A 16 years old high school senior, who is preparing to take up theatre major in her college wants to consult her physician for evaluation of excess hair growth over her legs and hands. She also has growth of hair over her upper lip. Her periods have been regular since her menarche which was when she was 12 years old. She works out regularly in a gym and takes ballet dancing classes.

 

1.  The initial work up of this girl will require:

(a) Serum total testosterone, Serum Cortisol and Serum TSH

(b) Serum free testosterone, Serum androstenedione and Serum TSH

(c) Serum total testosterone, Serum dehydroepiandrosterone, Serum Cortisol

(d) Serum free testosterone, Serum dehydroepiandrosterone, Serum Cortisol and Serum TSH.

Answer: The answer is (d). Serum total testosterone has no value in evaluation of hirsutism. It is the free component which is important. Serum dehydroepiandrosterone is to evaluate for adrenal source of hirsutism. Cushing’s syndrome and hypothyroidism can cause secondary hirsutism and so they also have to be ruled out.

 

2.  This girl has elevated androgen levels. The next step in the evaluation includes.

(a) Starting anti-androgens

(b) Starting estrogen-progesterone combination pills

(c) Ordering a Dexamethasone suppression test

(d) Ordering an ACTH stimulation test.

Answer: The answer is (c). The rationale for this study is that subnormal androgen suppression with normal adrenocortical suppression indicates a source of androgen other than an ACTH-dependent adrenal one. This can help to rule out the adrenal as a cause of the hyperandrogenemia. This is very important before starting therapy.

 

3.  A dexamethasone suppression test is done and it is found that the androgen is not suppressed but the cortisol level is suppressed. A pelvic ultrasound is done and is reported as normal. What is the clinical diagnosis in this girl?

(a) Cushing’s Syndrome

(b) Polycystic Ovarian Syndrome

(c) Van Wyk Grumbach Syndrome

(d) Adrenal carcinoma.

Answer: The answer is (b). In the dexamethasone suppression test if the androgen is not suppressed but the cortisol is suppressed, it indicates that the source of the androgen excess is not from the adrenal cortex. Therefore it is either a non adrenal tumor, or polycystic ovarian syndrome. Given the fact that many of polycystic ovarian syndrome patients have normal pelvic ultrasound, this patients probably has polycystic ovarian syndrome.