Selecting a Treatment Regimen
Figure 2. Congenital Adrenal Hyperplasia
All neonatal
After infancy
2-tier screen for
21-hydroxylase deficiency
Symptomatic
Early morning baseline
serum 17-OHP
High
Cosyntropin stimulation test
Equivocal?
Genetic
counseling?
Complete adrenocortical profile
Growing patients
Genotyping
Adult patients
ALL TREATED PATIENTS
SHOULD CARRY MEDICAL ID
NCCAH
Symptomatic
NCCAH
CAH
CAH
Early infancy
Treat if
symptomatic
MC and
NaCl
D/C if symptoms
resolve?
Feminizing surgery for
severely virilized females
(Prader stage ≥ 3)
Monitor for growth,
bone age, androgen
and GC excess
Tablet HC
maintenance
(dose < endogenous
suppression)
+ fludrocortisone
HC or
long-acting GC
+/− fludrocortisone
Increase GC during
major stress
Monitor PE and
hormones ≤ yearly
Men
Periodic US screen
for testicular tumors
Confirm
+17-OHP
with ACTH
stimulation test
Treat patientimportant
hyperandrogenism
or infertility
