2. • PRIMARY AMENORRHEA
• Definition: A young girl who has not yet
• menstruated by her 16 years of age is having primary
• amenorrhea rather than delayed menarche. The normal
• upper age limit for menarche is 15 years.

3. CAUSES:
• A. Hypogonadotropic hypogonadism
• (i) Delayed puberty — delayed GnRH pulse
• reactivation.
• (ii) Hypothalamic and pituitary dysfunction —
• Gonadotropin deficiency due to stress, weight
• loss, excessive exercise, anorexia nervosa,
• chronic disease (tuberculosis).
• (iii) Kallmann’s syndrome — inadequate GnRH pulse
• secretion — reduced FSH and LH .
• (iv) Central nervous system tumors — craniopharyngioma
• → reduced GnRH secretion →
• reduced FSH and LH.

4. • B. Hypergonadotropic hypogonadism
• (i) Primary ovarian failure (see p. 463).
• (ii) Resistant ovarian syndrome (see p. 463).
• (iii) Galactosemia: Due to premature ovarian failure
• (see p. 464).
• (iv) Enzyme deficiency (17 α hydroxylase deficiency)
• — characterized by ↓ cortisol and ↑
• ACTH, ↑ mineralocorticoids production.
• There is hypertension with hypernatremia and
• hypokalemia. The individual may be 46 XX or 46
• XY with primary amenorrhea and no secondary
• sexual characters.
• (v) Others — Gonadotropin receptor mutations
• – rarely FSH and/or LH levels are high as the
• respective receptor may be absent or mutated.

5. • C. Abnormal chromosomal pattern
• yy Turner’s syndrome (45 X) (Figs 27.2A and B).
• yy Various mosaic states 45 X/46 XX.
• yy Pure gonadal dysgenesis (46 XX or 46 XY)

6. • D. Developmental defect of genital tract
• yy Imperforate hymen (Fig. 4.2).
• yy Transverse vaginal septum.
• yy Atresia upper-third of vagina and cervix (Fig. 4.5).
• yy Complete absence of vagina (Fig. 28.1).
• yy Absence of uterus in MRKH syndrome
• E. Dysfunction of thyroid and adrenal cortex
• F. Metabolic disorders
• yy Juvenile diabetes.
• G. Systemic illness
• yy Malnutrition, anemia

7. • Weight loss
• yy Tuberculosis.
• H. Unresponsive endometrium
• yy Congenital
• −− Uterine synechiae (tubercular

8. INVESTIGATIONS