Reproductive Case Histories Parents were concerned about their 16-year-old son f

Question

Reproductive Case Histories

Parents were concerned about their 16-year-old son for the following reasons: he had no deepening of his voice, scanty pubic and axillary hair growth, absence of beard and mustache growth, small penis, poor muscular development, and psychosocial immaturity.

Laboratory evaluation indicated the following:

Serum testosterone

100 ng/dL

Sperm count

10 million/mL semen

The following tests were performed:

Clomiphene (a nonsteroidal, weak estrogen agonist that stimulates the release of gonadotropins) 100 mg/day for seven days: 0% increase in LH (50% is normal)

Gn-RH (100 µg I.V.): 0% increase in LH in twenty minutes (300% is normal)

HCG (5000 I.U., I.V.): 50% increase in plasma testosterone one to three days after injection

This person was subsequently treated with FSH at 25-75 U three times/week and HCG as described above. Sperm count and testosterone levels were both near normal after two months of treatment, and primary and secondary sex characteristics appeared.

1. What is the endocrine disorder in this individual?
2. Is this a primary or secondary disorder? Why?
3. Why is HCG used in the treatment?
4. Both FSH and HCG are needed in the treatment. Explain Why

Serum testosterone

100 ng/dL

Sperm count

10 million/mL semen

Explanation / Answer

!. The endocrine disorder in this individual is Kallmann syndrome. The syndrome is also called hypogonadotropic hypogonadism (HH).

2. The disorder is a secondary disorder ( a secondary disorder results from understimulation or overstimulation by the pituitary)

3. Gonadotropin (HCG) is required in the early stages of gestation for sexual differentiation of babies after birth. HCG treatment changes the testicular volume and helps their enlargement. Testicular volume may have psychosocial impact if the person becomes sexually active. HCG treatment may result in maturation of spermatognoia and increase serum testosterone. To increase the testes volume, serum testosterone, and sperm ocunt, HCG is given.

4. Follcile stimulating hormone is secreted by anterior pituitary gland. LH and FSH are required for normal development of testes. HCG is required for development of testes volume whereas FSH is required for proper functioning of testes during puberty (or for normal fertility function). FSH stimulates spermatogenesis.

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