ABSTRACT
The nonclassic form of adrenal hyperplasia (NCAH) has been increasingly recognized
in adolescent or adult hyperandrogenic patients. It is now widely accepted that neither
the clinical presentation nor the androgen plasma levels can be used for the screening
or diagnosis of NCAH in hyperandrogenic women, especially those presenting with a
phenotype like that in polycystic ovary syndrome. Therefore, the measurement of a
follicular morning level of serum 17-hydroxyprogesterone (17-HP) should be included
in the initial investigation of all hyperandrogenic women, including those with premature
pubarche. Levels of 17-HP lower than 2 ng/mL (6.0 nmol/L) and greater than 4 ng/mL
(12.0 nmol/L) have good predictive negative and positive values, respectively. The
adrenocorticotropic hormone test is useful only when the morning follicular unsuppressed
17-HP level falls between 2 and 4 ng/mL (6 to 12 nmol/L). Adrenal insufficiency and
adrenal hyperplasia are more theoretical than real complications of NCAH. On the other
hand, the polycystic ovary syndrome and infertility are frequently associated with
NCAH. For the treatment of hyperandrogenism, the conventional treatment by glucocorticoid
therapy is challenged by cyproterone acetate, but it is still indicated when patients
wish a pregnancy. It can be questioned whether the low risk of virilized female newborns
in untreated women with NCAH justifies prenatal diagnosis and treatment.
KEYWORDS
Adrenal hyperplasia - hyperandrogenism - adolescent girls - 17-hydroxyprogesterone
- ACTH test
