CONSTITUTIONAL PRECOCIOUS PUBERTY
 
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CONSTITUTIONAL PRECOCIOUS PUBERTY

 

DEFINITION:

A disorder characterized by the orderly development of secondary sexual characteristics prior to the age of 8 and 8.5 years in females and males, respectively.

EPIDEMIOLOGY:

  • incidence: accounts for a majority of cases:
    • 67-93% in females with true precocious puberty
    • 36-50% in males with true precocious puberty
  • age of onset:
    • prior to 8 years of age (by definition)
  • risk factors:
    • F > M
    • sporadic or familial

PATHOGENESIS:

1. Background

  • due to the premature awakening of the hypothalamic-pituitary-gondal axis by an unknown mechanism
  • considered a true or central (complete) precocious puberty:
    • gonadotropin-dependent
    • development of all secondary sexual characteristics (isosexual)
    • increased size and activity of gonads
  • represents a normal orderly development of secondary sexual characteristics but earlier

CLINICAL FEATURES:

1. Clinical Course

  • extremely variable
  • may occur at any age <8 years of age
  • may complete sexual maturity rapidly or slowly
  • characteristics may remain stationary or regress

2. Females

  • breast development +/- pubic hair
  • development of external genitalia
  • axillary hair
  • menstruation
    • initially irregular and usually anovulatory
    • bone age of 12 years
  • growth spurt -> short stature ultimately

3. Males

  • enlargement of testes (>2 cc) and penis
  • development of pubic hair
  • acne
  • frequent erections, spermatogenesis, noctural emissions
  • deepening voice
  • growth spurt -> short stature ultimately
    • testicular volume 10-12 cc at peak of growth spurt

INVESTIGATIONS:

1. Imaging Studies

1. Skeletal X-Rays

  • advanced bone age

2. Pelvic Ultrasound

  • enlargement of the uterus
  • multicystic appearance of ovaries

2. Serum

  • elevated testosterone (M) and estradiol (F)
  • elevated FSH and LH on serial determinations
  • pulsatile secretion of FSH and LH
  • LHRH stimulation test
    • brief LH response as in puberty
    • best test for evidence of central precocious puberty

MANAGEMENT:

1. LHRH Analogues

1. Luprolide Acetate (Lupron Depot)

  • 0.2-0.3 mg/kg (max. 7.5 mg) IM q4weeks
  • a decapeptide with one amino acid altered at positions 6 or 10
  • minimal side effects: painful, abscess at injection site, and may initially see an acceleration of sex features
  • expensive: $400/injection
  • suppress hypothalamic-pituitary-gonadal function
    • down-regulates receptors
  • regression of secondary sexual characteristics
    • cessation of menses
  • delays short stature by delaying the closure of epiphyses and normalizes rate of growth

 

 

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