Reproductive Endocrinology

Question 1

What causes testicular feminization?

Answer 1

Genetic male that phenotypically looks female due to androgen insensitivity

• Deficiency of 5a-reductase is most common cause

–> Absence of Testosterone and Mullerian inhibiting Substance permits female pattern to develop although abdominal testes will be present

–> cannot activate testosterone, therefore, external virilization and male sexual maturation at puberty do not occur

Question 2

What causes congenital adrenal hyperplasia?

Answer 2

21-Hydroxylase Deficiency

–> Aldosterone and cortisol cannot be produced, due to hydroxylase deficiency. therefore, no ACTH negative biofeedback occurs resulting in increased androgen production

–> Results in masculinity of genetic females

Question 3

What is gonadarche?

Answer 3

Activation of gonadal functions

-> Cause: GnRH driven pituitary LH/FSH secretion

Question 4

What is Thelarche?

Answer 4

Breast Development

• Cause: ovarian estrogen

Question 5

What is adrenarche?

Answer 5

Activation of adrenal androgen secretion causing the appearance of pubic and axillary hair
- includes changels in sweat glands and composition of sweat leading to adult body odor

• Cause: uncertain - ot ACTH or gonadotropin mediated

Question 6

What is peripherial vs central precocious puberty?

Answer 6

Peripheral: caused by source of androgens outside of brain hormone release

Central: Due to increased release of LH by pituitary gland

Question 7

How does testing LH help determine peripheral vs central precocious puberty?

Answer 7

• *Normal = rules out “precocious” puberty**
  interpretation: normal development for this child
• *High = central precocious puberty**
  treatment: suppression of HPG axis with super active GnRH analogs
• *Low = peripheral precocious puberty**
  treatment: identify source of sex steroids (adrenal, Rx, diet) and treat accordingly

Question 8

What are causes of central precocious puberty?

Answer 8

• Tumors (astrocytomas, gliomas, germ cell tumors secreting HCG)
• Hypothalamic hamartomas
• Acquired CNS injury caused by inflammation, surgery, trauma, radiation therapy, or abcess
• Congenital anomalies (i.e. hydrocephalus, arachnoid cysts, suprasellar cysts)

Question 9

What are the components of the adenohypophysis?

Answer 9

AKA anterior lobe of pituitary

Pars tuberalis

Pars distalis (pars anterior)

Pars intermedia

Question 10

What are the components of teh neurohypophysis?

Answer 10

AKA posterior lobe of pituitary

Pars nervosa (infundibular process)

Infundibulum (neural stalk)