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. Normal linear growth pattern
  2. 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
  3. No Sexual Development
    1. No penis or Testes enlargement
    2. No Breasts, clitoris or ovary enlargement

V. Differential Diagnosis

VI. Imaging

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

VII. Labs

  1. Labs may be deferred initially
  2. Slight increase in Serum Dehydroepiandrosterone (DHEA) level
  3. Other adrenal steroid Hormones normal (e.g. Cortisol, Aldosterone)
  4. Sex Hormone levels normal (Estradiol, Testosterone)
  5. Normal Luteinizing Hormone (LH) and Follicle Stimulating Hormone (FSH)
  6. ACTH Stimulation Test normal
    1. No Congenital Adrenal Hyperplasia
  7. GnRH Stimulation Test normal
    1. Consistent with prepubertal pattern
  8. 17-Hydroxyprogesterone
    1. Elevated in <5% of cases (due to mild congeital adrenal hyperplasia)

VIII. Management

  1. Reassurance
  2. Indications for pediatric endocrinology
    1. Pathologic adrenal causes suspected (esp. age <6 years)
    2. Virilization
    3. Associated other signs of pubertal development
    4. Increased Growth Velocity
    5. Advanced Bone Age

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