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Delayed Puberty


  1. Normal sequence of puberty
    1. Accelerated growth
    2. Thelarche - mean 9.8 yrs
    3. Adrenarche - mean 10.5 yrs
    4. Menarche - mean 12.8 yrs
    Delayed puberty (physiologic) is a rare condition, most girls that have not shown signs of puberty by age 17 most likely suffer from a genetic or hypothalamic-pituitary disorder. Most U.S. females have entered puberty by age 13.

  2. Work Up
    • History
      • General health
      • Height and weight records
      • Pubertal experience of older siblings and parents
      • Relevant behavior - eating disorders, extreme exercising, drug use (legal and illegal) w/o visual or olfactory disturbances
    • Physical exam
      • Ht. and Wt. with vital signs
      • Tanner staging
      • Signs of chronic illness
      • Is there a cervix and uterus
      • Is the adenexae palpable
      • Neurological exam - including vision and smell
      Lab
      • X-rays for bone age
      • Adrenal and gonadal steroid measyrements
      • Skull imaging if appropriate
      • Thyroid studies
      • Gonadotropin and prolactin levels
      • Karyotype

  3. Classification of Delayed Puberty
    1. Hypergonadotropic Hypogonadism:
      Accounts for 43% of cases
      Increased gonadotropins without gonadal response
      1. Ovarian failure, abnormal karyotype: 60%
             Most common is 45 XO (Turners)
             46 XX with structurally abnormal X chromosome
      2. Ovarian failure, normal karyotype: 40%
             46 XX with gonadal dysgenesls, either streaks or complete absence
             46 XX with 17 alpha-hydroxylase deficiency
                  - hypertension, hypernatremia, hyqokalemia
                  - decreased 17-hydroxyprogesterone and cortisol
                       20% of sickle cell disease pts. have delayed puberty with hypergonadotropism
    2. Hypogonadotropic hypogonadism
      Accounts for 31% of cases
      Decreased levels of LH and FSH (LH< 6 IU/mL)
      1. Reversible: 58%
        • Physiologic delay - usually follows a familial pattern
        • Weight loss/anorexia - either from chronic disease, eating disorder, or excessive exercise.
        • Primary hypothyroidism
        • Congenital adrenal hyperplasia
        • Cushings syndrome
        • Prolactinomas
      2. Irreversible: 42%
        • GnRH deficiency
        • Hypopituitarism
        • Congenital CNS defects
        • Other pituitary tumors - malignant or benign
        • Craniopharyngioma - most common neoplasm
        • Other pituitary adenomas
    3. Eugonadism
      Accounts for 26% of cases
      1. Mullerian agenesis
      2. Vaginal septum
      3. Imperforate hymen
      4. Androgen insensitivity syndrome - Genotypical male Phenotypical female Inappropriate positive feedback.

  4. Treatment
    1. Removal or correction of primary etiology when possible
    2. In XY individual, proper timing of gonad removal followed by steroid hormone replacement.
    3. For patients with physiologic delay, simple reassurance appropriate.
    4. In patients with hypogonadism, hormonal replacement to initiate development of secondary sexual characteristics:
      1. start at 0.3-.05 E2 daily for 6 mos to 1 yr;
      2. then begin sequential estrogen/progesterone therapy.
      3. In patients who are sexually active, OCPs are probably best choice
 
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