Selecting a Treatment Regimen
ÎÎIncrease the GC dosage of CAH patients in situations such as febrile
illness (> 38.5°C), gastroenteritis with dehydration, surgery accompanied
by general anesthesia, and major trauma (1|⊕⊕).
ÎÎDo NOT increase GC doses in mental and emotional stress, minor illness,
and before physical exercise (1|⊕).
ÎÎDo NOT use stress doses of GC in patients with NCCAH unless their
adrenal function is suboptimal or iatrogenically suppressed (1|⊕).
ÎÎPatients who require treatment should always wear or carry medical
identification indicating that they have adrenal insufficiency (2|⊕).
ÎÎMonitor treatment by consistently timed hormone measurements (1|⊕).
ÎÎIn order to avoid adverse effects of over-treatment, do NOT completely
suppress endogenous adrenal steroid secretion (1|⊕⊕).
ÎÎMonitor height, weight, and physical examination regularly. Assess bone
age with annual x-ray after age 2 years (2|⊕).
Treatment of NCCAH
ÎÎTreat NCCAH children with inappropriately early onset and rapid
progression of pubarche or bone age, and adolescent patients with overt
virilization (2|⊕⊕).
ÎÎDo NOT treat asymptomatic individuals with NCCAH (1|⊕⊕).
ÎÎGive previously treated NCCAH patients the option of discontinuing
therapy when symptoms resolve (2|⊕⊕).
Complications of CAH
ÎÎClosely monitor all GC-treated patients for iatrogenic Cushing syndrome
(1|⊕⊕).
ÎÎDo NOT routinely evaluate bone mineral density in children (2|⊕).
ÎÎReserve adrenal imaging for those patients who have an atypical clinical or
biochemical course (2|⊕).
Feminizing Surgery
ÎÎFor severely virilized (Prader stage ≥ 3) females, consider clitoral and
perineal reconstruction in infancy performed by an experienced surgeon
in a center with similarly experienced pediatric endocrinologists, mental
health professionals, and social work services (2|⊕⊕).
ÎÎConsider neurovascular-sparing clitoroplasty and vaginoplasty using total
or partial urogenital mobilization (2|⊕).
Experimental Therapies
ÎÎConsider growth hormone treatment of children with CAH who have a
predicted height standard deviation of ≤ –2.25 in appropriately controlled
trials (2|⊕).
ÎÎDo NOT use experimental treatment approaches outside of formally
approved clinical trials (1|⊕⊕).
