeital adrenal hyperplasia)

VII. Management

  1. Reassurance

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Ontology: Premature adrenarche (C0342546)

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, ソウハツセイフクジンヒシツキノウショウガイ
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