Precocious Puberty

Precocious Puberty is a topic covered in the Select 5-Minute Pediatrics Topics.

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Basics

Description

  • In most populations, the mean age of onset of puberty is 10.5 years in girls and 11.5 years in boys.
  • The first sign of puberty in girls is most commonly breast development (thelarche), followed by pubic hair development (pubarche), and then menarche, which generally occurs 2–3 years after thelarche; in boys, the first sign is usually testicular enlargement, followed by pubarche and penile growth.
  • Precocious puberty has traditionally been defined as physical signs of sexual development before age 8 years in girls and age 9 in boys (2.5–3 standard deviations below the mean age of onset of puberty).
  • Recent guidelines propose lowering the age considered to be normal for sexual development in girls to as young as age 6 years in black girls and age 7 years in white girls. These new guidelines have not been universally adopted.
  • When evaluating precocious puberty, the entire clinical picture, including rate of pubertal progression and the presence of any neurologic symptoms, must be considered.

Epidemiology

  • Occurs in ∼1 in 5,000 children
  • Precocious puberty is up to 10 times more common in girls than boys.
  • Racial differences observed in girls: African-American girls may enter puberty 1 year sooner on average than Caucasian girls. Racial difference not present in males.
  • Increased incidence in internationally adopted children and in children born premature or small for gestational age
    • In girls: 80–90% of central precocious puberty are idiopathic.
    • In boys: Precocious puberty is more likely to be associated with underlying pathology.
    • ∼50% of affected boys have idiopathic central precocious puberty.

Genetics

  • Genetic causes include the following:
    • Familial male precocious puberty (testotoxicosis): sex-limited, autosomal dominant inheritance of luteinizing hormone (LH) receptor activating mutation
    • McCune-Albright syndrome: sporadic, postzygotic, somatic mutation in the stimulatory subunit of G-protein receptor; precocious puberty more common in girls

Pathophysiology

  • Central precocious puberty can be associated with CNS disorders.
  • Peripheral precocious puberty
    • Arises from peripheral sex hormone sources, including gonadal and adrenal disorders, abdominal or pelvic tumors, or exogenous sex steroids
    • Can progress to central precocious puberty due to maturation of the hypothalamic–pituitary axis by sex steroids

Etiology

  • Central precocious puberty (gonadotropin-releasing hormone [GnRH] dependent)
    • Associated with gonadotropin (LH and/or follicle-stimulating hormone [FSH]) levels that are elevated beyond the normal prepubertal range. Results from activation of hypothalamic–pituitary–gonadal axis.
    • Physical changes are typically those of normal puberty for a child of that sex.
  • Peripheral sex hormone effects (peripheral precocious puberty or GnRH-independent; less common)
    • Gonadotropin-independent elevation of sex steroids arising (i) directly from gonads and/or adrenals, (ii) through stimulation of gonads by GnRH-independent mechanism, or (iii) from an exogenous source
    • Physical changes reflect predominant excess hormones (estrogenic or androgenic) and are often markedly discordant from normal pubertal development.

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Basics

Description

  • In most populations, the mean age of onset of puberty is 10.5 years in girls and 11.5 years in boys.
  • The first sign of puberty in girls is most commonly breast development (thelarche), followed by pubic hair development (pubarche), and then menarche, which generally occurs 2–3 years after thelarche; in boys, the first sign is usually testicular enlargement, followed by pubarche and penile growth.
  • Precocious puberty has traditionally been defined as physical signs of sexual development before age 8 years in girls and age 9 in boys (2.5–3 standard deviations below the mean age of onset of puberty).
  • Recent guidelines propose lowering the age considered to be normal for sexual development in girls to as young as age 6 years in black girls and age 7 years in white girls. These new guidelines have not been universally adopted.
  • When evaluating precocious puberty, the entire clinical picture, including rate of pubertal progression and the presence of any neurologic symptoms, must be considered.

Epidemiology

  • Occurs in ∼1 in 5,000 children
  • Precocious puberty is up to 10 times more common in girls than boys.
  • Racial differences observed in girls: African-American girls may enter puberty 1 year sooner on average than Caucasian girls. Racial difference not present in males.
  • Increased incidence in internationally adopted children and in children born premature or small for gestational age
    • In girls: 80–90% of central precocious puberty are idiopathic.
    • In boys: Precocious puberty is more likely to be associated with underlying pathology.
    • ∼50% of affected boys have idiopathic central precocious puberty.

Genetics

  • Genetic causes include the following:
    • Familial male precocious puberty (testotoxicosis): sex-limited, autosomal dominant inheritance of luteinizing hormone (LH) receptor activating mutation
    • McCune-Albright syndrome: sporadic, postzygotic, somatic mutation in the stimulatory subunit of G-protein receptor; precocious puberty more common in girls

Pathophysiology

  • Central precocious puberty can be associated with CNS disorders.
  • Peripheral precocious puberty
    • Arises from peripheral sex hormone sources, including gonadal and adrenal disorders, abdominal or pelvic tumors, or exogenous sex steroids
    • Can progress to central precocious puberty due to maturation of the hypothalamic–pituitary axis by sex steroids

Etiology

  • Central precocious puberty (gonadotropin-releasing hormone [GnRH] dependent)
    • Associated with gonadotropin (LH and/or follicle-stimulating hormone [FSH]) levels that are elevated beyond the normal prepubertal range. Results from activation of hypothalamic–pituitary–gonadal axis.
    • Physical changes are typically those of normal puberty for a child of that sex.
  • Peripheral sex hormone effects (peripheral precocious puberty or GnRH-independent; less common)
    • Gonadotropin-independent elevation of sex steroids arising (i) directly from gonads and/or adrenals, (ii) through stimulation of gonads by GnRH-independent mechanism, or (iii) from an exogenous source
    • Physical changes reflect predominant excess hormones (estrogenic or androgenic) and are often markedly discordant from normal pubertal development.

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