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Key Features

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  • Defined as pubertal development occurring below the age limit set for normal onset of puberty

  • More common in girls than in boys

  • Many girls showing signs of puberty between 6 and 8 years of age have a benign, slowly progressing form that requires no intervention

  • Age of pubertal onset may be advanced by obesity

  • Central precocious puberty in girls is generally idiopathic but may be secondary to a central nervous system (CNS) abnormalities, including but not limited to

    • Hypothalamic hamartomas

    • CNS tumors

    • Cranial irradiation

    • Hydrocephalus

    • Trauma

  • Peripheral precocious puberty (GnRH-independent)

    • Occurs independent of gonadotropin secretion

    • Can be caused by ovarian or adrenal tumors, ovarian cysts, late-onset congenital adrenal hyperplasia, McCune-Albright syndrome, or exposure to exogenous estrogen

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Clinical Findings

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  • Usually starts with breast development followed by pubic hair growth and menarche

  • Girls with ovarian cysts or tumors generally have signs of estrogen excess such as breast development and possibly vaginal bleeding

  • Adrenal tumors and congenital adrenal hyperplasia produce signs of androgen excess which include pubic hair, axillary hair, acne, and increased body odor

  • Children with precocious puberty usually have accelerated growth and skeletal maturation, and may temporarily be tall for age

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Diagnosis

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  • If the bone age is advanced, further laboratory evaluation is warranted

  • In girls with an ovarian cyst or tumor, estradiol levels will be markedly elevated

  • In central precocious puberty,

    • Random follicle-stimulating hormone (FSH) and luteinizing hormone (LH) concentrations may still be in the prepubertal range

    • If so, documentation of the maturity of the hypothalamic-pituitary axis depends on demonstrating a pubertal LH response after stimulation with a GnRH agonist

  • In peripheral precocious puberty, the LH response to GnRH stimulation is suppressed by feedback inhibition of the hypothalamic-pituitary axis by the autonomously secreted gonadal steroids

  • In girls with pubic or axillary hair but no breast development, androgen levels (testosterone, androstenedione, dehydroepiandrosterone-sulfate) and 17-hydroxyprogesterone should be measured

  • When a diagnosis of central precocious puberty is made, an MRI of the brain should be done to evaluate for CNS lesions

  • When laboratory test results suggest peripheral precocious puberty, an ultrasound of the ovaries and/or adrenal gland may be indicated

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Treatment

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  • Two most common GnRH analogs that downregulate pituitary GnRH receptors and thus decrease gonadotropin secretion are leuprolide, which is given as a monthly intramuscular injection, and histrelin subdermal implant, which is replaced annually

  • In a girl with an ovarian cyst, intervention is generally not necessary, as the cyst usually regresses spontaneously

  • Surgical resection and possibly chemotherapy are indicated for the rare adrenal or ovarian tumor

  • Aromatase inhibitors, which block the production of estrogen, may be effective in treating McCune-Albright syndrome

  • Attention to the psychological needs of the patient and family is essential

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