18 4
adrenarche/isolated premature pubarche. Fasting plasma insulin and glucose
should be measured in children with obesity. If a child has premature pubarche
along with other signs of androgenization (clitoromegaly in girls and penile
enlargement in boys) or bone age is significantly advanced, child should be
actively evaluated for pathological causes of premature pubarche. The evalua-
tion includes 17α-hydroxyprogesterone (17-OHP), serum DHEAS, and total
testosterone. A baseline serum 17-OHP of >2 ng/ml mandates ACTH stimula-
tion test; serum 17-OHP level 10–100 ng/ml suggests a diagnosis of late-onset
congenital adrenal hyperplasia, and serum 17-OHP level >100 ng/ml confirms
the diagnosis of simple virilizing CAH due to 21α-hydroxylase deficiency.
Serum testosterone >0.6 nmol/L and DHEAS >115 μg/dl suggest androgen
excess, while a serum testosterone >6.94 nmol/L and DHEAS >700 μg/dl sug-
gest virilizing ovarian and adrenal neoplasm, respectively. A scheme of workup
for premature pubarche is illustrated below (Fig. 6.4).
Premature pubarche
Bone age
Significantly advanced* Normal/Slightly advanced
- Premature adrenarche
- Isolated premature
pubarche
Total
Testosterone
(>0.6nmol/L)
DHEAS
(>115 μg/dl)
17 OHP
(>2 ng/ml)
CAH • Virilizing ovarian/adrenal neoplasm
- Glucocorticoid resistance syndrome
- Exogenous androgen exposure
* Bone age>2.5 SD for chronological age
Fig. 6.4 Approach to a child with premature pubarche
6 Precocious Puberty